Abstract
We explored the relationship between seizure activity (SA) and/or chronic epilepsy
(CE) and short-term neurodevelopmental outcomes following fetal myelomeningocele (fMMC)
surgery. Retrospective databases and a parental questionnaire focusing on common complications
of hindbrain herniation associated with MMC were used to determine the incidence of
seizures following fMMC surgery. The Bayley Scales of Infant Development II was used
to evaluate the neurocognitive outcomes. The available 3-year outcome data were used
for analysis. 54 children underwent fMMC closure at our institution between 1998 and
2003. 48 (89%) families participated. The shunt rate was 50% (n=24). Seizures developed
in 8/48 (17%) children, 2 (8%) non-shunted and 6 (25%) shunted (P=0.07). Of those
six, 3 developed CE. Neurodevelopmental scores in the average range were found in
both non-shunted and 3 shunted fMMC children. The remaining 3 shunted toddlers had
CE and significant neurodevelopmental delays. Of those, 2 had severe intracranial
hemorrhage and one developed frequent apneic spells in combination with epilepsy.
The incidence of seizures in fMMC children was similar to previously reported data
of postnatally repaired MMC patients. SA alone without CE was not associated with
a worse neurocognitive outcome. The occurrence of severe acquired intracranial injury
and CE, however, appeared to be correlated with adverse neurocognitive outcome following
fMMC surgery.
Key words
myelomeningocele - spina bifida - fetal surgery - seizure - epilepsy - neurodevelopmental
outcome - ventriculomegaly
References
1
Bartoshesky LE, Haller J, Scott RM. et al .
Seizures in children with meningomyelocele.
Am J Dis Child.
1985;
139
400-402
2
Battaglia D, Acquafondata C, Lettori D. et al .
Observation of continuous spike-waves during slow sleep in children with myelomeningocele.
Childs Nerv Syst.
2004;
20
462-467
3
Battibugli S, Gryfakis N, Dias L. et al .
Functional gait comparison between children with myelomeningocele: shunt versus no
shunt.
Dev Med Child Neurol.
2007;
49
764-769
4
Brown TM, Ris MD, Beebe D. et al .
Factors of biological risk and reserve associated with executive behaviors in children
and adolescents with spina bifida myelomeningocele.
Child Neuropsychol.
2008;
14
118-134
5
Chadduck W, Adametz J.
Incidence of seizures in patients with myelomeningocele: a multifactorial analysis.
Surg Neurol.
1988;
30
281-285
6
Danzer E, Finkel RS, Rintoul NE. et al .
Reversal of hindbrain herniation after maternal-fetal surgery for myelomeningocele
subsequently impacts on brain stem function.
Neuropediatrics.
2008;
39
359-362
7
Danzer E, Flake AW.
In utero repair of myelomeningocele: rationale, initial clinical experience and a
randomized controlled prospective clinical trial.
Neuroembryol Aging.
2006/2007;
4
165-174
8
Danzer E, Gerdes M, Bebbington MW. et al .
Lower extremity neuromotor function and short-term ambulatory potential following
in utero myelomeningocele surgery.
Fetal Diagn Ther.
2009;
25
47-53
9
Danzer E, Gerdes M, Bebbington MW. et al .
Preschool neurodevelopmental outcome of children following fetal myelomeningocele
closure.
Am J Obstet Gynecol.
2010;
202
450e1-450e9
10
De Wit OA, den Dunnen WF, Sollie KM. et al .
Pathogenesis of cerebral malformations in human fetuses with meningomyelocele.
Cerebrospinal Fluid Res.
2008;
5
1-9
11
Johnson MP, Sutton LN, Rintoul N. et al .
Fetal myelomeningocele repair: short-term clinical outcomes.
Am J Obstet Gynecol.
2003;
189
482-487
12
Lu GC, Steinhauer J, Ramsey PS. et al .
Lethal pulmonary hypoplasia after in-utero myelomeningocele repair.
Obstet Gynecol.
2001;
98
698-701
13
Nalin A, Frigieri G, Cordioli A. et al .
The risk of convulsions: a longitudinal study of normal babies and infants with neonatal
damage in the first 6 years of life.
Childs Nerv Syst.
1990;
6
254-263
14
Rintoul NE, Sutton LN, Hubbard AM. et al .
A new look at myelomeningoceles: functional level, vertebral level, shunting, and
the implications for fetal intervention.
Pediatrics.
2002;
109
409-413
15
Ronen GM, Buckley D, Penney S. et al .
Long-term prognosis in children with neonatal seizures: a population-based study.
Neurology.
2007;
69
1816-1822
16
Scher MS, Aso K, Beggarly ME. et al .
Electrographic seizures in preterm and full-term neonates: clinical correlates, associated
brain lesions, and risk for neurologic sequelae.
Pediatrics.
1993;
91
128-134
17
Talwar D, Baldwin MA, Horbatt CI.
Epilepsy in children with meningomyelocele.
Pediatr Neurol.
1995;
13
29-32
18
Yoshida F, Morioka T, Hashiguchi K. et al .
Epilepsy in patients with spina bifida in the lumbosacral region.
Neurosurg Rev.
2006;
29
327-332
19
Zhang G, Raol YS, Hsu FC. et al .
Long-term alterations in glutamate receptor and transporter expression following early-life
seizures are associated with increased seizure susceptibility.
J Neurochem.
2004;
88
91-101
Correspondence
Mark P. Johnson,MD
The Center for Fetal Diagnosis and Treatment
The Children's Hospital of Philadelphia
5th Wood CenterThe Children's Hospital of Philadelphia
34th Street and Civic Center Boulevard
PA 19104 Philadelphia
USA
Telefon: +1/215/590 2747
Fax: +1/215/590 2447
eMail: johnsonma@email.chop.edu