Abstract
Purpose: The aim of this study was to evaluate recent trends in the epidemiology, diagnosis and treatment of congenital anomalies of the esophagus.
Methods: A questionnaire survey regarding esophageal atresia (EA) and congenital esophageal stenosis (CES) treated over the last 10 years was carried out at pediatric surgical centers in the Kyushu, Okinawa, and Yamaguchi areas.
Results: Twenty out of 29 institutions (69%) answered our questionnaires. One hundred and ninety patients with EA and 39 patients with CES were thus recruited in this study. Five centers treated no patients with EA, one center treated 1–5 patients, 7 centers 6–10 patients, 5 centers 15–20 patients, and 2 centers 20–25 patients. Forty-seven out of 174 (27%) patients were diagnosed prenatally. Regarding the surgical approach, posterolateral incisions were performed in 60% of the centers. The overall survival rate was 73.7% in all patients. The survival rate in patients who underwent operation was 90.9%. Sixty-three percent of the infants weighed less than 2,500 g, and 14.7% weighed less than 1,500 g. Among the 39 patients with CES, the causes of CES were as follows: tracheobronchial remnants in 12 patients, fibromuscular stenosis in 10 patients, and membranous stenosis in 3 patients. The incidence of associated anomalies was 35.9%, and that associated with esophageal atresia was 15.4%. The onset of symptoms occurred in late infancy in 56.8% and in the neonatal period in 21.6%.
Conclusions: EA patients with primary anastomosis demonstrated a favorable prognosis. CES patients also developed symptoms and all such patients were diagnosed in the neonatal period.
