Esophageal atresia: Factors influencing survival - Experience at an Indian tertiary centre

Abstract

Objective:To study the clinical profile of the cases of esophageal atresia (EA) and/or tracheoesophageal fistula (TEF) and various factors affecting the surgical and early postoperative management and their outcome.Materials and Methods:A prospective analysis of 127 cases of EA from February 2004 to May 2006 was performed. Waterston prognostic criteria were used for grading.Results:EA with TEF was the commonest type in 117 cases (92%). Associated congenital anomalies were present in 52 (41%) patients, the commonest being the cardiac anomalies, which was followed by the gastrointestinal anomalies. VACTERL was found in 6 (5%) cases. Prematurity, associated congenital anomalies, gap between esophageal ends and preoperative respiratory status were the significant factors affecting the survival (P = < 0.001). Primary extrapleural repair was the surgical approach in most of the patients. Azygos vein was preserved in 46 cases and no retropleural drainage was used in 27 cases. Staged procedures were performed in 19 cases, including 6 cases of isolated esophageal atresia. Pneumonitis and sepsis were the most common early postoperative complications (42%). Hypoxia and cardiorespiratory arrest were the most common causes of mortality (11 cases). Anastomotic leak complicated 13 cases, including 9 major and 4 minor leaks. Major leak followed by sepsis caused 7 deaths. Survival as per Waterston criteria was 100% in group A, 83% in group B and 22% in group C.ConclusionFactors affecting the survival are major or life-threatening associated anomalies, long gap, pneumonia and sepsis at presentation or that acquired during hospitalization and major leaks. The high incidence of low birth weight, delayed diagnosis, poor referral, low-socio economic status and lack of advanced neonatological back up are important contributory factors to poor outcome.

A scoring system to predict mortality in infants with esophageal atresia

“…The diagnosis of EA was based on the clinical manifestations and chest X-rays using air as the contrast in the proximal pouch to avoid aspiration of the contrast fluid (esophagography) or chest computed tomography with 3-dimensional reconstruction. [ 6 , 18 ] The classification of the EA anomalies was based on the Gross classification system. [ 18 ]…”

“…The gap length between 2 esophageal pouches was classified as a short gap (<1 cm or 1 vertebral body), an intermediate gap (1–3 cm or 1–3 vertebral bodies), or a long gap (>3 cm or 3 vertebral bodies). [ 6 ] Congenital heart disease (CHD) was diagnosed by 2-dimensional echocardiography performed by pediatric cardiologists who were also responsible for the categorization of the cardiac defects. Major CHD was defined as disease that might have an impact on the patient's well-being or the surgical course.…”

“…In most current reports excluding neonates with lethal congenital anomalies, neonates with EA have an expected survival rate of more than 90%. [ 3 , 4 ] In addition to fatal malformations, factors such as prematurity, [ 3 ] low birth weight, [ 3 – 7 ] sepsis, [ 8 – 10 ] respiratory system complications, [ 6 , 8 , 11 , 12 ] anastomotic leak, [ 6 , 13 ] chromosome abnormality disorders, [ 14 ] low socioeconomic status, and delayed diagnosis [ 6 ] are associated with high mortality in EA.…”

Simultaneous single-staged repair of anorectal malformation with tracheoesophageal fistula: Lessons learned

“…[1415] The improved outcomes are mainly due to advances in better antenatal care, neonatal intensive care, nutritional support, and improvement of anesthetic and surgical techniques. Along with these advances, concepts of the prognosis and therapeutic strategy for EA management have been changing.…”

Eyelid Coloboma in a Newborn with VACTERL Association

“…Morbidity is signifi cant following repair of trachea-oesophageal abnormalities. Anorectal and vertebral anomalies are associated with variable but poor functional outcomes in later life 15 .…”