Split cord malformations: an experience of 203 cases.

METHODS

A total of 203 patients with split cord malformation (SCM) were operated on at our centre between March 1989 and October 2003. Patients' demographic profile, radiological and operative details, complications and surgical outcome were evaluated retrospectively.

RESULTS

The mean age of the patients was 7.4 years and the female to male ratio was 1.3:1. The dorsolumbar and lumbar regions were the most common sites. One hundred thirty-two patients had type I SCM and 71 had type II SCM. One or more skin stigmata were present in 60% cases, hypertrichosis being the most common. Asymmetric weakness of the lower limbs and sphincter disturbances were present in 73 and 33% cases, respectively. Twenty-seven patients were asymptomatic. Of the symptomatic cases, 40.5% showed improvement in motor power, whereas 31% regained continence. The neurological status was unchanged in 48%. Seven percent of the patients showed neurological deterioration immediately after surgery.

CONCLUSIONS

SCMs are rare malformations of the spinal cord. We present the largest series so far reported in the world literature. The risk of developing neurological deficits increases with age; hence, all patients with SCM should be surgically treated prophylactically even if asymptomatic.