Pre-operative clinical deterioration and long-term surgical outcomes in 41 patients with split cord malformation type 1.

Purpose: To document the pre-operative rate of clinical deterioration in a cohort of patients with split cord malformation type 1 (SCM 1) and the early- and long-term surgical outcome in these patients.

Methods: Data from 41 patients with SCM 1 operated upon by the same surgeon (VR) between January 2008 to June 2023 were retrospectively reviewed with respect to history of clinical deterioration prior to surgery and early and long-term surgical outcomes.

Results: The mean age of the patients at presentation was 79.3 months and the male to female ratio was 1:1.93. Twelve (29%) patients had congenital deficits whereas 4 (10%) patients had no neurological deficits. Twenty-six (63%) patients had kyphoscoliosis and 25 (61%) patients had motor dysfunction. Thirty-three (81%) patients (8/12 (67%) with congenital deficits) had clinical deterioration prior to surgery. By the age of 2 years, 56% of patients had clinical deterioration. After surgery, 18 (55%) patients with progressive symptoms had improvement in one or more of their symptoms on long-term follow-up (mean, 63.4 months). There were no predictors of surgical outcome.

Conclusions: Since over half of our patients with SCM 1 developed progression of congenital deficits or developed deficits by the age of 2 years, surgery should be performed as soon as possible in these children. On long-term follow-up after surgery, improvement can be expected in over half the patients.