Esophageal lung associated with VACTERL anomaly: a case report

Abstract

Esophageal lung is an extremely rare type of bronchopulmonary foregut malformation in which the main stem bronchus is anomalously connected to the esophagus instead of the trachea and is supplied by branches of pulmonary artery. Less than 25 cases have been reported so far in literature. We hereby report first case of esophageal lung associated with VACTREL anomaly. An 8-month-old female child with weight of 4.7 kg presented with recurrent lower respiratory infections. She was diagnosed to have esophageal lung on basis of clinical examination, chest X-ray f/b computed tomogram of chest and esophagobronchoscopy. There was a bifid vertebra at D9 level with scoliosis, an ectopic left kidney and dextrocardia with patent foramen ovale. Thus, this case was classified as having VACTREL association. Patient was successfully operated for excision of the hypoplastic lung via thoracotomy. She recovered well and gaining weight at 3 months f/u without any respiratory infection since surgery. High index of suspicion is necessary for the diagnosis of esophageal lung when common causes of recurrent respiratory infections are ruled out.

Bronchiectasis without lower respiratory symptoms in the presence of multisystem anomalies – a clinical clue to diagnose esophageal lung anomaly

“…Multiple congenital anomalies have been reported in association with CBPFM. Group-I is always associated with EA/TEF [6,9,[11][12][13]. In the index case, we noticed following associated anomalies -scoliosis with vertebral segmentation anomalies C-3/4 and D-1/2, 13 pairs of ribs and inferior crossed fused ectopia of left kidney.…”

Esophageal lung with rare associated vascular and anorectal malformations

“…No abnormal narrowing in the rest of the esophagus and no fistula communicating with the trachea or left bronchus was found. There have been case reports of CBPFM with VACTERL (vertebral defects, anal atresia, cardiac defects, tracheoesophageal fistula, renal anomalies, and limb abnormalities) association [13] , [14] , [15] , as well as a report of CBPFM with pulmonary artery sling [16] . However, to our knowledge, this is the first reported case of CBPFM presenting as imperforate anus, a right esophageal lung with an absent right bronchus (group II), pulmonary artery sling, and an interrupted inferior vena cava with azygos continuation.…”

Esophageal lung-clinical images

“…[ 1 ] Till date, <25 case reports have emerged in the English literature. [ 2 ] In this anomaly, one of the bronchi communicates directly with the esophagus instead of the trachea. The right side lung is frequently involved, possibly owing to the proximity of the right bronchus to the esophagus.…”