Split cord malformation (scm) in paediatric patients: outcome of 19 cases

Abstract

There had been considerable debate regarding the surgical outcome of neuro-orthopaedic syndromes (NOS) and neurological syndromes in cases of split cord malformation (SCM). On retrospective analysis of 19 cases of SCM, thirteen were grouped under (Pang) type I and 6 in type II. Their age ranged from 1 month to 9 years (mean 3.5 years). 14 of these were male children. The NOS without neurological signs was detected in 6 cases where as pure neurological signs without NOS were seen in 8 patients. However, the rest 5 had mixed picture of NOS and neurological dysfunction. Nine of 19 cases presented with cutaneous stigmata, mainly in the form of hairy patch. 18 cases had other associated craniospinal anomalies i.e. hydrocephalus, meningomyelocoele, syrinx, dermoid, teratoma etc. Detethering of cord was done in all cases by removal of fibrous/bony septum. Associated anomalies were also treated accordingly. Follow up of these cases ranged from 6 months to 6 years. Six cases of NOS group neither showed deterioration nor improvement, and remained static on follow up. However, four of 8 children with neurological signs showed improvement in their motor weakness, and 1 in saddle hypoaesthesia as well as bladder/bowel function. In 5 cases of mixed group, two had improvement in their weakness and one in hypoaesthesia, but no change was noticed in NOS of this group as well. Hence surgery seemed to be effective, particularly in patients with neurological dysfunction.