Type II congenital pulmonary airway malformation in an esophageal lung

esophageal lung. Can respir J 2013;20(3):e52-e54. A seven-month-old girl, born prematurely (birth weight 1000 g) from a twin pregnancy, was admitted to hospital due to recurrent pneumonia and atelectasis. She experienced cough and respiratory distress during feeding. The right hemithorax was smaller than the left, with diminished breath sounds and dullness. Chest x-rays revealed decreased lung volume and multiple radiolucent images in the right lung, as well as overdistention of the left lung. An esophagogram revealed three bronchial branches arising from the lower one-third of the esophagus, corresponding to the right lung and ending in a cul-de-sac. A diagnosis of esophageal lung was established. On bronchography, the right lung was absent and the trachea only contin ued into the left main bronchus. Echocardiography and angiotomography revealed agenesis of the pulmonary artery right branch. The surgical find ing was an esophageal right lung, which was removed; the histopathologi - cal diagnosis was type II congenital pulmonary airway malformation in an esophageal lung.

T he esophageal lung or esophageal bronchus is characterized by an aberrant bronchus or lung that arises from the esophagus, with total absence of ventilatory function in the involved pulmonary tissue (1)(2)(3)(4)(5). The present report describes an unusual case of a type II congenital pulmonary airway malformation (CPAM) that occurred in an esophageal lung (6).

CASE PrESEntAtion
The patient was a seven-month-old girl born after 34 weeks' gestation in a twin pregnancy, with a birth weight of 1000 g. In the neonatal period, she developed pneumonia and required mechanical ventilation for 40 days, and was discharged eight days later. She was hospitalized on three more occasions due to pneumonias and was sent to the Hospital de Pediatría, Centro Médico Nacional Siglo XXI (Mexico DF) for evaluation and treatment.
On admittance, her weight was 2900 g, oxygen saturation was 92% on room air, with respiratory distress, asymmetrical thorax due to a smaller size of the right hemithorax. She arrived without an orogastric tube and, thus, previous oral feeding was assumed, but she experienced an episode of choking after trying to initiate by oral route.
A chest radiograph revealed a pencil-like tapering of her tracheal air column, and the right lung was smaller and with multiple radiolucent images compared with the left lung, which was overdistended ( Figure 1). Bronchoscopy revealed 20% subglottic stenosis and progressive diminution of tracheal lumen and, thus, the carina could not be visualized. Endoscopy showed incompetent hiatus. In contrasted images, the barium swallow and a Valsalva manoeuvre revealed a three-branch ramification emerging from the lower one-third of the esophagus, which ended in a cul-de-sac, establishing the diagnosis of esophageal lung ( Figure 2). An echocardiogram showed probable agenesis of the right pulmonary artery, neoformation of blood vessels in the esophageal lung and venous return through pulmonary veins draining to the right atrium. Angiotomography with three-dimensional reconstruction clearly showed diminution of the tracheal diameter, which continued with the left main bronchus, and the esophageal lung emerging from the esophagus (with an orogastric tube inside); the right lung had clInIco-paThologIc conferences ©2013 Pulsus Group Inc. All rights reserved A seven-month-old girl, born prematurely (birth weight 1000 g) from a twin pregnancy, was admitted to hospital due to recurrent pneumonia and atelectasis. She experienced cough and respiratory distress during feeding. The right hemithorax was smaller than the left, with diminished breath sounds and dullness. Chest x-rays revealed decreased lung volume and multiple radiolucent images in the right lung, as well as overdistention of the left lung. An esophagogram revealed three bronchial branches arising from the lower one-third of the esophagus, corresponding to the right lung and ending in a cul-de-sac. A diagnosis of esophageal lung was established. On bronchography, the right lung was absent and the trachea only continued into the left main bronchus. Echocardiography and angiotomography revealed agenesis of the pulmonary artery right branch. The surgical finding was an esophageal right lung, which was removed; the histopathological diagnosis was type II congenital pulmonary airway malformation in an esophageal lung.
The intraoperative findings were right lung with main bronchus arising from the esogaphus, with remnant veins in its hilum and lacking systemic arterial blood supply. A right pneumonectomy was performed. The histopathological analysis reported a type II congenital cystic adenomatoid malformation (Figure 6), corresponding to type II CPAM in the current nomenclature (6).