Pulmonary capillary haemangiomatosis diagnosed during life

A 12-year-old girl was admitted for evaluation of recurrent haemoptysis for 3 months. A chest radiograph showed minimal signs of an interstitial pattern in the right basal region and a small left lung. Bronchoscopy did not reveal the location of the haemorrhage. but a ventilationperfusion scan established a defect in the left lower lobe, lingula and part of the pars superior of the upper lobe. Pulmonary function tests suggested a restrictive defect; the arterial blood gas analysis a t rest was normal. A coagulopathy could be excluded. Echocardiography revealed no evidence for a congenital abnormality, nor for an increased pulmonary arterial pressure. Recurrent severe haemoptysis necessitated a left pneumonectomy. There were no complications post-operatively. Two and a half years