Weber-Rendu-Osler disease: pulmonary arteriovenous malformation with a shunt revealed after five episodes of purulent meningoencephalitis

Hereditary hemorrhagic telangectasis or I Weber-Rendu-Osler disease is asso ciated with the presence of capillary malformations with pulmonary visceral shunts. These shunts are the cause of recurrent infections of the nervous system by loss of the anti-infectious lung filter. Hereditary hemorrhagic t elangiectasis was diagnosed in a 68-year-old woman with a history of epista xis, cutaneous telangectasis, purulent and pyogenic brain abscesses and men ingitis. Outcome was favorable with antibiotic therapy. Ventilation as well as chest x-I-ay, brain scan and fiver ultrasongraphy were normal. Blood ga ses showed a PO, at 63 mmHg in ambient air and 62 mmNg with FiO(2) = 1. The re was no dyspnea or cyanosis nor any apparent polycythemia. Pulmonary angi ography showed and arteriovenous malformation in the lower-right lobe. Endo vascular embolization was achieved with coils and N-butyl-cyano-acrylate gl ue which enabled angiographic occlusion and normalization of gas exchange: on ambient air the PaO2 = 71 mmHg and on FiO(2) = 1, PaO2 was 359 mmHg. A t rue shunt was suspected on account of the association of hereditary hemorrh agic telangiectasis and recurrent meningitis. The diagnosis was suspected o n blood gases alone.