Regression of microcephaly as a protective factor of neuropsychomotor development in fetal surgery for occipital encephalocele

Purpose: Encephaloceles are neural tube closure defects characterized by herniation of intracranial contents through the skull, with a mortality rate of 33.3%. Approximately 50% of patients who survived have some degree of neuropsychomotor developmental impairment or seizures. This study aimed to analyze the relationship between good neuropsychomotor development (NPMD) in patients undergoing fetal occipital encephalocele correction and the reversal of microcephaly, comparing these outcomes with those observed in patients who underwent postnatal surgery.

Methods: The 22 participants were categorized into two groups: 10 in the fetal group (FG) and 12 in the postnatal group (PNG). During the study, 1 patient was excluded from the FG and 2 patients were excluded from the PNG, totaling 19 patients in the study. All patients were diagnosed, evaluated, and monitored by the same healthcare service between July 2012 and July 2018. All participants were subjected to a careful developmental assessment using the Bayley Scale of Infant Development, Second Edition (BSID-II), up to 2 years and 11 months of age. Additionally, CP measurements were taken during the first year of life to monitor their progress. The relationship between microcephaly reversal and NPMD was studied.

Results: The CP adjusted for gestational age showed a tendency toward the reversal of progressive microcephaly after correction of encephaloceles in the FG. We found a statistically significant difference in the median BSID-II score between the PNG and FG. Patients in the FG maintained normal CP development in the first year of life, whereas those in the PNG remained microcephalic.

Conclusion: The reversal of microcephaly in the FG directly influences good NPMD and can be considered a protective factor.