Terminal myelocystocele.

Abstract

The authors describe the cases of eight patients with terminal myelocystoceles and report on their initial diagnoses, neuroimaging findings, surgical procedures, and clinical follow-up examinations. There were seven girls and one boy. The initial age at diagnosis ranged from prenatal age (determined by imaging) to 14 years. Operative correction was performed from 6 weeks to 14 years of age. One patient presented with a large prevertebral (intraabdominal) meningocele. Four patients have associated abdominal wall defects and are significantly handicapped. Two are wheelchair bound and the other two ambulate with prosthetic devices; all are incontinent. The four patients without abdominal wall defects are fully ambulatory; one wears an ankle-foot orthosis. One patient has normal bowel and bladder functions, and the other three require intermittent clean catheterization. No patient to date has had clinical evidence of retethering, although a low-set conus medullaris and residual lipoma were visible on postoperative neuroimaging. No patient in this series had hydrocephalus or Chiari malformation. It is concluded that patients with myelocystoceles without abdominal wall defects have a favorable neurological outcome when compared with those having ventral wall defects.