As malformations are due to excessive growth of abnormally formed blood or lymphatic vessels, some medications may be used to stop growth such as Sirolimus. This is a drug also used to treat some cancers as it stops cell growth. It will work for some patients but not others so it needs to be monitored, or gene testing performed.
The drug needs to be monitored as it can have some side effects. New medications are being researched.
Surgical treatment is the mainstay of treatment to get a malformation under control or even to cure.
Treatment includes performing a venogram – this is direct injection of a dye into the blood vessels to see the extent and pattern of the type of malformation and direct treatment.
Most malformations will be treated by: Ethanol Embolisation: This involves directly cannulating all the abnormal vessels and measuring the volume of fluid (blood or lymph) and slowly injecting the same amount of ethanol. Ethanol is very powerful and the aim is that it denatures the inside of the malformation and causes it to seal off. Ethanol may have local and systemic side effects and treatments need to be carefully planned and performed under a General Anaesthetic. Although aggressive, this is the most likely treatment to enable cure. Other endovenous treatments that may be used or foam sclerosant injection, laser or radiofrequency ablation. This all depends on the type of malformation present and the type of drainage the malformation has.
Surgical treatment needs to be repeated every 3-4 weeks to obtain the best results, and depending on the size of the malformation, may range from 4 to over 10 treatments. Serial MRI’s every 6-12 months are done to assess the progress and response to treatment. Once the actual malformation has been treated, one may require plastic surgery to optimise the aesthetic of the ‘cured’ malformation.
Treatment with ethanol does carry some risks, but if performed by an experienced team and all necessary precautions taken, side effects are very uncommon. Obviously the location and size may have an impact on risks and outcomes.
Complications may include:
- Nerve injury
- Most will resolve when swelling subsides. Permanent nerve damage only in 1%.
- Wound formation / skin breakdown
- Thrombosis or bleeding
- Deep Vein Thrombosis
- Cardiac or pulmonary complications
- Preventative measure taken to avoid this:
- Procedure done under general anaesthetic to monitor
- Steroids given
- Volume, dose and response carefully monitored
Outcomes of Treatment
Vascular malformations are notoriously difficult to treat, but there is an increasing trend to be more aggressive in treating them in order to obtain cure or reduce them significantly. There is a shift of thinking to not only treat life or limb threatening malformations but ones that may impact on a patients psychosocial status, overall wellbeing or quality of life.
Dr Redman has trained with Professor Baumgartner, Professor Wayne Yakes, Professor Krassi Ivancev and Professor Raul Mattassi to try and establish the best treatment protocols for patients suffering with malformations. She is also part if the Red Cross Children’s Hospital malformation discussions, working with colleagues to build up best practices for malformations on South Africa. They require multiple treatments to achieve successful results. However, with a multidisciplinary team approach good outcomes can be achieved.