Resumo

SIEGERT-OLIVARES, Augusto Ignacio et al. Pulmonary aplasia: report of two cases. Bol. Med. Hosp. Infant. Mex. [online]. 2015, vol.72, n.1, pp.66-70. ISSN 1665-1146.  https://doi.org/10.1016/j.bmhimx.2015.02.004.

Background: Pulmonary aplasia is a rare disorder of lung development characterized by the presence of a rudimentary main bronchus in the absence of lung and pulmonary artery. It is generally associated with other congenital defects and must be suspected in the presence of a total radiopaque hemithorax. Case reports: We describe two cases of pulmonary aplasia diagnosed in the Hospital Infantil de México "Federico Gómez" in the last 5 years. The first case was a female who presented respiratory distress from birth with a radiopaque left hemithorax in which the diagnosis of pulmonary aplasia was completed with bronchoscopy and lung scan. Her evolution has been insidious, characterized by chronic respiratory symptoms, oxygen dependence and pneumonias. The second case is a 5 year old female, who remained asymptomatic untill the age of two years when she was hospitalized for gastroeteritis. She underwet chest X-rays with findings suspicious of right diaphragmatic hernia. She was then transferred to our hospital. She underwent surgery at which time diaphragmatic eventration and no ipsilateral lung were found. The diagnosis was completed with a blind bottom main right bronchus in bronchoscopy. Conclusions: Pulmonary aplasia is an uncommon pathology. Due to great variability in clinical presentation, there must be a high index of suspicion in the presence of a fully radiopaque hemithorax. Among the diagnostic methods, X-rays, tomography and lung scan are useful. Bronchoscopy is required for diagnostic confirmation. Surgical removal of the stump and translocation of the diaphragm have been proposed as surgical options.

Palavras-chave : Pulmonary aplasia; Congenital malformation; Bronchoscopy.

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