Organ System Anomalies Associated With Congenital Scoliosis: A Retrospective Study of 305 Patients

Design Retrospective analyses of congenital scoliosis patients at 2 tertiary care pediatric hospitals. Objective This study objectives were (1) to report the rates of anomalies of 10 organ systems in congenital scoliosis patients and (2) to determine whether the presence of a single organ system anomaly increases the rate of nonspinal organ system defects. Summary of background data Intraspinal, cardiac, renal, and gastrointestinal anomalies have been reported to occur at higher rates in congenital scoliosis than the normal population. It is unknown whether the presence of 1 organ system defect increases the risk of nonspinal organ system anomalies. Methods All patients diagnosed, evaluated in the outpatient setting, with congenital scoliosis who were below 18 years of age at time of presentation with available cardiac echo, renal ultrasound, or magnetic resonance imaging were included in this study. Results There were 305 patients (161 females, 53%) whose mean age was 7+3 years. In total, 84% of patients were observed to have at least 1 organ defect. Overall, 22% of patients had 1 organ defect, 19% had 2, 18% had 3, and 18% had ≥4 organ defects. There was an average of 2.2 anomalies per patient (range=0 to 8). Intraspinal anomalies were documented in 43% of patients; syrinx occurred most frequently. Urogenital anomalies were documented in 39% of patients; solitary kidney was most prevalent. Cardiac anomalies were documented in 54% of patients; ventricular septal defect was most common. In 12% of patients, the triad of spinal, urogenital, and cardiac defects was observed. Multiple organ systems were found to have significant associations (P Conclusions Cardiac anomalies were the most common defect (54%), and occurred at a rate >2 times higher than previously reported. The high rate of intraspinal, cardiac, and urogenital defects makes magnetic resonance imaging, echocardiography, and renal ultrasound a critical part of evaluation for all congenital scoliosis patients. Level of evidence Level II.