Brain abscess in patients with hereditary hemorrhagic telangiectasia: Case report and literature review

Hereditary hemorrhagic telangiectasia (HHT), or Osler–Weber–Rendu disease, affects multiple organ systems. Brain abscess is a potential complication, and this disease carries a high mortality. In the setting of HHT the abscess most likely results from paradoxical septic emboli or bacterial seeding of an ischemic portion of the brain after paradoxical sterile emboli. Brain abscess is the diagnosis that must be ruled out in patients with HHT presenting with new onset neurologic symptoms. The clinician can be misled by seemingly benign and nonspecific symptoms, signs, and laboratory test results. Appropriate diagnostic imaging with computed tomography or magnetic resonance imaging of the head is mandatory. We present a case of brain abscess in a patient with HHT presenting to the Emergency Department. The review of the literature deals with the pathophysiology and manifestations of HHT with particular focus on the pathologic and clinical features, and management of cerebral abscess in this setting. Differences between patients with brain abscess with or without HHT are highlighted.