Occiput-cervical fusion for symptomatic atlantoaxial subluxation in a 32-month-old child with Down syndrome: a case report

Abstract

Atlantoaxial subluxation in Down syndrome rarely becomes neurologically symptomatic in very young children. The authors present a 32-month-old girl with Down syndrome, who had tetraporesis due to an atlantoaxial subluxation. She was treated with halo immobilization and partial reduction of the subluxation at ®rst, followed by removal of posterior arch of the atlas and posterior fusion with wire ®xation. Halo immobilization was continued for 3 months after operation. After 1-year follow-up, her motor functions were normal, and the dynamic roentgenogram of the cervical spines showed good stability. The authors recommend posterior fusion and postoperative halo immobilization for the treatment of the symptomatic atlantoaxial subluxation in young Down syndrome patients, even in a 32-month-old child.

Eponym

“…Both facts cause ligament hypermobility with distension and abnormal laxity of ligaments surrounding the articulation, resulting from the direct spread of inflammation from ENT area, which joined to the muscle spasm, may cause the subluxation [1,3,6,17]. The laxity of ligaments can explain the association in reported cases with Down syndrome and Marfan syndrome [10,12,16]. Other high-risk groups include Klippel-Feil syndrome, osteogenesis imperfecta, neurofibromatosis, and any syndromes associated with spinal instability [11].…”