High cervical spine instability in children with down syndrome: A report of six cases and review of current management

Abstract

High cervical instability has been well documented to be associated with Down syndrome. The clinical presentation of 6 children with Down syndrome and atlantoaxial instability who required surgical stabilization was reviewed. Torticollis, frequent falls and hyperreflexia were common preoperative findings. A high index of suspicion and a thorough clinical examination is very important. In addition to lateral flexion-extension views, CT scan with 3 dimensional reconstruction and MRI are useful preoperative investigative tools. Surgery is indicated in symptomatic patients. Fusion from occiput to axis with postoperative external immobilization till union offers the best results, Asymptomatic children may not have their sports activities restricted even if their screening X-rays are found to be abnormal.