Understanding split cord malformation: from pathophysiology to long-term outcomes

Abstract

Split cord malformation (SCM) is a rare congenital anomaly of the spinal cord, frequently associated with tethered cord syndrome and orthopedic, urological, and neurological sequelae. Surgical management of SCM remains controversial, particularly regarding timing of intervention, selection criteria for asymptomatic cases, and expected outcomes for different SCM types. This systematic review aims to synthesize current evidence regarding surgical indications, techniques, outcomes, and prognostic factors in SCM treatment. We conducted a comprehensive systematic review of the literature, selecting 30 studies comprising over 1,200 patients who underwent surgery for SCM. Outcomes assessed included neurological, urological, and orthopedic status, complication rates, timing of surgery, and comparative outcomes between type I and type II SCM. Study quality was assessed using the Newcastle-Ottawa scale. Early surgical intervention, especially in type I SCM, was consistently associated with superior outcomes. Preoperative neurological status emerged as the strongest predictor of postoperative results, with early surgery preventing progression and facilitating functional improvement. Urological and orthopedic outcomes similarly benefited from timely intervention. Type II SCM demonstrated a more benign course, with selective surgical indications. Overall complication rates were low, with transient neurological worsening being the most common adverse event. Intraoperative neurophysiological monitoring contributed to enhanced surgical safety. Early surgical correction remains the cornerstone of SCM management, particularly in type I cases. Delayed intervention is associated with reduced potential for neurological recovery. Further prospective studies are needed to refine patient selection criteria, optimize long-term outcomes, and guide management of adult and type II SCM presentations.