Right sided lung agenesis with esophageal atresia and tracheoesophageal fistula

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Amit Kumar.

Consultant Pediatrician, Mahavir Vaatsalya Aspatal, Patna, Bihar, India.

DR. Amit Kumar, Bank Colony, Pharmacy College Road, Bailey Road, Patna-801503. India.
Email: [email protected]

A term male baby, vaginally delivered, with birth weight of 2.8 kg presented on D3 of life with provisional diagnosis of congenital pneumonia in view of opaque right hemi thorax. There was no significant antenatal event. On presentation, there was marked respiratory distress and excessive frothing from mouth. The orogastric (OG) tube could not be passed beyond 10 cm. There was no audible breath sound over right hemithorax. Chest X-ray showed opacification of right hemithorax with hyperinflated left lung and curved OG tube was visible. (Figure 1) CECT chest was done which showed similar findings. Baby underwent surgical repair where diagnosis of absent right lung and type c tracheoesophageal fistula (TEF) were confirmed. With right posterolateral thoracotomy approach, fistula was ligated and divided. Esophago-esophageal anastomosis was performed. Baby was kept on mechanical ventilation, but could not be weaned off and succumbed on D6 of life.

Figure 1: Chest X-ray showing opacification of right hemithorax with hyperinflated left lung and curved orogastric tube

The association of unilateral lung agenesis with esophageal atresia and tracheoesophageal fistula is extremely rare with a very lethal outcome. (1) Agenesis of lung is believed to be caused by failure to maintain development balance between the two lung buds. Congenital underdevelopment of the lungs was classified by Schneider. (2) Combination of TEF with pulmonary agenesis had been universally fatal until 1985 when first documented case of long term survival was reported. (3) Since then around 16 cases have been reported to survive beyond neonatal period. Verma et al reported a case who is surviving till 10 yrs of age for the same condition after surgery. (4)

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Funding None
Conflict of Interest None

  1. Harmon CM, Coran AG, O’ Neil J. Paediatric Surgery. 7th ed; 2012: 893-919.  [CrossRef]
  2. Schneider P, Schawatbe E. Die Morphologie der Missbildungen Des Menschen Under Thiere. Jena: Gustav Fischar. 1912; 3 Part.2: 817–822.
  3. Benson JE, Olen MM, Fletcher BD. A spectrum of bronchopulmonary anomalies associated with tracheoesophageal malformation. Pediatr Radiol. 1985; 15: 377-80.  [CrossRef]  [PubMed]
  4. Verma S, Mahajan JK, Rao KLN. Esophageal atresia and tracheoesophageal fistula with unilateral pulmonary agenesis-hypoplasia..J Neonatal Surg 2013; 2: 21.  [PubMed]

DOI: https://doi.org/10.7199/ped.oncall.2014.26

Cite this article as:
Kumar A. Right Sided Lung Agenesis with Esophageal Atresia and Tracheoesophageal Fistula. Pediatr Oncall J. 2014;11: 50. doi: 10.7199/ped.oncall.2014.26

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