Long-term neurological cognitive, behavioral, functional, and quality of life outcomes after fetal myelomeningocele closure: a systematic review

Abstract

Background: Myelomeningocele is a lifelong condition that features several comorbidities, such as hydrocephalus, scoliosis, club foot, and lower limb sensory and motor disabilities. Its management has progressed over time, ranging from supportive care to early postnatal closure to prenatal closure of the defect. Recent research discovered that fetal myelomeningocele closure (fMMC) provided superior neurological outcomes to those of postnatal closure. When performed at 12 months of age, fMMC can avert or delay the need for a ventriculoperitoneal shunt and reversed the hindbrain herniation. Moreover, fMMC reportedly enhanced motor function and mental development at 30 months of age. However, its long-term outcomes remain dubious.Purpose: This systematic review aimed to determine the long-term neurological cognitive, behavioral, functional, and quality of life (QOL) outcomes after fMMC. Methods:The PubMed, Directory of Open Access Journals, EBSCO, and Cochrane databases were extensively searched for articles published in 2007-2022. Meta-analyses, clinical trials, and randomized controlled trials with at least 5 years of follow-up were given priority.Results: A total of 11 studies were included. Most studies revealed enhanced long-term cognitive, behavioral, functional, and QOL outcomes after fMMC. Conclusion:Our results suggest that fMMC substantially enhanced patients' long-term neurological cognitive, behavioral, functional, and QOL outcomes.

Abnormal prenatal brain development in Chiari II malformation

“…The long-term neurological outcome, executive functioning, and adaptive behavioral skills following fetal myelomeningocele (fMMC) surgery are significantly better than postnatal repair ( Danzer et al, 2016 ). Also, fMMC substantially enhanced patients’ long-term neurological, cognitive, behavioral, and functional outcomes ( Siahaan et al, 2023 ). However, currently, guidelines for surgical intervention in fetuses are based on the severity of the malformation.…”