A Rare Case Report of Esophageal Lung Associated with VACTERL Anomaly

Case Report

J Pediatr & Child Health Care. 2017; 2(1): 1011.

A Rare Case Report of Esophageal Lung Associated with VACTERL Anomaly

Loganathan PK¹*, Nair V³, Albrashdi Y³ and Boulton J4

¹Department of Neonatology, Queen Elizabeth University Hospital, Scotland

²Department of Neonatology, University Hospital of Crosshouse, Scotland

³Department of Radio-Diagnosis, Alberta Children’s Hospital, Calgary, Canada

4Department of Pediatrics, Section of Neonatology, Alberta Children’s Hospital, Canada

*Corresponding author: Prakash Kannan Loganathan, Department of Neonatology, Queen Elizabeth University Hospital, Maternity Building, Glasgow, Scotland

Received: December 20, 2016; Accepted: January 19, 2017; Published: January 23, 2017


Background: Communicating Broncho-pulmonary-foregut malformation is one of the rare anomalies and esophageal lung is one among them.

Case Report: We report on a preterm infant with right esophageal lung associated with VACTERL anomaly.

Conclusion: This case is to report this rare association, and its unique clinical presentation and diagnosis.

Keywords: Esophageal lung; Broncho-pulmonary foregut malformation; VACTERL


CBPFM: Communicating Broncho-Pulmonary Foregut Malformation; CT: Computed Tomography; ECHO: Echocardiogram; TEF: Tracheo-Esophageal Fistula; VACTERL: Vertebral defects, Anal atresia, Cardiac, Tracheo-Esophageal Fistula (TEF) with Esophageal atresia, Renal dysplasia and Limb deformities; VATER: Vertebral defects, Anal atresia, Tracheo-Esophageal Fistula (TEF) with Esophageal atresia and Renal dysplasia


Anomalous origin of bronchus from the esophagus is rare and also known as the esophageal lung. It is one of the rare anomalies in the group of “Communicating broncho-pulmonary-foregut malformation”. The esophageal lung is associated with higher incidence of esophageal atresia, Tracheo-Esophageal Fistula (TEF) and VACTERL (Vertebral defects, Anal atresia, Cardiac defects, Tracheo-esophageal fistula, Renal anomalies, and Limb abnormalities) [1]. We report an extremely rare case of a preterm infant with right esophageal lung associated with VACTERL anomaly. This rare association, difficulty in diagnosis and unique clinical presentation is the focus of this case report.

Case Report

A 30week preterm female infant was born to a 23-year-old mother after the uneventful pregnancy. Her antenatal scan showed mild dilation of cerebral lateral ventricles, with bilateral foot abnormality, and probably sacral dysplasia. She was delivered in the special care baby unit by cesarean section, indicated for fetal distress. She was intubated at birth and post-intubation chest X-ray showed the complete collapse of the Right lung with mediastinal shift (Figure 1). After receiving one dose of surfactant and baby was transferred to tertiary neonatal intensive care unit for ongoing management. At admission she was noted to have dysmorphic features like low set ears, arthrogryposis, right lower limb dysplasia (fibula agenesis) with poly-syndactyly, and bifid great toe on the left leg. As a part of congenital anomaly work up we carried out various investigations. Chest X-ray showed adequately expanded right lung, multiple fused and segmented thoracic spine vertebrae (Figure 2). Echocardiogram (ECHO) showed type 1 truncus arteriosus with large VSD, and dysplastic truncal valve. She had a normal cranial ultrasound and renal ultrasound showed right kidney hydronephrosis. The gastric tube could not be passed beyond 8cm and the presence of dilated stomach on abdominal radiography confirmed the diagnosis of esophageal atresia with tracheaesophageal fistula (Figure 2). On day 3 of life fistula was ligated along with gastrostomy tube insertion and during bronchoscopy baby was noted to have small stenotic right main bronchus. Post surgery she noted to have a complete collapse of the right lungs. There was no improvement with antibiotics, lung recruitment strategies and alpha dornase nebulisation. Chest Ultrasound showed minimal pleural effusion, not accounting for the complete collapse. With computed tomography scan, right main bronchus and upper lobe bronchi were identified but could not identify bronchus intermedius and its branches. Right middle lobe and lower lobes are completely opacified. Right lung had normal pulmonary artery supply and pulmonary vein drainage. While planning for repeat bronchoscopy, babe also had issues with the progression of gastrostomy tube feeding. Contrast study through gastrostomy tube showed the abnormal orientation of right bronchus intermedius, which formed a communication with the distal part of the esophagus (Figure 3). With the preferential passage of contrast into the bronchus rather than duodenum and gastric outlet obstruction was suspected. With the presence of vertebral, cardiac, Tracheo-esophageal fistula, right kidney hydronephrosis and limb anomalies- VACTERL association was diagnosed. In view of truncus arteriosus and the need for right pneumonectomy, Baby was transferred to cardio-thoracic surgery center. CT angiogram showed markedly hypoplastic left pulmonary artery. With the constellation of multiple anomalies, multi-disciplinary consultations were made with cardiac surgery, cardiology, General surgery, neonatology and family. The decision was taken for palliative care and the baby passed away comfortably on day seventy-one of life. The parents deferred autopsy.