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[Epistaxis in Rendu-Osler-Weber disease treated with selective embolization--case report]
Magdalena Jarzabek1, Piotr Trojanowski, Maciej Szajner
1Zakład Radiologii Zabiegowej i Neuroradiologii, Uniwersytet Medyczny w Lublinie. magda.jarzabek@yahoo.co.uk
Przeglad Lekarski
|January 2, 2013
Summary
Hereditary hemorrhagic telangiectasia (HHT), or Osler-Weber-Rendu syndrome, causes recurrent nosebleeds. This case study shows successful low-invasive treatment using intravascular arterial embolisation.
Area of Science:
- Vascular Medicine
- Genetics
- Interventional Radiology
Background:
- Hereditary hemorrhagic telangiectasia (HHT), also known as Osler-Weber-Rendu syndrome, is an autosomal dominant disorder affecting endothelial cells.
- Pathophysiology involves abnormal blood vessel development, leading to telangiectasias and arteriovenous malformations (AVMs) in various organs.
- Affecting 1 in 5,000-10,000 people, HHT commonly presents with recurrent epistaxis, impacting quality of life.
Observation:
- A patient with HHT experienced frequent, severe nosebleeds.
- Current treatments for HHT primarily manage symptoms, lacking a cure for the underlying cause.
- No established guidelines exist for effective HHT therapy due to its rarity.
Findings:
- The patient's recurrent epistaxis due to HHT was effectively managed.
- A minimally invasive intravascular arterial embolisation procedure was employed.
- Interventional radiology successfully treated the vascular abnormalities causing the bleeding.
Implications:
- Low-invasive intravascular arterial embolisation offers a promising treatment option for HHT-related epistaxis.
- This approach addresses a significant symptom of HHT, improving patient outcomes.
- Further research into interventional radiology techniques may provide new therapeutic avenues for rare vascular disorders.
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