Health-related quality of life in adult patients with esophageal atresia—a questionnaire study

Abstract

Background/Purpose Long-term sequelae caused by associated anomalies or respiratory and gastrointestinal disorders are common after the repair of esophageal atresia (EA). The aim of this study was to assess the effect of these sequelae on the health-related quality of life (HRQoL) of patients with EA. Methods A questionnaire including a 36-item Gastrointestinal Quality of Life Index, a 15-item Respiratory Symptoms–Related Quality of Life Index, and a 36-item psychosocial survey and a symptoms query was sent to 159 patients with EA with or without tracheoesophageal fistula (TEF), who were operated on between 1949 and 1979, and to 400 healthy control subjects. A 36-item Health Survey Form was sent to patients with EA only and the results were compared with values of the general Finnish population. Results A total of 128 patients with EA (80%) and 162 control subjects (41%) returned the questionnaire. Median age [patients with EA, 38 (range, 24-54) years; control subjects, 36 (20-56) years] and sex distribution (M/F of patients with EA, 57:70; control subjects, 63:99) were comparable. Of the 128 patients, 115 (91%) had EA with a distal TEF, 8 (6%) had EA without TEF, 3 (2%) had EA with proximal and distal TEF, and 2 (1%) had TEF without EA. The types of esophageal repair were distributed as follows: primary end-to-end anastomosis (n = 112), colon interposition (n = 10), gastric tube (n = 3), thoracic skin tube (n = 1), and fistula closure only (n = 2). Thirty-eight patients had significant associated anomalies. Gastrointestinal Quality of Life Index scores did not differ statistically between patients with EA and control subjects. However, the incidence of regurgitation (17% vs 10%) and dysphagia (10% vs 2%) was significantly higher in patients with EA ( P < .5). Low Respiratory Symptoms–Related Quality of Life Index was found in 10 of 128 patients with EA and in 3 of 163 control subjects ( P < .05). Psychosocial survey scores and the incidence of acquired diseases did not differ between the groups ( P = NS). The 36-item Health Survey Form indicated low HRQoL in 19 (15%) of patients with EA (expected value, 16%). Of the 19 patients with EA with low HRQoL, 8 (42%) had significant congenital or EA-associated diseases and 11 (58%) had acquired diseases. The type of EA or esophageal conduit was not related to HRQoL. Conclusions Most adult survivors of EA or TEF repair have a normal quality of life. Morbidity from esophageal functional disorders and respiratory disorders with or without acquired diseases impairs HRQoL in 15% of patients with EA.