Characteristics of mucocutaneous vascular malformations drawn from a decade of a multidisciplinary committee experience

Abstract

Vascular malformations (VM) are congenital, benign, and relatively frequent lesions. Scant data have been published about the epidemiology, clinical presentation, and treatment of VM from a dermatologist's perspective. The substantial differences between subtypes, broad range of specialists consulted and confusing nomenclature used over previous years may hamper a correct diagnosis. The main objective of this study is to describe VM epidemiology. As a secondary endpoint we evaluate clinical characteristics, clinical‐radiological correlation and treatment approaches. We carried out an observational, descriptive, retrospective study. Cases presented to the multidisciplinary committee of our hospital from 2009 to 2019 were retrieved. Electronic medical records, monthly committee reports and the iconographic archive were reviewed and statistically analyzed. Overall, venous malformations (VeM) are the most frequent VM, followed by capillary malformations (CM), arterioVeM and lymphatic malformations (LM). Considering only patients under 16, CMs are the most frequent ones. Capillary and LMs are larger than venous or arteriovenous. While CMs are usually asymptomatic, symptomatic cases are threefold more frequent in the other subtypes. Decisions on active or conservative management depend on VM size but not location or patient age. CMs are mainly treated with laser therapy; venous with sclerotherapy or surgery; arteriovenous with surgery and lymphatic with surgery or sirolimus. Dermatologists play an important role in VM diagnosis and management. Our 10‐year multidisciplinary experience should contribute to the literature and represent a practical resource for clinicians and researchers.

Clinical phenotype of adolescent and adult patients with extracranial vascular malformation

“…Most published studies focus on a pediatric or mixed age population. 16,21,22,30 Those that report the prevalence in the pediatric population partially overlap with our findings of a predominance of VM, 20,22 but differ in the proportion of other ISSVA subgroups.…”

“…Consequently, single-center studies do not always have a representative distribution of their patient population, 16,17 do not show age disaggregated data 17,18 or present one region preferably. 19,20 Many publications are limited to pediatric population 21,22,23 rather dominated by lymphatic 24 and complex malformations associated with other anomalies 25,26 or a particular subgroup of CVM with or without genetic assessment. 17,20 Furthermore, the classification of patients often lacks clearly described clinical criteria for diagnosis, the classification used or the handling of unclear diagnoses.…”

“…19,20 Many publications are limited to pediatric population 21,22,23 rather dominated by lymphatic 24 and complex malformations associated with other anomalies 25,26 or a particular subgroup of CVM with or without genetic assessment. 17,20 Furthermore, the classification of patients often lacks clearly described clinical criteria for diagnosis, the classification used or the handling of unclear diagnoses. 16,17,20,27 As a result, despite the some descriptions of the above-mentioned populations, it becomes almost impossible to compare them.…”

“…17,20 Furthermore, the classification of patients often lacks clearly described clinical criteria for diagnosis, the classification used or the handling of unclear diagnoses. 16,17,20,27 As a result, despite the some descriptions of the above-mentioned populations, it becomes almost impossible to compare them.…”

Update on lasers in pediatric dermatology: how primary care providers can help patients and families navigate appropriate treatment options and timelines

“…These patients require multidisciplinary care, often with a combination of medical, surgical, and vascular interventions, as well as laser therapy whenever appropriate. Procedural interventions for venous malformations primarily consist of embolization or sclerotherapy, and surgery for glomovenous malformations [17]. Imaging is recommended prior to treatment both to determine the full extent of the malformation, and to exclude a high-flow vascular malformation that requires occlusion of feeder vessels prior to laser [18].…”