Diagnostic imaging of congenital pulmonary aplasia in a dog

A 2-year-old, female Pomeranian dog was referred for dyspnea. Thoracic radiographs revealed left-sided mediastinal shift, increased soft tissue opacity in the caudal aspect of left thorax with loss of the left diaphragmatic silhouette, and dorsal elevation of mediastinal structures and heart from the sternum by lung tissue. The left main bronchus was visualized as an air-bronchogram and observed to abruptly discontinue at the level of the 10th rib. Thoracic computed tomography (CT) revealed absence of the left lung parenchyma and left pulmonary vessels with a rudimentary left main bronchus. The case was congenital pulmonary aplasia diagnosed via radiography and CT.

Aplasia pulmonar: a propósito de dos casos / Pulmonary aplasia: report of two cases

Introducción: La aplasia pulmonar es un raro trastorno del desarrollo pulmonar. Se caracteriza por la presencia de un bronquio principal rudimentario en ausencia de pulmón y arteria pulmonar. Generalmente se asocia con otros defectos congénitos, y debe sospecharse ante la opacidad del hemitórax. Casos clínicos: Se describen dos casos de pacientes con aplasia pulmonar diagnosticados en el Hospital Infantil de México Federico Gómez en los últimos 5 años. La primera paciente presentó dificultad respiratoria desde el nacimiento. Se observó radiopacidad total del hemitórax izquierdo, y se completó el diagnóstico de aplasia pulmonar con gammagrafía pulmonar y broncoscopia. La evolución ha sido insidiosa, con sintomatología respiratoria crónica, dependencia de oxígeno y neumonías recurrentes. La segunda paciente, de 5 años de edad, permaneció asintomática durante los primeros 2 años de vida. Fue hospitalizada por gastroenteritis infecciosa, y la radiografía sugirió hernia diafragmática derecha. Se intervino y se encontró eventración diafragmática derecha y ausencia de pulmón ipsilateral. Se completó el diagnóstico con broncoscopia que mostró bronquio principal derecho con saco ciego terminal. Conclusiones: La aplasia pulmonar es una entidad infrecuente. Debido a la variabilidad en la presentación clínica debe tenerse un alto índice de sospecha ante el hallazgo de la radiopacidad total del hemitórax. Los métodos diagnósticos que se utilizan son radiografía, tomografía y gammagrafía. Para confirmar el diagnóstico se requiere realizar broncoscopia. La escisión del muñón y la traslocación diafragmática se han descrito como opciones quirúrgicas de tratamiento.

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.

Unilateral Pulmonary Aplasia: A Case Report.

Chest X-Ray findings of unilateral lung or lobar collapse with a shift of mediastinal shift towards the affected side may prompt differential diagnoses of suspected foreign body aspiration, mucus plug occlusion, and bronchial mass lesions. We must also consider the rare condition of pulmonary agenesis. It is one of the rare congenital abnormalities in the development of the lungs in which there is complete absence of a lung. We report a three month old child with right sided pulmonary aplasia.