Long-term changes of health-related quality of life in patients with peripheral vascular malformations - a prospective observational study.

PURPOSE: The aim of this observational study was to assess health-related quality of life (HRQOL) changes in patients with vascular malformations, over a period of almost eight years, and to assess clinical and demographic characteristics possibly associated with HRQOL changes. METHODS: Eighty out of 111 patients who were included in a previously published comparative HRQOL study accepted inclusion in this follow-up study. HRQOL at baseline and follow-up was assessed with the Short-Form 36-item questionnaire (SF-36). Median observation time was 7.9 years. Linear mixed models and linear regression models were applied to assess HRQOL change and possible associations with demographic and clinical variables. RESULTS: The median age of the patient cohort at baseline evaluation (n = 111) was 27.0 years. Ninety-six out of 111 (86.5%) patients were diagnosed with venous malformations. Significantly higher SF-36 scores at follow-up were found for the physical domains Role limitations due to (RLDT) physical problems (difference=13.5; 95% CI [1.6, 25.3]) and Bodily pain (difference=11.3; 95% CI [3.8, 18.8]). No deterioration of HRQOL was found in any domain. In multivariate analyses, female gender, muscle/bone involvement, and higher age were associated with a positive relative change in SF-36 in the domains Physical functioning, RLDT physical problems, and RLDT emotional problems, respectively. Invasive treatment was not associated with long-term HRQOL change. CONCLUSIONS: Over a period of almost eight years, significant improvement of SF-36 scores was observed in the physical domains RLDT physical problems and bodily pain. Female gender, muscle/bone involvement, and higher age were associated with HRQOL improvement in certain domains.

Regression of a venous malformation during angiotensin-converting enzyme inhibitor treatment for hypertension.

Recent studies have reported that components of the renin-angiotensin system (RAS) are expressed in venous malformations by embryonic stem cell-like subpopulations. It has been hypothesized that these cells are sustained by the RAS and, therefore, could be a novel therapeutic target, using medications such as angiotensin-converting enzyme inhibitors. A young man with a symptomatic intramuscular venous malformation of the upper limb, and hypertension was treated with an angiotensin-converting enzyme inhibitor. After 8 months of treatment, we registered a considerable volume reduction of the venous malformation and a reduction in pain. Our observation warrants further research on the link between the RAS and venous malformations.

Portal Vein Embolization Using N-Butyl Cyanoacrylate-Glue: What Impact Does a Central Vascular Plug Have?

PURPOSE: To examine if the addition of a central vascular plug (CVP) to portal vein embolization (PVE) with N-butyl cyanoacrylate-glue (NBCA) increases future liver remnant (FLR) growth. MATERIAL AND METHODS: This is a single-center retrospective study of 115 consecutive patients with colorectal liver metastases undergoing PVE in 2013-2019. All patients were embolized with NBCA as the main embolic agent. In 2017-2019 NBCA was combined with a CVP in the central part of the right portal vein. Growth of the FLR and standardized FLR (sFLR) including degree of hypertrophy (DH) and kinetic growth rate (KGR) were analyzed, as well as procedure data such as use of cone-beam CT (CBCT), dose area product (DAP), fluoroscopy time and contrast dose. RESULTS: A total of 40 patients (35%) underwent PVE with a combination of CVP and NBCA. The DH was higher in these patients after 4 weeks, mean 13.6% (SD 7.8) vs. 10.5% (SD 6.4; p = 0.022), verified in multivariate analysis (coefficient 4.1, p = 0.015). A CVP did not significantly increase the resection rate (90% vs 82%, p = 0.4). Cone beam CT was used in 65 patients (57%). Use of CBCT did not affect FLR growth, and fluoroscopy time and contrast doses were not different in patients having a CBCT or not. Slightly lower DAP (median 3375 vs. 4499 cGy*cm2; p = 0.09) was seen in procedures where CBCT was used. CONCLUSION: A CVP in addition to NBCA embolization was associated with increased growth of the FLR compared to NBCA alone.

Quality of life in patients with vascular malformations outside the central nervous system: Comparison with the general Norwegian population.

PURPOSE: The aim of this study was to assess Health-Related Quality of Life (HRQoL) in a population of patients with vascular malformations outside the central nervous system (CNS) and to compare the results with data from a national reference population. METHODS: In total, 111 consecutive patients above 14 years of age and referred for the first time to the national vascular malformation center from September 2011 to December 2012 were included. HRQoL was assessed using the Short-Form 36-item questionnaire (SF-36), which is a validated questionnaire with eight domains, covering both physical and mental aspects of HRQoL. The results were compared with national reference values. Possible association between HRQoL and selected demographic and clinical variables was analyzed using linear regression analysis. RESULTS: The sample consisted of 47 men (42.3%) and 64 women (57.7%). The median age of patients was 27 years (range 14-63 years). Ninety-six patients (86.5%) were diagnosed with venous malformations and nine patients (8.1%) with arteriovenous malformations. Six patients had other types of malformations (9%). The patients had significantly lower SF-36 scores in all domains, except for General health, than the general population. There was a significant association between muscular involvement and lower SF-36 scores in the physical domains Bodily pain and Role limitation due to physical problems. CONCLUSIONS: Our data suggest that patients with vascular malformations outside the CNS have impaired quality of life when compared with the general population. Muscular involvement seems to be associated with worse HRQoL in the physical aspects.