Pediatric Notes: Spring 2017

H A S B R O

C H I L D R E N ’ S

H O S P I TA L

Pediatric N O T E S SPRING 2017 Dear Colleagues,

in this issue...

• Acute Appendicitis • New Cleft Palate Repair Improves Speech Outcomes • Pediatric Clinical Decision Unit • Pediatric News - No Hit Zone • Announcements

We hope you will find this issue of Pediatric Notes both helpful and informative. We thank you again for entrusting your patients to our care and hope that you find our collaborative approach to medical care a benefit to your patients.

Phyllis Dennery, MD Pediatrician-in-Chief

Francois Luks, MD Interim Pediatric Surgeon-in-Chief

Acute Appendicitis Francois Luks, MD, Interim Pediatric Surgeon-in-Chief and Christopher Muratore, MD

Acute appendicitis is the most common surgical emergency in the pediatric population. In the US over 300,000 appendectomies are performed each year, over 70,000 operations in patients younger than 18 years. Operative appendectomy has been the gold standard treatment for acute appendicitis for over 100 years. At present, the procedure is performed with low morbidity and virtually zero mortality throughout the world. Surgical therapy has transformed a previously potentially fatal condition to a commonplace illness with a mortality rate close to zero.

Most recent reports suggest that the morbidity rates in children are approximately 2.7% in non-perforated (non-ruptured) and 16% percent in perforated appendicitis. Current trends in modern medicine and surgery include a push to drive down even single digit morbidity percentages and to maximize cost effectiveness in medical care.

This has caused a new analysis of the treatment of many medical and surgical diseases. Non-operative treatment of early uncomplicated appendicitis could, if successful, help reduce healthcare costs and avoid many emergency surgical interventions.

The traditional emergency surgical management of appendicitis has been challenged. In a 2012 survey of members in the American Pediatric Surgical Association (APSA) only 4% of 484 physicians polled considered non-perforated appendicis to be an emergent procedure, most indicated that “urgent surgery within a day” was a reasonable approach to treatment.

Supporting this paradigm shift are several studies that demonstrated no increase in morbidity and mortality of appendicitis, and no increase in perforation, when appendectomy was postponed overnight. This shift away from emergent surgical nature of appendicitis has enabled the consideration of alternative management strategies for the treatment of acute non-perforated appendicitis.

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HASBRO CHILDREN’S HOSPITAL Pediatric Notes

New Cleft Palate Repair Improves Speech Outcomes Albert S. Woo, MD, Chief of Pediatric Plastic Surgery

Cleft lip and palate deformities are the most common congenital anomalies affecting the head and neck, accounting for approximately 1 in 690 live births in the United States. Even after cleft palate repair, about 20% of children continue to have abnormal speech characterized by hypernasality and nasal emission, called velopharyngeal insufficiency (VPI). This problem is primarily thought to be associated with the fact that the levator veli palatini muscles of the palate are in the wrong position. Even after surgical correction, however, the speech results have been less than ideal.

In such situations, additional surgery is frequently offered. Traditional options have included obstructive, non-anatomic surgeries such as a pharyngeal flap or sphincter pharyngoplasty. While speech results have generally been good from these traditional procedures, they also subject young patients to significant risks of obstructive sleep apnea. Surgeons have therefore focused on revising cleft palate repairs rather than on performing these other procedures, to minimize the risk of sleep apnea and other complications while also improving speech.

Formerly Chief of Pediatric Plastic Surgery at St. Louis Children’s Hospital and Washington University in St. Louis, I was hired at Hasbro Children’s Hospital in 2016 to head the cleft team. In 2009, I had developed a new cleft palate repair designed to improve speech outcomes in both primary and secondary operations. The results have been nothing short of surprising.

Improvement in Speech for Children who had Undergone Initial Cleft Palate Repair The Woo Palatoplasty was initially offered as a secondary procedure for children who still had abnormal speech despite having already

undergone an initial cleft palate repair in infancy. These results were compared against the Furlow double-opposing Z-plasty technique, which is the most common palatal redo operation performed in the United States.

In non-syndromic patients, the Woo Palatoplasty achieved 95% success compared to 63% success with Furlow. When the results were refined further to examine only patients considered ideal for the Furlow technique, the Woo Palatoplasty had a 100% success rate compared to 67% with the other procedure.

These results were published in Plastic & Reconstructive Surgery in October 2014.

Better Results with Initial Cleft Palate Repair

Spurred by our success, this operation was offered to infants undergoing cleft palate repair for the first time, typically performed around 1 year of age. Despite the common finding of roughly 20% of children suffering from VPI with traditional operations, since its initial offering in 2009, none of the non-syndromic patients who have had this procedure have needed additional surgery.

These dramatic results are thought to be due to the Woo Palatoplasty’s focus on overlapping the palatal musculature, which are thought to be overly long in cleft palate patients. The findings were also published in Plastic & Reconstructive Surgery in July 2015. The benefits of this surgical technique were also supported independently by computer models of cleft palate repair developed at the University of Virginia.

Decreased Need for Speech Therapy

In general, approximately 75% of cleft palate patients traditionally require some degree of speech therapy, as these children are taught how to use their repaired palates to produce

HASBRO CHILDREN’S HOSPITAL Pediatric Notes normal speech. A recent study has demonstrated that those who had undergone Woo Palatoplasty had a 57% decrease in cleft-related indications for speech therapy. These findings were recently published in the Annals of Plastic Surgery in March 2017 and described in a CME on cleft palate repair in the January 2017 issue of Plastic & Reconstructive Surgery.

Improved Speech for Cleft Patients

The philosophy of the multidisciplinary cleft team at Hasbro Children’s Hospital is that all children born with a cleft should

be able to achieve normal speech, without having to struggle with hypernasality. Our goals are to minimize the number of surgical procedures in our patients while also optimizing their final outcomes – not just in speech, but in appearance, growth, function, and development. It is our hope that we can provide a patient-centered, holistic perspective in the care of those born with clefts, as we follow them into adult, or even beyond. This new procedure provides an effective tool in our armamentarium to help us achieve these ends.

Pediatric Clinical Decision Unit Laura Chapman, MD, Associate Medical Director for Pediatric Emergency Medicine

Hasbro Children’s Hospital has opened a new “short-stay” inpatient unit for pediatric patients. The Pediatric Clinical Decision Unit (CDU) began admitting patients on November 1, 2016. The CDU is a 6-bed unit designed for children with acute conditions that require brief inpatient observation level admissions. Typical admission diagnoses include asthma, bronchiolitis, dehydration, cellulitis, abdominal pain and acute allergic reactions. This unit in staffed by pediatric emergency medicine physicians, advance practice practitioners, and pediatric emergency room nurses.

Goals of the CDU:

• Provide high quality care for patients who need brief admissions

• Create additional admission beds at Hasbro Children’s Hospital

• Decrease wait time and improve patient flow in the emergency department • Streamline the admission and discharge process for patients requiring short admissions

Patients admitted to the CDU must meet certain criteria in terms of diagnosis, acuity level, age and anticipated

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length of stay. In general, children admitted to the CDU have straight-forward diagnoses and are expected to meet discharge criteria within 48 hours. All patients who are admitted to the CDU initially present to Hasbro Children’s Hospital via the emergency department; there are no direct admissions or transfers accepted to the CDU. On occasion, a child may be admitted to the CDU and then develop the need for a longer admission. These children are then simply admitted to the general inpatient medical or surgical services. Subspecialty services provide consultation on CDU patients as needed.

The physical design of the CDU is state-of-the-art, providing families with Tempur-Pedic® beds, smart televisions, dimmable task lighting, USB outlets for phone charging, and a shower room. There is also a family room that that is shared with the pediatric sedation service, where family members can find a quiet space to relax, enjoy some coffee and other nourishment, and use the public-access computers and television.

The response from families who have stayed in the CDU thus far has been overwhelmingly positive. We are pleased that children and their families now have another option when it comes to needing an admission at Hasbro Children’s.

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HASBRO CHILDREN’S HOSPITAL Pediatric Notes

Acute Appendicitis continutued from cover

Initial non-operative treatment of perforated appendicitis has been well reported in children and has demonstrated equivalence in efficacy (if not always in cost). Non-surgical treatment of early, uncomplicated appendicitis, however, has not been considered until very recently.

Within the last 24 months, 6 small studies have attempted to emulate the adult experience with antibiotic only treatment of acute appendicitis. Initial success ranged from 75 to 90% but long term data is lacking. In addition, these studies largely fail to find a statistically significant difference in overall cost when compared with operative management. This is likely owing to combination to study design, variable alteration of hospitalization and prolonged course of intervenous antibiotics.

In a small randomized study in children at Hasbro Children’s Hospital (HCH), we demonstrated that non-operative treatment is 88% successful in eliminating the signs and symptoms of early appendicitis in appropriately selected patients with confirmed acute appendicitis (approximately 20% of patients with appendicitis). Patients were hospitalized for less than a day and could return to their regular routine within days. The experience was rated more positively by patients and parents than that of similar contemporary control patient who underwent either laparoscopic or open appendectomy. When successful the overall cost of non-operative treatment was less than half that of urgent appendectomy ($1,365 versus $4,130).

Initial failure of non-operative treatment was 12% similar to that of other pediatric and adult surgeries.

A legitimate concern with non-operative studies is whether the correct diagnosis is being treated. The false positive rate of appendicitis has fallen dramatically in the last few decades. No longer is a 10 to 15% normal appendectomy rate acceptable as the risk of missed appendicitis is not the potentially lethal complication it was a century ago. Today, the false positive rates of 4 to 5% in children are accepted as the norm. In a recent study, we demonstrated that the negative appendectomy rate could be reduced below 2% using a combination of clinical exam, imaging and leucocytosis. Thus the current management of appendicitis in children at HCH allows the diagnosis of early disease with near 100% accuracy.

Nevertheless, any surgical intervention is associated with pain, discomfort and the risk of complications. It affects the overall health related quality of life and because of the high prevalence of appendicitis in children represents a significant healthcare burden. With the creation of incentives like the Patient Centered Outcome Research Institute, (PCORI) in 2010, an intentional shift is being made towards greater patient input in complex medical decisions.

In our current multidisciplinary RCT we aim to compare urgent appendectomy and non-operative management of early appendicitis in children. The overarching goal is to determine whether children with early appendicitis can be offered a trial of non-operative management. The proposed study capitalizes on the expertise and infrastructure of the National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK), in close cooperation with the Pediatric Emergency Care Applied Research Network (PECARN), and the geographic and patient diversity of its member institutions. The unique partnership between NIDDK, PECARN (the first federally funded multicenter Pediatric Emergency Medicine network of its kind) and academic pediatric surgeons ensures acceptance of the protocol at all levels of pediatric care and wide applicability of its results. Given its prevalence, there is the potential to radically reduce the burden of appendicitis, not only on patients and their families, but on society and health care systems as well.

References:

Chau DB, et al, Patient-centered outcomes research in appendicitis in children: Bridging the knowledge gap, J Pediatr Surg (2015), http://dx.doi.org/10.1016/j.jpedsurg.2015.10.029

Hartwich J, et al, Nonoperative treatment of acute appendicitis in children: A feasibility study, J Pediatr Surg (2015), http://dx.doi.org/10/1016/j.jpedsurg.2015.10.024

HASBRO CHILDREN’S HOSPITAL Pediatric Notes

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No Hit Zone Brett Slingsby, MD

The Lawrence A. Aubin Sr. Child Protection Center plans to establish a No Hit Zone policy at Hasbro Children’s Hospital, which has been successfully implemented in multiple hospitals and clinics across the country. A No Hit Zone is a policy in which no adult shall hit another adult, no adult shall hit a child, no child shall hit an adult and no child shall hit another child within the hospital setting. The goal is to assist in lowering the frequency of disruptive behaviors in hospital and clinic settings and elevate standard of care by reinforcing an environment of comfort and safety for patients, families, and staff working in our facility. Further, we hope to set an example to the community that violence and hitting are unacceptable forms of punishment, and provide awareness, education and training tools to health care professionals and families on how to deescalate disruptive and potentially harmful behaviors.

The American Academy of Pediatrics and the American Academy of Child and Adolescent Psychology both recommended against corporal punishment when disciplining a child. Corporal punishment has been shown to be less effective than other disciplinary measures and teaches children that hitting is the way to solve problems. Literature states that spanking and/or hitting increases aggression and anger instead of teaching responsibility, and encourages that acting in a physical

manner is an appropriate way to deal with anger. The Centers for Disease Control and Prevention’s plan for reducing child maltreatment focuses on the safe, stable, nurturing relationships between caregivers and children, through the establishment of positive reinforcement techniques and behavioral interventions.

Implementing the No Hit Zone in Rhode Island will provide all hospital staff with policies, education, and materials on corporal punishment in the medical setting and how to efficiently intervene when these behaviors are observed. Further, our goal is to provide education to parents to learn alternative, age-appropriate forms of discipline. We will create posters and advertisements that will be distributed throughout the hospital to inform patients and families that hitting is not allowed. Furthermore, training to staff about what to do when hitting or threatening behaviors are observed will be provided. Through this initiative, we are hoping to make Hasbro safer for patients, families and staff and, ultimately, establish community awareness that corporal punishment is ineffective and harmful.

Brett Slingsby, M.D., is a pediatrician with Hasbro Children’s Hospital, specializing in child maltreatment at its Lawrence A. Aubin, Sr. Child Protection Center.

Announcements Lee Polikoff, MD, is an Attending in the Pediatric Intensive Care Unit at Hasbro Children’s Hospital. In addition to his clinical responsibilities, he is an active clinical researcher, serving as both principal investigator for research conducted at this institution, and site investigator in several multi-center research projects in collaboration with the Pediatric Acute Lung Injury and Sepsis Investigators (PALISI) consortium. His research projects include The National Emergency Airway Registry for Children Project (known as “NEAR4KIDS”), a national collaborative to improve outcomes during endotracheal intubation of critically ill pediatric patients, The Prevalence of Deep Venous Thrombosis in Critically Ill Adolescents, a study undertaken to elucidate risk factors associated with Deep Venous Thrombosis in critically ill adolescent patients; The Sepsis-Induced Red Cell Dysfunction (SIRD) study, a multicenter study which seeks to characterize morphologic changes and functional alterations in Red Blood Cells in pediatric patients with Septic Shock; the Fluid Overload Assessment and Titration Trial (FLOAT), a pilot study to test

the efficacy and feasibility of a fluid management protocol in patients with Pediatric Acute Respiratory Distress Syndrome; and the Nutrition Practice and Outcomes for Children on Non-Invasive Ventilation trial (NPO-NIV) study, assessing feeding algorithms and outcomes for pediatric patient on non-invasive ventilation.

Dr. Polikoff recently returned from the 46th Society of Critical Care (SCCM) Annual Congress, where along with his collaborators, he was awarded the Bronze Research Snapchat Award for their presentation entitled “Impact of Noninvasive Ventilation Use on Safety of Tracheal Intubation: A Multicenter Study.” This research was undertaken with seven other collaborators from six other institutions. Moreover, Dr. Polikoff gave two other oral presentations; the first entitled “Focused Genetic Testing for Rare Genetic Diseases Causing Pediatric Status Epilepticus,” a study undertaken here at Hasbro Children’s Hospital with collaboration among Pediatric Critical Care, Pediatric Pain and Palliative Care, and Pediatric Genetics. Dr. Polikoff further presented preliminary data from The Prevalence of Deep Venous Thrombosis in Critically Ill Adolescents Study.

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