Diastematomyelia

Diastematomyelia is a rare congenital malformation, accounting for approximately 5% of all congenital spinal anomalies. It is characterized by a longitudinal split of the spinal cord into 2 hemicords and often presents alongside other neural tube defects. Diastematomyelia is classified into 2 types: type 1, which involves a bony or cartilaginous septum separating the hemicords, and type 2, where no septum is present. Clinical manifestations include hypertrichosis, back pain, lower limb motor and sensory deficits, foot deformities, and scoliosis, though some patients may remain asymptomatic. Diagnosis is typically made with CT or MRI. Surgical correction is the standard treatment, even in asymptomatic patients, to prevent future neurological decline.