Abstract

Objective The purpose of this study was to investigate the neurological outcome of patients with meningomyelocele treated with fetal closure in Zurich. Methods 14 patients were included, of two data were collected retrospectively, 12 were followed prospectively by a detailed evaluation protocol. We investigated the anatomical spinal level in utero before surgery by in utero MR imaging. The functional spinal level at birth, 3 months, 6 months and 12 months of age was compared to the anatomical level in utero. Chiari II-malformation and remission of hindbrain herniation over the course was investigated by MRI. Data of head circumference, shunt placement, bladder dysfunction and development were collected. Our results were compared to the results of the MOMS study (A randomized trial of prenatal versus postnatal repair of myelomeningocele. Adzick et al. NEJM, 2011 Mar 17;364(11):993–1004). Results Anatomical level in utero ranged between L1 to L5. According to the anatomical level the functional level after surgery was two or more levels better in 28%, level of function one level better in 21%, no difference was seen in 43%, level of function two level worse in 7%. Interestingly 75% of our patients had partial motor innervation below the functional level. Before surgery Chiari II-malformation was detected in all 14 patients, after fetal surgery we had no evidence of hindbrain herniation in any. In 46.1% shunt placement was necessary. 66% had neurogenic bladder dysfunction. Conclusion Our results from this small cohort show nearly the same results as reported in the MOMS trial, especially regarding shunt placement. A part of our patients showed a relevant partial motor innervation below the level, it is to discuss if this could be a result of fetal intervention, just as the low frequency of shunt placement and complete demission of hindbrain herniation.

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