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Clinical communication| Volume 20, ISSUE 3, P247-251, April 2001

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

  • Sandy L Dong
    Affiliations
    Division of Emergency Medicine, Faculty of Medicine and Dentistry, University of Alberta, Edmonton, Alberta, Canada
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  • Stuart F Reynolds
    Affiliations
    Division of Emergency Medicine, Faculty of Medicine and Dentistry, University of Alberta, Edmonton, Alberta, Canada
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  • Ivan P Steiner
    Correspondence
    Reprint Address: Ivan P. Steiner, MD, MCFP (EM), FCFP, 1G1.64 WMC Health Sciences Centre, 8440–112 Street, Edmonton, Alberta, Canada, T6G 2B7
    Affiliations
    Division of Emergency Medicine, Faculty of Medicine and Dentistry, University of Alberta, Edmonton, Alberta, Canada

    Department of Family Medicine, Faculty of Medicine and Dentistry, University of Alberta, Edmonton, Alberta, Canada
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      Abstract

      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.

      Keywords

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      References

        • Haitjema T
        • Westermann C.J.J
        • Overtoom T.T.C
        • et al.
        Hereditary hemorrhagic telangiectasia (Osler–Weber–Rendu Disease).
        Arch Intern Med. 1996; 156: 714-719
        • Peery W.H
        Clinical spectrum of hereditary hemorrhagic telangiectasia (Osler–Weber–Rendu disease).
        Am J Med. 1987; 82: 989-997
        • Guttmacher A.E
        • Marchuk D.A
        • White R.I
        Hereditary hemorrhagic telangiectasia.
        N Engl J Med. 1995; 333: 918-924
        • Smith C.R
        • Bartholomew L.G
        • Cain J.C
        Hereditary hemorrhagic telangiectasia and gastrointestinal hemorrhage.
        Gastroenterology. 1963; 44: 1-6
        • Porteous M.E.M
        • Burn J
        • Procter S.J
        Hereditary haemorrhagic telangiectasia.
        J Med Genet. 1992; 29: 527-530
        • Román G
        • Fisher M
        • Perl D.P
        • Poser C.M
        Neurological manifestations of hereditary hemorrhagic telangiectasia (Rendu–Osler–Weber Disease).
        Ann Neurol. 1978; 4: 130-144
        • Saunders W.H
        Permanent control of nosebleeds in patients with hereditary hemorrhagic telangiectasia.
        Ann Intern Med. 1960; 53: 147-152
        • Jahnke V
        Ultrastructure of hereditary telangiectasia.
        Arch Otolaryngol. 1970; 91: 262-265
        • Plauchu H
        • de Chadarevian J.P
        • Bideau A.J
        • Robert J.M.L
        Age-related clinical profile of hereditary hemorrhagic telangiectasia in an epidmiologically recruited population.
        Am J Med Genet. 1989; 32: 291-297
        • Haitjema T
        • Disch E
        • Overtoom T.T.C
        • Westermann C.J.J
        • Lammers J.W.J
        Screening of family members of patients with hereditary hemorrhagic telangiectasia.
        Am J Med. 1995; 99: 519-524
        • Reilly P.J
        • Nostrant T.T
        Clinical manifestations of hereditary hemorrhagic telangiectasia.
        Am J Gastroenterol. 1984; 79: 363-367
        • McCaffrey T.V
        • Kern E.B
        • Lake C.E
        Management of epistaxis in hereditary hemorrhagic telangiectasia.
        Arch Otolaryngol. 1977; 103: 627-630
        • Assar O.S
        • Friedman C.M
        • White R.I
        The natural history of epistaxis in hereditary hemorrhagic telangiectasia.
        Laryngoscope. 1991; 101: 977-980
        • Bernard G
        • Mion E
        • Henry L
        • Plauchu H
        • Paliard P
        Hepatic involvement in hereditary hemorrhagic telangiectasia.
        Gastroenterology. 1993; 105: 482-487
        • Eagel W.J
        • Perlberger R
        • Kauffmann R.H
        Portosystemic encephalopathy in hereditary hemorrhagic telangiectasia.
        Am J Med. 1988; 85: 858-860
        • Zentler-Munro P.L
        • Howard E.R
        • Karani J
        • Williams R
        Variceal haemorrhage in hereditary telangiectasia.
        Gut. 1989; 30: 1293-1297
        • White R.I
        • Lynch-Nyhan A
        • Terry P
        • et al.
        Pulmonary arteriovenous malformations.
        Radiology. 1988; 169: 663-669
        • Ference B.A
        • Shannon T.M
        • White R.I
        • Zawin M
        • Burdge C.M
        Life-threatening pulmonary hemorrhage with pulmonary arteriovenous malformations and hereditary hemorrhagic telangiectasia.
        Chest. 1994; 106: 1387-1390
        • Stanley I.M
        • Hunter K.R
        Neurological manifestations of hereditary hemorrhagic telangiectasis.
        BMJ. 1970; 3: 688
        • Dyer N.H
        Cerebral abscess in hereditary haemorrhagic telangiectasia.
        J Neurol Neurosurg Psychiatry. 1967; 30: 563-567
        • Härkönen M
        Hereditary hemorrhagic telangiectasia (Osler–Weber–Rendu Disease) complicated by pulmonary arteriovenous fistula and brain abscess.
        Acta Med Scand. 1981; 209: 137-139
        • Kikuchi K
        • Kowada M
        • Sasajima H
        Vascular malformations of the brain in hereditary hemorrhagic telangiectasia (Osler–Weber–Rendu Disease).
        Surg Neurol. 1994; 41: 374-380
        • Press O.W
        • Ramsey P.G
        Central nervous system infections associated with hereditary hemorrhagic telangiectasia.
        Am J Med. 1984; 77: 86-92
        • Hall W.A
        Hereditary hemorrhagic telangiectasia (Osler–Weber–Rendu Disease) presenting with polymicrobial brain abscess.
        J Neurosurg. 1994; 81: 294-296
        • Stern W.E
        • Naffziger H.C
        Brain abscess associated with pulmonary angiomatous malformation.
        Ann Surg. 1953; 138: 521-531
        • Ogino M
        • Inoue H
        • Harada S
        • Horinouchi H
        • Nakamura T
        Cerebellar abscess associated with pulmonary arteriovenous fistula and hereditary hemorrhagic telangiectasia.
        Neurol Med Chir (Tokyo). 1996; 36: 575-579
        • Nicolosi A
        • Hauser W.A
        • Musicco M
        • Kurland L.T
        Incidence and prognosis of brain abscess in a defined population.
        Neuroepidemiology. 1991; 10: 122-131
        • Grigoriadis E
        • Gold W.L
        Pyogenic brain abscess caused by streptococcus pneumoniae.
        Clin Infect Dis. 1997; 25: 1108-1112
        • Seydoux C.H
        • Francioli P
        Bacterial brain abscesses.
        Clin Infect Dis. 1992; 15: 394-401
        • Svanteson B
        • Nordstrom C.H
        • Rausing A
        Non-traumatic brain abscess.
        Acta Neurochir (Wien). 1988; 94: 57-65
        • Mathisen G.E
        • Johnson J.P
        Brain abscess.
        Clin Infect Dis. 1997; 25: 763-781
        • Yildizhan A
        • Pasaoglu A
        • Kandemir B
        Effect of dexamethasone on various stages of experimental brain abscess.
        Acta Neurochir (Wien). 1989; 96: 141-148
        • Schroeder K.A
        • McKeever P.E
        • Schaberg D.R
        • Hoff J.T
        Effect of dexamethasone on experimental brain abscess.
        J Neurosurg. 1987; 66: 264-269