Neonatal Left Pulmonary Artery Occlusion and Postinfarction Cysts of the Left Lung: Cause and Effect?

Excerpt

An 11-day-old male presented with tachypnea and fever of 2 days’ duration. He was found to be desaturated (85% saturation by pulse oximetry on room air) and he had no breath sounds on the left hemithorax. Chest x-ray showed extensive hyperlucency on the left with shift of the mediastinum to the right. Follow-up computed tomography (CT) scan with contrast revealed a large, lobulated, thick-walled cavity occupying most of the left hemithorax and connecting with the bronchial tree at the level of the left lower lobe (Fig. 1). Echocardiogram performed preoperatively demonstrated a large, highly echogenic mass at the take-off of the left pulmonary artery (LPA) with almost complete absence of the left pulmonary arterial blood flow (Figs. 2 and 3). Thrombosis of the LPA as the primary cause of the pulmonary pathology was considered likely and was confirmed at operation and subsequent histopathology examination, which showed more than 90% occlusion of the LPA with organizing thrombi, muscular hypertrophy, and dystrophic calcification. Multiple organizing thromboemboli involving distal pulmonary vessels with associated parenchyma infarction and pulmonary hypoplasia provided evidence for the intrauterine formation of the thrombus. A causal relationship between pulmonary infarction and cyst formation has been previously suggested [2]. Further inquiry into the family history revealed the presence of deep venous thrombosis in the mother and maternal grandmother, suggesting a pathogenetic link between the thrombotic tendency in the mother and the intrauterine LPA thrombus in the patient with subsequent postinfarction lung cysts [1]. The patient’s coagulation workup was normal, and he is currently well after a left pneumonectomy.

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