Spontaneous Esophageal Perforation 37 Years after Primary Repair of Esophageal Atresia

Introduction

Esophageal atresia (EA) with tracheoesophageal fistula (TEF) is a common congenital anomaly, affecting 1 in 2 400 to 4 500 live newborns [7]. It has been demonstrated that it occurs due to impaired normal tracheal development, with subsequent development of the foregut into a trachea rather than an esophagus during organogenesis [12].

Long-term follow-up of patients with EA with or without TEF has shown that gastrointestinal and respiratory symptoms are the most significant problems, with a reported incidence of 30–60 % in adult survivors. The most important gastrointestinal disorders are gastroesophageal reflux (GER) and dysphagia. Reflux symptoms may be alleviated over time; however, some patients develop chronic esophagitis and Barrett's esophagus [4], [17], [18]. Respiratory symptoms are thought to be more severe in childhood and eventually improve in adolescence.

Spontaneous esophageal rupture, or Boerhaave's syndrome, is a rare condition. In many instances it is associated with violent retching and vomiting that causes a sudden increase in intra-esophageal pressure [8]. Although cases of cervical esophageal rupture have been reported [1], the left side of the lower esophagus is affected in 90 % of patients. We report the first case of spontaneous esophageal perforation in an adult after primary repair of EA with TEF.

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