Focal spinal nondisjunction in primary neurulation: Limited dorsal myeloschisis and congenital spinal dermal sinus tract

Abstract

Spinal dysraphic lesions due to focal nondisjunction in primary neurulation are commonly encountered in paediatric neurosurgery, but the “fog-of-war” on these conditions was only gradually dispersed in the past 10 years by the works of the groups led by the senior author and Prof. Kyu-Chang Wang. It is now clear that limited dorsal myeloschisis and congenital spinal dermal sinus tract are conditions at the two ends of a spectrum; and mixed lesions of them with various configurations exist. This review article summarizes the current understanding of these conditions’ embryogenetic mechanisms, pathological anatomy and clinical manifestations, and their management strategy and surgical techniques.