Cervicothoracic spinal dysraphism: Unravelling the Pandora’s box

Abstract

Introduction:Cervicothoracic spinal dysraphism (CTSD) is relatively rare with reported incidence of 1%–6.5%. The entity has a separate spectrum of associations with other anomaly such as split cord malformation, Chiari malformation, and corpus callosum agenesis as compared with its lumbosacral counterpart. In this study, we have highlighted the associated anomalies (AAs). To the best of our knowledge, this study is one of the largest series reported before.Materials and Methods:In this study, we included the patients with spinal dysraphism operated between December 2007 and December 2017 at the Department of Neurosurgery. All patients underwent neurological and radiological examinations followed by surgical excision of the sac and exploration of the intradural sac. Neurological, orthopedic, and urological abnormalities were analyzed in our retrospective description. The last available follow-up in hospital records was taken for outcome assessment.Results:Of 34 cases, 18 were women and 16 were men. Seven of nine patients in cervical group had AAs, whereas 22 of 25 patients in dorsal group had AA. In this study, we taken 6 patients with Chiari malformation, 4 with corpus callosum agenesis, 7 with hydrocephalus, and 16 with split cord malformation. The follow-up ranges from 1 year to 11 years with a mean of 59.45 months.Conclusion:The management strategy and association with other congenital anomalies separate CTSD as different clinical entity as compared with their lumbosacral counterparts. These patients show relatively favorable outcome with regard to neurological, orthopedic, and urological symptoms. Early surgical intervention after proper radiological and clinical evaluation is recommended.

Etiology and Pathophysiology of the Spina Bifida

“…Only about 0-5% of cases occur in the cervical spine, 5-10% in the thoracic spine, 20-30% in the thoracolumbar junction, 20-30% in the lumbar, 30-50% in the lumbosacral level and 5-15% in the sacral spine [10]. Altogether cervicothoracic spinal dysraphisms are rare, with an incidence of only 1-6,5% [11]. Myelomeningocele occurs in approximately 1 in 1200 to 1400 births.…”