Prevalence of scoliosis in patients with Fontan circulation

Objective: To examine the prevalence of scoliosis in patients with Fontan circulation. Design: Retrospective cohort (case-only), level IV study. Setting: A comprehensive paediatric centre. Methods: We examined the radiographs of 194 patients who underwent Fontan completion surgery between 1998 and 2011 at a median age of 1.3 years. Main outcome measure: We collected data on the age at the last available radiograph and when scoliosis was diagnosed; we used the first available radiograph with scoliosis. We also measured the magnitude and direction of the spinal curve in patients with scoliosis. Results: Median age at radiography was 3.4 years. Nineteen patients (9.8%) developed scoliosis; none of them underwent thoracotomy. Most of the patients with scoliosis were older than 5 years of age at scoliosis diagnosis and the female to male ratio was 5 : 3. The major curve was right thoracic in 12 patients (63.2%), left thoracic in four patients (21.1%) and high thoracic in three patients (15.8%). For every 1 year increase in age there was a 27% increased risk of developing scoliosis. Conclusions: There was a high prevalence of scoliosis in patients with Fontan circulation. We recommend interdisciplinary monitoring of these patients to diagnose spinal curve deformities in a timely manner.