Neuropediatrics 2016; 47 - FV01-09
DOI: 10.1055/s-0036-1583714

Preliminary Results in Children with Myelomeningocele after Fetal Surgery: Data from the Zurich Cohort

D. A. Wille 1, A. Klein 1, 2, L. Mazzone 3, U. Moehrlen 3, M. Meuli 3, B. Latal 4, C. Rethmann 5, B. Plecko 1
  • 1Department of Pediatric Neurology, University Children`s Hospital Zurich, Zurich, Switzerland
  • 2Department of Pediatric Neurology, University Children`s Hospital Basel, Basel, Switzerland
  • 3Department of Pediatric Surgery, University Children’s Hospital Zurich, Zurich, Switzerland
  • 4University Childrens Hospital Zurich, Child Development Center Zurich, Switzerland
  • 5Department of Neuroradiology, University Childrens Hospital Zurich, Zurich, Switzerland

Background: The purpose of this study was to investigate the neurological outcome of patients with meningomyelocele treated with fetal closure.

Methods: A total of 29 patients were included, 27 were followed up prospectively, 2 retrospectively. Presence of Chiari II malformation and anatomic lesion level were assessed with fetal and postnatal MRI. The functional spinal level was determined by a pediatric neurologist after birth and at 3, 6, 12, and 24 months. Cognitive development was assessed at 1 (Griffiths) and 2 years of age (Bayley III) and time of shunt placement was noted.

Results: Anatomical level in utero was between L1 to L5. The functional level after birth had a range from L3 to S1. 19 of 27 (70%) showed a partial innervation below the functional level. Before surgery, Chiari II-malformation was detected in all 29 patients, while after fetal surgery complete reversal of hindbrain herniation was observed in 27/29 (93%). Shunt placement was necessary in 38% (11 of 29). At 1 year, cognitive developmental age ranged from 7 to 13 months (average: 10.2), at 2 years it ranged from 17 to 24 months (average 21).

Conclusion: In this cohort of fetally operated children with myelomeningocele, shunt placement was necessary only in 38%, comparable to the results of the fetal surgery arm in the MOMS trial. It is to be discussed if the partial motor innervation below the functional level, the low percentage of shunt placement, the remission of hindbrain herniation and the favorable cognitive outcome might be the result of fetal intervention.