Osler-Weber-Rendu syndrome treatment

Osler-Weber-Rendu syndrome treatment

Osler-Weber-Rendu syndrome treatment focuses on reducing bleeding from blood vessel lesions, and sometimes surgery or other targeted interventions to remove arteriovenous malformations in organs. Chronic bleeding often requires iron supplements and sometimes blood transfusions.

Surgery is indicated mainly for the evacuation of hematoma or diagnosis, especially when favorably located. Also, consider surgery for recurrent hemorrhages (rupture has been reported even after normal angiography) or medically intractable seizures. Stereotactic radiosurgery has not had a satisfactorily high enough benefit to risk ratio to justify its use 1).


The risk of treatment of brain AVMs in patients with Osler-Weber-Rendu syndrome is quite low for appropriately selected patients with treatment individualized to radiosurgerymicrosurgery, or a combination of embolization and microsurgery 2).


Currently, conventional heparin and warfarin remain first choice anticoagulants. If newer anticoagulants are considered, although study numbers are small, at this stage Apixaban appears to be associated with lesser bleeding risk than Rivaroxaban 3).

References

1)

Lindquist C, Guo W-Y, Kerlsson B, Steiner L. Radiosurgery for Venous Angiomas. J Neurosurg. 1993; 78:531–536
2)

Woodall MN, Nakaji P, Spetzler RF. Benefits of Treating Arteriovenous Malformations in Hereditary Hemorrhagic Telangiectasia: A Retrospective Analysis of 14 Patients. World Neurosurg X. 2019 Mar 9;3:100029. doi: 10.1016/j.wnsx.2019.100029. eCollection 2019 Jul. PubMed PMID: 31225521; PubMed Central PMCID: PMC6584483.
3)

Shovlin CL, Millar CM, Droege F, Kjeldsen A, Manfredi G, Suppressa P, Ugolini S, Coote N, Fialla AD, Geisthoff U, Lenato GM, Mager HJ, Pagella F, Post MC, Sabbà C, Sure U, Torring PM, Dupuis-Girod S, Buscarini E; VASCERN-HHT. Safety of direct oral anticoagulants in patients with hereditary hemorrhagic telangiectasia. Orphanet J Rare Dis. 2019 Aug 28;14(1):210. doi: 10.1186/s13023-019-1179-1. PubMed PMID: 31462308; PubMed Central PMCID: PMC6714298.

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