The congenital dislocated spine has been defined as the potentially most serious form of congenital kyphosis with an abrupt single-level displacement of the spinal canal. A retrospective chart review was conducted on 19 patients with this deformity.
To delineate the clinical and radiologic characteristics of this entity, and to analyze the outcome after treatment.
An anterior failure of formation was the basic feature. Kyphosis was variable. Vertebral displacement in the frontal plane was present in seven patients, and sagittal displacement was constant. Mechanical instability was seen in 17 patients. Neurologic impairment was identified in 12 patients, and congenital paraplegia was seen in eight patients. An acute paraplegia occurred after minor trauma in one patient.
Seventeen patients were treated surgically. Thirteen patients underwent complete circumferential stabilization through anterior strut grafting and posterior fusion without instrumentation, usually before age 3 years. Neurosurgical decompression was done in four patients.
The average follow-up period was 8 years, 6 months. Nonunion of the posterior fusion mass was detected and successfully treated in five patients. A solid fusion seemed to be obtained in all patients at last follow-up evaluation. The neurologic status after neurosurgical decompression remained unchanged in three patients and was improved temporarily in one patient.
Avoidance of neurologic morbidity requires early diagnosis and stabilization. The authors recommend early anterior strut grafting and posterior fusion. Exploration of the posterior fusion mass should be done systematically.
From the Department of Orthopaedic Pediatric Surgery, Hˆpital Saint-Vincent-de-Paul, Paris, France.
Presented at the 30th Annual Meeting of the Scoliosis Research Society, September 13-17, 1995, Asheville, North Carolina.
Acknowledgment date: September 13, 1995.
First revision date: January 2, 1996.
Acceptance date: January 4, 1996.
Device status category: 1.
Address reprint requests to: Reinhard D. Zeller, MD; Department of Pediatric Orthopaedics; Hôpital Saint-Vincent-de-paul; 82, Avenue Denfert Rochereau; 75674 Paris Cedex 14; France