Treatment

HHT-Treatment-Image

 

It is advised that any treatment for HHT be carried out by a HHT Specialist or someone who understands the complexities of HHT. Screening by an HHT expert is the best way to determine what course is best for you. If your Doctor decides that your AVMs need treated, there are several treatment options available.

The recommended treatment for AVMs depends almost entirely on the size of the malformation and its location in the body. AVMs can develop and change over time, so periodic screenings are important to ensure your safety and health.

These are the treatments available but your HHT specialist will decide which is best for you.

These treatments may vary country to country and evolve with the progression of science.

Your ENT specialist will discuss all suitable options open to you. It’s important to remember that what works for one person may not always work for another!

Lung AVMs

Embolisation – In this procedure, a catheter is used to place a small medical device inside an artery. This device blocks the AVM and reduces or stops the blood flow to relieve the pressure on the walls of the blood vessel.

Surgical – The surgical procedure for Lung AVMs removes the part of the lung containing the AVM.

Nose

90% of HHT patients experience epistaxis (nose bleeding). However, the severity, frequency and duration vary from patient to patient and family member to family member. There is no one treatment that works for everyone.

Some treatments that are on offer are as follows:

Topical Therapies include creams & gels such as Tranexamic acid or Estrogen cream or Bevacizumab cream (Avastin)

Local Therapies include both bevacizumab (Avastin) and sclerotherapy by injection.

Surgical Therapies include cautery, laser, coblation, septodermoplasty, Young’s procedure (closure of the nose) & estrogen medication, for women only.

Gastro-Intestinal Tract

AVMs are also common in the GI Tract, usually in the stomach and upper portion of the small intestine. When necessary, intestinal AVMs can be treated by laser, tranexamic acid, IV Avastin and other angiogenesis inhibitors. The most significant problem with intestinal AVMs is anaemia caused by chronic bleeding. Anaemia can be treated separately from the AVM with iron supplements and blood and iron transfusions.

Brain AVMs

Embolisation –  In this procedure, a catheter is used to place a small medical device or quantity of glue inside an artery. This device blocks the AVM and reduces or stops the blood flow to relieve the pressure on the walls of the blood vessel.

Surgical –  The surgical procedure for Brain AVMs removes the AVM or places a clip on the site of the malformation.

Gamma Knife radiosurgery – This procedure uses focused radiation to destroy the AVM tissue, and is only used for Brain AVMs.

Liver AVMs

Liver AVMs are common but typically do not require treatment. If treatment is required, it is essential that an HHT specialist is consulted regarding the best option. Procedures to treat liver AVMs can be very invasive and have the potential to worsen the condition of the patient if an HHT specialist is not involved.

Intravenous (IV) Iron

A haematologist may consider intravenous (IV) iron if a patient is intolerant of oral iron or has ongoing blood loss. There are several IV iron products that can be recommended, consult your HHT specialist for dosage amounts and time required to administer the dose.

Treatment