About this Research Topic

Background

Oesophageal Atresia-Tracheoesophageal Fistula (OA-TOF) is one of the most common digestive malformations occurring in 1 in 2,400 to 4,500 births worldwide. Since the first successful primary repair by Cameron Haight in 1941, postoperative outcomes have changed. The focus has shifted from mortality to morbidity and quality of life issues. OA-TOF is no more just a neonatal surgical problem but a life-long problem. The improvement of survival observed over the previous two decades is multifactorial and largely attributable to advances in neonatal intensive care, neonatal anesthesia, ventilatory and nutritional support, antibiotics, early surgical intervention, surgical materials and techniques. Indeed, mortality is currently limited to those cases with coexisting severe life-threatening anomalies. The diagnosis of OA-TOF is most commonly made during the first 24 h of life but may occur either antenatally or may be delayed. The primary surgical correction for OA-TOF is the best option in the absence of severe malformations. There is no ideal replacement for the esophagus and the optimal surgical treatment for patients with long-gap OA is still controversial. The primary complications during the postoperative period are leak and stenosis of the anastomosis, gastroesophageal reflux, esophageal dysmotility, fistula recurrence, respiratory disorders including tracheomalacia and “cyanotic spells” and deformities of the thoracic wall. Other than respiratory problems, nutritional and gastrointestinal issues are prevalent not only in the first years of life but also in adolescence and adulthood. Gastroesophageal reflux disease (GORD), peptic esophagitis, gastric metaplasia and Barrett esophagus, anastomotic strictures (early as well as late), feeding disorders, dysphagia, esophageal dysmotility are the most frequent GI short and long- term complications encountered in children and adolescents. These GI omplications significantly affect their quality of life. Concerns in adults include oesophageal adenocarcinoma and epidermoid carcinoma which have been recently been reported. This highlights the need for careful multidisciplinary follow up not only in childhood but also after transition to adulthood. Data regarding long-term outcomes and follow-ups are limited for patients following OA-TOF repair. The determination of the risk factors for the complicated evolution following OA-TOF repair may positively impact long-term prognoses. Much remains to be studied regarding this condition.

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