Chronic vomiting and recurrent pneumonia in an adolescent female

A 17-year-old girl presented with episodic vomiting associated with chest pain, a 20-pound weight loss over the past year, and multiple hospitalizations for pneumonia. She was bradycardic, cachectic (<3rd percentile), pale, and had anterior cervical lymphadenopathy. CT angiography suggested an obstructive vascular ring (formed by a right aortic arch with an aberrant left subclavian artery), diverticulum of Kommerell, and a tracheoesophageal fistula (TEF). She underwent left thoracotomy with ligation and division of the ligamentum arteriosum to relieve the vascular ring. Flexible bronchoscopy at the time demonstrated a large H-type TEF. Eight weeks later, she had the TEF closed via a right cervical incision and recovered uneventfully. Our case is unique, with symptomatic presentation of a congenital TEF and vascular ring in a teenager. Such major congenital anomalies are rarely discovered outside of childhood, and TEFs virtually always (>90%) present as neonatal emergencies secondary to esophageal obstruction. They have a high incidence of associated abnormalities, cardiovascular being the most common. Unexplained recurrent respiratory symptoms in an otherwise normal child with dysphagia should prompt the clinician to evaluate patients for foreign bodies, reflux and other more common problems. Unusual etiologies, however, do occur - as in this case - and warrant more complex workup.