Neurologic Complications of Osler-Weber-Rendu Syndrome: A Case Report and Literature Review (P3.058)

OBJECTIVE: Preventing the neurologic complications of Osler-Weber-Rendu Syndrome (OWRS), also known as hereditary hemorrhagic telangiectasia, by early diagnosis and appropriate treatment. BACKGROUND: OWRS is a rare genetic disorder characterized by telangiectasias and excessive bleeding. Brain abscess and ischemic stroke are rare but potentially serious complications, caused by paradoxical embolism from pulmonary arteriovenous malformations (AVMs). DESIGN/METHODS: We describe case of a 56 year old woman with past medical history of OWRS who presented to emergency department for suspected stroke. MRI revealed a brain abscess which was treated with antibiotic therapy. A pulmonary angiogram revealed recanalized arteriovenous malformation in the left lobe of lung which was embolised 15 years ago. RESULTS: Endovascular embolization was done to avoid future neurologic complications and routine follow-up was indicated to prevent recanalization of embolised AVM and other complications of the procedure. CONCLUSIONS: Brain abscess or ischemic stroke must be expected in patients with history of OWRS who present with neurologic manifestations. Family members of such patients should be screened for OWRS with appropriate genetic testing. All the patients with OWRS, whether symptomatic or not, should be screened for pulmonary AVMs. If found, pulmonary AVM’s should be treated to prevent paradoxical embolism which can result in brain abscess or ischemic stroke, both of which can be potentially fatal. Endovascular embolization is the treatment of choice for pulmonary AVMS and a long term follow-up is recommended after the procedure to prevent any complications. Those patients treated for pulmonary AVMs, likely should be screened for recanalization with a pulmonary angiogram at routine intervals, suggested to be every 10 years. Our case illustrates that CT angiogram can be insufficient for this task. Study Supported by: Self Disclosure: Dr. Farooq has nothing to disclose. Dr. Mowla has nothing to disclose. Dr. AbdelRazek has nothing to disclose. Dr. Iqbal has nothing to disclose. Dr. Sawyer has nothing to disclose.