CERVICAL MYELOMENINGOCELE IN ADULTHOOD

Abstract

Cervical myelomeningocele is an extremely rare condition, accounting for only 1 to 5% of all neural tube defects. These lesions are usually diagnosed in childhood. Here, we report a case of a cervical myelomeningocele diagnosed and treated in adulthood. A 52-year-old man presented with a 3-year history of progressing weakness and paresthesia in his upper limbs. Physical examination revealed a posterior midline neck mass covered with normal skin. Magnetic resonance imaging showed a soft-tissue mass tethering the cord by a stalk extending from the dorsal spinal cord to the dome of the lesion. Syrinx was evident cranially and caudally to the origin of the posterior stalk. Surgical resection of the sac and intradural exploration were performed. The subdural space was explored, and the tethered structures were released. Histological examination showed small foci of meningothelial cells with psammoma bodies and rare thin fascicle of glial tissue dispersed in hyaline tissue. Immunohistochemical stains against glial fibrillary acidic protein and S100 confirmed the presence of bands of astrocytic tissue. The patient demonstrated early improvement of neurological deficits. Six months after surgery, he was asymptomatic and magnetic resonance imaging showed resolution of the syrinx. We believe the syrinx in this patient was caused by a blockade of flow in the central canal and around the spinal cord as a result of the tethered cord. The untethering procedure resulted in the collapse of the syrinx followed by resolution of neurological deficits.