AV. Di Blasio*a (Dr), I. Boailchuka (Dr)


* andrejunin2008@hotmail.com

A two years old boy from the north of Argentina, previously healthy, with diagnosis of pneumonia of upper left lobe was admitted to Children Hospital, Ricardo Gutierrez because of hemoptysis. Discarding TBC and other regional illness, in early chest computed tomography shows consolidation in left upper lobe, ground glass opacities. In the period of non bleeding, efficient videobronchoscopy navegate through the airways shows ingurgitation and remodeling pulmonary venules in mains bronchus, in right upper lobe (B1), left upper and lower lobe (LUL; B6). Hemodynamic, Angio tomography and cardiovascular tomography features with low risk pulmonary hypertension, pulmonary venous stenosis, with severe stenosis near by left atrium. Biopsy of apical left lobe and lower left lobe presents proliferation of capillaries into alveolar walls, interlobular septa, pulmonary interstitium and pleura, confirmed with CD34 pulmonary capillary hemangiomatosis/veno occlusive disease. It is a rare entity, with a few cases in the literature. At his 8 years old, living ordinary life in his home, makes this case report the only one known and it is not following the common pattern of this rare pathology. The natural evolution of this illness is severe pulmonary hypertension and dead. No definitive treatment include lung transplantation and angiogenesis inhibitors, meanwhile treatment are in progressive improvement.

Conclusion: Hemoptysis is an infrequent and severe symptom in childhood. Video bronchoscopy in non bleeding period, has an invaluable rate of diagnosis of pulmonary pathology and in this particular case, an important tool to aim at Veno occlusive disease.


Pulmonary capillary haemangiomatosis in a premature infant - Cicero J T A Silva - 2005 Jun

Congenital pulmonary capillary hemangiomatosis in a newborn, Sandra L Sposito Cavallo, 2017 Feb

Pulmonary veno-occlusive disease, David Montani , Edmund M Lau; 2016 May

Disclosure of funding source(s): none