The goal of this clinical trial is to explore if enalapril can be used to treat painful venous malformations. The main question it aims to answer are: \- Can enalapril reduce pain and volume of the malformation and increase quality of life in patients with painful venous malformations? Participants will: Receive a dose of enalapril 5 mg once daily. If it doesn't have effect the dose of enalapril will be increased to 10 mg daily after 3 months. The treatment duration will be 12 months, with an additional follow-up of 12 months. The visit frequency will be after 1, 3, 6,9 and 12 months and then a follow up visit at 18 and 24 months. Approximately 15 participants will be screened to achieve minimum 10 enrolled participants.
Venous malformations are congenital defects of vasculature and may be disabling for patients throughout life, mostly because of disfigurement and pain. The patients remain challenging to treat as standard of care like sclerotherapy and compression garment is often inadequate in relieving the symptoms. A chance observation has given reason to believe that enalapril may have the potential to reduce pain and volume in venous malformations. A young man with a symptomatic intramuscular venous malformation of the upper limp, and hypertension was treated with an angiotensin-converting enzyme (ACE) inhibitor (enalapril). After 8 months of treatment, it was registered a considerable volume reduction of the malformation and a reduction in pain. Studies have reported that embryonic stem cell-like subpopulations in venous malformations express components of the renin-anigotensin system (RAS). It has been hypothesized that such primitive cells could be a novel therapeutic target by manipulation of the RAS using ACE-inhibitors. The aim of the study is to explore if the chance observation of reduced pain and volume of a venous malformation after enalapril treatment is a coincidence, or if enalapril may play a future role in the treatment of venous malformations.
Study Type
INTERVENTIONAL
Allocation
NA
Purpose
TREATMENT
Masking
NONE
Enrollment
12
Each participant will receive a dose of enalapril 5 mg once daily. If the participant doesn't have effect of the intervention after 3 months, the dose of enalapril will be increased to 10 mg daily, if the tolerance of enalapril is good. Lack of effect will be defined as unchanged or increased NRS score. Treatment duration will be 12 months.
Oslo University Hospital, Rikshospitalet
Oslo, Norway, Norway
RECRUITINGTo explore the change of venous malformation related pain
Changes in NRS (Numeric rating scale). NRS is an analogue scale with numbers from 0-10, where 0 represents no pain and 10 represents the worst imaginable pain.
Time frame: From baseline and to the end of treatment at 12 months.
To explore volume change of the venous malformation
Volume will be assessed by MRI. To estimate the volume of the venous malformation the area of the malformation on multiple adjacent MRI slices will be added and multiplied by the slice thickness. The volume change at follow-up imaging is calculated according to this formula: (Volume at baseline - volume at follow up)/ (volume at baseline) x 100 = percentage volume change.
Time frame: From baseline to the end of treatment at 12 months.
To explore change in quality of life in patients with venous malformations
Quality of life, will be assessed by the SF-36 (Short Form) questionnaire. The SF-36 consists of 36 questions measuring quality of life in 4 mental domains and 4 physical domains, supplying general information about life quality. The score of the SF-36 ranging from 0-100. Higher score ranging better health status.
Time frame: From baseline to the end of treatment at 12 months.
To explore change in quality of life in patients with venous malformations
Quality of life, will be assessed by the OVAMA score (Outcome measures for vascular malformations). The OVAMA score is a disease specific patient-reported-outcome-measures consisting of 23 questions measuring quality of live with a general symptom scale with 6 items, a head and neck symptom scale with 8 items, and an appearance scale with 9 items.
Time frame: From baseline to the end of treatment at 12 months.
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