Split cord malformation associated with myelomeningocele.

OBJECT: The coexistence of split cord malformations (SCMs) and myelomeningoceles (MMCs) can be misdiagnosed or ignored and can cause spinal cord tethering. The authors investigated cases involving the coexistence of SCMs and MMCs in patients from the Children's Hospital Medical Center in Tehran.

METHODS

Of the 330 patients with MMCs who underwent operations at the Children's Hospital Medical Center between January 2001 and June 2005, 33 (10%) had an associated SCM. These 33 cases were retrospectively reviewed.

RESULTS

Eighteen patients (55%) were female, and the mean age of the patients at presentation was 2.9 months. In 17 of the 33 patients, the SCM occurred at the level of the neural placode. A Type I SCM was found in 26 children. Two patients had hypertrichosis. Eight patients had unilateral leg paresis. The MMC sac was located in the lumbar region in 14 cases. Two patients had double spinal dysraphism with meningoceles at the thoracic level. All patients underwent simultaneous repair of both lesions at the time of surgery.

CONCLUSIONS

Patients with MMCs, especially those with unusual manifestations such as unilateral paresis or skin lesions, should undergo a preoperative clinical examination to check for the presence of an SCM. Use of spinal magnetic resonance imaging can help in identifying the associated abnormalities. The neural placode and the rostral and caudal spinal cord segments should be carefully inspected for dysraphic lesions such as SCMs during the operation to repair the MMC. In this paper, the authors hope to show the wisdom of identifying these anomalies at the time of the initial repair of the MMC.