Pulmonary capillary haemangiomatosis causing pulmonary arterial hypertension: a clinicians conundrum

Description 

Pulmonary capillary haemangiomatosis (PCH) is a rare cause of pulmonary hypertension which poses a considerable diagnostic challenge. Here, we 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 (figure 1A). CT angiography was performed to diagnose the aetiology of pulmonary artery hypertension. It revealed dilated central pulmonary arteries (figure 1B) with multiple ill-defined centrilobular nodules diffusely distributed in both lungs (figure 1C,D). 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.

Figure 1

Frontal chest radiograph (A) revealing prominent pulmonary artery segment with reticulonodular opacities predominantly involving the lower zones. No Kerley lines were noted....

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