Abcesso Cerebral num Doente com Rendu-Osler-Weber

A telangiectasia hemorragica hereditaria e uma doenca familiar rara, descrita pela primeira vez no final do seculo 19, caracterizada por telangiectasias mucocutâneas e viscerais, e por malformacoes arteriovenosas. Manifestacoes neurologicas ocorrem em 8 a 12% dos doentes e resultam em mais de metade dos casos de malformacoes arteriovenosas pulmonares. A telangiectasia hemorragica hereditaria e uma das causas mais frequentes de abcessos cerebrais recorrentes, multiplos e bilaterais, tendendo estes a ocorrerem na 3a para a 5a decada. Apresentamos o caso de uma mulher de 88 anos admitida por prostracao, febre e insuficiencia respiratoria, com a suspeita de metastizacao pulmonar de neoplasia oculta, cuja investigacao revelou, entretanto, um abcesso cerebral secundario a malformacoes arteriovenosas pulmonares. Apesar da antibioterapia e da drenagem cirurgica do abcesso, a paciente veio a falecer. O prognostico apos drenagem dos abcessos sem resseccao das lesoes pulmonares e desfavoravel, podendo sua elevada mortalidade refletir um atraso diagnostico pela falta de reconhecimento dessa associacao. Hereditary hemorrhagic telangiectasia is a rare familial disorder, first described in the late 19th century characterized by multiple mucocutaneous and visceral telangiectases and arteriovenous malformations. Neurologic manifestations occur in 8 to 12% of patients and result from pulmonary arteriovenous malformations in 50% of cases. Hereditary hemorrhagic telangiectasia is one of the most frequent causes of recurrent, multiple and bilateral cerebral abscess and these tend to occur in the 3rd to 5th decade. We present the case of an 88-year-old woman admitted for prostration, fever and respiratory failure who was thought to have pulmonary metastasis of an unknown tumor but investigation revealed a brain abscess secondary to pulmonary arteriovenous malformations. Despite antibiotics and surgical drainage the patient died. The prognosis of brain abscess drainage without resection of the pulmonary lesions is not favorable. The high mortality of brain abscess in these patients may reflect a delay in diagnosis due to lack of recognition of this association.