Adult Complex Spinal Dysraphism With Situs Inversus Totalis: A Rare Association and Review

Abstract

First published report of an adult complex spinal dysraphism with situs inversus. To describe a previously asymptomatic adult patient of multiple vertebral anomalies with cervical split cord malformation type II, tethering of the spinal cord (cervical and lumbar), and intraspinal arachnoid cyst along with dextrocardia and situs inversus. Only 5 cases (fetus, 1; neonates, 3; child, 1) of spinal dysraphism with dextrocardia or situs inversus have been reported. All these cases have had associated multiorgan developmental anomalies usually incompatible with survival and requiring multidisciplinary care. The case has been described and relevant literature reviewed. The patient was operated for cervical and lumbar levels in the same sitting. A C4-C5 laminectomy was performed, 2 hemicords enclosed in the same dural sac were visualized, dorsal paramedian nerve roots and the tethering arachnoid bands were cut, and the arachnoid cyst wall was partially excised. This was followed by L4-L5 laminectomy and detethering by sectioning of the thickened filum terminale. The patient showed significant neurologic improvement after surgery. The present case is a rare instance in which there has been an association of adult onset occult spinal dysraphism along with situs inversus totalis. Successful management requires appropriate understanding of embryology, anatomy, and imaging and has implications in neurosurgical and perioperative anesthetic care.