Isolated Congenital Bronchoesophageal Fistula in a Pediatric Patient: Case Report

Abstract

Abstract Introduction/Objective Congenital bronchoesophageal fistula (BEF) results from an undue passageway caudal to the foregut lung anlage. BEF may be associated with esophageal atresia and commonly involves the right lung. Herein we present a pediatric case of isolated congenital BEF masqueraded by severe dyspeptic symptoms. Methods A five-year-old child with a longstanding history of gastroesophageal reflux was hospitalized because of acute onset sharp lower chest/epigastric abdominal pain associated with emesis. Imaging disclosed a left lung abscess and empyema that responded to intravenous antibiotics, chest tPA instillation and drainage. After resolution of her empyema, she experienced severe dysphagia, chocking sensation, vomiting, and intermittent hematemesis. Esophogram disclosed a fistulous tract to the left lower chest cavity. Esophagoscopy revealed severe esophagitis and a stricture just proximal to a 0.6 to 0.8 cm ostium of a fistulous tract at 26 cm from the incisors, 2 cm above the gastroesophageal junction. Results A 103 g, 12 x 7.5 x 5.0 cm thoracoscopic left lower lobectomy showed a focally ulcerated fistulous tract that entered a 1.9 cm in diameter cyst that connected to a basilar-medial segmental bronchus. The passage was lined by stratified squamous epithelium, continuous with columnar ciliated epithelium, and was surrounded by intense chronic inflammation. The fistulous lumen contained partially degraded vegetable matter. Diagnosing BEF can be clinically challenging. Symptoms are often intermittent, alternating between digestive and pulmonary in nature. Often, cough, gastroesophageal reflux, pneumonia, and bronchopulmonary suppuration can lead to medical management that is only masking, not definitively treating the underlying pathology. While tracheoesophageal fistulas are relatively common in presentation, and usually diagnosed in the newborn period, BEFs are rare and with varied clinical expression, which may explain delayed diagnosis. Conclusion BEF is a rare anomaly that may manifest ambiguous symptoms, thus delaying the diagnosis and options for earlier, less invasive curative interventions.