Weber-Rendu-Osler disease: pulmonary arterio-venous malformation with shunt disclosed after 5 occurrences of purulent meningococcal encephalitis
Hereditary hemorrhagic telangectasis or Weber-Rendu-Osler disease is associated 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 telangiectasis was diagnosed in a 68-year-old woman with a history of epistaxis, cutaneous telangectasis, purulent and pyogenic brain abscesses and meningitis. Outcome was favorable with antibiotic therapy. Ventilation as well as chest x-ray, brain scan and liver ultrasongraphy were normal. Blood gases showed a PO2 at 63 mmHg in ambient air and 62 mmHg with FiO2 = 1. There was no dyspnea or cyanosis nor any apparent polycythemia. Pulmonary angiography showed and arteriovenous malformation in the lower right lobe. Endovascular embolization was achieved with coils and N-butyl-cyano-acrylate glue which enabled angiographic occlusion and normalization of gas exchange: on ambient air the PaO2 = 71 mmHg and on FiO2 = 1, PaO2 was 359 mmHg. A true shunt was suspected on account of the association of hereditary hemorrhagic telangiectasis and recurrent meningitis. The diagnosis was suspected on blood gases alone.