Neurosurgical strategy based on the type of occult spinal dysraphism in omphalocele-exstrophy-imperforate anus-spinal defects complex: A review of 10 cases.

Abstract

Background: Omphalocele-exstrophy-imperforate anus-spinal defects (OEIS) complex is a rare, life-threatening congenital malformation primarily treated with abdominogenital repair. The optimal indication and timing of neurosurgical interventions for the associated spinal cord lesions remains insufficiently studied. We reviewed spinal dysraphism in OEIS to evaluate the best timing for neurosurgical intervention.

Methods: We retrospectively reviewed 10 patients with OEIS, analyzing their clinical and imaging data, as well as surgical and pathological findings.

Results: Terminal myelocystocele (TMCC) and spinal lipomas were observed in 5 patients each. Of the spinal lipomas, one had a single filar lipoma, while four had double lipomas (3 caudal and dorsal; 1 filar and dorsal). TMCC manifested with severe lower limb motor dysfunction in addition to abdominogenital disorder at birth, with the cyst-induced lumbosacral mass increasing over time. Spinal lipomas were less symptomatic except for abdominogenital issues and demonstrated minimal growth of the intraspinal lipoma over time. Untethering surgery was performed in 8 patients (5 TMCC; 3 spinal lipomas) at a median age of 3 (range, 2-10) months for TMCC and 6 (range, 2-14) months for spinal lipomas. One TMCC patient (surgery at 10 months) experienced postoperative cerebrospinal fluid leakage, necessitating three reoperations.

Conclusion: Magnetic resonance imaging is essential to diagnose spinal cord malformations accurately. The necessity and timing of surgical intervention differ between TMCC and spinal lipomas. Since TMCC lesions tend to enlarge, surgery should be performed as soon as the patient's abdominogenital condition stabilizes. For spinal lipomas, surgery should be considered carefully based on the patient's neurological condition.