Cervical myelomeningocele

Abstract

Objective: Cervical myelomeningocele (cMMC) is a rare disease. Only a few series have been published regarding cMMC. Different issues regarding the etiology, classification, clinical, surgical, and pathological aspects of cMMC are still a matter of conflict.

Methods: Sixteen children operated on for cMMC between July 2000 and 2003 were followed by the neurosurgical service at Children's Hospital Medical Center in Tehran. The patients were followed up for 2 to 5 years (median, 3 yr).

Results: The studied patients were nine boys and seven girls, ages 1 day to 4 months. Neurological examination was normal in all but two patients. All children had a normal anal fold, could void spontaneously, and showed no evidence of gross orthopedic deformity. We found eight patients with hydrocephalus, four patients with Chiari II malformation, two patients with syringomyelia, one patient with diastematomyelia at the level of cervical hemimyelomeningocele, and one patient with associated sacral myeloschisis. A thorough urological evaluation was planned for 13 patients, which confirmed bladder dysfunction in 10 (71%) patients. All infants had midline lesions, which consisted of a protruding sac from the back of neck, covered with purplish rudimentary or dysplastic skin at the dome. All patients underwent surgical resection of the sac and intradural exploration to release any adhesion and to exclude other associated anomalies.

Conclusion: Cervical myelomeningocele differs structurally and clinically from myelomeningocele in distal areas and has a more favorable outcome. We think that some trivial neurological deficits in cMMC are caused by the late and limited neurulation abnormality during its development. We advise thorough preoperative evaluation of the brain, spinal column, and urinary system. Intradural exploration to release any potential adhesion bands as well as correcting associated anomalies is recommended in all cMMC operations.