Split cord malformation types I and II: a personal series of 131 patients

A personal series of 131 patients with split cord malformation (SCM) operated on is presented.

Age, gender, symptoms and signs, radiological and operative findings, complications, associated anomalies, outcome, and pathological specimens were analyzed.

There were 88 girls (73 %) and 43 boys (27 %). The female predominance was slightly more remarkable in type I SCMs than in type II SCMs. The presenting symptoms can be summarized as skin lesions, spina bifida aperta, scoliosis or kyphoscoliosis, sphincter disturbance, foot deformities and weakness, and/or atrophy in the lower extremities. The ages of patients with neurological deficits and orthopedic deformities were significantly older than those without deficits (P = 0.030). The duration of symptoms was longer in the patients with neurological deficits and orthopedic deformities than that in those without deficits (P = 0.00095). In six patients, composite SCMs were present. Only one patient with a type II SCM did not have an associated spinal cord lesion. A type I SCM was more frequently encountered in patients with spina bifida (P < 0.0005). Transient postoperative complications were seen in 29 patients (22 %). There was no permanent complication. Retethered cord syndrome developed in five patients with a type I SCM.

The risk of neurological and orthopedic deficits increases with the age of the patient. The risk of permanent deficit after surgery is very low. The whole spine must be examined for additional lesions. All patients should be surgically treated when diagnosed, especially before the development of orthopedic and neurological manifestations, and all associated lesions should also be treated at the same session.