Split cord malformation in children undergoing neurosurgical intervetion in India : a descriptive study

To describe the clinical parameters, radiological findings and surgical outcome of 49 cases of split cord malformation (SCM). Forty nine patients of SCM were operated between June 1989 and June 2002, at Sanjay Gandhi postgraduate institute of medical sciences, Lucknow, India out of total 155 cases of spinal dysraphism. Detailed clinical evaluation with magnetic resonance imaging (MRI), preferably craniospinal, was main tool of investigation. All patients underwent excision of bony spur or fibrous septum, repair of associated myelomeningocele sac, when present, excision of associated spinal lesions and detethering of cord. Seven patients needed shunt operation. Patients having minimum 11⁄2 month of follow up were included in this analysis. Mean follow-up period was 3.4 years. Female to male ratio was 1.04: 1, with mean age of 4.8 years. The commonest cutaneous manifestation was hypertrichosis in 16 (32.7%) cases and among the neuro-orthopedic syndromes, most frequent were scoliosis in 16 (32.7%) and congenital talipes equino varus in 11 (22.4%) cases. The commonest clinical indicators were lower limb weakness, graded sensory loss, sphincter involvement, and autoamputation of toes and trophic ulcer in 24, 20, 14, two and four patients, respectively. Common imaging findings were low lying cord in 28, neural placode in 19, and SCM with and without meningomyelocele (MMC) sac in 20 and 29 cases, respectively. Common sites of occurrence of SCM were dorsal region in 19 (38.8%) and lumbar in 14 (28.6%) patients. Postoperative complications were cerebrospinal fluid leak, pseudomeningocele, wound infection, and meningitis in 12, eight, four and six patients, respectively. Two patients died in the postoperative period. On average follow up of 3.4 years, motor weakness improved in 10 children and remained static in 14, whereas sensory dysfunction improved in 13 and remained static in seven, and sphincteric function improved in 10 and was static in four cases. Backache dramatically relieved in both children and in all four patients, trophic ulcer completely healed. SCM cases may have association with MMC in significant number of patients hence craniospinal MRI remains the choice, as this will not only delineate complex spina bifida and cord anatomy, but also screen the entire neuraxis for other associated cranial and spinal anomalies. (J Pediatr Neurol 2004; 2(1): 21-27).