Spontaneous hemothorax: first manifestation of pulmonary arteriovenous malformation in a patient with hereditary hemorrhagic telangiectasia
Parrilla Quinones, F.; Marmorato Rivera, R.; Calderon, R.E.; Vargas Otero, P.; Hidalgo Rios, A.
Boletin de la Asociacion Medica de Puerto Rico 103(4): 41-44
2011
ISSN/ISBN: 0004-4849 PMID: 22737829 Document Number: 702616
Hereditary Hemorrhagio Telangiectasla (HHT) is a rare inherited autosomal dominant disorder characterized by systemic fibrovascular dysplasia making vessel walls more liable to spontaneous ruptures and injuries. Epistaxis is the first and the most common symptom; however patients may have a variety of serious complications due to vascular involvement of internal organs. In this report we present a case of a 39 year-old woman with recurrent epistaxis and family history of HHT who presented with right spontaneous hemothorax. Pulmonary angiography disclosed multiple pulmonary artery aneurysms with subsequent coil embolization. Considering that pulmonary arteriovenous malformations are associated with considerable morbidity and mortality, patients and family members with suspected HHT should be screened for these vascular malformations.