Object: 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.
Methods: 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.
Conclusions: It is concluded that patients with myelocystoceles without abdominal wall defects have a favorable neurological outcome when compared with those having ventral wall defects.