Outcomes of Posterior Spinal Fusion in Pediatric Patients with Down Syndrome.

BACKGROUND: Trisomy 21 or Down syndrome is associated with multiple orthopaedic manifestations. Although cervical instability is the most common spinal condition associated with Down syndrome, the prevalence of scoliosis has been estimated at 4.8% to 8.7%. Very few prior studies have documented the role of spinal fusion in this population, and all have included ≤10 patients.

METHODS: An institutional review board-approved multicenter retrospective analysis of patients with Down syndrome treated with spinal fusion between January 2009 and December 2019 was performed by cross-referencing Current Procedural Terminology (CPT) and International Classification of Diseases, Ninth and Tenth Revisions (ICD-9 and ICD-10) codes. Patients were followed for ≥2 years, with a mean follow-up of 3.77 years. Clinical and radiographic outcomes were collected, and complications were documented using the Clavien-Dindo-Sink (CDS) classification.

RESULTS: A total of 23 patients were included: 96% had ≥1 medical comorbidities, including 16 (70%) with congenital heart disease, of whom 88% had previous cardiac surgery, and 10 (44%) with thyroid disorders. All 23 patients underwent posterior spinal fusion. The mean estimated blood loss was 617 ± 459 mL, the mean length of the surgical procedure was 290 ± 92.7 minutes, and the mean length of hospital stay was 6.03 ± 2.91 days. The major Cobb angle measured 61.7° ± 17.6°, which corrected to 19.4° ± 14.8° (68.6% correction; p < 0.001), with well-maintained correction at 2 years of 22.0° ± 10.3° (64.3% correction; p = 0.158). Thirteen (57%) of 23 patients had a change in curve of >5°. There were no intraoperative complications; however, 12 patients (52%) sustained postoperative complications (e.g., need for reoperation, implant failure, and pulmonary complications), including 6 patients with CDS type 3 or 4 (e.g., wound dehiscence, late superficial abscess, pleural effusion, pseudarthrosis, and readmission for hypoxia). Four patients (17%) required a revision surgical procedure. One patient (4%) required an unplanned intensive care unit admission.

CONCLUSIONS: Although instrumented spinal fusion can effectively correct spinal deformity in these patients, complications are more frequent than in children with adolescent idiopathic scoliosis, with over half of patients sustaining a complication.

LEVEL OF EVIDENCE: Prognostic Level III. See Instructions for Authors for a complete description of levels of evidence.