What is Spina Bifida and Hydrocephalus? by Lieven Bauwens – Secretary General Sofia, Bulgaria 24 November 2011
What I would like to cover
What is IF? What is Spina Bifida? What is Hydrocephalus? Treatment
What is IF?
International Federation for Spina Bifida and Hydrocephalus
Domains
Global umbrella organisation 44 national / regional members (organisations of people with SB/H or their parents) / 41 countries Human Rights Prevention International Solidarity Network Development
“HQ” in Brussels, small office in Kampala
What is IF?
IF represents people with Spina Bifida and Hydrocephalus
One of 7 “Key EU networks” of people with disabilities for the European Commission Consultative status at the UN (ECOSOC) Participatory status at the Council of Europe Actively seeking partnerships with FFI, WHO, CDC, Unicef, OHCHR and others For Africa: Fortification-project (training, advocacy) with Akzo Nobel and FFI – www.smarterfutures.net
www.ifglobal.org
What is IF?
An global network of dedicated people, of knowledge
Parents, professionals and adults with the impairments, national and regional organizations Annual conferences
WWW: website, monthly newsflashes Workshops
2011: Guatemala 2012: Sweden 2013: Turkey, Vietnam
2010: Surgery and parent groups (Dar Es Salaam); Standards Harmonization workshop (Nairobi); Capacity Building (Dublin); Conference on Preconception Care and Preconception Health (Brussels) 2011: workshop on prevention (Amsterdam (with Eurordis) / Brussels), Transition (Leuven), QA/QC (Dar es Salaam), Multidisciplinary Care (Kampala), Seminar/roundtable (Kinshasa) 2012: workshop on QA/QC (Addis Abeba), Workshop and Conference (Stockholm)
Stimulating research
What is Spina Bifida?
Spina Bifida
(and related Hydrocephalus)
Anencephaly
Small defect, a lot of damage Dr Liptak: “the most complex congenital malformation compatible with life”
Nerves interrupted Paralysis below the lesion Incontinence for stool and urine Mobility problems Loss of sensation and risk of pressure wounds Hydrocephalus / secondary malformations (eg. Chiari)
Resulting in a lot of medical needs
BUT: life is more than the medical deficit
Concentrate on the abilities and not only at the disabilities Medical interventions should be limited to absolute minimum. Less can be more! Conservative is not always a bad word.
Not shunting vs ETV/CPC versus shunting CIC versus urological surgical interventions Prevention of pressure wounds Training is self control Qualitative technical aids
AND: primary prevention (folic acid supplementation and fortification)!
Prevention
Large proportion of Spina Bifida can be prevented by taking Folic Acid (up to 70%) Up to 91% prevention with other B-vitamins Daily intake of 0.4 mg of folic acid,
at least two months prior to the conception and the first months of pregnancy
Parents at extra risk should take daily 4 mg
Prevention of NTD Different strategies Supplementation
But: unplanned pregnancies, taking pills before conception
Fortification
But: not in Europe
Diet
But: not possible to get enough FA through diet
Oral
contraceptive + Folic Acid Other
What are neural tube defects (NTDs)?
Role models
Hydrocephalus
P
f o on i t uc d ro
Circulation / function
Absorption of CSF CSF = Cerebrospinal fluid
F S C
What causes Hydrocephalus? ď ˝
Structural abnormality (congenital)
What causes Hydrocephalus? ď ˝ ď ˝
Structural abnormality (congenital) Obstructing mass (tumor)
What causes Hydrocephalus?
Structural abnormality (congenital) Obstructing mass (tumor) Inflammatory scarring (infection)
What causes Hydrocephalus?
Structural abnormality (congenital) Obstructing mass (tumor) Inflammatory scarring (infection) Hemorrhage (prematurity)
Golden standard: shunts ď ˝
ď ˝
A tube is implanted that drains CSF from the brain into another body cavity where it can be absorbed. The most common site is the abdominal cavity
If care is unavailable‌
1956
Contra shunts
Shunts prohibitively expensive (*) Increased morbidity in emerging countries? Difficult access to treatment for malfunction
North American Shunt Design Trial
Drake et al. Randomized trial of cerebrospinal fluid shunt valve design in pediatric hydrocephalus. Neurosurgery 43:294305, 1998. 39% shunt failure within 1 year 53% shunt failure within 2 years 8.1% infection rate Type of shunt valve used made NO DIFFERENCE
Standard differential pressure valve (no anti-siphon characteristics) Delta valve [Medtronic] (anti-siphon characteristics) Orbis-Sigma valve [Cordis] (resistance increases w/pressure)
l’ embarras du choix…
(of “Western” shunts…)
Chhabra VP Shunt
the “IF shunt” – by Surgiwear
J Neurosurg (Pediatrics 4) 102:358–362, 2005 Comparison of 1-year outcomes for the Chhabra and Codman-Hakim Micro Precision shunt systems in Uganda: a prospective study in 195 children Benjamin C. Warf, M.D. CURE Children’s Hospital of Uganda, Mbale, Uganda, East Africa Object. The author investigated the 1-year outcomes for shunt treatment of hydrocephalic children in Uganda, com-paring the results of using the inexpensive Chhabra shunt ($35 US dollars), widely used in East Africa, to those of using the Codman-Hakim Micro Precision Valve shunt ($650). Methods. The results in 195 consecutive children (mostly infants) in whom shunts were placed were studied prospectively. In Group 1, 90 patients randomly received either the Chhabra or Codman shunt as primary treatment for hydrocephalus. In Group 2, 105 patients received the Chhabra shunt when endoscopic third ventriculostomy could not be per-formed or had failed. The end points of the study were shunt malfunction, shunt migration, wound complication, death, or no problem at 1 year. Of all patients, 9.7% were lost to follow up and 15.9% died before 1 year. The occurrence of complications in all patients were infection 9.7%, migration/disconnection 6.3%, wound complication 5.7%, valve malfunction 3.4%, ventricular catheter obstruction 2.8%, and peritoneal catheter obstruction 1.1%. There was no statistically significant difference in any outcome category for patients receiving the Codman or Chhabra shunts (p =0.2463–1.0000). Conclusions. Ventriculoperitoneal shunt insertion for treatment of hydrocephalus can be performed in a developing country with results similar to those reported in developed countries. No difference in outcome was noted between the two shunt types. No advantage was found in using a shunt system that, in this setting, is prohibitively expensive. KEY WORDS • hydrocephalus • shunt • developing country • outcome • pediatric neurosurgery See the Editorial and Response in this issue, p. 357.
Results w/1 year follow up Outcome
Chhabra
Codman
Total
p value
N=140
N=36
N=176
No problem
54%
58%
54.5%
0.7083
Dead 1 yr
15.7%
17%
15.9%
1.0000
Infection
9.3%
11%
9.7%
1.0000
Wnd comp
5.7%
5.6%
5.7%
1.0000
Prox obst
2.9%
2.7%
2.8%
1.0000
Distal obst
1.4%
0
1.1%
1.0000
Migration
5.7%
8.3%
6.3%
0.6982
Valve mlfx
4.2%
0
3.4%
0.3448
Comparisons of one year outcomes No difference between shunts and better results than in North American Shunt Design Trial
Primary shunt failure (including infection)
Chhabra: 23.5% Codman: 22.0% North American Shunt Design Trial: 39%
$35 - 60 USD
$650 USD
ETV to Prevent Shuntdependency ď ˝
A minimally invasive endoscopic procedure to create an opening from inside the brain that bypasses the obstruction, allowing the CSF to escape and be absorbed normally.
ETV
Why ETV?
50% of shunts fail in 2 years 5-10%/year life-time shunt failure rate The “safety net” for urgent shunt maintenance does not exist in much of the world Untreated shunt failure in shunt-dependent children is usually fatal ETV/CPC avoids shunt-dependency in
>70% of MM and other congenital causes of hydrocephalus 80% of PIH (and PHH?) when cistern is clear
THEREFORE, ETV/CPC seems particularly suited to the situation of children in developing countries
Shunt vs ETV Shunt
Relatively inexpensive (not if Western shunt is used) Capacity is relatively available Prone to fail and to infect Will always be necessary
ETV
Expensive hardware and training Lack of capacity Lower failure and infection rates After 6 months: sustainable results
Questions?
Thank you!
www.ifglobal.org