Management of pediatric congenital atlantoaxial dislocation: a report of 23 cases from northern India.

From July 1996 to August 2001, we operated on 23 children (age 4-12 years) with congenital atlantoaxial dislocation (AAD). Sixteen had reducible AAD and 6 had the fixed type, while a single patient had basilar invagination. Preoperatively, these children were divided into four functional grades depending on the level of neurological deficits and dependence on others for activities of daily living (Di Lorenzo grades I-IV). There were only 2 patients with no deficits (grade I), while 15 of the 23 children presented with severe neurological deficits and were completely dependent for all activities of daily living (grade IV). Stigmata suggestive of congenital AAD were documented in 6 children and radiological osseous anomalies in 18 children. Transoral decompression and posterior stabilization was performed in 7 children, while 16 patients underwent the posterior stabilization procedure only. All patients were immobilized with a hard cervical collar postoperatively. Six children developed one or other treatable complications, of whom reoperation was indicated in 3, but could only be undertaken in 2 children. Retightening of the wire was done in 1 and further decompression of the odontoid process was carried out in the other. There was no postoperative deterioration in patients with no preoperative deficits (grade I). Ten out of the 15 children who were completely dependent for all activities in the preoperative period (grade IV) improved, and 6 children achieved normalcy or near normalcy (grade I or II). Three children deteriorated in the postoperative period, of whom 2 subsequently improved at follow-up. There were 2 deaths in the series; both these patients belonged to the severely compromised group (grade IV) and in addition had respiratory compromise. Bony fusion at the posterior stabilization site could be seen in 14 out of 16 patients screened. The various problems faced by the authors in managing these very fragile and severely compromised children with cervicomedullary compression due to congenital atlantoaxial dislocation in a setting with limited resources are discussed. Copyright 2002 S. Karger AG, Basel