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[Universal organ involvement in Rendu-Osler-Weber disease: interdisciplinary diagnosis and interventional therapy]
J Kirchner1, A Zipf, C F Dietrich
1Institut für Allgemeine Röntgendiagnostik, Johann Wolfgang Goethe-Universität, Frankfurt am Main.
Zeitschrift Fur Gastroenterologie
|November 1, 1996
Summary
Hereditary hemorrhagic telangiectasia (HHT) can cause severe bleeding in multiple organs. This case highlights the importance of advanced imaging and embolization for managing HHT complications.
Area of Science:
- Vascular Medicine
- Gastroenterology
- Radiology
Background:
- Hereditary hemorrhagic telangiectasia (HHT), also known as Morbus Rendu-Osler-Weber, is a genetic disorder causing abnormal blood vessel formation.
- Patients often present with recurrent bleeding from gastrointestinal and nasopharyngeal sites, necessitating frequent blood transfusions.
Observation:
- A 63-year-old patient with HHT experienced severe bleeding requiring extensive red blood cell transfusions.
- Endoscopy identified a jejunal bleeding source, while colonoscopy revealed no lesions.
- Angiodysplastic alterations in the ethmoidal arteries caused epistaxis, and a portovenous malformation was found in the liver.
Findings:
- Selective mesentericography showed arterial dilatations and early venous filling in the duodenum.
- Coeliacography revealed parenchymal liver deposits, indicative of vascular abnormalities.
- Embolization of ethmoidal arteries successfully terminated epistaxis, enabling subsequent aortic valve replacement.
Implications:
- This case demonstrates the widespread organ involvement characteristic of HHT.
- It underscores the value of employing complementary diagnostic and therapeutic modalities for comprehensive HHT management.
- Early and accurate diagnosis of HHT-related vascular malformations is crucial for effective treatment and improved patient outcomes.
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