Syndromic Scoliosis: National Trends in Surgical Management and Inpatient Hospital Outcomes

Abstract

Study Design. Retrospective cohort study. Objective. Evaluate the trends in management and inpatient outcomes in patients with syndromic scoliosis undergoing spinal deformity correction. Summary of Background Data. Syndromic scoliosis (SS) refers to scoliosis that is most commonly associated with systemic disease including Ehler Danhlos syndrome (EDS), Marfan syndrome (MF), Down syndrome (DS), Achondroplasia (AP), and Prader-Willi syndrome (PWS). Limited data exist evaluating hospital outcomes in patients with SS undergoing spinal deformity correction. Methods. The Kids’ Inpatient Database (KIDS) was queried from 2001 to 2012 to identify all pediatric patients with scoliosis undergoing spinal fusion. These patients were then sub-divided into two cohorts: (1) patients with idiopathic scoliosis (IS) and (2) patients with syndromic scoliosis. Trends in surgical management, and postoperative morbidity and mortality were assessed. Length of stay and total hospital charges were additionally analyzed. A sub-analysis to characterize outcomes in each syndrome was also performed. Results. An estimated 1071 patients with SS were identified and compared with 24,989 pediatric patients with IS. MF (36.8%), Down syndrome (16.0%), and PWS (14.9%) were the most common diagnoses among patients with SS. Between 2001 and 2012, there was a significant decline in the number of anterior procedures performed in both cohorts. Conversely, the number of posterior based procedures increased. SS was associated with increased major complications (2.7% compared with 1.0% in IS; P < 0.001) and minor complication rates (41.0% compared with 28.5% in IS; P < 0.001). Patients with AP incurred the highest rate of major complications (10.7%), minor complications (60.8%), and intraoperative durotomies (6.1%). Total hospital charges increased significantly over the 12-year span. Conclusion. Trends in management of syndromic scoliosis have paralleled that of idiopathic scoliosis. Syndromic scoliosis is associated with increased risks with surgical deformity correction. Further prospective studies are warranted to evaluate the reasons for these differences. Level of Evidence: 3

Musculoskeletal Diseases in Marfan Syndrome – A Nationwide Registry Study.

“…Compared to the background population, a relatively high number of patients with MFS had spinal surgery and it was performed at signi cantly younger age and mostly in women. These ndings are in accordance with other observational studies [12,21]. Scoliosis bracing for children and adolescents can be effective but growth-friendly spinal surgery may be necessary in many cases which includes staged surgery and growth friendly spine implants [22,23].…”

“…This huge burden of musculoskeletal disorders will affect most patients with MFS during their lifetime [9]. Many young patients will choose to have corrective surgery of the chest wall [10,11] and others will need treatment of their scoliosis at some point in life [12]. The aim of the present study was to assess the overall burden of musculoskeletal disease in patients with MFS.…”

Clinical Observations and Treatment Approaches for Scoliosis in Prader–Willi Syndrome

“…In addition, up to 62% of patients with PWS have low bone mineral density and other musculoskeletal manifestations [11,[42][43][44][45][46]. The combination of the PJK/DJK and bone weakness leads to a rate of hardware pull out/failure/rod fracture between 17% and 31% [9,40,41,47,48]. The consequences can be catastrophic [49].…”

Orthopaedic Management of Loeys-Dietz Syndrome: A Systematic Review

“…Future clinical studies will be important to understand how orthopaedic outcomes may coincide or differ among such patients and to what extent previous data from patients with other connective tissue disorders may inform care and prognosis for patients with LDS. 43 - 45 …”