eMedicine Specialties > Dermatology > Diseases of the Vessels
Osler-Weber-Rendu Syndrome: Treatment & Medication
Updated: May 6, 2009
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Treatment
Medical Care
One third of the cases of Osler-Weber-Rendu syndrome, or hereditary hemorrhagic telangiectasia (HHT), are mild, one third are moderate, and one third are severe.
- In mild cases of HHT, no treatment is necessary.
- Individual skin lesions may be obliterated with cautery or dye laser surgery.14
- In severe cases of HHT, recurrent epistaxis is treated surgically with nasal septum skin transplants by using skin taken from the lower trunk.
- Severe cases of HHT may respond to estrogen therapy.15
- Pulmonary hemorrhage may be stopped with surgery by using silicone balloon tamponade or other means.
- Antibiotic prophylaxis should be considered during treatment procedures because transient bacteremia may seed arteriovenous malformations.
- Buscarini et al report on the use of bevacizumab to treat complicated liver vascular malformations.16
Surgical Care
See Medical Care above.
Consultations
- A dermatologist may be consulted.
- An internal medicine specialist may be helpful.
- A neurologist may offer assistance.
Activity
- Patients should restrict their activity during episodes of acute bleeding.
- Patients with severe anemia should restrict their activity as well.
Medication
Mild forms of Osler-Weber-Rendu syndrome, or hereditary hemorrhagic telangiectasia (HHT), do not require treatment. Hormone, including antiestrogen, therapy with tamoxifen for the treatment of epistaxis due to HHT has produced good responses, although its use remains controversial.17
Iron salts
Iron replacement therapy provides symptomatic relief for anemia.
Ferrous sulfate (Feosol, Fero-Gradumet)
A nutritionally essential inorganic substance.
Adult
300 mg PO qd
Pediatric
Not established
Absorption enhanced by ascorbic acid; interferes with tetracycline absorption; food and antacids impair absorption
Documented hypersensitivity
Pregnancy
A - Fetal risk not revealed in controlled studies in humans
Precautions
GI tract upset; iron toxicity with ingestion of large amount (can be fatal, especially in children); IV administration can cause several reactions, including headaches, malaise, fever, generalized lymphadenopathy, arthralgia, and urticaria; can cause severe anaphylaxis and phlebitis at infusion site
Hormones
Estrogen therapy may be beneficial in some women with HHT and may be used to decrease the amount of bleeding. Oral contraceptives have been shown to be more effective than estrogen alone in mucosal bleeding.
Estradiol (Estrace, Climara, Dura-Estrin)
Increases synthesis of DNA, RNA, and many proteins in target tissues. Norethindrone acetate and ethinyl estradiol are options.
Adult
0.625-2 mg/d PO or transdermal patch; use lowest effective dose for long-term therapy
Ethinyl estradiol: 30 mcg PO qd
Norethindrone: 1.5 mg PO qd
Pediatric
Not established
May reduce hypoprothrombinemic effects of anticoagulants; coadministration of barbiturates, rifampin, and other agents that induce hepatic microsomal enzymes may decrease estrogen levels; corticosteroid levels may increase with concurrent ethinyl estradiol; use of ethinyl estradiol with hydantoins may cause spotting, breakthrough bleeding, and pregnancy; increase in fluid retention caused by estrogen intake may reduce seizure control
Documented hypersensitivity; thrombophlebitis; undiagnosed vaginal bleeding; coronary artery disease; active liver disease; history of stroke; carcinoma of the breast; ophthalmic vascular disease; pregnancy
Pregnancy
X - Contraindicated; benefit does not outweigh risk
Precautions
Caution in hepatic impairment, migraines, seizure disorders, cerebrovascular disorders, breast cancer, and thromboembolic disease
Antifibrinolytics
Used to enhance hemostasis when fibrinolysis contributes to bleeding.18
Aminocaproic acid (Amicar)
Inhibits fibrinolysis via inhibition of plasminogen activator substances and, to lesser degree, through antiplasmin activity. Used to prevent or treat mucosal bleeding caused by bleeding disorders or trauma.
Adult
3.5 g IV initially, then 1 g/h until bleeding stops; not to exceed 8 h treatment duration
3.5 g/dose PO tid/qid for 3-4 d
Topical: Insert gauze soaked in a 10% solution of aminocaproic acid into nasal cavity
Pediatric
50-100 mg/kg IV infused over 30-60 min, then 30-50 mg/kg/h until bleeding stops; not to exceed 8 h treatment duration
50 mg/kg/dose PO tid/qid for 3-4 d
Topical: Administer as in adults
Coadministration with estrogens may cause increase in clotting factors, leading to hypercoagulable state
Documented hypertensively; evidence of active intravascular clotting process; disseminated intravascular coagulation (DIC) because aminocaproic acid can be fatal in patients with DIC, differentiate between hyperfibrinolysis and DIC
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Reduce dose in cardiac, renal, or hepatic disease
More on Osler-Weber-Rendu Syndrome |
| Overview: Osler-Weber-Rendu Syndrome |
| Differential Diagnoses & Workup: Osler-Weber-Rendu Syndrome |
Treatment & Medication: Osler-Weber-Rendu Syndrome |
| Follow-up: Osler-Weber-Rendu Syndrome |
| Multimedia: Osler-Weber-Rendu Syndrome |
| References |
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References
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Further Reading
Keywords
Osler-Weber-Rendu syndrome, hereditary hemorrhagic telangiectasia, HHT, morbus Osler, Rendu-Osler-Weber syndrome, Rendu-Osler syndrome, Osler's disease, Osler disease
Treatment & Medication: Osler-Weber-Rendu Syndrome