Spinal muscular atrophy: Classification, aetiology, and treatment of spinal deformity in children and adolescents

SummarySpinal muscular atrophy is a hereditary neurological condition, which presents with symmetrical limb and trunk weakness. Spine deformity is the most frequent orthopaedic manifestation of the disease in patients who survive beyond the first year of life. Scoliosis in this group of severely disabled children decreases their sitting tolerance, causes pain from impingement of the ribs against the pelvis, affects ambulatory ability, and creates further respiratory compromise accelerating their death. Spinal arthrodesis is the only treatment that has a well-documented positive impact in restoring trunk balance and preserving function. This is associated with significant technical challenges and a high rate of life-threatening complications. A comprehensive review of the condition and a strategy for treating spinal deformity are presented in this paper.