Pulmonary capillary haemangiomatosis causing pulmonary arter
Pulmonary capillary haemangiomatosis (PCH) is a rare cause of pulmonary hypertension which poses a considerable diagnostic challenge.

Published in BMJ, the authors describe the case of a 14-year-old boy with gradually progressive exertional dyspnoea and cough for past 4 months. Frontal chest radiograph demonstrated prominent pulmonary artery segment with diffuse nodular opacities in bilateral lung fields.

CT angiography was performed to diagnose the aetiology of pulmonary artery hypertension. It revealed dilated central pulmonary arteries with multiple ill-defined centrilobular nodules diffusely distributed in both lungs. No interlobular septal thickening, pulmonary thromboembolism, pleural effusion or any intracardiac/extracardiac shunt was seen. A provisional diagnosis of PCH was made which was subsequently confirmed on a lung biopsy.

Learning points
• Pulmonary capillary haemangiomatosis (PCH) is an uncommon cause of pulmonary hypertension with non-specific clinical presentation, overlapping with primary pulmonary hypertension and pulmonary veno-occlusive disease.

• Characteristic imaging findings include the presence of diffuse, ill-defined, centrilobular nodules or ground-glass opacities with scarce septal lines and the presence of haemorrhagic pleural effusions.

• Precise diagnosis is crucial as a standard treatment of pulmonary hypertension, including vasodilator therapy or prostacyclin infusion, can have catastrophic consequences in these patients.


Read more here: http://casereports.bmj.com/content/2018/bcr-2018-227574.full
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