Congenital broncho-oesophageal fistula in the adult.

Abstract

The case of a 46-year-old man with congenital broncho-oesophageal fistula is presented. The patient had had recurrent chest infections since childhood and a recent history of heartburn and flatulence. A barium-swallow examination showed a small sliding hiatal hernia and an oesophageal diverticulum communicating via a fistula with the apical and posterior segmental bronchi of the left lower lobe. Bronchography showed bronchiectasis in this lobe. At thoracotomy resection of the broncho-oesophageal fistula and left lower lobectomy were performed. The patient's recovery was uneventful. Twenty published cases are reviewed. The classification of congenital broncho-oesophageal fistulae should take account not only of the type of fistula but also of the type and size of the bronchus with which the fistula communicates, the age of the patient at the first appearance of symptoms, and the duration of symptoms.The common origin of the respiratory tract and the oesophagus from the same embryonic structures sometimes gives rise to anomalous communications such as tracheo-oesophageal fistula and bronchooesophageal fistula. Most patients with tracheooesophageal fistula associated with oesophageal atresia present in infancy.' In patients who have congenital fistula without oesophageal atresia symptoms are usually insidious in onset and the patients may present in adult life."' The rarity of congenital broncho-oesophageal fistula in adults has been emphasised by various authors and by 1979 there were probably no more than 75 published cases. A barium-swallow examination showed a small sliding hiatal hernia and an oesophageal diverticulum in the middle third of the oesophagus. The contrast medium spilled into the left lower lobe through a fistulous communication between the oesophageal diverticulum and the apical and posterior basal segmental bronchi (fig 1). Bronchography showed bronchiectasis in the left lower lobe, particularly noticeable in the apical and posterior segments. There was no evidence of sequestration or leakage of the contrast medium into the oesophagus. Bronchoscopy showed an inflamed orifice of the apical segmental bronchus of the left lower lobe. At oesophagoscopy the oesophagus appeared normal and the broncho-oesophageal fistula could not be located. Methylene blue (3 ml) injected into the left-lower-lobe bronchus did not appear in the oesophagus.At operation (a left thoracotomy) there was a fistulous communication between the mid-portion of the oesophagus and the apical segment of the lower lobe (fig 2). The fistula was about 2 cm long and 0-4 cm in diameter. The fistula was excised and the oesophageal end repaired. Left lower lobectomy was performed. The small hiatal hernia was not repaired. The postoperative period was uneventful

Unilateral Bronchiectasis and Esophageal Dysmotility in Congenital Adult Tracheoesophageal Fistula.

“…Different theories account for the absence of symptoms in earlier stages of life: a) delay in rupture of an occlusive membrane that covers the tract until adulthood; b) presence of a mucosal fold that functions as a valve; after years of chronic inflammation, the valve becomes dysfunctional and the fistulous tract opens; c) the tract, which is oblique, closes during swallowing, preventing chronic aspiration; d) the tract is endowed with a layer of smooth muscle, which upon contraction can occlude the lumenof the tract. The contraction, subsequent closure, and the obliquity of the tract can all be reasons for the late appearance of the symptomsuntil later in the adulthood, as was seen in the present patient (9). The above explanations, in conjunction with the physician unfamiliarity, can postpone the diagnosis until the third or fourth decade of life.…”

“…Most of the cases have been reported in patients in their thirties with no predilection toward either gender. Coughand chronic respiratory tract infections are symptoms that may raise suspicion (8,9). Some patients may have subtle symptomsor be even asymptomatic until later into adulthood.…”

“…The majority have cough on swallowing liquids or have food in the sputum as their cardinal respiratory manifestations (9). Due to spillage of gastric contents into the respiratory tract, repeated bouts of respiratory tract infections occur which in the long-run could result in bronchiectasis and lung abscesses (10).…”

An Interesting Rare Case of Benign Congenital Oesophago- Bronchial Fistula Presenting in Adulthood – A Case Report

“…Non-malignant oesophageal respiratory fistulas are relatively rare and may be congenital or acquired from trauma [8] BEFs commonly communicate with the right lung, and fewer cases have been reported of left lung BEFs. This has been hypothesized to be due to a thinner muscle layer and lack of soft tissue envelope around the esophagus below the carina, causing a predilection for fistula formation in the right lung lobes [9] BEF may, however, be silent for several decades for various reasons: (1) an occlusion of the opening by an oesophageal tissue fold or a 'flap valve'; (2) the presence of a membrane that subsequently ruptures; (3) the action of gravity (upward direction of the fistula from the oesophagus to the bronchi) preventing spillage of the oesophageal contents into the respiratory tree; (4) adaptation of patients to the minimal symptoms, and (5) spasm of the smooth muscle in the fistula wall [10,11,15] . However, none of these are well supported by pathologic or radiological findings .…”

“…A BEF is typically associated with repeated and persistent respiratory infections, which can lead to the development of bronchiectasis and coughing bouts when eating [11] . Hemoptysis is also seen occasionally.…”