Osler-Weber-Rendu Disease Presenting as Recurrent Portosystemic Encephalopathy in a 75-year-old Female Patient.
- Author:
Junghoon HA
1
;
Byoung Kwan SON
;
Sang Bong AHN
;
Young Kwan JO
;
Seong Hwan KIM
;
Yun Ju JO
;
Young Sook PARK
;
Yoon Young JUNG
Author Information
- Publication Type:Case Reports
- Keywords: Hereditary hemorrhagic telangiectasia; Hepatic encephalopathy
- MeSH: Aged; Brain/diagnostic imaging; Electroencephalography; Female; Hepatic Encephalopathy/*diagnosis; Humans; Magnetic Resonance Imaging; Pedigree; Telangiectasia, Hereditary Hemorrhagic/*diagnosis; Tomography, X-Ray Computed; Vascular Malformations/etiology
- From:The Korean Journal of Gastroenterology 2015;65(1):57-61
- CountryRepublic of Korea
- Language:English
- Abstract: Osler-Weber-Rendu disease is a rare autosomal dominant disorder of fibrovascular tissues, characterized by a classic triad of mucocutaneous telangiectasias, recurrent hemorrhages, and a familial occurrence. Portosystemic encephalopathy in a patient with Osler-Weber-Rendu disease is rare, but we experienced a case presenting with recurrent portosystemic encephalopathy in Osler-Weber-Rendu disease. We report on a case of a 75-year-old female presenting with an altered mentality. Initial studies including brain imaging study did not reveal any specific cause for her mental status. She was diagnosed with the rare disease after a series of tests and received conservative treatment. Her neurological status recovered fully without complication after conservative treatment and she was discharged after 18 hospital days. This case demonstrated an extremely rare case of Osler-Weber-Rendu disease presenting as portosystemic encephalopathy treated successfully with conservative treatment. For patients who have shown hepatic encephalopathy without a definite cause, we recommend evaluation for the possibility of Osler-Weber-Rendu disease. Conservative treatment based on treatment of advanced liver cirrhosis could be an alternative solution.
