Children With Esophageal Atresia : Long term outcome

Abstract

Abstract

Purpose The aim of the study was to report long term outcome in esophageal atresia (EA) patients by a study of the literature. Special emphasis has been put on the potential association between esophageal atresia, gastroesophageal reflux, esophagitis and development of esophageal cancer. In addition, long term function in 21 adolescents with EA treated at Rikshospitalet University Hospital was assessed.

Methods A Pub Med search for the period January 1988 until September 2008 was made identifying studies using the terms esophageal atresia, gastroesophageal reflux, respiratory function, scoliosis, quality of life, long term outcome and esophageal cancer. The terms were combined. 21 EA adolescents were included in a patient study. Symptoms from the respiratory and gastrointestinal tract as well as quality of life were assessed at long term follow up by a specialized nurse and a pediatric surgeon different from the surgeon operating the patients in the neonatal period.

Results In long term follow up studies 30 % - 80 % of EA patients report symptoms from the respiratory tract, and pulmonary function is impaired in 10 % – 70% of patients. Dysphagia was reported in 30% - 60% of the patients and GER in 10% to 70% of patients. Chest wall asymmetry was reported in 25% of EA patients in one study. A majority of EA patients report a good quality of life. However increased risk for learning, emotional and behavior problems was reported in one study. Also, one study found that mental health and psychosocial functioning may be associated with declined health and esophageal dilatations. 6 cases of esophageal cancers in EA patients have been reported. On long term follow up of 21 adolescents, 16/ 21 (76%) reported symptoms from the respiratory tract. 6/ 21 (29 %) reported symptoms of gastroesophageal reflux and dysphagia was reported by 10/21 (48 %). 7/21 (35%) were below the 25% percentile on weight by height adjustments. A majority of EA patients (16/21, 76%) have a good quality of life.

Conclusion Respiratory tract symptoms in 76% of EA adolescents on follow up seem high compared to the reports in the literature. This may be due to a rather extensive detailed questionnaire. Prevalence of dysphagia and GER were the same in the Norwegian population as reported in the literature. Six cases of esophageal carcinomas have been identified in EA patients. In general, EA patients do not seem to have increased risk of esophageal cancer. However, more research is required to confirm this. In spite of the challenges EA patients may have in the early years, most patients enjoy a good quality of life.

Key words Esophageal atresia – long term outcome – respiratory function – GER – esophageal cancer