Spinal fusion in children with osteogenesis imperfecta: A nationwide retrospective comparative cohort study over a 12-year period

Background: Osteogenesis imperfecta (OI) is an uncommon disorder frequently associated with spinal deformity. Relatively little is known about the effect this disorder has on the outcomes of spinal fusion for spinal deformity. The purpose of this study was to evaluate the differences in treatment and outcomes of this unique patient population. Methods: The nationwide HCUP Kids' Inpatient Database (KID) from 2000 to 2012 was queried to identify patients with OI who underwent spinal fusion surgery for treatment of scoliosis. Demographic variables were obtained along with length of stay, mortality, length of fusion, transfusion rates, and hospital charges. Control data consisting of pediatric patients who underwent spinal fusion for adolescent idiopathic scoliosis (AIS) were acquired and compared. Results: A total of 60 patients (average age of 13.2 yr) with OI underwent spinal fusion during the study period. Of the patients who underwent fusions, 63.3% had involvement of nine or more vertebrae, which was significantly higher than patients with AIS (35.7%: P<0.001). Patients with OI also had a higher average length of stay (8.1 vs. 5.7 days: P<0.001). Nearly half of the patients with OI required blood transfusions, which was significantly more than patients with AIS (43.3% vs. 30.4%; P=0.029). Patients with OI also experienced significantly higher hospital charges versus controls ($173,652 vs. $46,953; P<0.0001). Conclusions: Spinal deformity surgery in patients with OI is a technically demanding procedure with high morbidity and high cost. In this nationwide series, a higher rate of blood transfusions, longer hospital stays, and significantly higher hospital charges were identified compared to patients with AIS.