Diagnosis, treatment, and functional prognosis in myelomeningocele: a single-center study from 2012 to 2019

Abstract

A trial that investigated prenatal versus postnatal repair of myelomeningocele (Management of Myelomeningocele Study: MOMS) reported a decrease in the combined rate of hydrocephalus and Chiari malformations. Some facilities in Japan have begun their preparations to be equipped to perform fetal surgeries for myelomeningocele (MMC). To perceive the current state of MMC in Japan before the introduction of fetal surgery, we investigated 35 cases of MMC diagnosed in our institution from 2012 to 2019. Among these cases, 32 cases were diagnosed before birth and three cases were diagnosed after birth. Among the 32 cases diagnosed before birth, abortion was performed in six cases. Among the 29 remaining cases, hydrocephalic surgery was performed in 24 (82.8%) cases and foramen magnum decompression was performed in four (13.8%) cases. Furthermore, in 63.2% alive cases, the patients later gained the ability to walk, and in 92.3% alive cases, the developmental quotient was >50. The study showed that appropriate postnatal treatment results in a relatively good functional prognosis, therefore, safer fetal treatment and maternal management are required. To promote fetal surgery for MMC in Japan, it is necessary to improve fetal diagnosis technology for obstetricians. Moreover, prenatal counseling of parents by a multidisciplinary team is mandatory, mainly by pediatric neurosurgeons who follow-up patients with MMC and are aware of the long-term prognosis of MMC.