Published online November 9, 2009
PEDIATRICS (doi:10.1542/peds.2008-3704)
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Articles

Natural History of Spinal Anomalies and Scoliosis Associated With Esophageal Atresia

Saara J. Sistonen, MDa,b, Ilkka Helenius, MD, PhDa, Jari Peltonen, MD, PhDa, Seppo Sarna, PhDc, Risto J. Rintala, MD, PhDa and Mikko P. Pakarinen, MD, PhDa

aSection of Pediatric Surgery, Hospital for Children and Adolescents, Helsinki University Central Hospital, Helsinki, Finland;
bDepartment of Surgery, Päijät-Häme Central Hospital, Lahti, Finland; and
cDepartment of Public Health, University of Helsinki, Helsinki, Finland

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° was observed in 56 patients, >20° in 11 patients, and >45° in 1 patient. The risk for scoliosis of >10° was 13-fold (OR: 13 [95% CI: 8.3–21]), and the risk for scoliosis of >20° 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.

Key Words: congenital abnormalities/anomalies • outcome • scoliosis

Abbreviations: CI, confidence interval

Accepted Jun 15, 2009.


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