Split cord malformation associated with myelomeningocele

Area of Science:

• Pediatric Neurosurgery
• Spinal Dysraphism
• Congenital Malformations

Background:

• Split cord malformations (SCMs) and myelomeningoceles (MMCs) are congenital spinal anomalies.
• The coexistence of SCMs and MMCs can lead to misdiagnosis and spinal cord tethering.
• Identifying associated SCMs in patients with MMCs is critical for effective management.

Purpose of the Study:

• To investigate the incidence and characteristics of split cord malformations (SCMs) coexisting with myelomeningoceles (MMCs).
• To emphasize the importance of preoperative evaluation and intraoperative inspection for associated SCMs in MMC patients.
• To highlight the benefits of simultaneous surgical repair of both SCMs and MMCs.

Main Methods:

• Retrospective review of 330 patients with MMCs treated between January 2001 and June 2005.
• Identification of 33 patients (10%) with coexisting SCMs.
• Analysis of patient demographics, SCM type and location, associated anomalies, and surgical outcomes.

Main Results:

• 33 patients (10%) with MMCs had associated SCMs.
• The mean age at presentation was 2.9 months; 55% were female.
• Type I SCM was most common (26 patients), often at the neural placode (17 patients).
• Associated findings included unilateral leg paresis (8 patients) and skin lesions (2 patients).
• All patients underwent simultaneous repair of both MMC and SCM.

Conclusions:

• Patients with MMCs, particularly those with atypical signs like unilateral paresis or skin lesions, require thorough preoperative clinical examination for SCMs.
• Spinal magnetic resonance imaging (MRI) aids in detecting these associated anomalies.
• Careful intraoperative inspection of the neural placode and spinal cord is essential to identify and address dysraphic lesions like SCMs during MMC repair.