International Journal of Medical Reviews and Case Reports (Int J Med Rev Case Rep) Year 2017:1-3

Table of Cont ent s - Int J Med Rev Case Rep. Year: 2 0 1 7 , Volume: 1 , Issue: 1 -3 , pp: 1 -5 5 ISSN: 2 5 3 4 -9 8 2 1 – Edit or-in-Chief: Dr. Ivan Inkov Review Art icle 1 . Los hombres y los accident es de t ránsit o: un vist azo al riesgo, conduct a de riesgo, not ificación de accident es, educación y formación profesional Cint ia Rodrigues, Thiago Vendramini, Carlos Górios, Cleo Chinaia, Rodrigo Armond, Jane Armond, Pat ricia Colombo-Souza, Elias Jirjoss Ilias Int J Med Rev Case Rep. 2 0 1 7 ; 1 ( 1 ) : 1 -5 » Abstract » PDF» doi: 10.5455/IJMRCR.los-accidentes-de-transito 2 . Kikuchi-Fujimot o disease: case report and review Irami Araujo-Filho, Art ur Dant as Freire, Irami Araújo-Net o, Carolina Chianca Dourado Lemos, Let ícia Araújo Cost a Uchôa, Amália Cínt hia Meneses Rêgo, Marco Ant ônio Bot elho Soares, José Francisco Correia-Net o, Carlos André Nunes Jat obá, Ana Maria de Oliveira Ramos Int J Med Rev Case Rep. 2 0 1 7 ; 1 ( 1 ) : 6 -1 0 » Abstract » PDF» doi: 10.5455/IJMRCR.kikuchi-fujimoto-disease Case Report 3 . Pancreas Divisum Causing Recurrent Pancreat it is: A Case Report Nadeem Ahmed Siddiqui, Rizwan Sult an, Fareed Ahmed Shaikh Int J Med Rev Case Rep. 2 0 1 7 ; 1 ( 1 ) : 1 1 -1 3 » Abstract » PDF» doi: 10.5455/IJMRCR.pancreas-divisum-recurrent-pancreatitis 4 . Vulvar non-filarial elephantiasis associat ed wit h scabies Sarah Sabur, Saoussane Bounajma, Mounia Nasr, Samir Mazouz Int J Med Rev Case Rep. 2 0 1 7 ; 1 ( 1 ) : 1 4 -1 6 » Abstract » PDF» doi: 10.5455/IJMRCR.vulvar-non-filarial-elephantiasis-scabies 5 . Pit uit ary microadenoma wit h prolact in, cort icot ropic and t hyreot ropic deficiency: from infert ilit y t o pregnancy : About a case.

Diallo Moussa, Koulimaya Cyr Esperence Gombet , Diallo Ast ou Coly Niassy, Diouf Abdoul Aziz, Gassama Omar, Gueye Mame Diarra Ndiaye, Leye Yakham, Diouf Alassane Int J Med Rev Case Rep. 2 0 1 7 ; 1 ( 1 ) : 1 7 -1 9 » Abstract » PDF» doi: 10.5455/IJMRCR.pituitary-microadenoma 6 . Surgical Management Of Traumat ic Manubrio-St ernal Dislocat ion Wit h Locking Compression Plat e; A Case Report And Review Of Lit erat ure.

Fareed Ahmed Shaikh, Syed Shahabuddin, Haroon Rashid, Noman Shahzad Int J Med Rev Case Rep. 2 0 1 7 ; 1 ( 1 ) : 2 0 -2 2 » Abstract » PDF» doi: 10.5455/IJMRCR.surgical-management-traumatic-manubrio-sternal-dislocation Review Art icle 7 . Test icular Descend, How and Why: A Review Art icle Sujan Narayan Agrawal Int J Med Rev Case Rep. 2 0 1 7 ; 1 ( 2 ) : 2 3 -2 7 » Abstract » PDF» doi: 10.5455/IJMRCR.testicular-descend

Case Report

8 . Efficacy of Int ravenous immunoglobulin and phot ot herapy in t he management of ext remehyperbilirubinaemia: A Case Report Oluf unke Bosede Bolaji, Sandeep Dhamaraj, Colin Lumsden, Olusegun Joseph Adebami Int J Med Rev Case Rep. 2 0 1 7 ; 1 ( 2 ) : 2 8 -3 1 » Abstract » PDF» doi: 10.5455/IJMRCR.immunoglobulin-and-phototherapy-in-the-management-ofextreme-hyperbilirubinaemia 9 . Surgical management of severe t rauma involving group of muscles around t he brisket of Dongola breed of horse:A case report Abayomi Kayode Olaif a, Cecelia Omowunmi Ogunt oye, Adenike Olat unji Akioye Int J Med Rev Case Rep. 2 0 1 7 ; 1 ( 2 ) : 3 2 -3 5 » Abstract » PDF» doi: 10.5455/IJMRCR.dongola-breed-of-horse-severe-trauma-surgery 1 0 . A case report of delayed diagnosis of ut erine rupt ure following vaginal delivery Vanessa Falé Rosado, Sara Rocha, Cat arina Vasconcelos, Maria Luisa Mart ins, Maria José Alves Int J Med Rev Case Rep. 2 0 1 7 ; 1 ( 2 ) : 3 6 -4 0 » Abstract » PDF» doi: 10.5455/IJMRCR.uterine-rupture-delayed-diagnosis 1 1 . Misdiagnosis of an Acardiac Twin in t he First Trimest er, A Case Report .

Cat arina Vasconcelos, Vanessa Falé Rosado, Álvaro Cohen, Ana Teresa Mart ins, Rit a Torres Int J Med Rev Case Rep. 2 0 1 7 ; 1 ( 2 ) : 4 1 -4 5 » Abstract » PDF» doi: 10.5455/IJMRCR.acardiac-twin-first-trimester 1 2 . Spider procedure for sacral pressure sore reconst ruct ion:A case report .

Sarah Sabur, Imane El Aissaoui, Mohammed Raboune, Lamiaa Bensaida, Samir Mazouz, Noureddine Gharib, Abdellah Abassi Int J Med Rev Case Rep. 2 0 1 7 ; 1 ( 2 ) : 4 6 -4 8 » Abstract » PDF» doi: 10.5455/IJMRCR.Spider-procedure-for-sacral-pressure-sore-reconstruction Review Art icle 1 3 . Met a Analysis Of Ant iepilept ic Drugs Induced Choreoat het osis In Paediat ric Pat ient s Sriram Shanmugam, Lidhu Daniel, Jaleel Ahamed Int J Med Rev Case Rep. 2 0 1 7 ; 1 ( 3 ) : 4 9 -5 2 » Abstract » PDF» doi: 10.5455/IJMRCR.ANTIEPILEPTIC-DRUGS-INDUCED-CHOREOATHETOSIS Case Report 1 4 . Case report of Dengue encephalopat hy in pregnancy mimicking at ypical eclampsia – An insight of neurological manifest at ions of dengue in pregnancy Sasirekha Rengaraj, Sut hrasika Thiyagalingam Int J Med Rev Case Rep. 2 0 1 7 ; 1 ( 3 ) : 5 3 -5 5 » Abstract » PDF» doi: 10.5455/IJMRCR.dengue-encephalopathy-in-pregnancy-mimicking-atypicaleclampsia

REVIEW ARTICLE

LOS HOMBRES Y LOS ACCIDENTES DE TRÁNSITO: UN VISTAZO AL RIESGO, CONDUCTA DE RIESGO, NOTIFICACIÓN DE ACCIDENTES, EDUCACIÓN Y FORMACIÓN PROFESIONAL Cintia Leci Rodrigues∗,1 , Thiago Leão Vendramini∗∗ , Carlos Górios∗ ∗ ∗ , Cleo Chinaia♦ , Rodrigo de Eston Armond♦♦ , Jane de Eston Armond♦♦♦ , Patricia Colombo-Souza4 and Elias Jirjoss Ilias44 ∗ Maestría

en Salud Pública FSP/USP. Curso de Medicina de la Faculdade das Américas., ∗∗ Especialista en alcohol y drogas FMUSP/USP., ∗ ∗ ∗ Mastría en Ortopedia y Traumatología USP. Curso de Medicina del Centro Universitario São Camilo., ♦ Especialista en Gestión de Servicios de Enfermería UNIFESP. Curso de Medicina y Enfermería de la Universidade de Santo Amaro., ♦♦ Cursando maestría en Ciencias de la Salud de la Universidade de Santo Amaro., ♦♦♦ Doctora en Salud Pública FSP/USP. Stricto sensu Ciencias de la Salud de la Universidade de Santo Amaro., 4 Doctora en Nutricion UNIFESP. Stricto sensu Ciencias de la Salud de la Universidade de Santo Amaro., 44 Doctorado en Medicina (Cirugía) de la Facultad de Ciencias Médicas de la Santa Casa de São Paulo. Departamento de Cirugía, Facultad de Ciencias Médicas de la Santa Casa de São Paulo.

ABSTRACT Los accidentes de tránsito son un grave y complejo problema de salud pública. La literatura señala que los accidentes de tránsito están asociados confactores de comportamiento, seguridad de los vehículos y la precariedad del espacio urbano.Reducir el número de accidentes de tránsito es un reto para los gestores deárea. La educación en el tránsito debe considerar la vigilancia como una estrategia eficaz para cambiar el comportamiento del conductor,principalmente en relación con el exceso de velocidad y el consumo de alcohol asociado con la conducción.La ingeniería tiene un papel importante para promover un entorno seguro,en el que la convivencia de peatones, ciclistas y conductores sea posible. El escenario en que se configura los accidentes de tránsito muestra la necesidad de los profesionales de la salud reconsiderar sus prácticas con el fin de replantear la imagen de la víctima de accidentes, las formas de trabajo de prevención de accidentes, la educación en el tránsito de peatones y conductores, con el fin de promover la salud y la cultura de paz.

Existe la necesidad de políticas de salud públicay estrategias que hacen posible acceso a las acciones tanto en el campo preventivo como en el campo de la rehabilitación.

KEYWORDS accidentes de tránsito, prevención de accidentes, educación, notificación de accidentes.

Introducción:

Copyright © 2017 by the Bulgarian Association of Young Surgeons DOI: 10.5455/IJMRCR.los-accidentes-de-transito First Received: April 24, 2017 Accepted: May 11, 2017 Manuscript Associate Editor: George Baytchev (BG) Editor-in Chief: Cvetanka Hristova (BG) Reviewers: Ivan Inkov (BG) 1 Cintia Leci Rodrigues.Dirección: Calle Professor Cândido Nogueira da Mota Nº 409 Barrio Interlagos, São Paulo – SP, Brasil C.P. 04786-035. Correo electrónico: kikarodrigues@hotmail.com

Los accidentes de tránsito son un grave y complejo problema de salud pública. Han aumentado con el desarrollo económico y tecnológico de las sociedades modernas y pueden tener implicaciones sociales y económicas, ya que predominan en la gente joven y económicamente activa[1,2].

De conformidad, en São Paulo, durante el año 2014, los accidentes de tránsito fueron responsables del 2% de las muertes totales[3].

La bibliografía señala que los accidentes de tránsito están relacionados con factores de comportamiento, seguridad de los vehículos y la precariedad del espacio urbano. Se configuran-

Cintia Leci Rodrigues et al./ International Journal of Medical Reviews and Case Reports (2017) 1(1): 1-5

como causas importantes de mortalidad y la morbilidad debido al creciente número de vehículos, los cambios en el estilo de vida y el comportamiento de riesgo en la población general[2, 4,5].

Los factores asociados con accidentes de tránsito, implícita o explícitamente, son:el hombre; vehículo; vía y entorno; y en relación con la legislación y su cumplimiento. Según Almeida y Cols[5] el desglose de estos factoresy el estudio de sus asociaciones son necesarios para comprendery mejor intervenir en fenómeno accidente de tránsito. Es que su combinación puede aumentar la probabilidad de ocurrencia de los accidentesde manera diferente en ciertos lugares[5].

En este contexto, el objetivo de esta revisión fue preparar una reflexión sobre la importancia de los accidentes de tránsitoen el ámbito de la salud pública y, más en concreto, sobre la subjetividad en la evaluación de riesgo de los conductoresy su posible influencia en la aplicación de medidas de prevención de accidentes de tránsito.

Los accidentes de tránsito: la percepción de riesgo de los conductores

Reducir el número de accidentes de tránsito es un reto para los gestores de área[6].La gestión del tránsito es un asunto complejo.El sistema de transporte prioriza el uso de vía terrestrey en consecuencia favorece el movimiento y la expansión del número de los automóviles. Este hecho,combinado con la falta de infraestructura (aceras, vías, señalización), y otros problemas en el transporte (costo, la capacidad de los medios de transporte, la contaminación, la congestión)forman un escenario favorable para el aumento de las tasas de mortalidad y morbilidad.También tenemos que considerar que el tránsito se compone de personasy como tal hay que tener en cuentasu individualidad y el desarrollo constante,sus procesos que se componen de las funciones y actividades diarias,su contexto y el momento histórico en que está insertada[7].

El riesgo podría definirse como la probabilidad de que ocurra algo indeseable.Y los factores de riesgo son elementos con alta probabilidad de desencadenaro estar asociado con el desencadenamiento de un acontecimiento no deseado, no siendo necesariamente elemento causal. Por el contrario, los factores de protección son los recursos personales o socialesque tendría el papel de reducir o neutralizar el impacto del riesgo.La percepción de riesgo tendría la perspectiva del control preventivo de riesgos, y a través de la educación, influiría en el comportamiento perjudicial para la salud de la persona y el entorno en que viven [7,8].

El comportamiento del conductor se identificó como uno de los factores responsables de los accidentes de tránsito con respecto a la observación de señales de tránsito, velocidad, y las decisiones en el momento de adelantar a otro vehículoo cruzando una vía.

Se necesitan estudios sobre culturas y las condiciones de vida localespara la comprensión de las actitudes de los conductores,con el fin de desarrollar programas de capacitación, rehabilitación y educación[8].

El uso de alcohol y otras drogas entre los conductores:

Los conductores que conducen bajo la influencia del alcohol son una de las principales causas de accidentes de tránsito, de tal modo que la ingesta de pocas dosis alcohólicas es suficientepara aumentar la predisposición del conductor para emitir alguna conducta de riesgo[9].

El tema relacionado con los accidentes de tránsito provocados por conductores en estado de ebriedadempieza a tener una dimensión más amplia en la bibliografía.Por un lado, aunque se

realicen programas de prevención e intervención, como no conducir en estado de ebriedad constantemente, las investigaciones muestran que los accidentes de tránsito provocados por conductores en estado de ebriedad siguen presentando tasas altas en todo el mundo[9, 10]. Otros factores juntos y posibles tales como somnolencia, fatiga, estrés/ ansiedad, el uso de medicamentos (inadecuados y asociados con las bebidas alcohólicas)y la sensibilidad individual (susceptibilidad neurofuncional) combinados aumentan el riesgo de accidentes de tránsito[11].

El uso de alcohol sugiere la baja percepción de riesgo de los conductores. Los motivos de salud públicaque pueden subrayar las medidas preventivasson mayor vigilancia y leyes estrictas.La posición tolerante de algunas sociedades en relación con el consumo de alcohol- que es intensificado por fuertes campañas publicitarias[12].

Estudio de revisión sistemática realizada por Aguilera y cols[13] en relación con la legislación, leyes que establecen límites de concentración de alcohol en la sangre no fueron eficaces, sobre todo cuando no estén acompañados de otras medidas.Estas leyes tuvieron un impacto únicamente cuando se asocia con la suspensión de la licencia del infractor o prohibiciones más coercitivas. La vigilancia tiene un efecto significativo en la reducción de violaciones por conducir bajo los efectos del alcohol,por ejemplo, con el aumento de las operaciones policiales.

Los países con larga tradición en la vigilancia del conductor en estado de ebriedad, con leyes que establecen un límite bajo de concentración de alcohol en la sangre, y donde los conductores están en alto riesgo de ser sorprendido en el sistema de vigilancia y detención, cuyo trabajo está apoyado por los medios de comunicación, tienden a tener un bajo número de casos que involucren alcohol y la conducción[13].

Como se ha visto los accidentes de tránsito se han convertido como un problema de salud pública, en vista de altos costos financieros (hospitalización, rehabilitación de las víctimas)y social que resultan de ellos y, en cuanto a la discusión sobre conducir bajo los efectos del alcohol se ha formateado como un problema que requiere políticas públicas. El aumento de los accidentes proporciona una proyección esencialpara que este asunto se hiciera público en los medios de comunicación. Estos se nutren de la investigación, los datos estadísticos de organismos de tránsito, por estudiosos sobre el tema, campañas sociales, y otros que trabajan con el problema del alcohol y los accidentes de tránsito.

Según Almeida[14] hay una tensión en algunos campos de la actividad donde los intereses en conflicto están presentes en el tema de conduciren estado etílico. Por un lado, el problema de salud pública, por otra parte, la defensa del mercado libre y autorregulado.También debe analizar el papel de los medios de comunicación en este proceso, la regulación insuficiente de la publicidad de alcohol, principalmente entre los países en desarrollo y el cambio de posición en relación con el uso y la venta de bebidas alcohólicascomo un asunto de libre mercado[14].

La eficacia de las leyes de tránsito requiere una vigilancia eficaz junto con enfoques informativos/ educativos. Por lo tanto, su efectividad depende,por una parte, la percepción inmediata de la pena para los conductores. Aguilera y cols13mencionan que la prueba de alcohol en la sangre obligatoriay el programa de denuncia de conductor ebriomostraron una reducción de las colisiones, sin embargo revelan que el conductor cambia el comportamiento sólo cuando el siente que está siendo vigilado por la autoridad pública u observado por la sociedad[13].

Cintia Leci Rodrigues et al./ International Journal of Medical Reviews and Case Reports (2017) 1(1): 1-5

El comportamiento de riesgo: velocidad, violaciones de leyes, el tiempo de licencia:

La educación siempre ha sido considerada una de las principales estrategias de prevención.Sin embargo, el papel de la educación en la prevención del tránsito ha sido reevaluado, con la vigilancia como una estrategia más probable de impactoy la ingeniería la más eficaz para asegurar velocidades más lentas, por los cambios físicos en las vías (badenes, pasos de peatones elevados, ciclovías, entre otros)[13].

Iniciativas de normativa muestran cambios de conducta como por ejemplo,límite de velocidad y uso restringido del teléfono celular disminuyeronal mismo tiempo que la reducción de las tasas de colisiones y lesiones[13].

Varios autores, afirman que las intervenciones dirigidas a reducir la velocidad del tránsito se consideran esenciales para la prevención de los accidentes de tránsito.Entre esas, podemos mencionar la implementación de radares de control de velocidad.El uso de radar contribuye a la reducción de la velocidad media, disminución del porcentaje de vehículos que transitan en el exceso de velocidady la reducción de la velocidad desarrollada por los conductores.Uno de los problemas asociados con la aplicación de detectores de velocidad es la tendencia de algunos conductores para frenar al pasar por el radar y, luego, superar el límite de velocidad cuando se está fuera del alcance del mismo[13, 14,15].

La notificación de los accidentes de tránsito:

Al considerar las unidades de salud, incluidas las unidades de emergencia como los "serviços sentinelas", los profesionales de estas unidades deben ser capaces de identificar y llevar a cabo los procedimientos y encaminamientos asociados con casos de violencia, sobre todo con respecto a la notificación en el "Sistema de Vigilância a Violências e Acidentes (VIVA)", del Ministerio de Salud, en Brasil.Este sistema tiene por objeto determinar el alcance y el perfil de las causas externas asistida, lo que permite la aproximación de la situación real, especialmente en casos de lesiones menores que no determinaron la muerte u hospitalización, teniendo en cuenta el fuerte impacto de estos eventos en la salud, así como la gran demanda de estos casos en las unidades de emergencia[16].

En la ciudad de São Paulo, que se encuentra en la región sudeste de Brasil, durante el año 2015, se registraron 10.107 accidentes de tránsito, que se producen principalmente entre los automóviles (30,1%) y motocicletas (49,2%)[17].Las víctimas de accidentes son mayoritariamente hombres (73,3%) y jóvenes.En el mismo periodo, en 2,3% de los casos fueron reportados el consumo de alcohol y drogas entre las víctimas de accidentes trânsito[17]. Frente a casos de accidentes y desde la perspectiva de los "serviços sentinela", la unidad de emergencia es una de las "puertas de entrada" del Sistema de Salud que funciona como una oportunidad para el desarrollo del caso, lo que hace este sector estratégico para la investigación de indicadores de accidentes, sus diversas manifestaciones y consequências[16].

La notificación de accidentes ha sido una herramienta para los vínculos entre los sistemas de salud y garantía de los derechos, con la integración de las acciones de promoción de la salud, prevención y control de accidentes. Contribuyendo así a aumentar el conocimiento de los accidentes de tránsito, en la perspectiva del Sector de la Salud, para ayudar en la investigación de indicadores, así como subvencionando la expansión de las políticas, programas y prácticas destinadas a la prevención, para hacer frente a accidentes de tránsito y la reducción de daños. En este

sentido, contribuye a la consolidación de la red de atención completa a las víctimas de accidentes[18].

Educación vial:

La educación vial debe considerar la vigilancia como una estrategia eficaz para cambiar el comportamiento del conductor, especialmente en relación con el exceso de velocidad y consumo de alcohol asociado con la conducción.La ingeniería tiene un papel importante para promover un entorno seguro en el que sea posible la convivencia de peatones, ciclistas y conductores.Por último, la educación tenía carácter informativo y de apoyo para otras estrategias utilizadas[13].

Se entiende que los accidentes de tránsito se consideran un problema de salud pública responsable de generar altos costos para el sector de la salud y las consecuencias físicas y/ o psicológicas para las personas que sufren de este tipo de accidentes, además de las pérdidas sustanciales de las personas jóvenes que forman parte de la población activa, debido a la muerte o la invalidez como consecuencia de estos accidentes[19].

Para mitigar esta realidad, se observa la necesidad de una aplicación más estricta de las leyes de tránsito asociado a la inversión en la educación de los peatones y conductores de vehículos[19].

Formación de profesionales para hacer frente a los accidentes de tránsito:

Uno de los principales retos en el área de los accidentes de tránsito en Brasil es precisamente mejorar la práctica profesional en la salud para incluir los profesionales, para sostener científicamente la aplicación, el seguimiento, la evaluación o elaboración de políticas públicas de tránsito con el fin de intervenir en el comportamiento de los usuarios del tránsito y del transporte, ya sean peatones o conductores[20].

Se resalta la importancia de la formación permanente de los profesionales de la salud que trabajan en el área de emergencia, puesto que cuanto más rápida y cualificada sea la primera asistencia, mayor será la probabilidad de un buen pronóstico[19].

El escenario que se configura los accidentes de tránsito muestra la necesidad de profesionales de la salud de replantear sus prácticas con el fin de redefinir la imagen de la víctima de accidente, las formas de prevención de accidentes, la educación vial de peatones y conductores con objetivo de promover la salud y la cultura de paz.La formación de los profesionales en el tema; debe dar prioridad a la programación de trabajo; educación permanente para la creación de redes de apoyo y protección (incluyendo desde la prevención hasta el seguimiento de los casos en la emergencia, rehabilitación, asistencia psicológica a las víctimas y sus familias) sigue desafiando a los gestores y profesionales de la salud para exigir acciones concretas[21].

En Brasil, como los accidentes y la violencia son objeto de notificación obligatoria, hay una necesidad de acciones de carácter educativo e informativo, haciendo hincapié en la distinción entre notificación y denuncia y proporcionando un foro de discusión para los profesionales de diversos campos reflexionar sobre el miedo que impregna las notificaciones, su responsabilidad, el compromiso y la conducta ética delante de la persona que experimenta accidentes en su área de alcance, así como evaluar estrategias de apoyo[22].

El tema de los accidentes de tránsito, haciendo hincapié en la notificación obligatoria, debe incluirse en los contenidos de los planes de estudios de los espacios de formación, así como en entornos profesionales[22].

Cintia Leci Rodrigues et al./ International Journal of Medical Reviews and Case Reports (2017) 1(1): 1-5

Destaca la inclusión del tema en los contenidos de los planes de estudios de los cursos, por lo que los accidentes y la violencia se incorporan como objeto de la salud y así investigada durante el contacto con el cliente[22].

Consideraciones finales: La prevención de los accidentes de tránsito y sus consecuencias están directamente relacionados con la atención prehospitalaria y hospitalaria de las víctimas, la atención de rehabilitación y para, además, la vigilancia de los accidentes y la violencia, la adopción de medidas educativas y legislativas de seguridad vial que contribuya a la reducción de la morbilidad y la mortalidad por estas enfermedades.Por lo tanto, se recomienda una mayor inversión en la prevención de accidentes de transporte y rehabilitación de las víctimas con secuelas, apoyo psicológico y así reduciendo el impacto social de estos agravios.Existe la necesidad de políticas de salud pública y estrategias que dan acceso a las acciones tanto en el campo preventivo como en el campo de la rehabilitación[23].

Conflicto de intereses Los autores declaran que no existe conflicto de interés en este manuscrito.

References 1. Paiva L, et al.Estado de saúde e retorno ao trabalho após os acidentes de trânsito. Rev. Bras. Enferm. 2016; 69 (3): 443-450.

2. Paixão LMMM, Contijo ED, Mingoti SA, Costa DAS, Friche AAL, Caiaffa WT. Urban road traffic deaths: data linkage and identification of high-risk population sub-groups. Cad. Saúde Pública. 2015; 31 [Sup 1]:S92-S106.

3. Prefeitura Municipal de São Paulo. Secretaria Municipal de Saúde. Tabnet. Sistema de Informação de Mortalidade.

Disponível em: http://www.prefeitura.sp.gov.br. Acessado em: 26 set 2016.

4. Novo CF, Soares DP, Miolla JCS, Thielen IP. Percepção de risco do Motociclista Infrator. Psicol. cienc. prof. 2015; 35 (4): 991-1006.

5. Almeida RLF, Bezerra Filho JG, Braga JU, Magalhães FB, Macedo MCM, Silva KA. Via, homem e veículo: fatores de risco associados à gravidade dos acidentes de trânsito. Rev. Saúde Pública. 2013; 47 (4): 718-731.

6. Marques EV, Machado MA. Identificação dos fatores relevantes na decisão da alocação dos recursos econômicos visando um trânsito seguro. Rev. Adm. Pública. 2010; 44 (6): 1379-1404.

9. Leopoldo K, Leyton V, Oliveira LG. Uso exclusivo de álcool e em associação a outras drogas entre motoristas de caminhão que trafegam por rodovias do Estado de São Paulo, Brasil: um estudo transversal. Cad. Saúde Pública. 2015; 31 (9): 1916-1928.

10. Almeida ND, Roazzi A. Álcool e direção em universitários, comunicação persuasiva e prevenção.Psicol. cienc. prof.

2014; 34 (3): 715-732.

11. Abreu AMM, Lima JMB, Matos LN, Pillon SC. Uso de álcool em vítimas de acidentes de trânsito: estudo do nível de alcoolemia. Rev. Latino-Am. Enfermagem. 2010; 18 [Spec]: 513-520.

12. Boni R, Benzano D, Leukefeld C, Pechansky F. Uso de bebidas alcoólicas em postos de gasolina de Porto Alegre: estudo piloto. Rev. psiquiatr. Rio Gd. Sul. 2008; 30 (1): 65-68.

13. Aguilera SVU, Moysés ST, Moysés SJ. Intervenções de segurança viária e seus efeitos nas lesões causadas pelo trânsito: uma revisão sistemática. Rev Panam Salud Publica. 2014; 36 (4): 257-265.

14. Almeida NA. Os acidentes e mortes no trânsito causados pelo consumo de álcool: um problema de saúde pública. R. Dir. sanit. 2014; 15 (2): 108-125.

15. Oliveira DF, Friche AAL, Costa DAS, Mingotti SA, Caiaffa WT. Do speed cameras reduce speeding in urban areas?

Cad. Saúde Pública. 2015; 31 [Supl]: S1-S12.

16. Souza CS, Costa MCO, Carvalho RC, Araújo TM, Amaral MTR. Notificação da violência infanto-juvenil em Serviços de Emergência do Sistema Único de Saúde em Feira de Santana, Bahia, Brasil. Rev. bras. epidemiol. 2015; 18 (1): 80-93.

17. Prefeitura Municipal de São Paulo. Secretaria Municipal de Saúde. Tabnet. Sistema de Informação de Vigilância de Violência e Acidentes. Disponível em: http://www.prefeitura.sp.gov.br. Acessado em: 09 out 2016.

18. Souza CS, Costa MCO, Assis SG, Musse JO, Nascimento Sobrinho C, Amaral MTR. Sistema de Vigilância de Violências e Acidentes/VIVA e a notificação da violência infanto-juvenil, no Sistema Único de Saúde/SUS de Feira de Santana-Bahia, Brasil. Ciênc. saúde coletiva. 2014; 19 (3): 773-784.

19. Gomes ATL, Silva MF, Dantas BAS, Dantas RAN, Mendonça AEO, Torres GV. Caracterização dos acidentes de trânsito assistidos por um serviço de atendimento móvel de urgência. J. res. fundam. care. online. 2016;8 (2):4269-4279.

7. Fantoni R, Volpe FM.A subjetividade na avaliação do risco e sua influência nas políticas públicas de prevenção da violência no trânsito. Rev Med Minas Gerais. 2014; 24 [Supl 5]: S47-S56.

20. Silva FHVC. A psicologia do trânsito e os 50 anos de profissão no Brasil. PSICOLOGIA: CIÊNCIA E PROFISSÃO. 2012; 32 [num. esp.]: 176-193.

8. Polli GM, Thielene IP, Hartmann RC, Soares DP. Excesso de velocidade no trânsito: análise sob a perspectiva da moralida. Psicol. cienc. prof. 2010; 30 (1): 86-97.

21. Vieira Netto MF, Deslandes SF. As Estratégias da Saúde da Família no enfrentamento das violências envolvendo adolescentes. Ciênc. saúde coletiva. 2016; 21 (5): 1583-1595.

Cintia Leci Rodrigues et al./ International Journal of Medical Reviews and Case Reports (2017) 1(1): 1-5

22. Conceição JC, Gusmão MEN, Souza SS, Gomes NP. Elementos que dificultam a notificação da violência: percepção dos profissionais de saúde. Revista Baiana de Enfermagem.

2012; 26 (2): 468-477.

23. Andrade SSCA, Mello Jorge MHP. Estimate of physical sequelae in victims of road traffic accidents hospitalized in the Public Health System. Rev. bras. epidemiol. 2016; 19 (1): 100-111.

Cintia Leci Rodrigues et al./ International Journal of Medical Reviews and Case Reports (2017) 1(1): 1-5

CASE REPORT

KIKUCHI-FUJIMOTO DISEASE Irami Araújo-Filho∗, 1 , Artur Dantas Freire∗∗ , Irami Araújo-Neto∗∗ , Carolina Chianca Dourado Lemos∗∗ , Letícia Araújo Costa Uchôa∗∗ , Amália Cínthia Meneses Rêgo∗∗ , Marco Antônio Botelho Soares∗∗ , José Francisco Correia-Neto♦ , Carlos André Nunes Jatobá♦ and Ana Maria de Oliveira Ramos♦ ∗ Department

of Surgery, Potiguar University – Laureate International Universities – Natal, Rio Grande do Norte, Brazil., ∗∗ Potiguar University – Laureate International Universities – Natal, Rio Grande do Norte, Brazil., ♦ Department of Pathology, Potiguar University – Laureate International Universities, Natal, Rio Grande do Norte, Brazil.

ABSTRACT Objective:The development of associated febrile to lymphadenomegaly leads to several diagnostic hypotheses, among them the Kikushi-Fujimoto disease.Method:This review was set up by searching PubMed/Medline, Web of Science and Scopus database using the following key words: “Kikushi disease”, “Kikuchi-Fujimoto disease”, “histiocytic necrotizing”, “lymphadenitis”,“lymphadenopathy”. Results:We report a case in a young patient with a fever associated with the emergence of adenomegaly and weight loss in two months, associated with hepatosplenomegaly.

In laboratory tests showed anaemia with erythrocyte sedimentation rate (ESH) and lactate dehydrogenase (LDH) test elevated, widened mediastinum, with bilateral pleural effusion. Conclusion:Excisional biopsy of supraclavicular lymph node showed Kikuchi-Fujimoto disease on microscopic examination. After histopathological confirmation, were other causes of febrile adenomegaly apart, starting dose corticosteroid immunosuppressive therapy.

KEYWORDS Kikushi disease; Kikuchi-Fujimoto disease; histiocytic necrotizing lymphadenitis; lymphadenopathy.

Herpes virus type 6 and 8[3], Human Immunodeficiency virus (HIV), Parvovirus B19[4], paramyxovirus, parainfluenza viruses, Yersinia enterocolitis, and Toxoplasma[3].

Introduction Kikuchi’s disease, also called Kikuchi-Fujimoto disease or Histiocytic Necrotizing Lymphadenitis, was initially described in young women. Is a rare pathology, of unknown cause, benign, generally characterised by cervical lymphadenopathy and fever.

The histopathological examination of the lymph nodes involved differentiates Kikuchi disease of other more serious conditions.

Although the pathogenesis of Kikuchi disease is unknown, the clinical presentation is clear, and histological changes suggest an immune response of T cells and histiocytes to an infectious agent. Numerous agents urging the sickness have been proposed, including the Epstein-Barr Virus (EBV)[1,2] Human Copyright © 2017 by the Bulgarian Association of Young Surgeons DOI: 10.5455/IJMRCR.kikuchi-fujimoto-disease First Received: April 04, 2017 Accepted: May 11, 2017 Manuscript Associate Editor: George Baytchev (BG) Editor-in Chief: Cvetanka Hristova (BG) Reviewers: Ivan Inkov (BG) 1 Prof. Dr. Irami Araújo Filho, Full Professor of the Department of Surgery, PhD in Health Sciences. Full Professor of the Post-Graduate Program in Biotechnology at Potiguar University – Laureate International Universities, Natal, Rio Grande do Norte, Brazil. E-mail: irami.filho@gmail.com

Apoptotic cell death mediated by CD8 + cytotoxic T lymphocytes positive is the primary mechanism of cell destruction[5-7].

Although initially described in young women, the Kikuchi disease clearly also occurs in men. The proportion of men and women affected by three reviews was 1:4, 1:1,6 and 1:1,26, always with a predominance of women affected[8-10]. Most patients are under 40 years of age[11-14].

The most common clinical presentation of the disease is fever and cervical lymphadenopathy in a previously healthy young patient. Fever, usually low and persisted for about a week is a primary symptom in 30 to 50% of patients [8].

Methods: This review was set up by searching PubMed, Web of Science and Scopus database using the following key words: “Kikushi disease”, “Kikuchi-Fujimoto disease”, “histiocytic necrotizing”, “lymphadenitis”, “lymphadenopathy”.

Irami Araújo-Filho et al./ International Journal of Medical Reviews and Case Reports (2017) 1(1): 6-10

Case report The woman, 20 years old, he began to rub the frame with symmetrical fists, knees and ankles and daily 39oC fever. Two months after the onset of symptoms, cervical tumour arose and painful. Associated to the feverish, she referred 7% of body weight in two months. Was admitted to the hospital with the symptoms described, showing Traube’ space busy with splenomegaly (4cm of the costal edge left), hepatomegaly (6cm from the costal edge right) and generalized lymphadenopathy (axillary, supraclavicular chains left and cervical inguinal), being the largest lymph node found in the cervical region left measuring 06cm in your larger diameter, fibroblastic, painful, not acceded to plans. The laboratory tests revealed hemoglobin 9.2 g/dL; hematocrit 26.3%; leukocytes 3900/mm³ with 84% segmented and 8% of lymphocytes; 119000 platelets/mm³; erythrocyte sedimentation rate (ESR) 110 mm; 1.7 mg creatinine/dL; total protein 5 g/dL; albumin 2,4g/dL; AST 220 U/L; ALT: 79 U/L, GGT: 247 U/L, LDH:2046 IU/Ml; alkaline phosphatase 551 U/L, 0,6% reticulocytes and direct Coombs test negative. The serology for hepatitis B, C and HIV negative. Had an antinuclear factor (ANF) profile with core: no reagent; metaphase chromosomal plate reagent and antibody ribonucleoprotein no reagent.

The chest x-ray showed a widening of the mediastinum, pleural effusion and bilateral pulmonary consolidation right. Antibiotic therapy was initiated with Cefepime for eight days. During the diagnostic investigation was prompted computed tomography (CT) of total abdomen whose result revealed moderate ascites and lymphadenopathy in the various chains of retroperitoneal and bilaterally iliac lymph nodes. The chest CT evicted small bilateral pleural effusion, with atelectasis, mediastinal lymphadenopathy, bilaterally axillary and left the supraclavicular region. Held left supraclavicular lymph node biopsy, showing histolytic necrotizing lymphadenitis compatible with KikuchiFujimoto disease, represented in figures 1-4. Corticotherapy began in dose immunosuppressive therapy for 21 days. Evolved with dramatic improvement of the clinical picture, improvement of joint pain and significant reduction of adenomegaly.

Discussion: We report a case of Kikuchi-Fujimoto disease. The actual incidence of this disease is estimated at between 0.5%, and 5% of all analysed lymph histologically; Not found any infectious microorganism (virus or bacteria) to the case reported, but it is possible that the antigenic stimulus has been triggered by one of these[15].

Clinical condition After reviewing the literature on the topic, it was found that the most common symptoms were fever (35%), fatigue (7%) and joint pain (7%) [16]. The most common clinical and laboratory findings were lymphadenopathy (100%), rash (10%), arthritis (7%), hepatosplenomegaly (3%), leukopenia (43%), high sedimentation rate (40%) and anaemia (23%)[16].

Systemic symptoms may accompany fever and lymphadenopathy and seem to be more prominent in patients with extranodal involvement[17]. Systemic symptoms include night sweats, nausea, vomiting, weight loss (by about 10%) and diarrhea[15-17].

A variety of other symptoms and physical signs occur sporadically in patients with Kikuchi disease. These include chills, myalgia, arthralgia, pain in the chest and abdomen,

splenomegaly and hepatomegaly, which may be associated with abdominal lymphadenopathy, as was evidenced in the case reported[8].

The involvement of lymph nodes is usually cervical and located in Kikuchi disease[18]. The nodules are moderately increased in diameter (1-2cm) but occasionally are higher (≥ 7cm)[18]. They are typically firm, fibroelastic, discreet and furniture. The increased lymph node is often associated with a mild pain[18].

The increase in the size of the mediastinal lymph nodes is minimal and only. However retroperitoneal nodules may be involved[19,20]. The diagnosis is unlikely to be confirmed until the completion of lymph node biopsy in such patients that present with fever of unknown origin.

Laboratory tests Most patients with Kikuchi disease have a normal blood count[8], even though leukopenia is observed in 20-32%[9,21].

Atypical lymphocytes are reported in up to 25% of patients[18].

Other less common findings include thrombocytopenia, pancytopenia, and, in those with severe disease, chronic anemia[1,22].

The erythrocyte sedimentation rate can be normal but was elevated to more than 60 mm/h in 70% of patients in a series[19].

Other non-specific findings may include slightly abnormal liver function tests and high levels of lactate dehydrogenase[20].

Antinuclear antibodies (ANA), rheumatoid factor, lupus erythematosus and preparations are negative. Some patients initially diagnosed with Kikuchi disease presented later systemic lupus erythematosus (SLE)[8,23]. The FAN should be performed in patients with suspicion of Kikuchi disease that have features suggestive of LES to delete such a diagnosis. A study describes transient elevation anti-DNA antibody levels of protein antiribonuclear[9].

Diagnosis Lymph node biopsy makes diagnosis of the disease of KikuchiFujimoto. The biopsy should be performed, despite the selflimiting nature of this syndrome to rule out more serious conditions that require aggressive therapy such as lymphoma.

Kikuchi disease patients were diagnosed as having lymphoma treated with cytotoxic agents when doctors and pathologists still did not know in detail this entity[8]. Other pathologies that were confused with Kikuchi disease tuberculous adenitis, venereum lymphogranuloma and Kawasaki disease[24-26].

Though excisional biopsy is often recommended because it often breaks up a framework of lymphoma, the fine needle aspiration is increasingly useful in the hands of the experienced pathologist, using colours and cell block preparations, allowing diagnosis[27,28].

Pathology The histology of the lymph node in Kikuchi-Fujimoto disease can easily be differentiated from more well-known infectious conditions in the differential diagnosis of fever and Lymphadenopathy [8-11],[29]. Yellow necrotic foci can hardly be noticed in the cutting surface of the nodule. Microscopic examination usually shows outbreaks paracortical with necrosis and cellular infiltrate histiocytic. These outbreaks can be single or multiple. The capsule can be infiltrated, and perinodal inflammation is common.

The necrotizing process is often confined to circumscribed areas

Irami Araújo-Filho et al./ International Journal of Medical Reviews and Case Reports (2017) 1(1): 6-10

of fibrinoid Eosinophilic material with an irregular distribution of fragments of rubble nuclear[11].

The histological appearance changes as the disease progress.

Early biopsies on "proliferative phase" show follicular hyperplasia and paracortical expansion by T cells, B cells and monocytes and miscellaneous plasmacytoid histiocytes with numerous deep down apoptosis[30].

In "the proliferative phase," the presence of several blastic cells raises the differential diagnosis of lymphoma, infection with Epstein-Barr virus (EBV), and herpes simplex infection.

The preservation of nodal architecture, the mole, and polyclonal immunohistochemistry negative viral conditions exclude[30].

Subsequent biopsies on "Necrotizing phase" have shown without a neutrophilic infiltrate necrosis associated with the domain of histiocytes. The histiocytes often have nuclei in growing and contain dendrites phagocytosed. Immunohistochemical staining shows positive monocytes plasmacytoid and CD68 histiocytes with predominantly CD8 positive T lymphocytes[30]. The absence of neutrophils in "Necrotizing phase" is useful to distinguish this condition from LES and drug-induced lymphadenopathy.

Figure 1:Axillary lymph node blade, representing necrotizing lymphadenitis with nuclear fragmentation and reactive hyperplasia - Image with a magnification of 100x.

Figure 2: Axillary lymph node blade, representing necrotizing lymphadenitis with nuclear fragmentation - Image with a magnification of 400x.

Figure 3:Axillary lymph node blade, representing necrotizing lymphadenitis with nuclear fragmentation and reactive hyperplasia - Image with a magnification of 50x.

Radiology Differential diagnosis The differential diagnosis of the histological point of view includes LES, herpes simplex and lymphoma (non-Hodgkin’s lymphoma and Hodgkin’s Lymphoma). In the LES, bodies of hematoxylin and plasma cells are also seen. In herpes simplex, there are fewer surrounding mononuclear cells and neutrophils are commonly present. In contrast to the Kikuchi disease, necrosis associated with Hodgkin’s lymphoma usually includes neutrophils and large atypical cells (cell variants of Reed-Sternberg cells), positive for CD30, CD15, CD45. It is suggested that the plasmacytoid dendritic cells infiltrate in lymph nodes with higher frequency in Kikuchi-Fujimoto disease when compared to any other reactive lymphadenitis or B or T cell lymphoma, regardless of the size of the lesion. Thus, the predominance of plasmacytoid dendritic cells can be a useful indicator in the diagnosis of hepatological disease of Kikuchi[31].

Computed tomography (CT) of the affected lymph nodes typically demonstrates perinodal infiltration (81%) and homogeneous enhancement (83%)[32]. On ultrasound, the lymph nodes can present radiological features of malignancy[33].

Treatment No effective treatment for the disease has been established of Kikuchi-Fujimoto to the present. The signs and symptoms usually disappear within one to four months. Patients with severe or persistent symptoms are treated with high-dose glucocorticoids associated with intravenous immunoglobulin, or not showing results apparently promising[34,35]. There have been reports of success in the treatment of recurrent disease of Kikuchi-Fujimoto with hydroxychloroquine[36]. Affected patients should be followed for a few years because they may be affected of LES and relapses of the disease of Kikuchi-Fujimoto are applicants for several years, after the first episode of disease[22-26].

Irami Araújo-Filho et al./ International Journal of Medical Reviews and Case Reports (2017) 1(1): 6-10

Conclusion Excisional biopsy of supraclavicular lymph node showed Kikuchi-Fujimoto disease on microscopic examination. After histopathological confirmation, were other causes of febrile adenomegalias apart, starting dose corticosteroid immunosuppressive therapy.

Competing Interests Written informed consent obtained from the patient for publication of this case report and any accompanying images.

References 1. Yen A, Fearneyhough P, Raimer SS, Hudnall SD. EBVassociated Kikuchi’s histiocytic necrotizing lymphadenitis with cutaneous manifestations. J Am Acad Dermatol 1997; 36:342.

2. Hudnall SD, Chen T, Amr S, et al. Detection of human herpesvirus DNA in Kikuchi-Fujimoto disease and reactive lymphoid hyperplasia. Int J Clin Exp Pathol 2008; 1:362.

3. Huh J, Kang GH, Gong G, et al. Kaposi’s sarcomaassociated herpesvirus in Kikuchi’s disease. Hum Pathol 1998; 29:1091.

13. Payne JH, Evans M, Gerrard MP. Kikuchi-Fujimoto disease: a rare but important cause of lymphadenopathy. Acta Paediatr 2003; 92:261.

14. Ray A, Muse VV, Boyer DF. Case records of the Massachusetts General Hospital. Case 38-2013. A 30-year-old man with fever and lymphadenopathy. N Engl J Med 2013; 369:2333.

15. Infante MJ, Lovillo C, Santaella IO, Checa RM, González MR. Enfermedad de Kikuchi-Fujimoto como causa de linfadenopatías. An Pediatr (Barc). 2007;67(1):83-5.

16. Kucukardali Y, Solmazgul E, Kunter E, et al. KikuchiFujimoto Disease: analysis of 244 cases. Clin Rheumatol 2007; 26:50.

17. Kuo TT. Cutaneous manifestation of Kikuchi’s histiocytic necrotizing lymphadenitis. Am J Surg Pathol 1990; 14:872.

18. Kuo TT. Kikuchi’s disease (histiocytic necrotizing lymphadenitis). A clinicopathologic study of 79 cases with an analysis of histologic subtypes, immunohistology, and DNA ploidy. Am J Surg Pathol 1995; 19:798.

19. Norris AH, Krasinskas AM, Salhany KE, Gluckman SJ. Kikuchi-Fujimoto disease: a benign cause of fever and lymphadenopathy. Am J Med 1996; 101:401.

4. Yufu Y, Matsumoto M, Miyamura T, et al. Parvovirus B19-associated haemophagocytic syndrome with lymphadenopathy resembling histiocytic necrotizing lymphadenitis (Kikuchi’s disease). Br J Haematol 1997; 96:868.

20. Bailey EM, Klein NC, Cunha BA. Kikuchi’s disease with liver dysfunction presenting as fever of unknown origin.

Lancet 1989; 2:986.

5. Iguchi H, Sunami K, Yamane H, et al. Apoptotic cell death in Kikuchi’s disease: a TEM study. Acta Otolaryngol Suppl 1998; 538:250.

21. Song JY, Cheong HJ, Kee SY, et al. Disease spectrum of cervical lymphadenitis: analysis based on ultrasound-guided core-needle gun biopsy. J Infect 2007; 55:310.

6. Ohshima K, Shimazaki K, Kume T, et al. Perforin and Fas pathways of cytotoxic T-cells in histiocytic necrotizing lymphadenitis. Histopathology 1998; 33:471.

7. Ura H, Yamada N, Torii H, et al. Histiocytic necrotizing lymphadenitis (Kikuchi’s disease): the necrotic appearance of the lymph node cells is caused by apoptosis. J Dermatol 1999; 26:385.

8. Dorfman RF, Berry GJ. Kikuchi’s histiocytic necrotizing lymphadenitis: an analysis of 108 cases with emphasis on differential diagnosis. Semin Diagn Pathol 1988; 5:329.

9. Asano S, Akaike Y, Jinnouchi H, et al. Necrotizing lymphadenitis: a review of clinicopathological, immunohistochemical and ultrastructural studies. Hematol Oncol 1990; 8:251.

10. Lin HC, Su CY, Huang CC, et al. Kikuchi’s disease: a review and analysis of 61 cases. Otolaryngol Head Neck Surg 2003; 128:650.

11. Tsang WY, Chan JK, Ng CS. Kikuchi’s lymphadenitis. A morphologic analysis of 75 cases with particular reference to unusual features. Am J Surg Pathol 1994; 18:219.

12. Lee KY, Yeon YH, Lee BC. Kikuchi-Fujimoto disease with prolonged fever in children. Pediatrics 2004; 114:e752.

22. Smith KG, Becker GJ, Busmanis I. Recurrent Kikuchi’s disease. Lancet 1992; 340:124.

23. Patra A, Bhattacharya SK. SLE Developing in a Follow-Up Patient of Kikuchi’s Disease: A Rare Disorder. J Clin Diagn Res 2013; 7:752.

24. Mital D, Desai V, Chin K. Kikuchi-Fujimoto syndrome presenting to a sexual health clinic. Int J STD AIDS 2009; 20:140.

25. Dorfman RF, Warnke R. Lymphadenopathy simulating the malignant lymphomas. Hum Pathol 1974; 5:519.

26. Nieman RB. Diagnosis of Kikuchi’s disease. Lancet 1990; 335:295.

27. Tsang WY, Chan JK. Fine-needle aspiration cytologic diagnosis of Kikuchi’s lymphadenitis. A report of 27 cases. Am J Clin Pathol 1994; 102:454.

28. Mannarà GM, Boccato P, Rinaldo A, et al. Histiocytic necrotizing lymphadenitis (Kikuchi-Fujimoto disease) diagnosed by fine needle aspiration biopsy. ORL J Otorhinolaryngol Relat Spec 1999; 61:367.

29. Atwater AR, Longley BJ, Aughenbaugh WD. Kikuchi’s disease: case report and systematic review of cutaneous and histopathologic presentations. J Am Acad Dermatol 2008; 59:130.

Irami Araújo-Filho et al./ International Journal of Medical Reviews and Case Reports (2017) 1(1): 6-10

30. Ohshima K, Karube K, Hamasaki M, et al. Apoptosis- and cell cycle-associated gene expression profiling of histiocytic necrotising lymphadenitis. Eur J Haematol 2004; 72:322.

31. Kishimoto K, Tate G, Kitamura T, et al. Cytologic features and frequency of plasmacytoid dendritic cells in the lymph nodes of patients with histiocytic necrotizing lymphadenitis (Kikuchi-Fujimoto disease). Diagn Cytopathol 2010; 38:521.

32. Kwon SY, Kim TK, Kim YS, et al. CT findings in Kikuchi disease: analysis of 96 cases. AJNR Am J Neuroradiol 2004; 25:1099.

33. Youk JH, Kim EK, Ko KH, Kim MJ. Sonographic features of axillary lymphadenopathy caused by Kikuchi disease. J Ultrasound Med 2008; 27:847.

34. Jang YJ, Park KH, Seok HJ. Management of Kikuchi’s disease using glucocorticoid. J Laryngol Otol 2000; 114:709.

35. Lin DY, Villegas MS, Tan PL, et al. Severe Kikuchi’s disease responsive to immune modulation. Singapore Med J 2010; 51:e18.

36. Rezai K, Kuchipudi S, Chundi V, et al. Kikuchi-Fujimoto disease: hydroxychloroquine as a treatment. Clin Infect Dis 2004; 39:e124.

Irami Araújo-Filho et al./ International Journal of Medical Reviews and Case Reports (2017) 1(1): 6-10

CASE REPORT

PANCREAS DIVISUM CAUSING RECURRENT PANCREATITIS: A CASE REPORT Nadeem Siddiqui∗ , Rizwan Sultan∗ and Fareed Shaikh∗,1 ∗ Department

of Surgery Aga Khan University Hospital, Karachi, Pakistan.

ABSTRACT Background: Pancreas divisum is one of the rare causes of recurrent pancreatitis. Diagnosis is challenging and diagnostic failure leads to recurrent episodes of pancreatitis and ultimately pancreatic failure. Case: Here we are presenting a case of a young female with a history of recurrent attacks of upper abdominal pain which on work up was diagnosed as pancreas divisum. This patient was successfully treated with ERCP with minor papillotomy followed by stenting in major papilla. Written informed consent was taken from the patient before writing this case report.Conclusion: This report emphasises the need for early suspicion of disease, in patients with recurrent idiopathic pancreatitis, by clinician and efficacy of minimally invasive procedures (ERCP) as a definitive treatment option.

KEYWORDS Pancreas Divisum, Recurrent pancreatitis, Endoscopic Papillotomy

Introduction Pancreas divisum is one of the most commonly encountered congenital anomalies in the hepatobiliary system. The incidence is variable, but it ranges from 5% to 14% of the general population [1]. The disease occurs due to failure of fusion of the two embryonic parts of the pancreas i.e. non-fusion of dorsal and ventral ducts of pancreas resulting in openings at abnormal positions, This abnormal opening of primary pancreatic duct at minor papilla is not enough to completely drain the duct secretions resulting in stasis of enzymes and premature activation of these enzymes results in recurrent attacks of pancreatitis [2, 3].

Most of the patients are asymptomatic, and diagnosis is incidental. Abdominal ultrasound is not a very useful modality for diagnosis. Computed tomography (CT) of the abdomen may help in diagnosis, but imaging of choice is cholangiography (ERCP or MRCP). MRCP being better as it is noninvasive but ERCP has an advantage in its diagnostic as well as therapeutic capacity. Treatment is usually nonsurgical in the form of Copyright © 2017 by the Bulgarian Association of Young Surgeons DOI: 10.5455/IJMRCR.pancreas-divisum-recurrent-pancreatitis First Received: April 12, 2017 Accepted: May 09, 2017 Manuscript Associate Editor: George Baytchev (BG) Editor-in Chief: Cvetanka Hristova (BG) Reviewers: Ivan Inkov (BG) 1 [Department of Surgery Aga Khan University Hospital, Karachi, Pakistan. Email: fareed.shaikh@aku.edu

endoscopic sphincterotomy.

Here we are presenting a case of 24 years young female with a history of recurrent attacks of pancreatitis, which on workup is found to be secondary to pancreas divisum. She was successfully treated with endoscopic sphincterotomy with the pain-free postprocedure course. Report points out the need for early disease suspicion and efficacy of endoscopic sphincterotomy for the treatment of this disease.

Case Presentation: Written informed consent was taken before reporting this case report. Our patient was a 24 years old lady, presented in the emergency department with a complaint of sudden onset severe continuous epigastric pain for last four days. This pain was not radiating, aggravated by food intake and relieved only with opioid analgesics. The pain was also associated with non-bilious vomiting. She had an episode of similar pain about one year back for which she was admitted and managed as acute pancreatitis. She had a history of the acid peptic disease, anorexia, and malaise and weight loss of 6 Kg in last three years. On examination, she was dehydrated and jaundiced. Her pulse was 96/minute; BP was 117/68, afebrile. Abdominal examination revealed tenderness in an epigastric area without any mass or palpable viscera. Rest of systemic examination was unremarkable.

Her initial laboratory workup showed TLC of 10600/mm3, total bilirubin 1.2 mg% (0.2-1.2) with direct bilirubin 0.9 mg% (0.0 to 0.4), alanine aminotransferase of 71 IU/l (normal 0-31

Fareed Shaikh et al./ International Journal of Medical Reviews and Case Reports (2017) 1(1): 11-13

IU/l), aspartate aminotransferase of 251 IU/l (normal 0-34 IU/l), alkaline phosphatase of 85 IU/l (12-38 IU/l), serum amylase was 99 IU/l (25-125 IU/l) and serum lipase was 167 IU/l (3-60 IU/l).

Ultrasonography (US) of abdomen surprisingly revealed a hypoechoic solid mass in the head of the pancreas resulting in atrophy of pancreatic body and tail, dilatation of pancreatic duct and prominent common bile duct suggestive of the neoplastic process.

To further delineate this situation, CT scan of the abdomen was done which showed a hypodense mass involving the uncinate process of the pancreas causing atrophy of body and tail of pancreas without any infiltration into surrounding structures.

Pancreatic duct and common bile duct were prominent without dilatation of intrahepatic biliary channels appeared dilated.

(Figure 1) This CT scan was discussed in length with radiol-

which confirmed the presence of minor papilla about 5 mm proximal to the major papilla, major pancreatic duct was opening at minor papilla while minor pancreatic duct was opening at major papilla (figure 2), Minor papillotomy was done and plastic stent placed in major pancreatic duct. The patient tolerated the procedure well, and no immediate or delayed complication was identified.

Figure 2: ERCP showing presence of minor papilla about 5 mm proximal to the major papilla, major pancreatic duct was opening at minor papilla (upper figure) while minor pancreatic duct was opening at major papilla (lower figure).

Post procedure, the patient had a smooth recovery with significant improvement in abdominal pain and appetite. She was discharged on 3rd post procedure day. On clinic follow-up of 6 months, she has remained pain-free and leading a normal healthy life.

Discusion: Figure 1: CT scan showing hypodense mass involving the uncinate process of pancreas and dilated common bile duct and pancreatic duct. ogists as it is hard to differentiate between Pancreas Divisum and pancreatic malignancy. Finally, a provisional diagnosis of Pancreas Divisum was made as there was a convincing evidence of opening of dilated primary pancreatic duct at minor papilla.

ERCP was done as diagnostic as well as therapeutic measure,

Pancreatic Divisum is a congenital anatomical anomaly characterised by the lack of fusion of the ventral and dorsal parts of the pancreas during the eighth week of fetal development. This condition is found in 5% to 14% of the general population [1].

In a large retrospective study from India, pancreas divisum was more frequent in patients with pancreatitis than in those with biliary diseases or obscure abdominal pain (9 versus 2 percent).

The major pancreatic duct (Wirsung’s duct), drains the secretions from the head, body and tail of the exocrine pancreas, and ends at the major duodenal papilla (hepatopancreatic ampulla);

Fareed Shaikh et al./ International Journal of Medical Reviews and Case Reports (2017) 1(1): 11-13

the accessory pancreatic duct (Santorini’s duct) extends through the head of the pancreas, crosses Wirsung’s duct and ends at the minor duodenal papilla; both pancreatic outlets are located on the medial wall of the second part of the duodenum at a distance of approximately 10 to 15 mm from each other; the minor papilla are above, the major duodenal papilla below.

In Pancreatic Divisum, the dorsal pancreatic section drains into the minor duodenal papilla through the major pancreatic duct; the ventral pancreatic duct, the smaller part of the pancreas, merges with the common bile duct at the hepatopancreatic ampulla. There are two types of Pancreatic Divisum: complete (most common) and incomplete (much less common), in which the ventral and dorsal systems remain connected through smallcaliber branch ducts. Approximately 15 percent of cases of pancreas divisum are of the incomplete type. However, the clinical implications of incomplete pancreas divisum are the same as for classic (or complete) pancreas divisum, In PD, the increased incidence of acute and chronic pancreatitis is caused by inadequate drainage of secretions produced by the body, tail and part of the pancreatic head through an orifice which is too small [4, 5]. There is a group of patients with pancreas divisum who are subject to recurrent bouts of seemingly idiopathic pancreatitis [6]. In these patients, the minor papilla orifice is so small that excessively high intrapancreatic dorsal ductal pressure occurs during active secretion, which may result in inadequate drainage, ductal distension, pain, and, in some cases, pancreatitis. Although many times, patients with pancreas divisum remain clinically asymptomatic, other common forms of clinical presentations range from recurrent attacks of varying degree of pancreatitis, bowel obstruction, ascites, jaundice, shock and in its most severe form, can lead to shock. Alcohol seems to be the triggering factor for the attack of pancreatitis [6].

Our patient presented with upper abdominal pain secondary to pancreatitis.

References 1. Liao Z, Gao R, Wang W, et al. A systematic review on endoscopic detection rate, endotherapy, and surgery for pancreas divisum. Endoscopy 2009;41:439-44.

2. Vasile D, Grigoriu M, Turcu F, et al. [Pancreas divisumea rare cause of chronic pancreatitis]. Chirurgia (Bucur) 2007; 102:83-7.

3. Gonoi W, Akai H, Hagiwara K, et al. Pancreas divisum as a predisposing factor for chronic and recurrent idiopathic pancreatitis: initial in vivo survey. Gut (2011); doi:10.1136/gut.2010.230011.

4. Kamisawa T, Tu Y, Egawa N, et al. Clinical implications of incomplete pancreas divisum. JOP 2006; 7:625-30.

5. Ng WK, Tarabain O. Pancreas divisum: a cause of idiopathic acute pancreatitis. CMAJ 2009; 180:949-51.

6. Elena G, Dorin A, Roxana B, Gheorghe B. Pancreas divisum pancreatitis: a case report. Abdom Imaging (2011) 36:215–217.

7. Kamisawa T, T. Y. (2007). MRCP of congenital pancreaticobiliary malformation. Abdom Imaging, 129-133.

8. GA, L. (2003). Acute recurrent pancreatitis. Can J Gastroenterol, 381-383.

9. Kamisawa T (2004) Clinical significance of the minor duodenal papilla and accessory pancreatic duct. J Gastroenterol 39:605–615.

Diagnostic workup ranges for laboratory tests to imaging modalities. Laboratory workup may show raised amylase or lipase levels indicating an episode of pancreatitis, but they are not accurate for diagnosing Pancreas Divisum. Imaging modalities which may help in diagnosis include ultrasound, CT scan abdomen but definitive diagnosis is made with some form of cholangiography either ERCP or MRCP. PD is most of the times diagnosed from MRCP but the important point to note here is that currently used 64 slicer CT scan is a good modality to diagnose PD like in our case, especially when the diagnosis is not being suspected [7].

The usual therapeutic solution for symptomatic Pancreatic Divisum is a sphincterotomy of the minor duodenal papilla, which decongests Wirsung’s duct [8, 9]. Clinical improvement with such treatment has been observed in up to 75 percent of patients. Rarely, in selected cases only, is surgical treatment indicated: surgical sphincterotomy, draining or even partial pancreatectomies and their results are comparable with those achieved by endoscopic procedure [6].

Conclusion This report emphasizes the need of early suspicion of disease, in patients with recurrent idiopathic pancreatitis, by clinician and efficacy of minimally invasive procedures (ERCP) as definitive treatment option.

Fareed Shaikh et al./ International Journal of Medical Reviews and Case Reports (2017) 1(1): 11-13

CASE REPORT

VULVAR NON-FILARIAL ELEPHANTIASIS ASSOCIATED WITH SCABIES Sarah Sabur∗,1 , Saoussane Bounajma∗ , Mounia Nasr∗ and Samir Mazouz∗ ∗ Department

of Plastic Surgery, Ibn Sina teaching hospital, Mohamed V University, Rabat, Morocco.

ABSTRACT Vulvar elephantiasis is a very rare condition often caused by filariasis infection. Other causes like general inflammatory disease, cancer treatment, traumatic causes or other infections can also explain the occurrence of this condition. In some cases, it is due to unknown causes. In this study, we present the clinical case of a Moroccan youth female patient with no significant history, who presented vulvar elephantiasis associated with scabies with no evidence of filariasis infection, general inflammatory disease, malignancy, or other infection. This clinical case is unusual because of the large volume of lymphedema, its atypical localisation, its association with generalised scabies, and the absence of a well-defined aetiology, which may explain its occurrence.

KEYWORDS Elephantiasis, vulvar, scabies, lymphedema.

Introduction Elephantiasis describes a massive enlargement of a limb or the external genitalia because of chronic lymphedema [1]. The elephantiasis of the vulva is a rare condition often caused by filariasis in developing nations [3]. In this study, we present the clinical case of a 27-year-old patient with no significant medical history, who presented elephantiasis of the vulva evolved one year ago, associated with scabies. The patient did not show any evidence of filariasis infection or any other known cause which can explain the occurrence of this condition.

Case report: A female patient of 27-years-old, with no previous medical history, was admitted to our department for vulvar elephantiasis of one-year evolution associated with generalised itchy skin lesions. The general clinical examination found: BMI=26, normal body temperature, generalised, smooth and slightly elevated Copyright © 2017 by the Bulgarian Association of Young Surgeons DOI: 10.5455/IJMRCR.vulvar-non-filarial-elephantiasis-scabies First Received: March 22, 2017 Accepted: April 15, 2017 Manuscript Associate Editor: George Baytchev (BG) Editor-in Chief: Ivan Inkov (BG) Reviewers: Khadija Bellahammou (MA) 1 Sarah Sabur, Plastic Surgery Departement - Ibn Sina Teaching Hospital Mohamed V University Rabat, Morocco. sarah.kadhem77@gmail.com

Figure 1: Anterior view of the vulva.

Sarah Sabur et al./ International Journal of Medical Reviews and Case Reports (2017) 1(1): 14-16

Figure 4: the two masses after excision.

Figure 2: Posterior view of the vulva.

Figure 5: Vulvoplasty. tures were realised to close the residual substance loss (figure 5).

The pieces were sent to the anatomopathological study which returned for vulvar lymphedema, without any sign of malignancy.

The surgical follow-up was simple, but the patient presented again after two months with a new recurrence of lymphedema in the left labia majora (figure 6), without any associated general skin lesions.

Discussion: Figure 3: view of the left and right labia majora with the two masses.

erythematous papules, and scratch lesions. The vulvar examination found a voluminous pendulous swelling mass in the left labia majora with minimal skin inflammation, and moderate lymphedema in the right labia majora, (figure 1, 2). The clinical examination also found bilateral inguinal adenopathy, and lower limb varices. However, the clinical and radiological examinations did not reveal the presence of an abdominal or gynaecological mass. The biological testing showed an increasing number of circulating eosinophilic granulocytes. C-reactive protein (CRP) was also elevated (84 mg/L), and the routine biochemistry, glucose, serological testing for sexually transmitted infection (hepatitis B, HIV, syphilis) didn’t show any particularity. We also performed a Midnight peripheral blood smear for filarial worms, but the result was negative. The patient received an antiscabies treatment for her skin lesions, and she underwent into a complete excision of her vulvar masses, which carried out a mass of 2 kg from the left labia majora and a mass of 500 g from the right labia majora (Figures 3 and 4). Simple cutaneous su-

Lymphedema can be defined as swelling of soft tissues which are the result of the accumulation of protein-rich interstitial fluids caused by a low output failure of lymph [3].

The Impairment of lymphatic drainage can be due to congenital abnormal vessel development (primary lymphedema) or damaged lymphatic vessels from infection, trauma, surgery, ra-

Figure 6: Recurrence of lymphedema after one month.

Sarah Sabur et al./ International Journal of Medical Reviews and Case Reports (2017) 1(1): 14-16

diation, persistent inflammation, obstruction due to metastases or parasite infestation (secondary lymphedema) [1].

Lymphedema is frequently encountered in the limbs, less commonly in the face and the genital organs [3]. In its earliest stages, lymphedema is soft and pitting; however, with persistence, lymphedema has a brawny, non-pitting appearance likened to peau d’orange because of accentuation of skin folds and follicular ostia. Most of the swelling occurs in the subcutaneous tissue, but the skin exhibits the most changes, which, when severe, is termed elephantiasis [1]. Elephantiasis described a gross enlargement of the part of the body that has been involved. Review of the world’s literature reveals that chronic vulvar elephantiasis is a very rare condition [3].

The majority of cases of vulvar elephantiasis found in the literature are due to filariasis [3] [4]. Other causes include bacterial sexually transmitted infections (STI’s), especially lymphogranulomavenereum (LGV) and donovanosis; tuberculosis, haematological malignancies, and dermatological diseases and in some cases from unknown cause [4, 1, 2].

Lu et al. [1] presented a clinical series of 24 cases of localised lymphedema; he made a comparison between those patients and the patients with diffuse lymphedema. Lu found that the most frequent localisation in these patients is anogenital, and overall, women were more frequently affected by lymphedema, particularly in the anogenital region and trunk, than men.

The patients in Lu et al. series did not have any form of cancer treatment, they were significantly more likely to have history of trauma to the site (vaginal delivery), and less likely to have a coexisting, chronic inflammatory process (rosacea and Crohn’s disease), No etiology or associated disease was found in about one-third of all cases. Lu and al. Also, show in his study that 85% of patients were obese or overweight which can promote lymphedema by obstruction of the lymphatic canals by fat cells.

Antonio et al. [2] presented two clinical cases of unilabial vulvar lymphedema similar to the Lu and Al series, but unlike the Lu et al. series, they did not find any factors that may explain the onset of Lymphedema except obesity in one patient.

In our study the patient did not have any previous medical history or an apparent cause that can explain the vulvar elephantiasis, the research of filariasis was negative and also no sign of sexually transmitted infection, neither a sign of malignancy or inflammatory disease. Histological examination of the excised mass showed Acanthosis papillomatous epidermis surmounted by hyperkeratosis and orthokeratosis without cytonuclear atypia; in the dermis, we found many dilated lymphatic vessels of variable size. Important interstitial oedema without any sign of malignancy. The only abnormality she presented beside the vulvar elephantiasis was the skin lesions which were resolved after antiscabies treatment and, the slightly elevated level of circulating granulocyte and the CRP which may correlate with the previous scabies infestation.

Conclusion This clinical case is unusual because of the large volume of lymphedema, its atypical localisation, its association with generalised scabies, and the absence of a well-defined aetiology, which may explain its occurrence.

Acknowledgements Sabur Sarah wrote the case report. All authors have read and agreed to the final version of this manuscript and have equally contributed to its content and the management of the case.

Disclosure Statement There were no financial supports or relationships between the authors and any organisation or professional bodies that could pose any conflict of interests.

Competing Interests Written informed consent obtained from the patient for publication of this case report and any accompanying images.

References 1. Lu S, Tran TA, Jones DM, Meyer DR, Ross JS, Fisher HA, Carlson JA: Localized lymphedema (elephantiasis): a case series and review of the literature. J Cutan Pathol 2009.

2. Antonio A, Caleo A, Boscaino A, Mossetti G, Lannantuoni N, Vulvar lymphoedematous pseudotumours mistaken for aggressive angiomyxoma: Report of two cases.Gynecol.Obstet Invest 2010 ;69 :212-216.

3. Chaudhary R, Rathi S, Maheshwari A, Nigam S.Vulvar elephantiasis of filarial origin: A case report.Indian journal of clinical practice 2013;24 (2).

4. Ipyana HM, Bonaventura CTM, Januarices H.Vulvar filarial elephantiasis in a Tanzanian woman, a rare presentation of lymphatic filariasis. A case report and review of literature.Sudan Journal of Medical Sciences 2014; 9(4):256-270.

5. Kos M, Ljubojevic N, Ilic-Forko J, Babic D, Jukic S. [Elephantiasis of the vulva of an unclear aetiology: a case report].

Lijeniki Vjesn 1996;118(7-8):158–60.

Our patient presented a recurrence of lymphedema after excision. In Lu and al series the majority of lymphedematous tumours were cured with excision, and those that recurred or progressed were associated with factors known to aggravate lymphedema: obesity, cellulitis, signs of persistent inflammation. In our patient, we believe that persistent of inflammation can explain recurrence. However we planned to realise more investigation to explain the recurrence, but the patient refuse to go under supplementary examination.

Sarah Sabur et al./ International Journal of Medical Reviews and Case Reports (2017) 1(1): 14-16

CASE REPORT

PITUITARY PROLACTIN MICROADENOMA WITH, CORTICOTROPIC AND THYREOTROPIC DEFICIENCY: FROM INFERTILITY TO PREGNANCY: ABOUT A CASE Diallo Moussa∗,1 , Koulimaya Cyr Esperence Gombet∗ , Diallo Astou Coly Niassy∗∗ , Diouf Abdoul Aziz∗ , Gassama Omar∗∗ , Gueye Mame Diarra Ndiaye∗∗ , Leye Yakham∗ ∗ ∗ and Diouf Alassane∗ ∗ Department

of obstétric and gynecology of Pikine National Hospital., ∗∗ Department of obstétric and gynecology of Aristide Le Dantec Hospital., ∗∗ Department of internal medicine of Pikine National Hospital.

ABSTRACT Pituitary adenomas are benign tumours developed at the expense of different cellular populations of the pituitary gland.

Within the pituitary gland, several cell populations may be involved, but the lactotrophic cells remain the most frequently affected by this hyperplasia. For both sexes, the overall frequency of adenomas is 100 per million, of which 40% are prolactinomas. The stimulating effect of oestrogens (combined oral contraceptives and pregnancy) on lactotrophic cells has long been demonstrated, and in general, only large tumours (macroadenomas) have an evolving risk to be feared during pregnancy. The diagnosis rests on the one hand on the evidence of a hormonal hypersecretion of the cell population concerned as well as a hormonal deficiency of the other cell groups which can be compressed by the tumour.

On the other hand, this diagnosis uses hypophyseal magnetic resonance imaging (MRI) to distinguish, according to their size, microadenomas (diameter less than 10 mm) from macroadenomas (diameter greater than 10 mm) pituitary.

The risk of increasing the volume of the adenoma during pregnancy depends on the initial size of the tumour. This risk is evaluated at 2% for microadenoma and 15-35% for macroadenomas. However, the most severe complication during pregnancy remains acute paroxysmal growth or pituitary apoplexy by necrotic-haemorrhagic phenomena. The management is mainly based on prolactinoma on bromocriptine or cabergoline and sometimes surgery, urgently in the presence of a pituitary apoplexy or the presence of an evolutionary macroadenoma.

KEYWORDS: Pituitary microadenoma, infertility, pregnancy, prolactin

Introduction

Copyright © 2017 by the Bulgarian Association of Young Surgeons DOI:10.5455/IJMRCR.pituitary-microadenoma First Received: March 26, 2017 Accepted: March 30, 2017 Manuscript Associate Editor: George Baitchev (BG) Editor-in Chief: Cvetanka Hristova (BG) Reviewers: Derun Taner Ertugrul (TR) 1 Diallo Moussa, Department of Obstetrics and Gynecology, Centre Hospitalier National de Pikine Sis Camp de Thiaroye, Dakar, Senegal.; E-mail: moussadiallo25@hotmail.com

Pituitary adenomas are benign tumours developed at the expense of different cellular populations of the pituitary gland.

Within the pituitary gland, several cell populations may be involved, but the lactotrophic cells remain the most frequently affected by this hyperplasia. For both sexes, the overall frequency of adenomas is 100 per million [1], of which 40% are prolactinomas. The stimulating effect of oestrogens (combined oral contraceptives and pregnancy) on lactotrophic cells has long been demonstrated, and in general, only large tumours (macroadenomas) have an evolving risk to be feared during pregnancy. A significant aspect is represented by fertility disorders induced in both sexes and which sometimes constitutes the circumstance of

Diallo Moussa et al./ International Journal of Medical Reviews and Case Reports (2017) 1(1): 17-19

discovery. The diagnosis rests on the one hand on the evidence of a hormonal hypersecretion of the cell population concerned as well as a hormonal deficiency of the other cell groups which can be compressed by the tumour. On the other hand, this diagnosis uses hypophyseal magnetic resonance imaging (MRI) to distinguish, according to their size, microadenomas (diameter less than 10 mm) from macroadenomas (diameter greater than 10 mm) pituitary. The risk of increasing the volume of the adenoma during pregnancy depends on the initial size of the tumour. This risk is evaluated at 2% for microadenoma and 15-35% for macroadenomas [2]. However, the most severe complication during pregnancy remains acute paroxysmal growth or pituitary apoplexy by necrotic-haemorrhagic phenomena. The management is mainly based on prolactinoma on bromocriptine or cabergoline and sometimes surgery, urgently in the presence of a pituitary apoplexy or the presence of an evolutionary macroadenoma. We reported here a case of microadenoma found during the infertility assessment and followed during pregnancy and childbirth.

rest of the gland (Figure 2). In summary, the diagnosis of pituitary prolactin’s microadenoma was retained with corticotropic and thyrotropic insufficiency complicated by primary infertility. She had subsequently benefited from a cabergoline-based treatment at a dosage of 0.25 mg per week, levothyroxine due to 50 mg daily in two doses and 40 mg hydrocortisone. Nineteen months later, the patient, with very irregular follow-up, was admitted to maternity for the management of a pregnancy at 38 weeks of amenorrhea complicated by severe preeclampsia (blood pressure of 240/130 mmHg). A caesarian was performed in emergency and had resulted in the birth of a female newborn weighing 3100 g. The return home was allowed after 12 days without complications. Three months after the patient was stable with a normal diaper return.

Observation Ms K B, 36, nulliparous, was referred the management of a galactorrhoea without amenorrhea, and primary infertility of the unexplored 10-year-old couple. The interrogation found an age of onset of menars at 12 years, a regular menstrual cycle.

We showed a delay in ideation, pubic and axillary depilation and bilateral galactorrhea. Examination of the thyroid gland found a homogeneous goitre without clinical sign of hypothyroidism. There was no evidence of intracranial hypertension.

The patient had a body mass index of 27.04. The thyroxine (tetraiodothyronine) level was decreased to 9.96 pmol / L, a level of thyrotropin releasing hormone (usTSH) normal to 1.689 uUI / ml, a prolactin level of 45.42 ng/ml and a cortisol level of 78 ng/ml. Thyroid ultrasound noted a moderate left lobe heteronodular goitre. Pituitary magnetic resonance (MRI) imaging with gadolinium injection resulted in a 5.5 mm left micro-adenoma (Figure 1) with delayed contrast enhancement compared to the Figure 2:Coronal section of the brain passing the pituitary in T1 sequence with a dynamic injection, showing the delayed enhancement of the adenoma

Discussion

Figure 1: Coronal section through the pituitary gland (T2), showing a tumour of 5.5 mm diameter.

Also known as pituitary hyperplasia, it is defined as an absolute increase in a cell population within the pituitary gland, manifested radiologically by an increase in its size [3]. The overall frequency of adenomas is about 100 patients per million [1].

However, prolactin adenomas (or prolactinomas) remain the most frequent of pituitary tumours. In both sexes, this condition is responsible for impairment of reproductive function.

Indeed, hyperprolactinemia is responsible for suppression of the pulsatile secretion of gonadotropin-releasing hormone (GnRH), the positive feedback of oestradiol on gonadotropin secretion and inhibition of progesterone production by The granulosa of the ovary. Moreover, a mere duplication of its primary level (prolactin) is enough to produce these effects [4, 5]. The association with an insufficiency of other hormonal axes is rare [3].

Our patient simultaneously affected three cell populations (hyperprolactinemia, corticotropic insufficiency and thyrotropin) causing galactorrhea without amenorrhea with infertility of the couple.

Diallo Moussa et al./ International Journal of Medical Reviews and Case Reports (2017) 1(1): 17-19

Diagnosis

The diagnosis of adenoma is most often made before pregnancy [6]. The circumstances of the discovery are, in the woman in reproductive period, an amenorrhea-galactorrhea syndrome associated with infertility, a syndrome of Cushing and more rarely a tumour syndrome which makes suspicion a macroadenoma.

The pituitary hormone assay, especially prolactin, regains very high levels greater than 30 or 35 mg / L, which are also strongly correlated with the size of the adenoma. Pituitary MRI reveals an increase in the volume of the pituitary, specifies its size and its relationship with adjacent structures (optic chiasm and cavernous sinus) and delayed contrast with the rest of the gland as it was For our patient (Fig. 2). It is classical to distinguish the pituitary microadenomas, whose diameter is less than 10 mm, from macroadenomas more than 10 mm in diameter [7].

Exceptionally, the adenoma is discovered during pregnancy by a tumoral syndrome related to pituitary hyperplasia on a preexisting adenoma. The latter form provides complications such as apoplexy. The microprolactinoma is usually expressed by a hypointense image T1 and, in 4 cases out of 5, a T2 hyperintense signal. The hypersignal T2 may correspond to only a part of the adenoma [7].

Management

The management of prolactinomas relies on medical treatment and neurosurgical excision of the tumour. The latter, although assuring the definitive cure, is only a second intention proposed.

However, surgery becomes indispensable in the presence of a pituitary apoplexy or acute necrotic-haemorrhagic form and after the failure of the drug treatment. Moreover, the occurrence of pregnancy in these patients is only possible after a medical treatment that allowed standardisation of prolactin. It is based on the dopaminergic antagonist’s bromocriptine, cabergoline and quinagolide. These molecules, by stimulating the D2 receptors, are responsible for a cascade inhibiting the lactotrophic cell. Their effectiveness varies between 70 and 90%. With a rate of tumour growth rate of 1.4% of microadenomas during pregnancy, it is recommended to stop antidopaminergic drugs to discover a pregnancy and to propose a simple monitoring [8]. The pregnancy occurred in our patient after one year of treatment, and the treatment could not be interrupted because she had been lost sight of and no malformation was detected at birth.

as an amenorrhea-galactorrhea syndrome or infertility of the couple. Its association with pregnancy, on the one hand of the good evolution of the pathology, also raises the problem of excessive tumour growth but also exposes these patients to major complications such as pituitary apoplexy.

References 1. Fatfouta I, Delotte J, Mialon O, Isnard V, Bongain A. Adenome a prolactine : du desir de grossesse a l’ accouchement. journal de gynecologie obstetrique et biologie de la reproduction 2013;42:316—24.

2. Molitch ME. Pituitary Diseases in Pregnancy. Seminars in perinatology. 1998;22(6):457-70.

3. Sunita MC, Sousa D, Peter E, Ann IM. Pituitary hyperplasia: case series and literature review of an under-recognised and heterogeneous condition. Endocrinology, Diabete and Metabolism cases report May 2015 D: 15-0017 DOI: 10.1530/EDM-15-0017.

4. Melmed S, Casanueva F, Hoffman A. Diagnosis and treatment of hyperprolactinemia: an Endocrine Society clinical practice guideline. J Clin Endocrinol Metab ;:.

2011;96:273—88.

5. Casanueva F, Molitch M, Schlechte J. Guidelines of the Pituitary Society for the diagnosis and management of prolactinomas. Clin Endocrinol. 2006;65:265—73.

6. Soto-Ares G, Cortet-Rudelli C, C D, JP P. Adenomes hypophysaires et grossesse: considerations morphologiques en IRM. Journal de radiologie. 2002;83:329-35.

7. Bonneville J-F, Cattin F, Bonneville F. Imagerie desadenomes hypophysaires. Encyclopedie medico-chirurgicale Elsevier Masson SAS; 2009. p. 84-91.

8. Delemer B. Adenomes a prolactine : diagnostic et prise en charge. Presse Medicale. 2009;38:117-25.

9. Molitch M. Prolactin-secreting tumors: what’s new? . Expert Rev Anticancer Ther. 2006;6:S29—35.

Evolution and prognosis

In fact, during pregnancy, we see an increase in the lactotrophic cell population in the pituitary, which can reach half of its total cell population. This phenomenon is mostly observed during the second and third trimesters of pregnancy where oestrogen levels are highest. This suggests, according to its natural history, an aggravation of the symptomatology (intracranial hypertension) of the adenoma during the pregnancy. The evolutionary risk seems more important in the case of macroadenoma [9]. In our case, medical treatment had made it possible to correct the various disorders. It was followed by a pregnancy during which the patient presented no symptoms related to a complication of the adenoma (a headache, vomiting, visual disturbances).

Conclusion Pituitary adenomas are rare pathologies and have the particularity of involving several entities or cell groups of the pituitary gland. In women of childbearing age, it most often manifests Diallo Moussa et al./ International Journal of Medical Reviews and Case Reports (2017) 1(1): 17-19

CASE REPORT

SURGICAL MANAGEMENT OF TRAUMATIC MANUBRIO-STERNAL DISLOCATION WITH LOCKING COMPRESSION PLATE: A CASE REPORT AND REVIEW OF LITERATURE Fareed Shaikh∗,1 , Syed Shahabuddin∗ , Haroon Rashid∗ and Noman Shahzad∗ ∗ Department

Of Surgery, Aga Khan University Hospital, Karachi, Pakistan.

ABSTRACT Background: Manubriosternal joint dislocation as a result of trauma is rare with only few case reports published in the literature. Materials that have been used for fixation of displaced manubriosternal joint are steel wires, polydioxanone ropes, and plates with screws. Case: We present a case of manubriosternal dislocation in which fixation was done with locking compression plate. It is a case of 32 years old lady with a history of road traffic accident; car ran over her chest. She had bilateral lung contusions with multiple rib fractures on right side and manubriosternal joint dislocation of type-I. The patient was initially stabilised in high dependency unit, and once her contusions got better, she underwent fixation of her manubriosternal dislocation with locking compression plate. Post-operatively she remained pain-free and was discharged home. Conclusion: This case is an important addition to literature regarding options that can be used for fixation of manubriosternal joint dislocation.

KEYWORDS Manubriosternal joint, Plate fixation, Joint dislocation, Chest trauma

letter from the Institutional Ethical Review Committee was acquired (3368-Sur-ERC-14)

Introduction

Case Description:

Manubrio-sternal dislocation as a result of trauma is rare with only few case reports published in the literature till now [1].

The materials used for fixation of displaced manubrio-sternal joint are steel wires [1], Polydioxanone ropes [2], and Plates with screws [1, 3, 4].

We describe a case of the young lady with traumatic manubrio-sternal dislocation. She underwent fixation with locking compression plate. Informed consent was taken from the patient for reporting the case with images and an exemption Copyright © 2019 by the Bulgarian Association of Young Surgeons DOI: 10.5455/IJMRCR.surgical-management-traumatic-manubrio-sternal-dislocation First Received: March 26, 2017 Accepted: May 09, 2017 Manuscript Associate Editor: George Baytchev (BG) Editor-in Chief: Cvetanka Hristova (BG) Reviewers: Atsushi Sano (JP) 1 [Department of Surgery Aga Khan University Hospital, Karachi, Pakistan. Email: fareed.shaikh@aku.edu

We had a 32 years old woman who presented to our emergency department after sustaining blunt chest trauma as a result of road traffic accident. She was a motorcycle rear seat rider and had a head-on collision with a car. There was no history of loss of consciousness. She was found to be awake, alert and vitally stable. Airway, breathing and circulation were intact. On the subsequent survey, she was found to have a bruise over sternum with a palpable deformity at the manubrio-sternal joint that was tender to touch. Rest of the examination was unremarkable.

Her X-Ray chest and CT scan chest revealed multiple bilateral rib fractures, bilateral lung contusions and manubrio-sternal dislocation as shown in Figure 1. After initial management at the emergency department, she was kept in intensive care unit (ICU) and later in high dependency unit for the management of lung contusions. Over next four days of conservative management (Pain control, negative balance of fluids, O2 support and chest physiotherapy) her lung contusions improved. However,

Fareed Shaikh et al./ International Journal of Medical Reviews and Case Reports (2017) 1(1): 20-22

gical treatment for sternal fractures, but due to the rarity of cases and literature, no consensus has been reached at. In general undisplaced fractures are considered for non-operative treatment whereas displaced fractures, persistent pain affecting respiration, chronic non-union and sternal instability are the primary indications for surgical fixation as reported by Harston A et al.[6].

In our patient, the manubrium was anteriorly displaced categorising it to be type I and the mechanism were a direct traumatic impact [6, 7].

Although people have used different tools for fixing this type of fractures as mentioned above but consensus has not been established yet. The reason for preferring locking compression plate over the steel wires in our case was the former is much safer if performed under fluoroscopic guidance, taking care not to push screws much beyond posterior cortex; approximates bone closely and it requires minimal dissection of tissues, hence less interruptions in the blood supply of sternum as compared to latter [8-10]. Moreover, the previous experience with sternal wires, as reported by Salloum W et al.[1], was not good, as they had to remove the wires and apply a metallic plate for fixation two months after initial fixation by wires. In this regard CT scan helped us in measuring the distance between the outer and inner table of the sternum, hence the size of the screws to be used was decided preoperatively. The timing of fixation has long been debated, but Harston A et al.[6] reported that most of the sternal fractures had been surgically fixed during the same hospital stay as done in our case.

her sternal pain persisted restricting movement of upper limbs.

Manubrio-sternal dislocation being the only factor responsible, it was decided to fix the sternal dislocation.

Under general anaesthesia, in the supine position, the fracture site was approached using a vertical incision in the midline over the palpable deformity. The hematoma was evacuated; the fracture was reduced and fixed using locking compression plate with the help of screws. The length of the screws was predetermined with the support of CT scan as the distance between anterior and posterior cortices of the sternum. Fixation, alignment and position of screws were confirmed using intraoperative fluoroscopy and with lateral radiograph postoperatively Figure 2. She had unremarkable post-operative course; her pain improved with a better range of motion in upper limbs. She was discharged home on the third postoperative day. She is pain-free with full range of motion in upper limbs at follow-up in the clinic after six months of surgery.

Discusion: Traumatic dislocation of manubrio-sternal joint is rare and surgical correction requires an individualised decision. There is limited literature regarding large series regarding surgical fixation. Both wires and plates are being reported to be used with an acceptable outcome. Thirupathi and Husted [5] classified manubrio-sternal dislocations into two; Type-I, the rare variety, is one in which the body of the sternum is displaced posterior to manubrium, whereas, in type-II, the commoner variety, the body is displaced anteriorly. Direct trauma to the chest is the common mechanism involved in type-I, whereas deceleration injuries resulting in hyperflexion of the torso, rheumatoid disease and kyphosis are the predisposing factors for type-II. There has been a lengthy discussion on conservative vs. sur-

CONCLUSION: Manubrio-sternal dislocations are extremely rare. Due to the paucity of literature, a consensus has not been established yet on the ideal method of fixation. However, this case report will be a valuable addition to literature to achieve desirable outcome after surgical management of sternal fractures with titanium plate during the same hospitalisation.

AUTHORS’ STATEMENTS Competing Interests

References 1. Salloum W, Nikolaidis N, Weeden D. Manubriosternal Joint Dislocation–A Treatment Dilemma. The Internet Journal of Thoracic and Cardiovascular Surgery. 2009;15(1).

2. Lemaitre J, Koriche C, Massard G, Wihlm J. Manubriosternal Disjunction: a New Approach for Surgical Repair. Acta chirurgica Belgica. 2004:593-5.

3. Nijs S, Broos P. Sterno-manubrial dislocation in a 9-year old gymnast. Acta chirurgica Belgica. 2005;105(4):422.

4. Gaines RJ, Wilson A, Antevil J, Demaio M. Parallel plating for a sternomanubrial dislocation. Orthopedics.

2012;35(8):e1276-8.

5. Thirupathi R, Husted C. Traumatic disruption of the manubriosternal joint. A case report. Bulletin of the Hospital for Joint Diseases Orthopaedic Institute. 1981;42(2):2427.

Fareed Shaikh et al./ International Journal of Medical Reviews and Case Reports (2017) 1(1): 20-22

6. Harston A, Roberts C. Fixation of sternal fractures: a systematic review. Journal of Trauma and Acute Care Surgery.

2011;71(6):1875-9.

7. Chou SS, Sena MJ, Wong MS. Use of SternaLock plating system in acute treatment of unstable traumatic sternal fractures. The Annals of thoracic surgery. 2011;91(2):597-9.

8. Abdul-Rahman MR, Seong NK, Hee TG, Aljada ME, Reda TA, SuMin JO, et al., editors. Comminuted sternal fracture—a sternotomy wire fixation: report of 2 cases. The heart surgery forum; 2009: Carden Jennings.

9. Al-Qudah A. Operative treatment of sternal fractures. Asian Cardiovascular and Thoracic Annals. 2006;14(5):399-401.

10. Severson EP, Thompson CA, Resig SG, Swiontkowski MF. Transverse sternal nonunion, repair and revision: a case report and review of the literature. Journal of Trauma-Injury, Infection, and Critical Care. 2009;66(5):1485-8.

Fareed Shaikh et al./ International Journal of Medical Reviews and Case Reports (2017) 1(1): 20-22

CASE REPORT

TESTICULAR DESCEND, HOW AND WHY: A REVIEW ARTICLE SUJAN NARAYAN AGRAWAL∗, 1 ∗ Associate

professor, Department of Surgery. Late SBRKM Government Medical College, Jagdalpur (Bastar) C.G. PIN 494001, India.

ABSTRACT Background:The testis develops in the dorsal abdominal wall, and then descends to the scrotum. The development begins as early as the 6th week of intrauterine life and is completed by the fifth month of intrauterine life.

The testis may get arrested during its descent from dorsal abdominal wall to the scrotum. The anomalies of descent include cryptorchism (and its variant like anarchism, monarchism or partially descended testis), ectopic testis, persistent processus vaginalis and encysted hydrocele of the spermatic cord, and others. Cryptorchism is usually diagnosed during the newly born examination. The recognition of this condition, identification of associated syndromes, proper diagnostic evaluation and timely treatment by a surgical urologist is important to prevent adverse consequences like sterility, congenital hernia & hydrocoele, testicular carcinoma, and others. Objectives: The objective of this review is to study the role of gubernaculum in the testicular migration process. Material & Method: We performed a descriptive review of the literature about the role of the gubernaculum in testicular migration during the human fetal life. This article provides an overview of the role of gubernaculum and other factors responsible for gonadal migration. Results: In the first phase of testicular movement the gubernaculum enlarges to hold the testis near the groin and in the second phase the gubernaculum migrates across the pubic region to reach the scrotum. The proximal end of gubernaculum is attached to the testis and epididymis. The lower end reaches to the bottom of the scrotum. A failure in the proper functioning of gubernaculum causes cryptorchism. Rarely male gonads may deviate from primary pathway due to the presence of many tails of distal gubernaculum, and it may give rise to the ectopic testis. The processus vaginalis usually closes by birth. If it remains patent, it leads to a congenital hernia, hydrocele and encysted hydrocele. Conclusion: the gubernaculum presents a significant structure during testicular migration, and its failed mechanism gives rise to different pathological conditions.

KEYWORDS testicular descend, gonads, gubernaculum, processus vaginalis, undescended testis, congenital hernia/hydrocele

Introduction

Copyright © 2019 by the Bulgarian Association of Young Surgeons DOI: 10.5455/IJMRCR.testicular-descend First Received: May 20, 2017 Accepted: June 18, 2017 Manuscript Associate Editor: George Baytchev (BG) Editor-in Chief: Cvetanka Hristova (BG) Reviewers: Ivan Inkov (BG) 1 SUJAN NARAYAN AGRAWAL, Associate professor, Department of Surgery. Late SBRKM Government Medical College, Jagdalpur(Bastar) C.G. PIN 494001. INDIA, E-mail: drsujanagrawal@gmail.com

The urinary system consists of Kidney, Ureter, Urinary bladder and Urethra. The genital system consists of external and internal genitalia. Male internal genitalia is testis, vas deference or ejaculatory ducts and seminal vesicles, whereas the external genitalia is scrotum and penis. The female genital system consists of ovary, uterus and vagina. The testis is male, and ovaries are female gonads. The main difference lies in the fact that the male gonads have a well differentiated ductal system. The duct system is suppressed in the female, and germ cells develop on the surface of female gonads.

The urogenital system develops from urogenital mesoderm.

SUJAN NARAYAN AGRAWAL et al./ International Journal of Medical Reviews and Case Reports (2017) 1(2):23-27

Soon after the formation of three germ layers, the mesoderm layer is divided into several parts i.e. axial, paraxial, intermediate and lateral plates. This intermediate mesoderm gives rise to urogenital system. [1] The testis develops before birth in the dorsal abdominal wall. During the human fetal period, the testis migrates from the abdomen to scrotum traversing the abdominal wall and the inguinal canal between 15t h th to 28t h -week post conceptions. The development of gubernaculum is the single most important structure in the testicular migration process.

The gubernaculum is a cylindrical structure, covered by the peritoneum from all the sides except posteriorly, where the testicular vessels and vas difference passes to it. The proximal portion of the gubernaculum is adherent to the lower pole of the testis and the epididymis. During testicular migration, these structures move through the inguinal canal as a single unit. During the 8t h month it reaches the superficial inguinal ring, and during the 9t h month, it comes to rest at the bottom of the scrotum. In the scrotum it bulges into the lower part of the processus vaginalis, causing the invagination of the posterior wall of processus vaginalis.

The factors which contribute to the descent of testis includes Gubernaculum testis, the differential growth of abdominal wall, intra abdominal pressure and temperature, Calcitonin gene related peptide(CGRP), male sex hormones, insulin like hormone 3(INSL3) and maternal gonadotrophins. The descent of testis may become erratic and gives rise to undescended testis, ectopic testis, congenital hernia and hydrocoele etc. This paper discusses the process of migration, the role of Gubernaculum and consequence of the failure of this mechanism.

1. Testicular migration The testis develops before birth in the dorsal abdominal wall. The development begins as early as 6t h weeks of intrauterine life and is completed by the 5t h month of intrauterine life. [2] Testicular migration is a complex process wherein the testis migrates from its abdominal position to bottom of the scrotum. [3] During the human foetal period, the testis migrates from the abdomen to scrotum traversing the abdominal wall and the inguinal canal between 15t h and 28t h -week post conception (WPC). [4-5] 1.1 Intrauterine events In the early months of the intrauterine life, the scrotum is rudimentary. It has no cavity and testis is high-up on the posterior abdominal wall. Testicular migration happens in two distinct phases. The first phase corresponds to the testicular migration from the abdomen to the internal inguinal ring. Moreover, the second phase corresponds to the transposition of testes through the inguinal canal until their definite arrival at the scrotum. [6] The exact timing of the testicular migration is controversial.

Various authors have quoted this from as early as a 17t h week up to a 24rth week, but it is more agreed upon that the passage of testis through inguinal canal occurs very quickly.

1.2 The development of gubernaculum The development of gubernaculum is the single most important anatomical structure in the testicular migration process. Using contraction and shortening, it imposes traction forces on the testis.

The gubernaculum starts to develop in the human foetus during the 6t h week of gestation, the same period when the germinative cells are arriving at the genital ridge. [7-8]

In the 8t h week of gestation, the testis and mesonephros are linked to the posterior abdominal wall by a peritoneal fold. As the mesonephros degenerates, a portion of this fold, cranial to the testis, called the diaphragmatic ligament, also degenerates; it then turns in to the cranial position of the gonadal mesentery. This structure is known as the caudal gonadal ligament, which gives rise to gubernaculum testis.

Cranially the gubernaculum approaches the mesonephric duct, while distally it approaches the inguinal region. At this moment, the future inguinal canal is still only space in the musculature of the anterior abdominal wall, where only mesenchymal tissue exists.

The gubernaculum is a cylindrical structure, covered by the peritoneum from all the sides except posteriorly where the testicular vessels and vas deference passes. Macroscopically it looks like Wharton’s jelly of the umbilical cord. Histologically it is composed of undifferentiated cells with elongated shapes, surrounded by large quantities of extracellular material. It is impossible to identify smooth or striated muscle cells in it, except in its distal end and the peripheral portion. [9] The genital branch of the genitor-femoral nerve, provides, the nerve supply to gubernaculum.

Around the 8t h week of gestation, a portion of epithelium starts a small invagination from the coelomic cavity, across from the gubernaculum, slowly penetrating its mesenchymal structures. This invagination occurs bilaterally and is considered as a start of the vaginal process. The growth of the vaginal process divides the gubernaculum into three parts;1). The main gubernaculum, which corresponds to the portion covered by the visceral layer of the peritoneum of the vaginal process.

2). The vaginal gubernaculum- which corresponds to the portion that externally surrounds the parietal portion of the vaginal process.

3). The infra vaginal gubernaculum that corresponds to the caudal portion of the gubernaculum which has not been invaded by the vaginal process. [10-11].Both the gubernaculum and vaginal process change in harmony during testicular migration. The maintenance of this undifferentiated mesenchyme along the inguinal canal and scrotum is essential for the downward extension of the vaginal process to occur, during which it follows the pathway, created by the dilatation of the gubernaculum, forming the canal, through which the testis will reach to the scrotum.

1.3 Proximal gubernaculum The proximal portion of the gubernaculum is adherent to the lower pole of the testis and the epididymis, during testicular migration these structures move through the inguinal canal as a single unit. According to Johansson and bloom, [12] in this situation the proximal gubernaculum always adheres to the end of the vaginal process. Many studies have shown that the changes in the proximal insertion of the gubernaculum are associated with epididymal anomalies and can contribute to the occurrence of cryptorchism. [13] The proximal portion is necessary by uniting the scrotal region and serving as a guide for testicular migration. Not only that, but it also limits the mobility of testis and prevents testicular torsion.

1.4 Distal gubernaculum

SUJAN NARAYAN AGRAWAL et al./ International Journal of Medical Reviews and Case Reports (2017) 1(2):23-27

The insertion site of the distal gubernaculum is one of the factors involved in testicular ectopia. [14] Many studies have shown that the distal gubernaculum has six tails or extensions. i.e. abdominal, pubo-penile, femoral, perineal, contra lateral scrotal and scrotal.[15-16] It is speculated that these tails of the gubernaculum exists during the beginning of the foetal development and disappears during testicular migration. If any of these extensions of the distal portion exists, the individual may develop testicular ectopia. [16] The most accepted theory to explain testicular ectopia are;1). Failure of gubernaculum to dilate the inguinal canal, enabling the testis to migrate through other pathways and not to the scrotum.

2). Invasion of the gubernaculum by abdominal wall fascia near the inguinal canal, blocking the passage of testis to the scrotum and diverting it to ectopic sites.

3). The existence of multiple distal insertions of the gubernaculum testis, guiding the testis to ectopic sites. So the most accepted theory to explain testicular ectopia is the existence of multiple distal insertions of the gubernaculum.

1.5 The process of testicular migration The different parts of the gubernaculum undergo various changes during the testicular migration. The vaginal and infra vaginal portion become proportionately longer as the testis starts to descend into the scrotum. Their diameter also increases which helps to dilate the inguinal canal and to allow testis to pass through it. The growth of the gubernaculum can be divided into two phases. In the first phase, its volume increases and the second phase, it decreases in size, coinciding with the complete descent of the testis. [17] The cremaster muscle presents structural alterations during this period as well. The rhythmic contraction of this muscle guides the testis into the scrotum.

The first phase is characterised by the pronounced cell multiplication and accumulation of glycosaminoglycans, mainly hyaluronic acid. These substances act as hydrophilic agents and raise the quantity of water. There is also increase of extracellular material. The presence of myoblast intensifies, and there are changes in the number and arrangement of the collagen fibre and alteration in the elastic system.

In the second phase, the gubernaculum shrinks, particularly in length, frequently accompanied by the descent of the testis. This phenomenon appears to be androgen dependent and brings substantial degradation of the glycosaminoglycans previously accumulated in the extracellular material, with resulting dehydration of this space and condensation of the gubernaculum. Understanding the relationship between regression of the gubernaculum and descent of testis is vital to the comprehension of, how androgens control testicular migration. Studies have demonstrated the association of deficiency of androgens to failed regression of gubernaculum and thus leading to cryptorchism. [18-19] During the 8t h month it reaches the superficial inguinal ring, and during the ninth month, it comes to rest at the bottom of the scrotum, where it bulges forward into the lower part of the processus vaginalis and invaginates the posterior wall of the processus. Two layers of it now cover the testis. The coverings are called tunica vaginalis having outer parietal layer and inner visceral layer. Since the testis

invaginates the processus vaginalis, from the posterior aspect, the posterior border is not covered by tunica.

The orifice of communication between the cavity of the processus vaginalis and the peritoneal cavity is usually closed before birth. The cavity of the part of the processus that extends from the deep ring to the scrotum is usually obliterated during the first month after birth, and its wall becomes a fibrous thread ( the vestige of the processus vaginalis).

The part of the processus that reaches the scrotum persists as the tunica vaginalis testis. The cavity of tunica vaginalis is now entirely separated from the peritoneal cavity, but its wall is still connected with the peritoneum for a longer or shorter time, by the vestige of the processus. However, the vestige undergoes atrophy from below upwards and frequently, as already mentioned, it entirely disappears.

2. The factors responsible for, testicular descent.

The exact cause of descent of testis and processus vaginalis is still a subject of dispute. However, the following factors contribute to the descent of testis;2.1. Gubernaculum testis; - This is considered to be the most important musculo-fibrous structure and factor, which brings and guide testis to the scrotum. Failure of its proper mechanism gives rise to cryptorchism and ectopic testis.

[20] 2.2. Intra abdominal pressure; - tends to displace the testis downwards. In undescended testis, continuous intra abdominal pressure causes atrophy of testis, and they are more prone to developing malignancies.

2.3. The differential growth of abdominal wall.

2.4. Intra abdominal temperature; - the temperature in the abdomen is two degrees more than the scrotum. If the testis remains inside the abdomen (as in undescended testis), it hurts spermatogenesis.

2.5. Calcitonin gene related peptide (CGRP);- It is a neurotransmitter secreted by the genitofemoral nerve, supplying the muscular fibres of gubernaculum testis.

2.6. Male sex hormones-In the male Y chromosome has a gene for testis determining factor (TDF). Under the influence of TDF, the development of gonads proceeds in the male direction and testis develops, while in a female in the absence of TDF the development of gonads proceed in female guidance and ovaries develop.

2.7. Sexual development begins at the approximately 8t h week of gestation with the production of Mullerianinhibiting substance/anti-Mullerian hormone (MIS/AMH).

It triggers Mullerian duct regression along with testosterone, stimulating the Wolffian duct to persist and to form, the epididymis, vas deference and seminal vesicles. [21-22] Mullerian inhibiting substance (MIS/AMH) is a glycoprotein dimer (mw 140,000) produced by the Sertoli cells. It is also secreted in the Wolffian duct and then diffuses laterally into the adjacent Mullerian duct to trigger its regression in the male. It may also have some secondary role in the development of gubernaculum and has post natal functions in the ovarian cycle. [23] 2.8. Insulin like hormone 3 (INSL 3): - It is a protein with homology to the insulin that is produced by the Leydig cells. It stimulates the growth of the gubernaculum, which is important for the first phase of the testicular descent. [24]

SUJAN NARAYAN AGRAWAL et al./ International Journal of Medical Reviews and Case Reports (2017) 1(2):23-27

2.9. Calcitonin gene related peptide (CGRP); - it is a neurotransmitter secreted by the genitofemoral nerve, supplying the muscular fibres of gubernaculum testis. It provides a chemotactic gradient for the gubernaculum to follow. [25] 2.10. Maternal gonadotrophins; - However the gubernaculum testis is considered an important factor for the formation of processus vaginalis and in guiding testis to the scrotum. The descent is also required for the propagation of the species since intra abdominal pressure and temperature has an inhibitory effect on spermatogenesis.

Thus we see that the testicular descent is a complex and multifactorial event, and cryptorchism should be viewed as a disease with multiple aetiologies.

3. Why the ovaries do not descent to labia? (In Females) The initial phase of descent of ovaries is similar to that of the testis. There is the formation of gubernaculum of ovaries; there is also the formation of the inguinal canal and processus vaginalis. In females, the gubernaculum is attached to developing uterus close to fallopian tubes so it may be seen as two parts of gubernaculum.

1). The first part extends from the lower pole of the ovary to the uterus. This part forms the ligament of the ovary.

2). The other part extends from uterus to labia majora.

This part passes through inguinal canal and forms round ligament of the uterus.

The first part of descent of ovary up to the pelvic brim takes place because of the growth of posterior abdominal wall and pelvis.

Ovaries are prevented from entering the inguinal canal because of the attachment of gubernaculum to the uterus when uterus descends into the pelvic cavity the ovaries are also carried along with it. Since the ovaries do not traverse the inguinal canal, the processus vaginalis and inguinal canal remain small and rudimentary.

4. The pathology related to descent of testis The descent of testis may become erratic and gives rise to following pathological conditions:4.1. Related to gubernaculum testis:1). Undescended testis.

2). Ectopic testis.

4.2 Related to processus vaginalis:-

e. Scrotal- located high up in scrotum.

4.1.2. Ectopic testis In this condition, the testis frequently descends, from the posterior abdominal wall, negotiate through the deep inguinal ring to the inguinal canal and comes out through superficial inguinal ring. However, it fails to reach to the scrotum.It deviates from its path of normal descend. It is thought to be due to many tails of gubernaculum. There is a failure of primary gubernaculum functioning and activation of one of its “tail”, which pulls the testis to an unusual position. Thus ectopic testis may be found at abdominal, pubo-penile, femoral, perineal, or contra lateral scrotal positions.

4.2. Congenital hydrocele/hernia The processus vaginalis is a diverticulum of the abdominal peritoneum. It obliterates once the descent of testis is over.

If it is not obliterated as happens, then it may give rise to following pathological conditions:a. Congenital hydrocele: - It is a persistent congenital sac of the peritoneum, with a tiny communication between processus vaginalis and peritoneal cavity. It may contain fluid and thus presents as congenital hydrocele. b. Hydrocoel of cord: - The processus is obliterated at above and below having a cystic space shut off from peritoneal cavity above and cavity of tunica below. This is called the encysted hydrocele of cord. c. A congenital hernia: -The processus vaginalis fails to obliterate, and the communication to peritoneal cavity is sufficiently significant to permit passage of abdominal content into the sac. This condition is called congenital inguinal hernia.

5. What this paper contributes/Highlights • It highlights the mechanism of testicular descent to the scrotum, which is essential for proper spermatogenesis and propagation of species.

• It explains why the blood supply of testis and epididymis comes all the way from the abdomen.

• It describes the mechanism and probable causation of ectopic and undescended testis.

• It also highlights the factors responsible for a congenital hernia and hydrocele.

1). Congenital hernia.

2). Congenital hydrocoel.

4.1.1. Undescended testis The descent of testis may be arrested at any point in its journey, from lumber region to scrotum, depending upon the arrest of descending and its abnormal location. [26-28] The undescended testis may be found at:a. Lumber region -due to total failure of descending, b. Iliac region – where in the testis are situated at the entry of inguinal canal, near deep inguinal ring (seven-month position). c. Inguinal-situated within the inguinal canal. d. Pubic- located at the superficial inguinal ring.

Authors’ Statements Competing Interests The authors declare no conflict of interest.

References 1. Hutson J. Development of the urogenital system. In: Standring S (Ed) Gray’s anatomy, 40t h edn. Churchill Livingstone Elsevier, pp 1305–1325, 2008.

2. Veena Vidya Shankar, Roopa Kulkarni. Undescended Testes: Embryological and clinical importance. Int J Anat Res 2014; 2:456-458.

SUJAN NARAYAN AGRAWAL et al./ International Journal of Medical Reviews and Case Reports (2017) 1(2):23-27

3. Luciano A. Favorito, Suelen F. Costa, Helce R. Julio-Junior1, Francisco J. B. Sampaio. The importance of the gubernaculum in testicular migration during the human fetal period. IBJU 2014; 40: 722-729, 2014. doi: 10.1590/S16775538.IBJU.2014.06.02 4. Heyns CF, Hutson JM. Historical review of theories on testicular descent. J Urol. 1995; 153:754-767.

5. Sampaio FJ, Favorito LA. Analysis of testicular migration during the foetal period in humans. J Urol. 1998; 159:540-2.

6. Heyns CF. The gubernaculum during testicular descent in the Human fetus. J Anat. 1987; 153:93-112.

7. Heyns CF, Hutson JM. Historical review of theories on Testicular descent. J Urol. 1995; 153:754-767.

8. Wensing CJ. The embryology of testicular descent. Horm Res. 1988; 30:144-52.

9. Costa WS, Sampaio FJ, Favorito LA, Cardoso LE. Testicular Migration: remodelling of connective tissue and muscle cells in Human gubernaculum testis. J Urol. 2002; 167:21712176.

10. Ludwig KS. The development of the caudal ligaments of the mesonephros and of the gonads: a contribution to the development of the human gubernaculums (Hunteri). Anat Embryol (Berl). 1993; 188:571-7.

11. Frey HL, Rajfer J. Role of the gubernaculum and Intraabdominal pressure in the process of testicular descent. J Urol. 1984; 131:574-9.

12. Johansen TE, Blom GP. Histological studies of gubernaculum testis taken during orchiopexies. Scand J Urol Nephrol.

1988; 22:107-8.

13. Favorito LA, Sampaio FJ, Javaroni V, Cardoso LE, Costa WS.proximal insertion of gubernaculum testis in normal human foetuses and in boys with cryptorchidism. J Urol.

2000; 164:792-794.

19. Barthold JS, Kumasi-Rivers K, Upadhyay J, Shekarriz B,Imperato-Mcginley J. Testicular position in the androgen insensitivity syndrome: implications for the role of androgens in testicular descent. J Urol. 2000; 164:497-501.

20. Herman, J. G., Hawkins, N. V. Rider, W. D. Cryptorchidism and non-seminomatous testis cancer. International Journal of Andrology. 1981; 4: 123–130. doi:10.1111/ j .1365 -2605.1981.tb00662.x 21. Tong SY, Donaldson K, Hutson JM. Does testosterone diffuse down the Wolffian duct during sexual differentiation?

J Urol 1996; 155:2057–2059.

22. Handelsman DJ. Androgen action and pharmacologic uses.

In: DeGroot LJ, Jameson LJ (eds) Endocrinology Fifth edn (vol III) Elsevier Saunders, Philadelphia, PA, pp 3121–38, 2006 23. Seifer DB, MacLaughlin DT. Mullerian inhibiting substance is an ovarian growth factor of emerging clinical significance.

Fertil Steril, 2007; 88:539–46.

24. Zimmermann S, Steding G, Emmen J. Targeted disruption of the INSL3 gene causes bilateral cryptorchidism. Mol Endocrinol, 1999; 13:681–691.

25. Utson JM, Hasthorpe S. Testicular descent and cryptorchidism: the state of the art in 2004. J Pediatr Surg, 2005; 40:297–302.

26. Susan Standring. Male Reproductive system. In: Chapter 97, Neil R. Borley (ed). Gray’s Anatomy. 39t h ed. Edinburgh: Elsevier Churchill Livingstone, pp 1306. 2005 27. V.K.Mital, Brijendra K. Garg - Undescended Testicle. Indian Journal of Pediatrics 1972; 39:171 –174.

28. Sekabira J, Kaggwa S, Birabwa-Male D. Prevalence and patterns of undescended testis among primary school pupils in Kampala, Uganda. East & Central African Journal of Surgery 2003; 8: 25-27.

14. Nightingale SS, Al Shareef YR, Hutson JM. Mythical ‘Tails of Lockwood’. ANZ J Surg. 2008; 78:999-1005.

15. Attah AA, Hutson JM. The role of intra-abdominal pressure in cryptorchidism. J Urol. 1993; 150:994-996.

16. Ramareddy RS, Alladi A, Siddappa OS. Ectopic testis in children: experience with seven cases. J Pediatr Surg. 2013; 48:538-541.

17. Fentener van Vlissingen JM, Koch CA, Delpech B, Wensing CJ. Growth and differentiation of the gubernaculum testis during testicular descent in the pig: changes in the extracellular matrix, DNA content, and hyaluronidase, betaglucuronidase, and beta- N-acetylglucosaminidase activities. J Urol. 1989; 142:837-845.

18. Elder JS, Isaacs JT, Walsh PC. Androgenic sensitivity of the gubernaculums testis: evidence for hormonal/mechanical interactions in testicular descent. J Urol. 1982; 127:170-176.

SUJAN NARAYAN AGRAWAL et al./ International Journal of Medical Reviews and Case Reports (2017) 1(2):23-27

CASE REPORT

EFFICACY OF INTRAVENOUS IMMUNOGLOBULIN AND PHOTOTHERAPY IN THE MANAGEMENT OF EXTREME-HYPERBILIRUBINAEMIA: A CASE REPORT Olufunke Bosede Bolaji∗ , Sandeep Dhamaraj∗∗ , Colin Lumsden∗∗ and Olusegun Joseph Adebami∗ ∗ ∗, 1 ∗ Department

of Paediatrics and Child Health, 1Federal Teaching Hospital, Ido-Ekiti, Nigeria., ∗∗ Royal Preston Hospital, Lancashire Teaching Hospitals NHS Foundation Trust, United Kingdom., ∗ ∗ ∗ Department of Paediatrics and Child Health, Ladoke, Akintola University of Technology Teaching Hospital, Osogbo, Nigeria.

ABSTRACT Background: Neonatal jaundice is one of the leading causes of neonatal morbidity and hospitalisations worldwide, and in the developing countries particularly, it still represents one of the main causes of neonatal mortality.

While phototherapy is widely accepted as an effective treatment, adjunct modes of management such as the use of Intravenous Immunoglobulin (IVIG) are not so widely utilised in developing countries like Nigeria. This present case report is to show the usefulness of IVIG in the management of neonatal jaundice. Case summary: A case report of a 42-hour-old Caucasian term male neonate with extreme-hyperbilirubinaemia secondary to ABO incompatibility which was managed with a combination of guided phototherapy and Intravenous immunoglobulin (IVIG) is presented.

The patient responded well to treatment with no apparent immediate adverse effects. Conclusion: The implications for clinical practice regarding reduction in the frequencies of Exchange Blood Transfusion (EBT) and the duration of phototherapy are at this moment presented.

KEYWORDS Extreme-hyperbilirubinaemia, Intravenous immunoglobulin (IVIG), Guided phototherapy

Introduction Neonatal jaundice is one of the most common causes of morbidity in newborns globally, and severe neonatal jaundice is a frequent cause of hospitalisation or readmission for special care in the first week of life. [1] The approaches to management appear to differ considerably between developed and developing countries. [1,2] Exchange Blood Transfusion (EBT) and Copyright © 2019 by the Bulgarian Association of Young Surgeons DOI: 10.5455/IJMRCR.10.5455/IJMRCR.immunoglobulin-and-phototherapy-in-themanagement-of-extreme-hyperbilirubinaemia First Received: June 03, 2017 Accepted: July 15, 2017 Manuscript Associate Editor: George Baytchev (BG) Editor-in Chief: Cvetanka Hristova (BG) Reviewers: Ivan Inkov (BG) 1 Dr.Olusegun JosephAdebami, Department Paediatrics and Child Health, College of Health Sciences, Ladoke Akintola University of Technology, Osogbo, Nigeria and Neonatology Unit, Department Paediatrics and Child Health, Ladoke Akintola University of Technology Teaching Hospital, Osogbo, Nigeria, E-mail: ojadebami@lautech.edu.ng

phototherapy (PT) have traditionally been used to treat jaundice so as to avoid the associated neurological complications of severe hyperbilirubinaemia.[1] EBT is not without risk. Hence Intravenous Immunoglobulin (IVIG) has been suggested as an alternative therapy for isoimmunehaemolytic jaundice to reduce the need for exchange transfusion.[3] Some of the complications of exchange transfusion include haemodynamic instability, apnea, pulmonary haemorrhage, thrombocytopaenia, coagulopathies, hypoglycaemia, hypocalcaemia, electrolyte imbalance, vasospasm, vascular thromboses, hypertension, arrhythmias, sepsis, necrotizing enterocolitis and bowel perforation with morbidity rates varying from 2.8-5.2% per procedure.[3-5] While international guidelines for the management of neonatal jaundice are widely available and accessible globally; uniform practice guidelines are rare at all levels of healthcare delivery in Nigeria.[4] The threshold for EBT is often based on a TSB level of ≥ 20mg/dL and two-third of that level for commencing PT for term babies.[5] Intravenous Immunoglobulin (IVIG) is an alternative therapy which may be effective in treating isoimmunehaemolytic jaundice. In isoimmunehaemolysis, red blood cells are proba-

Olusegun Joseph Adebami et al./ International Journal of Medical Reviews and Case Reports (2017) 1(2):28-31

bly destroyed by an antibody-dependent cytotoxic mechanism mediated by Fc receptor bearing cells of the neonatal reticuloendothelial system. The putative mechanism of IVIG action is a nonspecific blockade of Fc receptors resulting in a decline in carboxyhaemoglobin levels. Carboxyhaemoglobin levels are a sensitive index of haemolysis and hence immunoglobulin could decrease haemolysis.[2] The use of IVIG as an adjunct for the management of extreme and severe hyperbilirubinaemia in developing countries is uncommon due to non-availability of the drug and sometimes the limited knowledge of its use. EBT is, therefore, a common modality of treatment for severe neonatal jaundice in developing countries like Nigeria.[5,6] Literature and data are sparse from these regions in the use of standardised guidelines and IVIG in the management of NNJ. Therefore, the present case is presented to illustrate the use and response of IVIG in the management of extreme hyperbilirubinaemia.

Case report: Complaint: A 42-hour-old Caucasian male neonate (gestational age 40 weeks, birth weight 3009g) presented at the neonatal unit of Royal Preston Hospital, Lancashire Teaching Hospitals NHS Foundation Trust, the UK with jaundice noticed on the 3r d day of life by the community midwife at 36 hours of life during the routine home visit.

Activity was good, and no other systemic symptoms were noticed. The patient was feeding well on exclusive breast milk. The patient was born by normal vaginal delivery to a 33-year-old mother. The mother’s and baby’s blood group were O positive and A positive respectively. He had been passing urine and opening bowels since birth. There were no risk factors for sepsis and no history of jaundice in the elder sibling. He had earlier been discharged from the postnatal ward at 24 hours of life. The total serum bilirubin (TSB) recorded by the midwife at home during the home visit with transcutaneous bilirubinometer was 421 micromol/L. By the published NICE guidelines for treatment threshold in neonatal jaundice, TSB value was on the exchange blood transfusion line. Hence, the baby was referred to the neonatal unit for possible exchange blood transfusion. At presentation, he was severely jaundiced, not pale, afebrile, not dehydrated, weight was 2800g, representing a 6% weight loss from birth weight. Systemic examination was normal, and there were no clinical signs of bilirubin encephalopathy.

Diagnosis: Severe neonatal jaundice (with TSB at exchange transfusion line) secondary to possible ABO incompatibility was made. Investigation results showed baby’s blood group as A Rhesus positive. Direct Coombs Test was strongly positive. Total serum bilirubin (TSB) taken immediately on admission was 433 mmol/L; 4 hours after the referral value. Haemoglobin values were 15.5 g/dL and 14.9 g/dL on the 1st and 2nd day of admission respectively. Reticulocyte counts were initially 16.09%. Blood film on admission also showed numerous spherocytes with polychromasia. Blood glucose and gases were largely normal and infection screens were not suggestive of infection. Serum electrolytes and urea were also normal. Other liver function tests were normal with albumin 36 g/L, Alkaline Phosphatase 159 U/L and Alanine transferase (ALT) 12 U/L. Interventions:The patient was admitted and started on quadruple phototherapy with three overhead phototherapy lamps and a Bilibed. Umbilical arterial and venous accesses were

Table 1 Timeline of care

Age

Level of jaundice

Treatment offered

(TSB mmol/L) 42 hour

Referral to hospital by

421

midwife Quadruple phototherapy 46 hour

433

IVIG 0.5mg/kg, IVF

52 hour

404

58 hour

354

Phototherapy lamps reduced to 3

64 hour

222

76 hour

211

Phototherapy

Day 4

169

Phototherapy discontinued

Day 5

150

Patient discharged

3 months

Follow up at clinic – Child was neurologically normal

obtained, and intravenous fluid was started at 120 ml/kg/day.

The patient was placed on nil per by mouth. IVIG was given at 0.5g/kg at admission. By 6 hours into admission, which was after the administration of IVIG, TSB had reduced to 404 micromol/L, (conjugated fraction 43micromol/L). When plotted on the NICE guideline jaundice treatment graphs, this value was four boxes below the exchange transfusion line. 12 hours into admission showed a further reduction in the TSB to 354 micromol/L (conjugated fraction 38) which was eight boxes below exchange transfusion line.Phototherapy lamps were thus reduced to 3. TSB 6 hours later was 250 micromol/L which was on the phototherapy treatment line. On the second day of admission, haematocrit was 14.9 g/dL, and reticulocyte was 13.99%.

Enteral feeds were restarted by the 2nd day of admission when the TSB had dropped significantly to 222 micromol/L, and this value was by now four boxes below phototherapy treatment line.

The next TSB value by the end of the 2nd day of admission was 211 mmol/L, which was seven boxes below the phototherapy treatment line. Phototherapy was then reduced to 2 lamps. TSB was 169 mmol/L on the 4t h day of life after which phototherapy was discontinued.

Outcome:The patient made a full recovery, as judged by the clinical appearance and the neurologic examination and the TSB values. He was subsequently discharged from the hospital on the 5t h day of life. Neurodevelopmental follow-up in the outpatient clinic at three months of age was normal.

Discusion: Extreme hyperbilirubinaemia is defined as a TSB greater than 428micromol/L (25mg/dL). [7] The patient’s total serum bilirubin (TSB) taken immediately on admission was 433 micromol/L, and this was due to ABO blood group incompatibility as the mother’s and baby’s blood group were O positive and A positive respectively.

Olusegun Joseph Adebami et al./ International Journal of Medical Reviews and Case Reports (2017) 1(2):28-31

ABO hemolytic disease remains the most common cause of severe and early jaundice in the newborns. ABO blood group incompatibility is present in 15-25% of pregnancies.[8] ABO feto-maternal red blood cell incompatibility induces an immune hemolysis after fetal transfer of haemolysing maternal anti-A or anti-B. High levels of consequent unconjugated hyperbilirubinemia may induce both acute and chronic neurological complications.[8,9] Although phototherapy (PT) and exchange transfusion (ET) are widely used in the management of severe jaundice, there is no standard or evidence basis for the thresholds for each intervention. It is also unclear what the safe upper limit of bilirubin level is or whether bilirubin level alone is a sufficient predictor of the need for exchange transfusion or as a determinant of neurodevelopmental outcome.[10] Many practitioners in developing countries use double set up or intensive phototherapy and defer EBT until the serum bilirubin has risen above high thresholds. Some paediatricians still use early rate of rising of bilirubin as an indicator for ET.[11] Intravenous human polyclonal immunoglobulin (IVIG) has been proposed for concomitant use with PT to reduce the need for ET in ABO haemolytic disease with positive direct Coomb’s test.[1] The exact mechanism of action of IVIG in HDN is still unknown. IVIG is thought to decrease haemolysis by blocking Fc receptor sites of reticuloendothelial cells preventing lysis of neonatal erythrocytes. IVIG competes with sensitised neonatal erythrocytes for the Fc receptor sites of the reticuloendothelial system to prevent further haemolysis (competitive inhibition) thus suggesting that early administration of IVIG is necessary for efficacy in immune hemolytic diseases of the newborn. [12] Recognising the potential benefits of IVIG over exchange transfusion is easy. The administration is less complicated and less labour intensive. As well as being a less invasive therapy, IVIG may also allow treatment of some infants in Level IIcentres or avoid delaying treatment while transferring infants to tertiary centres for exchange transfusion. Administration of IVIG has also been shown to reduce the duration of phototherapy. [13] This may particularly be helpful in developing countries where health care cost is related to duration of stay in the hospital and this cost is largely out of pocket expenditure.

Case reports and case series have reported the success of IVIG in the treatment of jaundice due to both Rhesus and ABO incompatibility.A systematic review of the use of IVIG in hemolytic disease of the newborn showed that significantly fewer infants required EBT when IVIG was administered in combination with PT compared to those who had PT alone. Also, hospital stay and duration of phototherapy were reduced. [14] However, there are still debates on the routine use of IVIG in ABO hemolytic disease of the newborn as some studies have failed to show the efficacy of IVIG in reducing the need for EBT. The reasons for this discrepancy have not been explained but it should be noted that in some of the studies that failed to show significant effects, IVIG was used more or less prophylactically for all apparently immunized infants, whereas in the studies that reported benefits, IVIG was used exclusively as a rescue therapy in infants headed for EBT.[15] Numerous guidelines for the management of neonatal jaundice have been published, the NICE guideline being just one of them. The AAP and Norwegian guidelines all state explicitly the thresholds for commencing or stopping phototherapy as well as those for EBT. These guidelines also vary for both gestational

age and birth weight.In sharp contrast to the practice in most high-income nations, national or local guidelines for the effective management of severe hyperbilirubinaemia are rare in the developing countries even though the disease burden is high. [16] The absence of harmonised protocols either for classification or management in most low and middle-income countries renders it difficult if not impossible for comparisons between locations.

As regards the long/life term effect of our choice of management, the patient had no sign of bilirubin encephalopathy at admission nor at follow-up at three months. We, therefore, had no reason to fear that the prognosis will not remain excellent.

Implications for practice Administration of IVIG to newborns with significant hyperbilirubinemia due to ABO haemolytic disease with positive direct Coomb’s test combined with the use of PT reduces the need for EBT and the duration of PT. The efficacy and good tolerance prompt consideration of IVIG as a therapeutic adjuvant to PT in severe hemolytic hyperbilirubinemia due to ABO incompatibility. Since it appears safe, it may also have a role in special circumstances such as parental refusal for exchange transfusion, failed cannulation for EBT or where appropriate blood components for exchange transfusion are unavailable.

This case report also encourages the employment of standard protocols which are widely available in determining the need for phototherapy or exchange blood transfusion as well as the use of alternative treatments like IVIG in cases of extreme hyperbilirubinaemia with isoimmunization. This maximises the chances of a favourable outcome for patients with severe neonatal jaundice regarding reducing the need for EBT and the duration of accompanying phototherapy.

Authors’ Statements Competing Interests The authors declare no conflict of interest.

References 1. Burke BL, Robbins JM, Bird TM, Hobbs CA, Nesmith C, Tilford JM. Trends in hospitalisations in neonatal jaundice and kernicterus in the United States, 1988-2005. Pediatrics 2009; 123:524-32 2. National Institute for Health and Clinical Excellence.

Neonatal Jaundice. (Clinical Guideline 98); 2010. Available from http://www.nice.org.uk/CG98. Last accessed 8th August 2016 3. Alcock GS, Liley. Immunoglobulin infusion for isoimmunehaemolytic jaundice in neonates.Cochrane Database Syst Rev. 2002; 3: CD003313 4. BO Olusanya, FB Osibanjo, CA Mabogunje, TM Slusher, SA Olowe. The burden and management of neonatal jaundice in Nigeria: A scoping review of the literature. NJCP 2016;19:1-17.

5. Ibekwe RC, Ibekwe MU, Muoneke VU. Outcome of Exchange blood transfusions done for neonatal jaundice in Abakaliki, South Eastern Nigeria. Journal of Clinical Neonatology. 2012;1:34-37

Olusegun Joseph Adebami et al./ International Journal of Medical Reviews and Case Reports (2017) 1(2):28-31

6. Owa JA, Ogunlesi TA. Why are we still doing so many exchange blood transfusion for neonatal jaundice in Nigeria.

World J Pediatr. 2009; 5:51-5 7. Nigerian consensus statement on newborn jaundice. Management of jaundice and prevention of extreme hyperbilirubinemia in infants ≼ 35 weeks gestation. Nigerian Expert Committee for severe hyperbilirubinemia (For NISONM) 8. Urbaniak SJ. ADCC (K Cell) lysis of human erythrocytes sensitized with Rhesus alloantibodies.II. Investigation into mechanism of lysis. Br J Haematol. 1979;42:315-328 9. Senterre T, Minon JM, Rigo J. Neonatal ABO incompatibility underlies a potentially severe hemolytic disease of the newborn and requires adequate care. Arch Pediatr.

2011;18:279-82 10. Ahlfors CE. Criteria for exchange transfusion in jaundiced newborns. Pediatrics 1994; 93:488-94 11. Greco C, Arnolda G, Boo NY, Iskander IF, Okolo AA, Rohsiswatmo R, et al. Neonatal jaundice in low- and middle- income countries: lessons and future directions from the 2015 Don Ostrow Trieste Yellow Retreat. Neonatology 2016;110:172-180 12. Cortey A, Elzaabi M, Waegemans T, Roch B, Aujard Y. [Efficacy and safety of intravenous immunoglobulins in the management of neonatal hyperbilirubinemia due to ABO incompatibility: a meta-analysis]. Arch Pediatr. 2014; 21:97683.

13. Miqdad AM, Abdelbasit, Shaheed MM, Seidahmed MZ, Abomelha AM, Arcala OP. Intravenous immunoglobulin G (IVIG) therapy for significant hyperbilirubinemia in ABO hemolytic disease of the newborn. J Matern Fetal Neonatal Med. 2004;16:163-6.

14. R Gottstein, RWI Cooke. Systematic review of intravenous immunoglobulin in hemolytic disease of the newborn. Arch Dis Chil Fetal Neonatal Ed 2003;88: F6-F10 15. Neonatal jaundice treatment and management. emedicine.medscape.com. accessed 8th August 2016.

16. BO Olusanya, Tinuade A Ogunlesi, Praveen Kumar, NemYun boo, Iman F Iskander, Maria Fernanda B de Almeida, et al. Management of late-preterm and term infants with hyperbilirubinaemia in resource-constrained settings. BMC Paediatrics 2015;15:39

Olusegun Joseph Adebami et al./ International Journal of Medical Reviews and Case Reports (2017) 1(2):28-31

CASE REPORT

SURGICAL MANAGEMENT OF SEVERE TRAUMA INVOLVING GROUP OF MUSCLES AROUND THE BRISKET OF DONGOLA BREED OF HORSE: A CASE REPORT Olaifa A.K∗,1 , Oguntoye C.O∗ and Olatunji-Akioye A.O∗ ∗ Department

of Veterinary Surgery and Radiology, University of Ibadan, Nigeria.

ABSTRACT Background: Equine wound management is challenging to the veterinarian in practice. This paper presents the successful management outcome of a large wound on the brisket of a seven and half years old Dangola Stallion horse sustained from a road traffic accident. Case Summary: There was traumatic shearing off of the skin including the dermis and fascia of the thoracic inlet beginning from the ventral caudal end of the neck to the cranial border of both shoulders and there were two open wounds on the medio-cranial aspect of the right tibial region just below the elbow joint. All physiological parameters were within normal range. Continuous suture and interrupted patterns were used during the surgical procedure until all the exposed muscles were apposed. Conclusion: The surgery was successful, with good post operative care, the wound healed properly.

KEYWORDS Horse, wound, accident, surgery, Dongola

Introduction Wounds constitute the most commonly encountered injuries in equine practice (Westgate et al., 2010; Pollock, 2011). Due to the horse’s nature and the environment in which it lives, wounds frequently involve a significant amount of tissue trauma. Legs caught in fences, panels, wire, or gates are a frequent occurrence, as are lacerations from steel siding, trailer accidents, kicks, and riding accidents. As a result, equine ambulatory practitioners typically see a relatively large number of cases presenting for wound care. Enormous variation exists in treatments, medications, bandages, and bandaging techniques applied to wounds in horses (Theoret, 2008; Gomez, 2008). In pathology, wounds Copyright © 2019 by the Bulgarian Association of Young Surgeons DOI: 10.5455/IJMRCR.dongola-breed-of-horse-severe-trauma-surgery First Received: May 13, 2017 Accepted: June 01, 2017 Manuscript Associate Editor: George Baytchev (BG) Editor-in Chief: Cvetanka Hristova (BG) Reviewer: Ivan Inkov (BG) 1 [Olaifa A.K, Department of Veterinary Surgery and Radiology, University of Ibadan, Nigeria Email:akolaifa@yahoo.com

remain a challenging clinical problem, with early and late complications presenting a common cause of morbidity and mortality (Natarajan et al., 2000). A wound is a breakdown in the protective function of the skin or loss of continuity of epithelium, with or without loss of underlying connective tissues, muscles, nerves, bones following injury to the skin, surgery, a blow, cut, chemicals, heat, cold, friction, shear force, pressure or diseases such as leg ulcers or carcinomas (Velnar et al., 2009; Fernandez and Griffiths, 2015). Wound healing is a homeostatic mechanism for restoration of physiological balance and is triggered by the interruption of the connection between adjacent cells or cell death. The healing process consists of a sequence of overlapping events including inflammatory responses, regeneration of the epidermis, shrinkage of the wound and finally connective tissue formation and remodelling (Leaper and Harding, 1998; Choucair and Phillips, 1997).

Indeed, it seems that of all the species dealt with by veterinary surgeons, the horse is particularly prone to wounds. Reasons for higher incidence of wounds in horses than other species may be due to the conditions in which horses are kept, the type of work they are involved in, and the potentially “flighty” equine temperament. (Pollock, 2011).Wound infection is a challenge to wound management and wound infection and dehiscence can

A.K Olaifa et al./ International Journal of Medical Reviews and Case Reports (2017) 1(2):32-35

occur in both surgically or trauma-induced wounds (Carter et al., 2003; Westgate et al., 2010). Factors that influence infection include integrity of the wound and its perfusion, the wound repair process, bacterial challenge and host immunity. The management protocol employed in equine wound may also determine the outcome of such wounds. Equine wound management is challenging to the veterinarian in practice (Carter et al., 2003).

This paper presents the successful management outcome of a large wound on the brisket of a horse sustained from a road traffic accident involving both the horse and its rider.

Case Presentation A seven and half years old Dangola Stallion was presented to the veterinary Teaching Hospital of the University of Ibadan following a road traffic accident involving it and its rider. The horse, owned by the mounting department of the State Police, was ridden on the highway and had collided with an on- coming vehicle. There was traumatic shearing off of the skin including the dermis and fascia of the thoracic inlet beginning from the ventral caudal end of the neck to the cranial border of both shoulders and there were two open wounds on the medio-cranial aspect of the right tibial region just below the elbow joint (figure 1). The horse also favoured both hind limbs. The temperature, respiratory rate, heart rate and pulse rate were 38.5º C, 72 breaths/min.

50 beats/min and 52/min respectively. All were within normal range. The mucous membrane was pink. Its weight was estimated as 220kg.

Figure 2: Wound repair in progress suture pattern until all the exposed muscles were apposed. The edges of the skin flap were trimmed and closed with 2-0 nylon suture (Ethicon, USA), using simple interrupted suture and continuous patterns. The two open wounds on the medio-cranial aspect of the right tibial region just below the elbow joint were also sutured using 2-0 nylon suture (Ethicon, USA), with simple interrupted suture pattern.

Post-operative care Tetanus toxoid was administered 1500i.u and penicillin- streptomycin antibiotic. (PENSTREP 20/25, Kepro B.V., Holland at a dosage of 1ml/25kg for 5 days) intramuscularly. Oxytetracycline spray (Holland) was sprayed on the wound after closure (figure2). The sutures were removed a week later following wound dehiscence although significant wound contraction had taken place (figures 3 and 4).

Figure 1: Wound presented to the Veterinary Teaching Hospital.

Management The horse was placed on intravenous fluid (15litres Hartman’s Solution, Danax®) and given xylazine hydrochloride (XYL-M2®, VMD, Holland) intramuscularly for both sedation and analgesia at a dosage of 2mg/kg body weight.

Figure 3: Wound repair completed.

Surgery The wounds were thoroughly irrigated with normal saline to remove all dirt and all loose and unviable flesh was trimmed.

Following site infiltration with lignocaine hydrochloride, the exposed muscles were closed layer by layer (figure2) using 2-0 and 1-0 chromic catgut sutures (Ethicon, USA), using continuous

Discusion: The wounds of this horse were closed primarily. Primary repair of the wound is the preferred treatment for wounds that involve detachment of skin with maintenance of an intact blood

A.K Olaifa et al./ International Journal of Medical Reviews and Case Reports (2017) 1(2):32-35

Table 1 Timeline

Dates

Medical History

Diagnostic testing

Interventions

No previous case

None

None

Physical examination, 12/02/2017

Wound from road accident

Respiratory rate, Heart

Surgical wound repair

rate, Rectal temperature

References 1. Choucair MM, Phillips TG (1997): What is new in clinical research in wound healing. Clin Dermatol, 15:45-54.

2. Leaper PJ, Harding KG (1998). Wounds, Biology and management Sanders.

3. Theoret C. Wound repair: problems in the horse and innovative solutions. In: Stashak TS, Theoret C, editors. Equine wound management. 2nd edition. Ames (AI): WileyBlackwell; 2008. p. 47–68. 4.

4. Gomez J. Bandaging and casting techniques for wound management. In: Stashak TS, Theoret C, editors. Equine wound management. 2nd edition. Ames (IA): WileyBlackwell; 2008. p. 623–58.

Figure 4: Showing wound management by 12th day. supply (Hansen, 2008). Tissue integrity and perfusion, wound repair processes, and bacterial challenge and host responses heavily influence infection. Excessive tension of sutured skin often also lead to complications of healing because of local ischemia with pressure necrosis of the surrounding skin and the pull through of sutures at the skin edge with subsequent wound disruption. Both surgical and traumatic wounds can fail to heal and become chronic but traumatic wounds are more commonly affected by healing difficulties. A large number of horse trauma wounds progress to chronicity making wound healing management plans more complex. Trauma wounds are very important in horses because of the high prevalence (Collins et al., 2000; Singer et al., 2003).The incidence of wound infection appears higher in horses than in small animals and man( Cruse and Ford, 1980; Levy et al.,1988). One factor responsible for this is the high number of resident microbes on horses’ skin (Westgate et al., 2010).

CONCLUSION: Careful management is important for successful treatment of horse wounds. Wounds should be assessed quickly and carefully and aseptic surgical techniques meticulously employed.

Post-operative care was done conscientiously and appropriate antibiotic treatment regimens employed.

COMPETING INTERESTS The authors declare no conflict of interest. Written informed consent obtained from the patients for publication of this article and any accompanying images.

5. Velnar T, Bailey T, Smrkolj V. The Wound Healing Process: an Overview of the Cellular and Molecular Mechanisms. J Int Med Res. 2009; 37:1528-42.

6. Fernandez R, Griffiths R. Water for wound cleansing."

Cochrane database of systematic reviews. 2015. CD003861.

PMID 22336796.

7. Griffiths DA, Simpson RA, Shorey BA, Speller DC, Williams NB. Single-dose peroperative antibiotic prophylaxis in gastrointestinal surgery. Lancet. 2003; 2(7981):325–328 8. R.ReidHanson Equine practiceSurgical Complications and Strategies Vet CLINICS OF NORTH AMERICA, 2008 Vol 24 No 3 9. Carter CA, Jolly DG, Worden CE Sr, Hendren DG, Kane CJ. Platelet-rich plasma gel promotes differentiation and regeneration during equine wound healing. ExpMolPathol.

2003; 74(3):244–255 10. Samantha J. Westgate, Steven L. Percival; Derek C. Knottenbelt, Peter D. Clegg, Christine A. Cochrane, Equine Wounds: What Is the Role of Infection and Biofilms?WOUNDS 2010; 22(6):138–145 11. Singer ER, Saxby F, French NP. A retrospective case-control study of horse falls in the sport of horse trials and three-day eventing. Equine Vet J. 2003; 35(2):139–145. 7.

12. Hernandez J, Hawkins DL. Training failure among yearling horses. Am J Vet Res. 2001; 62(9):1418–1422.

13. Collins MN, Friend TH, Jousan FD, Chen SC. Effect of density on displacement, falls, injuries, and orientation during horse transportation. ApplAnimBehav Sci. 2000; 67(3):169–179.

A.K Olaifa et al./ International Journal of Medical Reviews and Case Reports (2017) 1(2):32-35

14. Cruse PJ, Foord R. The epidemiology of wound infection.

A 10-year prospective study of 62,939 wounds. SurgClin North Am. 1980; 60(1):27–40. 15.

15. PB, Levy J, Dowd E, Eliot J. Surgical wound infection rates in dogs and cats: Data from a teaching hospital. Vet Surg.

1988; 17(2):60–64.

16. Natarajan S, Williamson D, Stiltz AJ, et al: Advances in wound care and healing technology. Am J Clin Dermatol 2000; 1: 269–275.

A.K Olaifa et al./ International Journal of Medical Reviews and Case Reports (2017) 1(2):32-35

CASE REPORT

A CASE REPORT OF DELAYED DIAGNOSIS OF UTERINE RUPTURE FOLLOWING VAGINAL DELIVERY Vanessa Falé Rosado∗, 1 , Sara Rocha∗ , Catarina Vasconcelos∗ , Maria Luisa Martins∗ and Maria José Alves∗ ∗ Maternidade

Dr. Alfredo da Costa, Rua do Viriato, Lisbon, Portugal.

ABSTRACT Introduction: A uterine rupture is still a rare event, but its incidence appears to be increasing, even in the unscarred uterus. In our case, the uterine rupture presented itself in an unscarred uterus and after a vaginal delivery.

Case report: A 36 years old women with three previous normal deliveries, comes to our hospital for assistance at 32 weeks with a poor pregnancy surveillance. After diagnosing Gestational Diabetes, she is admitted for therapeutic adjustment. She is discharged after achieving metabolic control but comes back a few days later with a stillbirth, born by vaginal delivery. Six days later she presents with: fever and pain; anaemia leukocytosis and a heterogeneous image on ultrasound. However, was decided to start intravenous antibiotics before choosing for surgery. Her condition worsens, and an exploratory laparotomy is done: a posterior uterine wall rupture that required a hysterectomy. Conclusion: Risk factors for uterine rupture were present (maternal age over 35, higher parity, fetal macrosomia) but the absence of any symptom, the regular examination after delivery, and mostly, an unscarred uterus, resulted in a delay in the diagnosis of more than one week, leading to catastrophic consequences: hysterectomy. This case reminds us that uterine rupture happens not only in case of previous uterine surgery, and these cases seem to be increasing because of the increase in other risk factors: advanced maternal age and diabetes with resulting fetal macrosomia.

KEYWORDS uterine rupture, hysterectomy, postpartum period

Introduction Uterine rupture represents a breach in the integrity of the uterine muscle, usually during labour although it can also happen before. It has been considered an entity with dramatic consequences both for the mother and the fetus, leading to severe haemorrhage on the mother and hypoxia in the fetus. Because it can have no symptoms, the diagnose may be delayed aggravating the prognosis. This obstetric emergency, considered rare, seems to be increasing due to the rising rate of uterine surgeries like cesareans and myomectomies (due to the delaying in Copyright © 2019 by the Bulgarian Association of Young Surgeons DOI: 10.5455/IJMRCR.uterine-rupture-delayed-diagnosis First Received: July 07, 2017 Accepted: July 22, 2017 Manuscript Associate Editor: George Baytchev (BG) Editor-in Chief: Cvetanka Hristova (BG) Reviewers: Ivan Inkov (BG) 1 Maternidade Dr. Alfredo da Costa, Rua do Viriato, Lisbon, Portugal., vanessasfrosado@hotmail.com

maternity). However, in our case, no previous uterine surgery was reported. This aspect combined with the lack of any symptom, resulted in a delay of more than one week in the diagnosis, which in turn led to disastrous consequences - hysterectomy. Better methods for predicting this disaster complication are needed, especially considering its increasing incidence also in the unscarred uterus, possibly due to the growing of other risk factors like advanced maternal age and gestational diabetes with fetal macrosomia.

Case report A thirty-six years old woman, melanodermic, natural from Africa, came to Lisbon, Portugal at 32 weeks of pregnancy to have the remaining pregnancy surveillance and delivery in Maternidade Dr Alfredo da Costa. Her personal and family background were irrelevant. As for previous deliveries, she mentioned three eutopic deliveries: in 2002 a healthy baby with 3800grs, in 2004 she referred a late premature delivery of a healthy baby with 3400grs and finally in 2014, a pregnancy complicated with gestational diabetes that resulted in a newborn

Vanessa Falé Rosado et al./ International Journal of Medical Reviews and Case Reports (2017) 1(2): 36-40

Table 1 Analysis of patients’ clinical evolution on antibiotic

therapy Day 0

Day 1

Day 2

Day 3

Day 5

Hb (gr/dL)

9,8

8,3

8,8

7,8

8,1

WC (cells/µL)

26 180

17 500

16 500

13 000

17 200

Table 2 Timeline of the patient.

Dates

N (%)

91

89

88

84

86

2002

CRP (mg/L)

397

316

315

239

260

2004

US image size

105x95x70

116x99x59

Relevant Past Medical History and Interventions Vaginal delivery in Africa, newborn with 3800 grs, without any interferences during pregnancy, intrapartum or postpartum Vaginal delivery in Africa, newborn with 3400 grs, without any interferences during pregnancy, intrapartum or postpartum

2014

Vaginal delivery in Africa, newborn with 3200 grs, diagnosis of Gestational Diabetes without posterior reclassification test

Dates (in 2016)

Summaries from Initial and Follow-up Visits The first appointment in our hospital; none of the

22 July

previous exams done in Africa, were available for consultation

Diagnostic Testing

Interventions

(including dates)

Ultrasound was done in Portugal on 18 July

Analysis with 75grs glucose

revealing a healthy baby

tolerance test was requested

with 32+5 weeks -Glucose tolerance

12 August

test positive:

Admission to Maternal-fetal

35+5 weeks

191/238/339

Infirmary for surveillance and

Asymptomatic

-Ultrasound that day:

therapeutic adjustment with

fetus on 95th

both insulin and metformin

percentile Adequate metabolic 22 August

37+1 weeks

control and daily

Cesarean scheduled to 6 of

Asymptomatic

cardiotocography

September

showing fetal well being 30 August

38+2 weeks

Stillbirth on ultrasound

No fetal movements

Figure 1: Ultrasound image at emergency department, eight days after delivery (U – uterus; HI – heterogeneous image) with 3200grs. A reclassification test of gestational diabetes was not done. All pregnancies were with the same partner. In her first appointment in Lisbon at 32+ 5 weeks, it was decided to repeat a glucose tolerance test (75grs glucose with glycaemia measurement at 0, 1 and 2 hours). The results were positive: 191, 239 and 339 mg/dL. An ultrasound was performed: the fetus was in the 95t h percentile (Hadlock et al) and the amniotic fluid level was normal. She was admitted to the hospital for maternalfetal surveillance, and glycemic control was achieved with diet, insulin and metformin. She was released at 37+ 1 with good metabolic control; a new appointment and elective cesarean for 39 weeks were scheduled.

At 38 weeks she came to the emergency department because of the absence of fetal movements for the last 12 hours: she was diagnosed with stillbirth. She went into spontaneous labour, and 10 hours later, after a breech delivery, she had a stillborn with 4525 grs. The family refused an autopsy.

After 48hrs of inpatient observation, she was discharged from the hospital.

She came back to the emergency department six days later with complaints of fever, pelvic pain and foul smelling vaginal discharge, which was confirmed by observation. On ultrasound, an image with heterogeneous echogenicity was found over the uterine fundus, measuring 105x95x70 mm. Blood analysis revealed low levels of haemoglobin (9,8gr/dL), leukocytosis (26 180/µL), neutrophilia (91%) and elevated C-reactive protein (397mg/L). She was initiated on endovenous antibiotic therapy with gentamycin, ampicillin and clindamycin. Analytic evolution is present in table 1.

Because of clinical deterioration, exploratory laparotomy was

Breech delivery 10 hours later: Dead fetus with 4525 grs

Asymptomatic

1 September

Clinical observation

48hrs after delivery, she is

normal: normal lochia,

discharged from Hospital with

normal uterine involution

metabolic control

and painless examination -Ultrasound: image with heterogeneous echogenicity was 8th day after delivery Fever, pelvic pain and 7 September

foul smelling vaginal discharge, which was confirmed by observation

found over the uterine fundus, with 105x95x70 mm -Anemia(9,8gr/dL), leukocytosis (26

Admitted to hospital and initiated ampicillin, gentamicin and clindamycin IV

180/µL), neutrophilia (91%) and elevated C-reactive protein (397mg/L)

Improvement of symptoms and clinical 8-10 September

observation: pyrexia, no pain at fundal uterine palpation 13th day after delivery

12 September

5th day with antibiotic Clinical deterioration: fever, pain

-Blood test improvements, -Ultrasound with

Maintain therapeutic

stable image (without increases in size) Deterioration of both

Exploratory laparotomy: vast

blood test and

uterine rupture of posterior wall

ultrasound image

and a hysterectomy was decided Discharged from hospital after therapeutic with meropenem, during three weeks because of

10 October

Vanessa Falé Rosado et al./ International Journal of Medical Reviews and Case Reports (2017) 1(2): 36-40

multiresistant Klebsiella pneumoniae isolated in the purulent exudate collected from the abdominal cavity during, surgery

Table 3 Medical History of eight cases of rupture (CS – cesarian; Leuco – leucodermic; melano – melanodermic; leucomelano -

leucomelanodermic) Obstetric and Gynecological History Dilatation #

Race

Age

&

≥3

Nulliparous

1 CS

≥ 2 CS

Deliveries

Curettage

Time since last

Other uterine

cesarian (in months)

surgery

1

Leuco

31

No

No

No

Yes

No

21

No

2

Melano

31

Yes (2)

No

No

Yes

No

48

No

3

Melano

29

No

No

No

Yes

No

3

No

4

Leuco

44

Yes (1)

Yes

No

No

No

-

No

5

Melano

26

No

No

No

No

15

No

6

Leucomelano

29

Yes (3)

No

No

Yes

No

84

laparotomic cerclage

7

Melano

36

No

No

Yes

8

Leuco

37

No

No

No

decided. On surgical exploration a large rupture was found on the posterior uterine wall; there was purulent exudate all over the abdominal cavity. A total hysterectomy was performed with unilateral adnexectomy due to technical difficulties.

After surgery, this patient stayed in the hospital for almost one month due to the isolation of a multiresistant Klebsiella Pneumoniae, which responded to Meropenem.

The mean age of this small sample was 33 years old (6 STD; min 26, max 44). Half were melanodermic and six had a previous cesarean. No case of prior myomectomy. Most went into spontaneous labour and most had a cesarean.

As for the rupture itself, some situations were identified as dehiscence and not as a complete disruption of the uterine wall.

This means that the serosa was still intact. The breach location was mostly on the previous historiography, therefore on the anterior uterine wall.

Finally, the timing of the diagnosis was mainly intrapartum; only one situation was diagnosed before, during the early third trimester, with inferior segment measurement on ultrasound; and the already mentioned rupture, that was diagnosed seven days after delivery.

Using the International Classification of Diseases 9t h Edition (ICD 66501 and 6651), we identified eight cases of uterine rupture in our hospital from January 2010 to September 2016 out of a total of 29 797 (average rate of cesarean of 28,5%).

Discussion The clinical significance of uterine rupture is growing, as its prevalence appears to be rising. However, it represents many challenges for modern obstetrics: its risk factors identification, its timely diagnosis and its prevention.

Many authors consider two types of uterine rupture: dehiscence and total rupture. Rupture would represent a disruption of both uterine muscle and visceral peritoneum while in dehiscence the peritoneum is intact.[1] Some authors prefer to consider dehiscence a partial rupture as opposed to total rupture.[2]

Yes

No

-

No

No

72

No

The incidence is somewhat difficult to define due to the scarcity of good quality studies since most of them are case reports. For this reason, uterine rupture rate varies from 1/10,000 to 1/16,840-19,765, in the unscarred uterus. In the event of a previous cesarean, then the rate goes higher as expected: 1/12354366 and as high as one /100. [3-8] Intrapartum diagnosis poses many obstacles as Holmgren et al. showed in a study of 36 cases of rupture.[9] This study shows which clinical sign led to the decision to perform a cesarean in a woman attempting trial of labor after cesarean (TOLAC): 30,5% severe variable decelerations, 19,4% prolonged fetal bradycardia, 22% maternal symptoms like pain and hypotension with no fetal heart rate concerning changes. The maternal pain was present in 25% of patients. This means that symptomatology is very varied and no symptom appears to be more frequent. This difficulty in intrapartum diagnosis increases time to delivery with possibly serious consequences for the mother and neonate According to this group, every additional minute to delivery enhances the risk of a neonatal adverse outcome by 8,8%. As for risk assessment, many clinical factors have been pointed out: maternal age equal or higher than 35 years, parity equal or higher than three, non-Western maternal origin, use of oxytocin, prostaglandins or transcervical balloon, scarred uterus (multiple previous cesarean section and type of previous hysterotomy closure), fetal macrosomia (birth weight ≥ 4 kg).[10-12] The one that reunites most agreement is the classical cesarean. In a study from 2012 by Gyamfi-Bannerman et al., three groups were compared: prior myomectomy, prior classical cesarean and previous lower segment transverse cesarean.[13] The main conclusions were that classical cesarean increased the risk of uterine rupture (adjusted OR 3,23), while previous myomectomy had no risk.

Despite these results, the group of prior myomectomy had some important differences from the groups of classical cesarean and lower segment transverse cesarean: lower rate of induction (1,1% vs. 2,6% for classical and 17,8% lower segment), lower rate of vaginal delivery (0% vs. 5,9% for classical and 44,2% for lower segment).

What is emerging, as a possibility to predict this disastrous

Vanessa Falé Rosado et al./ International Journal of Medical Reviews and Case Reports (2017) 1(2): 36-40

Table 4 Resume of type of delivery, type of rupture and neonatal outcomes of eight cases (time in labour in hours; CS – cesarian;

N.B.: newborn; AS – Apgar Score) Labor

#

1

Rupture Type

Spontaneous\

Time

induction/

in

cesarian

labor

spontaneous

4

Timing of

Site of Type

diagnosis

intrapartum

Delivery

uterine disruption

dehiscence

Other

Type

Weeks

complications

anterior

No neonatal

wall

intercorrence

NB

AS

weight

CS

38

2755

9//10

CS

39

2960

9//9

CS

39

3870

9//10

CS

38

3000

9//10

CS

39

3450

9//10

CS

35

2230

9//10

Breech

38

4525

0//0

CS

31

1580

9//9

broad 2

spontaneous

1

intrapartum

rupture

ligament

No neonatal

& lateral

intercorrence

wall 3

4

5

spontaneous

induction

2

6

elective

intrapartum

intrapartum

intrapartum

dehiscence

rupture

dehiscence

cesarian 6

7

8

spontaneous

spontaneous

spontaneous

3

2

5

intrapartum

postpartum

prepartum

rupture

rupture

dehiscence

complication, is lower uterine segment (LUS) measurement in women attempting TOLAC. According to Bujold et al., the measurement should be done between 35 and 38+ 6 weeks, using both the transvaginal and the transabdominal probes, to reduce interobserver variability.[14] Three measurements should be done and the smaller considered.

However, many debates surround this theme as for what should be measured and what the right cut-off value to consider. For some authors, full segment thickness is more predictive of uterine scar rupture. This means measuring both the myometrium and the bladder wall. However in a recent meta-analysis by Kok et al[15] measuring only the myometrium is as useful in predicting rupture. Another issue to debate is cut-off value: it has varied from 3,5 to 2 mm, and so far no precise value can be recommended.[16] In this case report the rupture occurred in an intact uterus, which makes it even harder to predict and diagnose and therefore with worst consequences as shown in a retrospective study

by Gibbins et al.[17] After exclusion of prednisone chronic use, connective tissue disease and multiple pregnancies, 146 cases of uterine rupture were selected; 20 of them in intact uterus.

After comparing these two groups (intact vs. scarred uterus), the major difference is the delay in the uterine rupture diagnosis: in women with a previous uterine scar, the delivery was more often a cesarean (91% vs. 58%), and for this reason the diagnosis was made intrapartum during the cesarean; as for intact uterus almost half women had a vaginal delivery and the diagnosis was delayed resulting in higher blood loss (2000 vs. 800mL, p <0,001), hysterectomy rates (35 vs. 2,4%, p < 0,001) and maternal

anterior

No neonatal

wall

intercorrence

posterior

No neonatal

wall

intercorrence

anterior

No neonatal

wall

intercorrence

anterior

No neonatal

wall

intercorrence

posterior

Hysterectomy;

wall

stillbirth

anterior

No neonatal

wall

intercorrence

morbidity (65 vs. 20%, p < 0,001).

As mentioned above, every obstetrician should be more and more aware of this condition because recent analysis shows its increasing incidence like this Norwegian study from 2015.[12] Deliveries from 21 hospitals from 1967 to 2008 were selected and divided into four groups that correspond to four decades: 6777, 78-88, 89-99, 00-08. The incidence increased abruptly in the last decade: from 0.9/10 000 in the second decade (1978–1988) to 6.1/10 000 in the fourth decade (2000–2008). This increase was mostly because of the scarred uterus group: 14.2/10 000 in the second decade to 66.8/10 000 in the fourth decade. Indeed, scarred uterus and labour augmentation with oxytocin were the main contributors to this increase in uterine rupture. In the intact uterus, after adjusting for prostaglandins and oxytocin use, the OR remains almost the same.

Finally, after a uterine rupture should we be tremendously scared of the next pregnancy? In a small study by Fox et al. with 60 pregnancies (20 after uterine rupture and 40 after uterine dehiscence), pregnancy outcomes were very good with no cases of repeated rupture or hysterectomy. [18] The only dehiscence repeated itself: 5% in the rupture group and 7,5% in the dehiscence group. All pregnancies were ended before 40 weeks: in the rupture group 75% ended between 36 and 37 weeks via cesarean and in the dehiscence group 93% between 36 and 39 weeks via cesarean.

Vanessa Falé Rosado et al./ International Journal of Medical Reviews and Case Reports (2017) 1(2): 36-40

Conclusion Uterine rupture is a serious complication that can be more easily suspected in the case of the previous cesarean; however, it can also happen in the intact uterus and, even though it is a rare event, factors like labour augmentation and prostaglandins use for labour induction can eventually lead to an increase in its prevalence.

In this particular case, some risk factors can be identified as fetal macrosomia, higher parity and maternal age over 35.

Still, she was discharged from the hospital 48hrs after delivery with no complaints and after a normal examination. Only one week later she comes back with pain and fever. This delay in diagnosis of more than one week after delivery led to very serious morbidity like mentioned above by Gibbins et al.

For all that it was mentioned, uterine rupture should be a diagnostic hypothesis to consider, especially if risk factors like older maternal age and previous uterine scar are present. An earlier clinical suspicion means a better obstetric outcome.

Authors’ Statements Competing Interests The authors declare no conflict of interest.

References 1. Landon MB, Hauth JC, Leveno KJ, et al. Maternal and perinatal outcomes associated with a trial of labour after prior cesarean delivery. N Engl J Med 2004;351: 2581-9.

2. Nahun GG, Pham KQ. Uterine rupture in Pregnancy. Medscape Reference, 25 Mar, 2016 [www.reference.medscape. com/article/275854-overview]. Accessed 2 March 2017.

3. Gardeil F, Daly S, Turner MJ. Uterine rupture in pregnancy reviewed. Eur J Obstet Gynecol Reprod Biol 1994;56:10710.2. Miller DA, 4. Goodwin TM, Gherman RB, Paul RH. Intrapartum rupture of the unscarred uterus. Obstet Gynecol 1997;89:671-3.

9. Holmgren C, Scott JR, Porter TF, Esplin MS, Bardsley T. Uterine Rupture With Attempted Vaginal Birth After Cesarean Delivery Decision-to-Delivery Time and Neonatal Outcome. Obstet Gynecol 2012; 119: 725–31 10. Kaczmarczyk M, Sparen P, Terry P, Cnattingius S. Risk factors for uterine rupture and neonatal consequences of uterine rupture: a population-based study of successive pregnancies in Sweden. BJOG 2007;114:1208–14.

11. Al-Zirqi I, Stray-Pedersen B, Forsen L, Vangen S. Uterine rupture after previous caesarean section. BJOG 2010;117:809–20 12. Al-Zirqi I, Stray-Pedersen B, Forsen L, Daltveit A, Vangen S. Uterine rupture: trends over 40 years. BJOG 2016;123:780–787.

13. Gyamfi-Bannerman et al. Risk of Uterine Rupture and Placenta Accreta With Prior Uterine Surgery Outside of the Lower Segment. Obstet Gynecol 2012;120:1332–37 14. Bujold E, Jastrow N, Simoneau J, Brunet S, Gauthier RJ. Prediction of complete uterine rupture by sonographic evaluation of the lower uterine segment. Am J Obstet Gynecol 2009; 201: 320.e1–6 15. Kok N, Wiersma IC, Opmeer BC, de Graaf IM, Mol BW, Pajkrt E. Sonographic measurement of lower uterine segment thickness to predict uterine rupture during a trial of labour in women with previous Cesarean section: a meta-analysis.

Ultrasound Obstet Gynecol 2014; 42: 132–139.

16. Jastrow N, Chaillet N, Roberge S, Morency AM, Lacasse Y, Bujold E. Sonographic lower uterine segment thickness and risk of uterine scar defect: a systematic review. J Obstet Gynaecol Can 2010; 32: 321–327.

17. Gibbins KJ, Weber T, Holmgren CM, et al. Maternal and fetal morbidity associated with uterine rupture of the unscarred uterus. Am J Obstet Gynecol 2015;213:382.e1-6.

18. Fox et al. Pregnancy Outcomes in Patients With Prior Uterine Rupture or Dehiscence. Obstet Gynecol 2014;123:785–9

5. Landon MB. Uterine rupture in primigravid women. Obstet Gynecol 2006;108:709-10.

6. Zwart JJ, Richters JM, Ory F, de Vries JI, Bloemenkamp KW, van Roosmalen J. Uterine rupture in The Netherlands: a nation- wide population-based cohort study. BJOG 2009;116:1069–78.

7. Landon MB, Hauth JC, Leveno KJ, Spong CY, Leindecker S, Varner MW, et al.; National Institute of Child Health and Human Development Maternal-Fetal Medicine Units Network. Maternal and perinatal outcomes associated with a trial of labour after prior cesarean delivery. N Engl J Med 2004;351:2581–9.

8. Landon MB, Spong CY, Thom E, Hauth JC, Bloom SL, Varner MW, et al.; National Institute of Child Health and Human Development Maternal-Fetal Medicine Units Network. The risk of uterine rupture with a trial of labour in women with multiple and single prior cesarean delivery.

Obstet Gynecol 2006; 108:12–20.

Vanessa Falé Rosado et al./ International Journal of Medical Reviews and Case Reports (2017) 1(2): 36-40

CASE REPORT

MISDIAGNOSIS OF AN ACARDIAC TWIN IN THE FIRST TRIMESTER: A CASE REPORT Catarina Vasconcelos∗, 1 , Vanessa Falé Rosado∗ , Rita Torres∗ , Ana Teresa Martins∗ and Álvaro Cohen∗ ∗ Maternidade

Dr. Alfredo da Costa - Centro Hospitalar Lisboa Central.

ABSTRACT Background:Twin reversed arterial perfusion (TRAP) sequence is a rare monochorionic twin pregnancy complication. It should be suspected in the first-trimester ultrasound when one fetus has no cardiac activity in monochorionic pregnancies. Doppler study is essential for early diagnosis of acardiac twin. This manuscript shows the importance of reassessing cases considered as vanishing twins in early pregnancy, to avoid a late diagnosis of acardiac twin, without timely treatment. Case Summary: In this case, the assumption of the death of one fetus in the first trimester, delayed the correct diagnosis of TRAP until 25 weeks, when the case was referred to our unit. We opted conservative management with weekly ultrasound surveillance, looking for features of heart failure in the pump twin. After suspecting fetal anaemia, at 32 weeks a healthy baby weighing 2100g was delivered along with an acardiac anceps with 518g. This is a case of expectant management, for lack of another option, to a successful end. Conclusion: Although twin reversed arterial perfusion (TRAP) sequence being a rare monochorionic twin pregnancies complication, it should be suspected in the first-trimester ultrasound, when detected monochorionic pregnancies and one embryo/fetus has no cardiac activity.

Follow up in the fetal death of one twin in monochorionic pregnancies must be done with Doppler to look for an acardiac twin. Because pump twin may develop a high-output cardiac failure and intrauterine fetal demise, for continuing pregnancies without poor prognostic criteria, it is important to maintain weekly ultrasound surveillance, with attention to the intervention criteria.

KEYWORDS TRAP sequence, acardiac fetus

Introduction TRAP sequence is a rare and exclusive complication of monochorionic twin pregnancies, with recent data on the incidence of 2.6% in these pregnancies.[1] It is characterised by a fetus that lacks cardiac structure and activity (acardiac twin), that is perfused and hemodynamically dependent (parasite) on the structurally normal co-twin (pump twin). This anomaly occurs Copyright © 2019 by the Bulgarian Association of Young Surgeons DOI: 10.5455/IJMRCR.acardiac-twin-first-trimester First Received: July 09, 2017 Accepted: July 22, 2017 Manuscript Associate Editor: George Baytchev (BG) Editor-in Chief: Cvetanka Hristova (BG) Reviewers: Ivan Inkov (BG) 1 Catarina Vasconcelos, Maternidade Dr. Alfredo da Costa - Centro Hospitalar Lisboa Central, E-mail:catv asconcelos@yahoo.com

through an artery-to-artery placental anastomosis, creating a “reversed” circulation, which provides perfusion of mixed or medium oxygenated blood from the pump twin to the recipient twin. It results in poor perfusion and tissue necrosis, with the evolution of a variety of structural abnormalities in the acardiac fetus.

The heart of the acardiac fetus may be completely absent (holo-acardius) or represented by a fundamental cardiac structure (pseudo-acardius). Four distinct morphological types have been described in literature: acardius acephalous: is the most common type (60-75%), with developed pelvis and lower limbs, may have arms, but thoracic organs and head are absent; acardius anceps: the most differentiated type of acardiac twins with established body and extremities, but only a partially formed head and face (20%); acardius acormus: in which only cephalic structures were detectable, is a sporadic type (10%); acardius amorphous: consists of a shapeless mass of tissue containing no recognizable human structures (5%).[2]

Catarina Vasconcelos et al./ International Journal of Medical Reviews and Case Reports (2017) 1(2):41-45

CASE REPORT: A 37-year-old, caucasian, spontaneous primigravida was referred to our prenatal unit at 25 weeks when twin reversed arterial perfusion (TRAP) sequence was diagnosed.

Early in the pregnancy she had performed the first ultrasound at ten weeks and five days: twin pregnancy was identified, with a second embryo without cardiac activity. At the 12 week ultrasound, one fetus had a crown rump length (CRL) of 61.3mm, described with normal morphology, 1.6mm of nuchal translucency and low risk in the combined screening test. At this time, the second fetus was not described, assuming a vanishing twin.

The morphological ultrasound at 20 weeks identified a normal female fetus and a heterogeneous mass of 71x24mm, with suggestive bone tissue, considering possible resorption of the second fetus.

At the time she arrived at our prenatal unit (25 weeks), was described as a monochorionic monoamniotic twin pregnancy, with one structurally normal fetus and a second twin anatomically abnormal and acardiac. The first twin presented a fetal estimation weight (EFW) of 846g, in the 50th percentile, normal fetal Doppler study and normal amniotic fluid index. The acardiac mass continued to grow (98x53x57mm), and TRAP sequence was diagnosed by Doppler study, which identified interstitial blood flow in the acardiac mass (figure 1).

continued EFW in the 50th percentile and at breech presentation.

No signs of high-output cardiac failure like polyhydramnios, cardiomegaly, ascites and fetal hydrops were found. The acardiac mass was 176x90x55mm, less than 25% of the healthy twin estimated weight (2073g). After inconclusive neurosonogram, a fetal magnetic resonance imaging (MRI) evaluated the natural brain development of the first twin, at 29 and 32 weeks.

Due to suspected fetal anemia, at 32 weeks and 5 days, she underwent caesarean section. Delivered a typical female live baby, weighting 2100g, Apgar score of 8 / 9 at one and five minutes respectively; and another female acardiac anceps: with 518g. The acardiac fetus had a rudimental structure: incompletely formed skeleton, edematous lower limbs, shortened upper limbs, abdomen with omphalocele and umbilical cord with two vessels, remnants of the chest and head structures (Figures 2 and 3).

Figure 2: Acardiac fetus delivered.

The healthy baby had mild anaemia (hemoglobin: 13.4 g/dl, haematocrit 38.9%), without the need for transfusion. She was admitted to the intensive/intermediate care unit, with a diagnosis of prematurity, hyperbilirubinemia and intra-periventricular haemorrhage grade I and discharged on the 17th day of life, clinically and neurologically well. Maintains neurodevelopment surveillance in our hospital centre.

Figure 1: Ultrasound of the acardiac fetus at 28 weeks, measuring 130x98x67mm.

DISCUSSION

Weekly ultrasound was performed with standard Doppler study and no signs of cardiac failure.

At 29 weeks, the peak systolic velocity (PSV) in the middle cerebral artery (MCA) of the healthy fetus reached 1.54 multiples of the median (MoM), with no signs of hemodynamic decompensation. Due to suspicion of fetal anaemia, a course of antenatal corticosteroids for fetal lung maturation was administered. Since then ultrasound surveillance started to be performed twice a week. The PSV in the MCA maintained under 1.5 MoM until 32 weeks and five days, reaching 1.69 MoM. The remaining Doppler studies were normal. The pump twin

Advances in first-trimester ultrasound have enabled diagnosis of TRAP sequence at earlier gestational age. TRAP should be suspected, when a monochorionic pregnancy is diagnosed with one fetus appearing anatomically normal, and the other lacks cardiac structures and activity. The confirmation is made with Doppler study: pulsatile flow in the umbilical artery towards the acardiac fetus or interstitial blood flow within the mass and supported by the observation of continuous growth of the acardiac twin in serial ultrasound examinations.[3] It may simulate an intrauterine death of one grossly abnormal monochorionic twin or a placental teratoma. In the described case, the first ultrasound identifies the second embryo without cardiac activity, and

Catarina Vasconcelos et al./ International Journal of Medical Reviews and Case Reports (2017) 1(2):41-45

Table 1 Timeline of interventions and outcomes.

From Obstetric Ultrasound to Delivery Out of our unit 10w

12w

In our prenatal unit 20w

25w

Once a

29w

week

Twice a

32w

32w

week Caesarean

Monochorionic monoamniotic twin, one structurally One fetus with

normal fetus One female

Two

normal

embryos

morphology

one

and low

without

risk in

cardiac

the

activity.

combined

fetus, normal morphology and a heterogeneous

screening

mass of 71x24mm.

test.

(EFW - 846g) and a second twin

Ultrasound

(98x53x57mm)

+

anatomically

Doppler

abnormal and

study

acardiac.

Doppler study identified interstitial blood flow in

Healthy fetus:

delivery

EFW in

because of

the 50t h

fetal

percentile and

breech

at breech

presentation.

Normal fetus:

presentation.

PSV in

PSV in the

Healthy

the MCA

MCA =

female live

= 1.54 MoM.

1.69 MoM.

baby, 2100g

No signs of

No signs of

AS-8/9. Mild

hemodynamic decompensation.

Fetal MRI: normal brain

Ultrasound + Doppler study

high-output

anaemia

cardiac failure.

(hemoglobin:

Acardiac mass:

13.4 g/dl,

176x90x55mm,

haematocrit

development

less than 25%

38.9%),

of

of the standard

without the

the pump

twin estimated

need for

twin.

weight

transfusion.

the acardiac mass.

(2073g).

Discharged on

Fetal MRI:

the 17th day of

normal brain

life, clinically

development of

and

the pump twin.

neurologically well.

Suspicion of fetal anaemia, a course of Resorption

antenatal

Suspicion of

Vanishing

of

corticosteroids

fetal anaemia.

twin?

the second

for fetal

Delivery

fetus?

lung

decision.

TRAP sequence.

Female acardiac anceps,with 518g.

maturation was administered.

AS - Apgar score; EFW - fetal estimation weight; MCA - middle cerebral artery; MRI - magnetic resonance imaging; MoM - multiples of median; PSV - peak systolic velocity; TRAP - twin reversed arterial perfusion; W- Weeks;

Catarina Vasconcelos et al./ International Journal of Medical Reviews and Case Reports (2017) 1(2):41-45

Figure 3: X-ray of Acardiac fetus. a vanishing twin was erroneously assumed in the first trimester.

The pump twin may develop a high-output cardiac failure and intrauterine fetal demise. This high cardiac output also increases perfusion of the fetal kidneys, resulting in overproduction of fetal urine and polyhydramnios, at risk of premature delivery.[2] The perinatal mortality rate for pump twin without treatment is high, around 55%, due to heart failure and preterm delivery.[4] After in utero therapy, the survival rate of the pump twin increases to 80-90%.

Indicators of poor prognosis have been described. A higher weight of the recipient twin is more likely associated with cardiac insufficiency and perinatal mortality of the pump twin.[2] When the ratio of the weight of the acardiac twin to the weight of the pump twin is greater than 70%, the risk of preterm delivery is 90%, and of congestive heart failure in the pump, twin is 30%. In comparison, when the ratio is less than 70% the corresponding risks are 75% and 10%, respectively. Estimation of acardiac fetus weight can be calculated using the following formula: Weight (grams) = (1.2 Ă&#x2014; longest length 2) - (1.7 Ă&#x2014; most extended lengths).[4] The alternative prognostic factor is the ratio of the abdominal circumference of acardiac twin to standard twin when â&#x2030;Ľ 1.0 is considered significant.[5] After diagnosis, the aim of management is to save the pump twin, as mortality is 100% for an acardiac twin. Exclusion of genetic abnormalities (determination of the karyotype) and ultrasound malformations of the pump twin, is advised before the management decision. It has been reported that the pump twin has a 9% risk of aneuploidy. To improve the outcome for the pump twin, at viable gestational age, fetal monitoring should be done with weekly ultrasound and Doppler fetal study. If there is evidence of pre-hydrops signs, the frequency should be twice a week. Fetal surveillance with Doppler studies of middle cerebral artery help in early diagnosis of anaemia in pump twin.[3,6] Fetal neurosonogram should be performed to evaluate brain development of the pump twin if cerebral lesions are suspected a fetal MRI is advised.

The correct and appropriate management is not yet estab-

lished. Antenatal intervention, delivery and expectant management are possible options. It is controversial regarding elective versus treatment of TRAP. Some support elective intervention due to improved outcomes after therapy and low sensitivity of prognosis factors to predict intrauterine fetal death.[7] It seems that the results are better if therapy is done before 16 weeks.

Others support expectant management with treatment when sonographic evidence of compromise is present in the pump twin (polyhydramnios, cardiac dysfunction, abnormal Doppler and hydrops), avoiding an intervention that itself can cause miscarriage.[2] Current, minimally invasive in utero interventions, to occlude vascular supply to the acardiac twin, can be performed through two modalities: extra-fetal (intertwine anastomosis or umbilical cord occlusion) and intra-fetal circulation. Occlusion of the umbilical cord of the acardiac twin can be performed through a fetoscope by laser or bipolar coagulation. The intra-fetal intervention modality is performed by radiofrequency ablation (RFA) or laser therapy, through a needle inserted under ultrasound guidance. RFA coagulates the abdominal wall of the acardiac twin, at the base of the umbilical cord, being preferred at gestational ages greater than 16 weeks. Laser needle is inserted towards fetal abdomen and the target fetal vessels, being preferred until 16 weeks.[3,8] Current small evidence, suggests ultrasound guided intrafetal techniques are associated with higher success rates and lower postoperative complications like preterm premature rupture of membranes and preterm labour. Laser and RFA are relatively comparable, with neonatal survival rates of 82% and 85%, respectively.[7,9] The choice of treatment should be based on operator experience, gestational age and accessibility.[3] The ideal timing of in utero intervention remains controversial; procedures have been reported between 12 and 27 weeks of pregnancy, is usually performed at the beginning of the second trimester (16 weeks). Knowing that the death of the pump twin occurs mostly until 16 weeks, preventive intervention at 12 weeks may be more advantageous. Larger studies are necessary to examine the best timing for preventive intervention with the higher pump twin survival.[2] In our case, the patient was diagnosed late in pregnancy (25 weeks), at the time she was referred to us we adopted an expectant management with weekly ultrasound surveillance, because of the higher risk of preterm delivery after treatment in late gestational age. No polyhydramnios, signs of cardiac failure or fetal hydrops in the pump twin were identified. The acardiac twin continued to grow but with an estimated weight proportion of less than 25%. Rohilla et al. defends conservative treatment when the acardiac twin is less than 25% the weight of the pump twin and no signs of heart failure. When the weight of the acardiac twin exceeds 70% of the pump twin, invasive intervention is justified.[10] The timing of delivery depends on clinical evolution and gestational age. TRAP sequence without poor prognostic criteria should deliver at 34-36 weeks of gestation. Caesarean delivery is indicated for general obstetrical indications.[3] When anaemia of the pump twin was suspected in this case,

by Doppler study of middle cerebral artery, a course of antenatal steroids was administered. The sign of fetal anaemia was our criteria to interrupt pregnancy after fetal pulmonary maturation. We delivered a healthy baby at 32 weeks and five days by caesarean section, for breech presentation.

Our case is an example of why ultrasound should be per-

Catarina Vasconcelos et al./ International Journal of Medical Reviews and Case Reports (2017) 1(2):41-45

formed by an experienced sonographer to avoid failure in early diagnosis of TRAP. In this sequence of late diagnosis, the approach of expectant management with tight surveillance to decide when to intervene or deliver allows good outcomes.

LEARNING POINTS/TAKE HOME MESSAGES • Although twin reversed arterial perfusion (TRAP) sequence being a rare monochorionic twin pregnancies complication, it should be suspected in the first-trimester ultrasound, when detected monochorionic pregnancies and one embryo/fetus has no cardiac activity.

• Follow up in the fetal death of one twin in monochorionic pregnancies must be done with Doppler to look for an acardiac twin.

• The appropriate management is not established: elective treatment and expectant management with treatment or delivery, are options. Early in utero therapy reduces the perinatal mortality rate. Intra-fetal intervention with laser or radio-frequency ablation is the preferred modality.

• Because pump twin may develop a high-output cardiac failure and intrauterine fetal demise, for continuing pregnancies without poor prognostic criteria, it is important to maintain weekly ultrasound surveillance.

• Criteria for intervention should be: Onset of Cardiac failure, hydrops of the pump twin or polyhydramnios and acardiac/pump twin weight ratio > 70%

8. Kilby M, Johnson A, Oepkes D. Fetal Therapy: Scientific Basis and Critical Appraisal of Clinical Benefits. Cambridge University Press. 2013; 194:197.

9. Cabassa P, Fichera A, Prefumo F, et al. The use of radiofrequency in the treatment of twin reversed arterial perfusion sequence: a case series and review of the literature. Eur J Obstet Gynecol Reprod Biol 2013; 166:127.

10. Rohilla M, Chopra S, Suri V, Aggarwal N, Vermani N. Acardiac-acephalus twins: a report of 2 cases and review of literature. Medscape J Med. 2008; 10:200.

Authors’ Statements Competing Interests The authors declare no conflict of interest.

References 1. Van Gemert MJ, Van den Wijngaard JP, Vandenbussche FP. Twin reversed arterial perfusion sequence is more common than generally accepted. Birth Defects Res A Clin Mol Teratol 2015; 103:641.

2. Pepe F, Teodoro MC, Luca C, Privitera F. Conservative management in a case of uncomplicated TRAP sequence: a case report and brief literature review. Journal of Prenatal Medicine 2015; 9(3/4):29-34 3. Mastrobattista JM, Lucas MJ. Diagnosis and management of twin revered arterial perfusion (TRAP) sequence. UpToDate Nov 2016.

4. Moore TR, Gale S, Benirschke K. Perinatal outcome of fortynine pregnancies complicated by acardiac twinning. Am J Obstet Gynecol 1990; 163:907.

5. Wong AE, Sepulveda W. Acardiac anomaly: current issues in prenatal assessment and treatment. Prenat Diagn.

2005;25:796-806.

6. Dubey S, Verma M, Goel P, Punia R. Twin Reversed Arterial Perfusion: To Treat or Not? Journal of Clinical and Diagnostic Research. 2017 Jan, Vol-11(1): QD05-QD07 7. Pagani G, D’Antonio F, Khalil A, Papageorghiou A, Bhide A, Thilaganathan B. [13] Intrafetal laser treatment for twin reversed arterial perfusion sequence: cohort study and metaanalysis. Ultrasound Obstet Gynecol. 2013;42:6-14 Catarina Vasconcelos et al./ International Journal of Medical Reviews and Case Reports (2017) 1(2):41-45

CASE REPORT

SPIDER PROCEDURE FOR SACRAL PRESSURE SORE RECONSTRUCTION: A CASE REPORT Sarah Sabur∗,1 , EL Aissaoui Imane∗ , Raboune Mohamed∗ , Bensaida Lamiaa∗∗ , Mazouz Samir∗ , Gharib Noureddine∗∗ and Abassi Abdellah∗∗ ∗ Department

of Plastic and Reconstructive Surgery, Mohammed V University Rabat, Morocco.,

∗∗ Plastic

Surgeon, Mohammed V Hospital, Tangier, Morocco.

ABSTRACT Background: Many forms of management have been described to treat a sacral pressure sore when the sacral pressure sore is type 4 the surgical treatment becomes necessary. Myocutaneous and fascio-cutaneous flaps can provide stable coverage of pressure sores. We propose in this article the use of a fascio-cutaneous flap named Spider flap in the coverage of sacral pressure ulcer in a young ambulant patient. Case report: A 43-years-old ambulant woman was addressed to our department to assure the management of a sacral pressure sore developed after an extended immobilisation in intensive care unit, the coverage was assured with modified 5- fascio-cutaneous flap Z-plasty technique called the spider procedure, the follow-up was nine months, with no sign of recurrence. Conclusion: The spider procedure has the advantage of tension-free closure, simple dissection with short operative time and no sacrifice of muscles function which can make this flap a useful solution in sacral pressure sore reconstruction.

KEYWORDS Spider procedure, Sacral defect pressure sore.

INTRODUCTION Pressure ulcers, also known as decubitus ulcers or bedsores, are defined as localised injuries to the skin and underlying tissue, usually over a bony prominence, as a result of pressure, or pressure in combination with shear and friction [1]. The sacral pressure sore is the most frequent form of pressure sores, and the treatment begins with prevention, but when it is classified as type 3 or 4 the surgical management is required, and it can be challenging especially for the ambulatory patients who developed this condition during an extended immobilisation in intensive care unit. For those patients, we should assure a good coverage of the wound with bulky and well vascularized healthy tissues to assure a good healing and assure a low rate of morbidity and disability.

Copyright © 2019 by the Bulgarian Association of Young Surgeons DOI: 10.5455/IJMRCR.Spider-procedure-for-sacral-pressure-sore-reconstruction First Received: June 10, 2017 Accepted: July 02, 2017 Manuscript Associate Editor: George Baytchev (BG) Editor-in Chief: Cvetanka Hristova (BG) Reviewers: Ivan Inkov (BG) 1 [Dr Sarah Sabur, Plastic and reconstructive surgery department, Ibn Sina teaching Hospital, Rabat-Morocco. Email:sarah.kadhem77@gmail.com

A lot of surgical techniques were proposed to treat this condition, we present our experience in the coverage of sacral defect occurred after pressure sore, with a spider procedure, the aim of this article is to propose the use of this simple technique which uses local tissue with tension-free closure in the coverage of medium to large size sacral pressure sore defect. Until now there is no references in the medical literature for the use of this flap in pressure sore reconstruction.

CASE REPORT: A 43 years old ambulant woman was addressed to our department to assure the management of a sacral pressure sore developed after an extended immobilisation in intensive care unit. At the time of consultation, the patient was on a physical therapy program to assure an optimal return of function. The clinical examination found a sacral pressure sore stage 4; the X-ray did not show any signs of osteomyelitis.

We scheduled surgery after a wound preparation with chemical and mechanical debridement, infection control, and improvement of nutritional parameters. The surgery was performed under spinal anaesthesia. We drew first the pattern of the spider procedure [Figure 1-2]. Then we realised a Surgical debridement until viable tissues were encountered.

The debridement consisted of a triangular incision of ulcer,

Sarah Sabur et al./ International Journal of Medical Reviews and Case Reports (2017) 1(2): 46-48

Figure 1: The pattern of spider procedure.

Figure 3: The final aspect after closure.

Figure 2: Technical drawing of spider procedure.

Figure 4: Nine months after surgery. underlying bursa, surrounding calcifications, the final defect was an equilateral triangle each side had the size of 9 cm. In depth, the defect arrives until contact of the bone.

The closure was assured then with the spider procedure which includes the use of a modified 5-flap Z-plasty technique to obtain maximum tissue relaxation for the tension-free closure of skin defect [Figure 3] [2] [3]. The skin flaps were elevated as fascio-cutaneous flaps. To prevent any fluid collection under the flaps, we put two drains. The patient was immobilised in ventral position for two weeks after the surgery. An antibiotic treatment depending on bacterial culture realised in the debridement tissue was prescript; the dressing was changed three times a week.

The postoperative period was uneventful for the patient.

Drains were used for five days. Follow-up of 9 months [Figure 4], did not show any recurrence.

DISCUSSION: Sacral pressure sores are commonly seen in neurologically compromised patients and in those who have received prolonged

anaesthesia or in sedated patients that remain in the supine position. The pressure wound management demands a multidisciplinary approach. For the invasive type pressure sores (type 3 and 4), in which there is full thickness tissue loss with or without exposed muscle, bones or tendons, surgery remains the best option [4], The goals of surgical management are to prevent progressive osteomyelitis, reduce protein loss, improve quality of life, improve function and hygiene, and reduce rehabilitation and wound care cost [1]. To achieve those goals, various methods are proposed to surgically treat the sacral pressure sore, depending on the size and the depth of the defect. The skin grafting is indicated if the defect is small and secondary to acute/short-term disability. However, the recurrence rate with this method is high (70%) [5]. Gluteus maximus flap remains the first choice for sacral pressure sore reconstruction [6].Theoretically The advantages of using myocutaneous flaps are elimination of the dead space because they are bulky flaps, providing a well-vascularized and cushioning tissue over the pressure bearing area and their vascularity helps fighting infection at the local site [7], but it compromises muscle function and

Sarah Sabur et al./ International Journal of Medical Reviews and Case Reports (2017) 1(2):46-48

should be avoided, especially in ambulatory individual [1]. It is also may be prone to postoperative skin breakdown because of tension [8], and the deneurotized muscle becomes atrophic and loses its dynamic function and the ability to absorb the pressure [9].

The fasciocutaneous flaps have the advantage of the reduction in donor site morbidity, minimal blood loss, decreased postoperative pain, shorter hospital stays, reduced costs, and preservation of muscle function.

Yamamoto et al. [10] reported that the use of the fasciocutaneous flap is expected to provide a better long-term result in the surgical reconstruction of pressure sores than the myocutaneous or muscle flap.Thiessen et al. [7] in their study showed that the complications and the recurrence rates were not associated with the type of the flap. Local fasciocutaneous flaps like Limber flap, Dufourmentel flap, are simple to realise and have stable circulation however they are inappropriate to cover a large skin defect in the sacral area because of the distortion and displacement of the adjacent mobile anatomic structures, so the defect closure is done with high tension [4].Sapountzis and all proposed the use of the reading man flap to cover pressure sores with The advantage of tension-free closure and the minimal additional healthy skin excision, but this flap is not able to fill the dead space [4].

Several perforator flaps have been proposed to cover sacral pressure sores, but they are more tedious to dissect [11] [12] and are more prone to venous congestion [13]. The free flap coverage indications include the absence of local flap tissue especially in the multiple recurrent pressure sore [1].

In our cases we used the spider procedure based on Z–plasty concept to reconstruct a sacral pressure severe defect (size 9 cm).

In this procedure, first, the existing defect is surgically converted to a triangle in shape, then using a modified 5-flap Z –plasty pattern. The flaps are outlined by transposing the elevated flaps in a Z-plasty manner. A tension free closure is achieved using tissue relaxation provided by opposing Z Plasty [3].

The advantage of this flap comparing to other local fasciocutaneous flaps indicated to cover large sacral defect with big cavity like bilateral VY advancement gluteal fascio-cutaneous flap and rotational gluteal fascio- cutaneous flap is that the tension in the mid-line is minime because of the extra tissue relaxation provided by opposing Z-plasty.

CONCLUSION: The spider procedure has the advantage of tension-free closure, simple dissection with short operative time and no sacrifice of muscles function which can make this flap a useful solution in sacral pressure sore defect reconstruction, Especially in a noparalyzed patient whom we want stable results with less donor site morbidity.

AUTHOR CONTRIBUTIONS Sarah Sabur wrote this article. All authors have read and agreed to the final version of this manuscript and have equally contributed to its content and the management of the cases.

DISCLOSURE STATEMENT There was no financial support or relationships between the authors and any organisation or professional bodies that could pose any conflict of interests.

FUNDING This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors

COMPETING INTERESTS The authors declare no conflict of interest.

Written informed consent obtained from the patients for publication of this article and any accompanying images.

References 1. Diaz S, Li X, Rodríguez L, Salgado CJ.Update in the Surgical Management of Decubitus Ulcers. Anaplastology. 2013; 2: 113.

2. Gemici K, Şentürk S. Surgical management of expansive sacrococcygeal pilonidal sinus with the spider procedure.

Eur J Gen Med.2015; 12(3); 203.207.

3. Mutaf.M, Temel M, Günal E .The spider procedure: Anew Zplasty based local flap procedure Ann Plast Surg.2012; 69 (5):555-9.

4. Sapountzis S, JoonPark H, Ye Ong Heo C.The reading man flap for pressure sore reconstruction.Indian J Plast Surg 2011 Sep-dec; 44(3):448-452.

5. Bauer J, Phillips.Pressure sores. Plast Reconstr Surg.2008; 121:1- 10.

6. Sarensen JL, Jorgesen B, Gottrup F. Surgical treatment of pressure ulcers.AM J Surg. 2004; 188:42-51.

7. Thiessen FE, Andrades P, Blondeel P, Hamdi M, Roche N, Stillaert F, et al.(2011). Flap surgery for pressure sores: should the underlying muscle be transferred or not? J Plast Reconstr Aesthet Surg.2011; 64:84-90.

8. Rubayi S, Chandrase Kharb S. Trunk, abdomen and pressure sore reconstruction.Plast Reconstr Surg 2011; 128: 201 e-215 e.

9. Lee JL, Pyon JK, Lim SY, Mun GH, Bang SL, Oh KS. Perforator- based bilobed flaps in patients with a sacral sore: Application of a schematic design. J Plast Reconstr Aesthet Surg. 2011; 64:790–5 10. Yamamoto Y, Tsutsumida A, Murazumi M, Sugihara T. Long-term outcome of pressure sores treated with flap coverage. Plast Reconstru Surg. 1997; 100: 1212–7 11. Koshima I, Moriguchi T, Soeda S, Kawata S, Ohta S, et al. The gluteal perforator-based flap for repair of sacral pressure sores. Plast Reconstr Surg.1993; 91: 678-683.

12. Nojima K, Brown SA, Acikel C, Arbique G, Ozturk S, et al.

Defining vascular supply and territory of thinned perforator flaps: part I. Anterolateral thigh perforator flap. Plast Reconstr Surg .2005; 116: 182-193.

13. Lee BT, Lin SJ, Bar-Meir ED, Borud LJ, Upton J .Pedicled perforator flaps: a new principle in reconstructive surgery.

Plast Reconstr Surg.2010; 125: 201208

Sarah Sabur et al./ International Journal of Medical Reviews and Case Reports (2017) 1(2): 46-48

REVIEW ARTICLE

META-ANALYSIS OF ANTIEPILEPTIC DRUGS INDUCED CHOREOATHETOSIS IN PAEDIATRIC PATIENTS Sriram Shanmugam∗∗ , Lidhu Daniel∗∗ and Jaleel Ahamed∗ ∗ Consultant

paediatrician, Sri Ramakrishna Hospital, Coimbatore, India., ∗∗ Department of Pharmacy Practice, College of Pharmacy, SRIPMS, Coimbatore, India.

ABSTRACT BACKGROUND The safety profile of anti-epileptic drugs (AEDs) is an essential consideration for the regulatory bodies, owners and prescribing clinicians. Meta-analysis has increasingly been used to identify adverse effects of drugs.

Efficacy studies are often too small to reliably assess risks that become important when a medication is in widespread use, so meta-analysis, which is a statistically efficient way to pool evidence from similar studies, seems like a natural approach. The safety profile of drugs is an important consideration, and it affects clinicians’ decisions to prescribe specific AED(s), as serious adverse effects can lead to chronic complications or even death. Less serious, but significant, adverse effects can significantly impact quality of life, leading to systematic illness which may increase the overall cost of treatment. METHODS An electronic search was performed in using Pubmed,Paediatric journals of Neurology, MEDLINE. RESULTS The literature search identified 30 unduplicated papers. Of these 11 papers were excluded by reading the abstracts and titles. Another ten papers were excluded from reading their complete text. We selected nine papers which comprised of case studies and observational studies. CONCLUSION The combination of different antiepileptic drugs has resulted in drug-induced choreoathetosis. A mainly increased risk was seen with combinations that have phenytoin and lamotrigine. This could be due to an additive or a synergistic effect on central dopaminergic pathways.

KEYWORDS: choreoathetosis, antiepileptics, meta-analysis

Introduction Adverse Drug Reaction (ADR) is defined as "Any noxious change, which is suspected to be due to the drug, occurs at doses normally used in man, requires treatment or decrease in dose or indicates caution in the future use of the same drug”.

Copyright © 2019 by the Bulgarian Association of Young Surgeons DOI:10.5455/IJMRCR.ANTIEPILEPTIC-DRUGS-INDUCED-CHOREOATHETOSIS First Received: July 31, 2017 Accepted: August 31, 2017 Manuscript Associate Editor: George Baytchev (BG) Editor-in Chief: Cvetanka Hristova (BG) Reviewers: Ivan Inkov (BG) 1 Department of Pharmacy Practice,college of Pharmacy,Sri Ramakrishna Hospital Campus,SRIPMS,Coimbatore.

Email: visitram@yahoo.com

Morbidity and mortality due to ADR are becoming a challenge to the healthcare system. Approximately 25% of ADRs have been reported in inpatients admitted to the hospital. This could be attributed to a multitude of factors like polypharmacy, drug interactions, lack of awareness, easy accessibility of drugs and increased co-morbid disease conditions. The unexpected ADRs for the new drugs are yet to be well documented; hence the ADR monitoring system will be beneficial for the treating physician.

Some adverse drug reactions have been identified after use by a large number of people in the phase IV clinical trial, so the documentation of ADR is more emphasised. In India, ADR monitoring system is still primitive due to lack of awareness and interest in reporting by the healthcare professionals. A voluntary reporting system could do active ADR monitoring in a hospital set-up. Pharmacovigilance plays an essential role in providing information about adverse drug reactions and drug safety in a hospital. The safety of drug prescribing has become a highly

Sriram Shanmugam et al./ International Journal of Medical Reviews and Case Reports (2017) 1(3):49-52

visible topic in medicine, due in part to research suggesting that there are essential ADRs caused by commonly used medications.Paediatric patients constitute a vulnerable group about rational drug prescribing since many new drugs are released onto the market without the benefit of even limited experience in this age group. This deficiency causes paediatricians to prescribe children drugs in an ’off-label’ manner often, thereby increasing the risk of drug toxicity. Adequate controlled clinical trials in children lack, mainly because of issues of cost and responsibility, and to regulations that frequently act as significant obstacles.

Moreover, until recently, the few clinical trials that had been performed involving children focused on the efficacy of drugs and rarely monitored their safety. Chorea (Latin for “dance”) is a hyperkinetic movement disorder usually due to basal ganglia injury or dysfunction. Movements are brief, irregular, unpredictable, and flow from one body part to another in a random fashion. Occasionally, they may be incorporated into a more purposeful movement to avoid social embarrassment. Chorea can occur in isolation, but usually appears in conjunction with slow, writhing, distal movements called athetosis (i.e., choreoathetosis).athetosis is considered to be a hyperkinetic movement disorder characterised by involuntary writhing movements of the distal extremities and perioral muscles. Most athetosis is secondary to lesions in the basal ganglia, whether from cerebral palsy, ischemia, or trauma. In cerebral palsy, athetosis commonly coexists with chorea and is called choreoathetosis. Meta-analysis is already a well-established methodological approach for evaluating the effectiveness of therapies. However, in contrast to the published experience of using meta-analysis to assess drug efficacy, the use of this method to also quantify the risk of treatments remains limited to date. A recently published meta-analysis on the incidence of ADRs in hospitalised patients shows that ADRs represent a significant public health issue, making these reactions between the fourth and sixth leading cause of death in the USA, even when the drugs are used in proper doses and for approved indications.

Although paediatric pharmacotherapy has recently come to the fore, so far no meta-analytical review has been performed to assess the risk of drugs in the paediatric population. Recently published drug surveillance studies allow an estimation of the overall incidence of ADRs in different child health care settings.

In this study, we systematically conduct a meta-analysis on drug-induced choreoathetosis in the paediatric population and provide a summary quantitative estimate of their occurrence.

Material and Method INCLUSION CRITERIA

• Age(neonates-14years), • Seizure disorder, • Combination of ant-epileptic drugs.

EXCLUSION CRITERIA

• Comorbid disease, • Pseudoseizure, • Age above 14 years.

LITERATURE RESEARCH

A sound meta-analysis is characterised by a thorough and disciplined literature search. A clear definition of hypotheses investigated provides the framework for an investigation.Typically, published papers and abstracts are identified by

a computerised literature search of electronic databases like PubMed (www.ncbi.nlm.nih.gov./entrez/query.fcgi), ScienceDirect (www.sciencedirect.com), Scirus (www.scirus.com/srsapp ), ISI Web of Knowledge (http://www.isiwebofknowledge.com), Google Scholar (http://scholar.google.com).

Individual or Aggregated Data

A 3-year-old boy was admitted for treatment of recurrent minor motor seizures complicating a recent otitis media. At this admission, he was still having seizures on a combination of lamotrigine, clonazepam, felbamate and a ketogenic diet. He was started on phenytoin and discharged. Within a few weeks, he developed generalised choreoathetoid movements, which increased with activity and disappeared during sleep. On discontinuation of phenytoin, there was a significant improvement in his chorea. Chorea as a result of lamotrigine therapy was noted as a rare side effect in trials. Published reports describing new onset choreoathetosis in two young patients treated. With lamotrigine.

A study describes three patients with new-onset choreoathetosis that developed while receiving lamotrigine and phenytoin in combination therapy. The first patient developed transient chorea when her usual lamotrigine and carbamazepine, were supplemented with phenytoin, which was loaded intravenously shortly before chorea development. At the time chorea developed, her phenytoin levels were in the normal range (16 µ ml-1 ).The third patient experienced the onset of choreoathetosis on a combination of anticonvulsants which included phenytoin and lamotrigine, in addition to felbamate and topiramate. The abnormal movements started shortly after adding phenytoin and significantly improved when phenytoin was discontinued since this patient used phenytoin in monotherapy before this without any abnormal movements. Similar movement disorders developed in two 8-year-old mentally disabled children while they were receiving phenytoin. Seizures after a diphtheriapertussis-tetanus immunisation had improved in each child at 1 to 2 months of age. A static encephalopathy ensued, characterised by mental retardation, ataxia, spasticity, and a mixed seizure disorder. Intermittent dystonia and choreoathetosis developed insidiously while serum phenytoin concentrations were in the therapeutic range. Sustained dystonia and choreoathetosis developed 2 hours after an oral provocation with phenytoin. The baseline abnormalities on the electroencephalogram remained unchanged during the choreoathetosis. Recognizable metabolic abnormalities known to be associated with similar movement disorders were excluded.

A study describes three patients with severe myoclonic epilepsy in infancy (SME) who suffer from choreoathetosis due to the adverse effect of phenytoin. Choreoathetosis appeared when these patients were 8, 19, and 21 years old, two days to 6 months after increasing the phenytoin dosage. Choreoathetosis disappeared when the phenytoin dosage was decreased. The two elder patients experienced the episodic and rather paroxysmal onset of long-lasting choreoathetosis, requiring the differential diagnosis of degenerative disease. In one of the patients, an ictal SPECT revealed decreased perfusion in the basal ganglia contralateral to the unilateral choreoathetosis. Polypharmacy, including carbamazepine and zonisamide, may have facilitated the onset of choreoathetosis. Phenytoin-induced choreoathetosis in the patients with SME is a vital differential diagnosis of degenerative disorders involving involuntary movements. The episodic and paroxysmal nature of this movement disorder can

Sriram Shanmugam et al./ International Journal of Medical Reviews and Case Reports (2017) 1(3):49-52

delay its diagnosis and effective treatment.

GABAergic mechanism.

Paroxysmal kinesigenic dyskinesia Paroxysmal kinesigenic dyskinesia (PKD) described three cases of brief dyskinetic episodes induced by sudden voluntary movements. Further descriptions followed and named the clinical entity paroxysmal kinesigenicchoreoathetosis (PKC). As a subtype of the primary dystonias, it is also called DYT10. PKC is a rare neurologic condition, and most cases are sporadic. Only 27% have a family history with an autosomal dominant inheritance. The attacks last seconds to minutes and can start between 1 and 40 years of age. Up to 100 attacks per day of dystonic posturing, choreoathetosis, and ballism can occur. About 42% of patients have additional afebrile seizures in childhood. Infantile convulsions and paroxysmal choreoathetosis- age onset less than one-year Childhood â&#x20AC;&#x201C;chromosome 16p12-q12. The rare adverse event of antiepileptic therapies includes choreoathetosis.polytherapy with antiepileptic drugs induces choreoathetosis.The commonly involved antiepileptic drugs include phenytoin, Phenobarbital, valproic acid, carbamazepine and also benzodiazepines.

Non-psychotic drugs that produce choreoathetosis arePhenobarbital, carbamazepine, benzodiazepines, valproic acid and phenytoin.A study report three patients who developed choreoathetoid movements on anticonvulsants. All those patients were using phenytoin and lamotrigine in combination as part of their anticonvulsant regimen when they developed chorea. No patient had these movements while on either phenytoin or lamotrigine monotherapy at normal, or toxic, concentrations.Drugs are causing chorea: There are many drugs which may cause choreoathetosis. Drug-induced chorea may be seen during the acute phase of treatment or may appear after some time.

Antiparkinsonian and antiepileptic drugs are most important causes of chorea. Levodopa-induced chorea is the most common cause of chorea in adults. Treatment includes withdrawal of the offending drug. It may take days to months before patiently is free from symptoms In this patient, it is also reasonable to conclude that neither lamotrigine nor phenytoin alone produced chorea, but the combination did. The third patient experienced the onset of choreoathetosis on a combination of anticonvulsants which included phenytoin and lamotrigine, in addition to felbamate and topiramate. The abnormal movements started shortly after adding phenytoin and significantly improved when phenytoin was discontinued since this patient used phenytoin in monotherapy before this without any unusual movements . Chorea is most likely due to the combination therapy. This patient has similar risk factors to other children described with drug-induced choreoathetosis. The mechanism by which these patients developed chorea is unknown. Pharmacokinetic interaction between anticonvulsants causing elevated high phenytoin levels is unlikely in our patients as lamotrigine is known not to elevate phenytoin levels.

The most consistent biochemical lesion in patients with Huntington chorea appears to be a loss of neurons in the basal ganglia that synthesise and contain GABA. The significance of this remains unknown. A variety of pharmacologic techniques have been attempted to increase CNS GABA levels. Valproic acid, which acts in part via a GABAergic mechanism, has, in a limited number of uncontrolled cases, ameliorated not only the agitation sometimes seen in persons with HD but also the movement problem. [17] However, no systematic studies have been conducted on the use of GABAergic agents to treat HD.

Discussion Anticonvulsant-induced choreoathetosis was first reported in 1962 with phenytoin. A literature search reveals about 80 cases.

Reported patients are frequently young and have organic brain abnormalities including mental retardation. More than one-half of the cases have occurred in association with toxic drug levels.

The use of phenytoin with other medications was reported to increase the risk of developing abnormal movements.

Pathophysiology

A simple model of basal ganglia function states that dopaminergic and GABAergic impulses from the substantia nigra and motor cortex, respectively, are funnelled through the pallidum into the motor thalamus and motor cortex. These impulses are modulated in the striatum via two segregated, parallel, direct and indirect loops through the medial pallidum and lateral pallidum/subthalamic nucleus. Subthalamic nucleus activity drives the medial pallidum to inhibit cortex-mediated impulses, thereby inducing parkinsonism. Absent subthalamic nucleus inhibition enhances motor activity through the motor thalamus, resulting in abnormal involuntary movements such as dystonia, chorea, and tics. VPA-induced chorea seems to require both high or toxic VPA serum concentrations and pre-existing brain injury. This case report highlights the importance of an accurate pharmacological history when approaching a patient with subacute onset of movement disorders because of the potential for recovery upon drug discontinuation.Moreover, the VPA, singly or in combination with phenytoin or carbamazepine should be used with caution in those with pre-existing basal ganglia injury.Pharmacokinetic interaction leading to increased free phenytoin level was suggested as a plausible explanation.

Both generalised and focal movements have been described. Duration of dyskinesia has been variable but has often responded to discontinuation of the anticonvulsants. Chorea is most likely due to the combination therapy. This patient has similar risk factors to other children described with drug-induced chorea. In combination, the effects of lamotrigine and phenytoin appear to be additive or synergistic, resulting in sufficient enhancement of dopaminergic activity to provoke clinically apparent choreoathetosis, this is rather a pharmacodynamic interaction between both anticonvulsants.the movement disorder is secondary to phenytoin and can occur at therapeutic serum concentrations.

Phenytoin is a central anticholinergic agent and a central stimulant of serotonin and may induce movement disorders as a result of altering these neurotransmitters in the brain. The variable expression of these movement disorders may relate to the nature of the preexisting striatal insult. Combined lamotrigine and phenytoin effects on central dopaminergic pathways might explain why our patients developed chorea when both anticonvulsants were present in combination. Neither direct enhancement of dopaminergic activity induced by phenytoin or indirect enhancement by lamotrigine alone was sufficient to provoke abnormal movements. In combination, the effects of lamotrigine and phenytoin appear to be additive or synergistic, resulting in sufficient enhancement of dopaminergic activity to provoke clinically apparent choreoathetosis, this is rather a pharmacodynamic interaction between both anticonvulsants.

Removal of one of the medications was sufficient to ameliorate or eliminate the clinical symptoms. These reported cases sug-

Sriram Shanmugam et al./ International Journal of Medical Reviews and Case Reports (2017) 1(3):49-52

gest that patients treated with a combination of lamotrigine and phenytoin may have an increased risk of developing dyskinetic movement disorders.

7. Phenytoin-induced dystonia and choreoathetosis in two retarded epileptic children Elias G. Chalhub.M.D. Darryl C. DeVivo, The official journal of American Academy of Neurology May 1976 vol. 26, issue no. 5, pg no 494.

Conclusion

8. Adverse motor effects induced by antiepileptic drugs G. Zaccara et al., US National Library of MedicineNational Institutes of Health, September 2004; vol6: issue 3, pg no 153-68.

We conclude that drug-induced choreoathetosis may be seen during the acute phase of treatment or may appear after some time Chorea is a rare side effect of anticonvulsants.The combination of different antiepileptic drugs has resulted in drug-induced choreoathetosis.Polytherapy with certain anticonvulsants may predispose patients to drug-induced choreoathetosis.It is essential for clinicians to evaluate both AEDs’ effectiveness and safety on an individual basis before the selection of the appropriate monotherapy or adjunctive AED therapy.

Acknowledgement We are very much delighted to connote our vehement indebtedness to Thiru. R.Vijayakumhar, our Managing Trustee, Dr.P.Sukumaran, Dean, Sri Ramakrishna Hospital & Dr T.K. Ravi, Principal, College of Pharmacy, Sri Ramakrishna Institute of Paramedical Sciences and all the teaching staff of the Department of Pharmacy Practice, College of Pharmacy, SRIPMS for the support, guidance and encouragement throughout the study.

Authors’ Statements Competing Interests Written informed consent was obtained from the patient for publication of this case report and any accompanying images.

There were no financial support or relationships between the authors and any organization or professional bodies that could pose any conflict of interests.

References 1. Choreoathetosis as a side effect of gabapentin therapy in severely neurologically impaired patients.Chudnow RS, Dewey RB Jr, Lawson CR, Archives of Neurology,1997Jul;volume54, issue 7pg.no:910-2.

2. Movement disorders in patients taking anticonvulsants C Zadikoff et al.Journal of Neurology, Neurosurgery and Psychiatry,2007 Feb; 78(2): 147–151.

9. Chorea Sanjay Pandey Journal of the association of physicians of India, July 2013, volume 61, pg no 35-47.

10. Movement Disorders II: Chorea, Dystonia, Myoclonus, and Tremor Jonathan W. Mink, Samuel H. Zinner, US national library of medicine-national institute of health, Vol.31, issue no.7 July 2010, pg no 287-94.

11. Chorea and related disorders R Bhidayasiri, D DTruong, post graduate medical journal 2004 Sep;80(947):527-34.

12. Study of Chorea in Children Debabrata Ghosh MD; Stephen Sreshta; Kohilavani Velayudam. International Parkinson and Movement Disorder Society,8 July 2016, Volume 4, Issue 2, Pages 231–236.

13. Lotze T, Jankovic J. Paroxysmal kinesigenicdyskinesias.

Seminars in pediatric neurology 2003; 10: 68-79.

14. Drug-induced movement disorders Denes Zadori, et al. , US National library of medicine –national institute of health,pubmed.gov, 2015 Jun, volume 6, pg no 877-890.

15. Valproate-Induced Generalized Choreoathetosis Alfonso Giordano, Marianna Amboni, and Alessandro Tessitore, The International Parkinson and Movement Disorder Society,28 JUL 2014, Volume-1-Issue-3.

16. Phenytoin-induced chorea in a pediatric patient: An interaction between phenytoin, phenobarbital and clobazam Manish Barvaliya et al., Indian Journal of Pharmacology, Vol. 43, No. 6, November-December, 2011, pp. 731-732.

17. Evidence for Efficacy of Combination of Antiepileptic Drugs in Treatment of Epilepsy Ehsan M. Sarhan, Matthew C. Walker, Caroline Selai, journal of neurology research, Volume 5, Number 6, December 2015, pages 267-276.

3. Reversible valproate-induced choreiform movements Dilek Ince Gunal1, MelihaGuleryuz, CananAykutBingol, Journal of Neurology, April 2002, Pages 205–206, VOLUME 11, ISSUE 3.

4. Valproate-Induced Chorea and Encephalopathy in Atypical Nonketotic Hyperglycinemia Peter F. Morrison, Raman Sankar, W. Donald Shields, Paediatric Journal of Neurology, Volume 35, Issue 5, November 2006, Pages 356–358.

5. Anticonvulsants-induced chorea: a role for pharmacodynamic drug interaction? MegdadZaatrehet al., US National Library of MedicineNational Institutes of Health, Volume 10, Issue 8, December 2001, Pages 596–599.

6. Transient chorea induced by phenytoin F. Filloux, J.A. Thompson, Journal of Paediatrics, Volume 110, Issue 4, April 1987, Pages 639-641.

Sriram Shanmugam et al./ International Journal of Medical Reviews and Case Reports (2017) 1(3):49-52

CASE REPORT

CASE REPORT OF DENGUE ENCEPHALOPATHY IN PREGNANCY MIMICKING ATYPICAL ECLAMPSIA – AN INSIGHT OF NEUROLOGICAL MANIFESTATIONS OF DENGUE IN PREGNANCY Sasirekha Rengaraj∗,1 and Suthrasika Thiyagalingam∗ ∗ Department

of Obstetrics & Gynaecology, JIPMER, Puducherry, India.

ABSTRACT Dengue is a global public health problem. Dengue in pregnancy carries a high maternal and perinatal morbidity and even mortality especially when the diagnosis is delayed. Often dengue with hemorrhagic shock syndrome is misinterpreted as HELLP syndrome and unnecessary obstetric interventions have to be avoided in such situations. There are case reports and guidelines on dengue hemorrhagic syndrome in pregnancy. However, rare atypical forms like neurological manifestations of dengue in pregnancy are rarely studied. Neurological events of dengue are increasingly observed nowadays. We are presenting a case report of atypical dengue (dengue encephalopathy) in pregnancy with a review of the literature. It was initially thought as meningitis then atypical eclampsia and HELLP syndrome. Whenever dengue infection presents in atypical form, strong clinical suspicion and early intervention improve the outcome.

KEYWORDS Dengue, dengue encecphaolopathy, atypical HELLP

Introduction Dengue fever is a significant global health problem worldwide, and 2/5th of world’s population is at risk[1]. Dengue fever, an Aedes aegypti mosquito-borne infection has an expanded clinical spectrum of asymptomatic infection to fatal dengue hemorrhagic fever and shock syndrome.

The impact of dengue on pregnancy needs to be better understood primarily regarding the maternal and perinatal outcome. The complications of dengue in pregnancy have been scarcely studied. There are increased risks of maternal haemorCopyright © 2019 by the Bulgarian Association of Young Surgeons DOI: 10.5455/IJMRCR.dengue-encephalopathy-in-pregnancy-mimicking-atypicaleclampsia First Received: May 20, 2017 Accepted: August 31, 2017 Manuscript Associate Editor: George Baytchev (BG) Editor-in Chief: Cvetanka Hristova (BG) Reviewers: Ivan Inkov (BG) 1 Associate Professor, Department of Obstetrics & Gynaecology, JIPMER, Puducherry,605006. sasirekha0226@gmail.com

rhage, preterm labour and stillbirths. Apart from that, Dengue infection as such in pregnancy can be a significant diagnostic challenge. This is very true in atypical presentations like dengue encephalitis /encephalopathy. Pregnancy-specific conditions like preeclampsia, HELLP syndrome can mimic such atypical presentations which can delay or misguide the clinician which often leads to poor maternal and perinatal outcome.

However, atypical forms such as neurological manifestations are not uncommon even in pregnancy. It was first reported in 1976, and worldwide literature search shows the various incidence of dengue encephalopathy from 0.5% to 6.2% with a mortality rate up to 40% [2]. Neurological manifestations of dengue are poorly studied especially in pregnancy.

Case Report Twenty-six years Mrs A, G2P1L1 monoamniotic twin pregnancy presented at 36+2 weeks to the emergency department with history of leaking P/V. It was spontaneous conception; she was booked at a private hospital. Her antenatal period was uneventful. There was no history of preeclampsia or anaemia. She was taken up for emer-

Sasirekha Rengaraj et al./ International Journal of Medical Reviews and Case Reports (2017) 1(3):53-55

gency LSCS given monoamniotic twin. Her previous Obstetric history was routine. There was no significant past and family history.

Emergency LSCS was done under SA. She one recording of 150/100 intraoperative, settled on its own. There were no other intraoperative complications. Both female babies weighed 2.2 & 2.1kg, and they were by M/S. She did not receive any antihypertensive drugs.

Within 6 hours of surgery, her dressing was soaked which required exploration of subcutaneous bleed under anaesthesia.

There was a brisk bleeder from the subcutaneous plane, and the same was ligated. She had one high spiking fever on a postoperative day 1. No clinical signs of puerperal sepsis. Her breasts were soft. Fundamental investigations were sent, and urine culture was sterile. Two days later she developed diffuse abdominal pain associated with vomiting which was non-bilious and non-projectile. There was no diarrhoea. Her Vitals were stable except for minimal dehydration.

The abdominal examination did not reveal any abnormality. It was soft, and there was minimal gaseous distension. On a postoperative day four, she started having signs of cerebral irritation with up drawing plantar. Her pupils were equal and reactive. Initial neuroimaging (CT) did not reveal any abnormality.

She was transferred to ICU and received supportive measures.

Her Bp was 110/60mmHg. The fluid imbalance was corrected.

With the correction of intra volume depletion, she developed altered mental status with signs of decerebrate rigidity. Hence further imaging was undertaken to rule out any cerebrovascular accidents but showed only diffuse cerebral oedema; received fluid therapy under close monitoring. Platelets and packed cell transfusion were given. Cerebral symptoms showed improvement with anti-cerebral oedema measures. Two days later her dengue serology report came and showed a positive result for NSN1 antigen and IgM Ab. Recovered well, had secondary suturing for wound infection. The patient was discharged from the hospital on Post op day 14 without any sequelae.

Discussion Dengue fever, which is caused by four similar but antigenically different serotypes of flavivirus has 50% mortality rate, especially if untreated [3]. There are 50-100 million new cases added every year. Even though India is an endemic area for recent dengue statistics have shown a case fatality ratio of <0.5% especially after the implication of national guidelines on clinical management of dengue fever. Even in India some areas has a case fatality rates between 3-5%.

Dengue fever in pregnancy can be confused with HELLP syndrome, SLE, thrombotic thrombocytopenic purpura (TTP), sepsis and DIC especially when it presents in atypical form [4].

Following viral infection, there is lifelong protection against similar subtype. However, subsequent infection with different subtype can be dangerous because it increases the chance of dengue hemorrhagic fever. The heterotypic antibodies form complexes, which attacks mononuclear phagocytes with enhanced efficiency. This antibody-dependent enhancement results in enhanced viral load replication which results in the severe form of dengue fever like dengue hemorrhagic fever and dengue shock syndrome. Similarly, it may also contribute to the development of atypical forms of dengue called dengue encephalopathy.

These neurological manifestations are associated explicitly with DENV-2 & 3. These serotypes were found in encephalitis, meningitis and myelitis. Even Den-4 was also detected in

Figure 1: Timeline for case report. brain cells by IHC in CSF of a patient with encephalitis. It could be due to direct viral infection of CNS or autoimmune reaction following dengue infection or due to metabolic/underlying hemorrhagic complication.

Atypical manifestations of dengue fever are associated with more severe grades of disease. Various case series have shown the importance of early and prompt proactive management observed nowadays and carried a high mortality. Few case series have been reported which shows different presentations like encephalopathy, encephalitis, neuropathy, and optic neuritis and Guillain Barre syndrome [5].

Even though rare, they have been increasingly reported in dengue epidemic regions. Classically it can occur in patients with few or no signs of previous dengue infection like this patient. Moreover, it can occur within 2-30 days after the onset of fever. There can be myelitis, myositis, radiculoneuritis and neuropathy. A headache, seizures and altered consciousness are the usual manifestations of dengue encephalitis [6]. However, these typical symptoms are seen in only less than 50% of patients with dengue encephalitis. Whenever patient presents with encephalitis, it should be one of the differential diagnosis in patients from dengue-endemic areas Diagnostic criteria for dengue encephalitis: • Fever • Acute signs of cerebral involvement • Presence of anti-dengue IgM/ dengue genomic material in serum/CSF MRI/CT findings may vary from cerebral oedema, haemorrhages to focal abnormalities in basal ganglia, hippocampus and thalamus.

It takes time to make an exact diagnosis of atypical manifestations of dengue fever like in our patient. It was initially thought of postoperative ileus, warranted surgery opinion later meningitis (complication following spinal anaesthesia) and then HELLP

Sasirekha Rengaraj et al./ International Journal of Medical Reviews and Case Reports (2017) 1(3):53-55

syndrome. The most crucial presentation was encephalopathy like features which include a headache, seizure, altered sensorium and behavioural disturbances. Various pathogenesis has been described. It could be due to cerebral oedema or cerebral haemorrhage. Even microcapillary haemorrhage and cerebral hypoxia play a role. Electrolyte disturbances like hyponatremia, hepatic failure or release of toxic products might be one of the reasons for neurological manifestations.

There is no convincing evidence for a demonstration of direct invasion to the central nervous system (CNS) however recent evidence shows dengue virus is capable of direct invasion onto CNS. The entry of the virus into brain seems to occur through infiltrates of infected macrophages7. Few animal studies have shown the cytokines which are released in dengue plays a role in creating a breach in BBB which result in CNS invasion. The neurological manifestations can vary from altered consciousness (most common), seizure, mental confusion; limb spasticity to focal neurological deficits (least familiar).The IgM antibody was even isolated from CSF. Usually, it is self-resolving. Supportive treatment is important. There is no specific treatment as such.

Fluid support and intense monitoring play a significant role in the outcome. Involvement of other systems such as hepatic failure, respiratory distress, hyperkalemia, acute kidney injury and metabolic acidosis may occur if untreated which increases the morbidity rate. Secondary infections like ventilator-associated pneumonia (VAP) may complicate the situation.

Even though misdiagnosis happened in our case, early intensive monitoring and fluid therapy along with ante cerebral oedema measures improved the outcome.

3. Rajagopala L, Satharasinghe RL, Karunarathna M BMC Res Notes. 2017; 10: 79. Published online 2017 Feb 2. doi: 10.1186/s13104-017-2391.

4. Lavanya R, Ravindra L. Satharasinghe, and Madhava Karunarathna A rare case of dengue encephalopathy complicating a term pregnancy J Community Med Health Educ DOI: 10.4172/2161-0711.C1.019.

5. Carod-Artal FJ, Wichmann O, Farrar J, Gascon J. Neurological complications of dengue virus infection. Lancet Neurol.

2013;12:906–19.

6. Solomon T, Dung NM, Vaughn DW, et al.

Neurological manifestations of dengue infection. Lancet.

2000;355(9209):1053–9.

7. Puccioni-Sohler M, Rosadas C, Cabral-Castro MJ. Neurological complications in dengue infection: a review for clinical practice. Arq Neuropsiquiatr. 2013;71 (9B):667–71.

Conclusion Dengue in pregnancy is a real threat to both obstetrician and patient. The classical presentation of dengue is unlikely to be missed. However, it is a significant diagnostic challenge when it presents in atypical form, especially during pregnancy. It can be easily misinterpreted with many other clinical conditions of pregnancy. Dengue should be the differential diagnosis even a pregnant women presents with fever and altered sensorium especially in endemic areas like India. A high index of clinical suspicion and prompt intervention in such situations improves the maternal and fetal outcome. Further studies are needed to evaluate dengue neurotropism in pregnancy.

Disclosure Statement There were no financial support or relationships between the authors and any organization or professional bodies that could pose any conflict of interests.

Competing Interests Written informed consent obtained from the patient for publication of this case report and any accompanying images.

References 1. Gupta E, Ballani N. Current perspectives on the spread of dengue in India. Infect Drug Resist 2014 ;7:337-42.

2. WHO. Dengue and Dengue haemorrhagic fever. Factsheet No. 117. Geneva: World health organization. 2008.

Sasirekha Rengaraj et al./ International Journal of Medical Reviews and Case Reports (2017) 1(3):53-55