Natural history of spinal anomalies and scoliosis associated with esophageal atresia

Abstract

Objective: Clinical characteristics of spinal deformities that commonly occur in patients with esophageal atresia (EA) are unclear. The objective of this study was to assess the incidence and natural history of spinal anomalies and scoliosis in patients with EA.

Methods: A population-based cohort of 100 adults who had an operation for EA in our hospital were examined clinically and radiographically for spinal deformities. The results were compared with data obtained from normal population-based controls.

Results: Vertebral anomalies were observed in 45 patients, predominating in the cervical spine in 38 patients. Any additional anomaly was the most significant risk factor (odds ratio [OR]: 27 [95% confidence interval (CI): 8-100]) for the occurrence of vertebral anomalies. Scoliosis of >10 degrees was observed in 56 patients, >20 degrees in 11 patients, and >45 degrees in 1 patient. The risk for scoliosis of >10 degrees was 13-fold (OR: 13 [95% CI: 8.3-21]), and the risk for scoliosis of >20 degrees was 38-fold (OR: 38 [95% CI: 14-106]) compared with those in the normal population. Thoracotomy-induced rib fusions (OR: 3.6 [95% CI: 0.7-19]) and other associated anomalies (OR: 2.1 [95% CI: 0.9-2.9]) were the strongest predictive factors for scoliosis. The general clinical course of spinal deformities was mild, and none of the patients had undergone spinal surgery.

Conclusions: The risk of scoliosis is 13-fold after repair of EA in relation to general population. Nearly half of the patients have vertebral anomalies predominating in the cervical spine. Most of these deformities were not diagnosed primarily or during growth. Spinal surgery is rarely indicated.