[Cerebromeningeal hemorrhages in Rendu-Osler disease. 2 cases treated by embolization]

Treatment for cerebral vascular malformations in patients with hereditary hemorrhagic telangiectasia, also called Osler-Weber Rendu disease, has been greatly improved with advances in interventional neuroradiology.

Two patients with Osler-Weber Rendu disease experienced intracranial hemorrhage. In the first case, the disease was diagnosed at the age of 60 years in a man with recurrent epistaxis and numerous angiomas on the face and trunk as well as in the buccal cavity. The first neurological signs appeared seven years later and brain MRI revealed a 2-3 cm temporo-occipital arteriovenous malformation. Six months later sudden onset headache following exertion led to a CT-scan which showed tetraventricular hemorrhage. The second case was a 63-year-old man referred to neurosurgery for cerebromeningeal hemorrhage. Clinical examination and history taking found several small skin angiomas and a family and personal history of epistaxis. CT-scan revealed a dural fistula in the posterior fossa. Good clinical outcome was obtained in both cases by endovascular embolisation.

Intracranial hemorrhage is observed in 2 to 3% of patients with Osler-Weber Rendu disease. Telangiectasia is not a pathognomonic sign and non-specific vascular malformations may be observed, particularly arteriovenous malformations or arterial aneurysms. Most can be reached by endovascular catheterism. Most of these usually unique malformations can be successfully treated by first-line embolisation.