BACKGROUND Slow-flow vascular malformations (SFVMs) are rare congenital anomalies that cause significant morbidity. Currently, no evidence-based therapy exists to improve SFVM symptoms and reduce its relapse in the long-term. Sclerotherapy is central to management and widely practiced, yet comprehensive international data are limited and no standardized guidelines exist. We aimed to evaluate current practice patterns, focusing on sclerotherapy and multidisciplinary care. METHODS An international cross-sectional survey was distributed to experts in the International Society for the Study of Vascular Anomalies (ISSVA) network. The questionnaire addressed treatment indications, sclerosant use and availability, anesthesia, coagulation management, risk communication, and case-based decision-making. Responses were analyzed descriptively. RESULTS Fifty-two physicians from 16 countries completed the survey. Respondents included interventional radiologists (69%), vascular medicine specialists (15%), and pediatric surgeons (8%) in academic centers (90%). Multidisciplinary consultation was reported by 92% of participants. Ethanol (67%), bleomycin (58%), and polidocanol (52%) were the most accessible, although ethanol was consistently rated as the most dangerous. Sclerosant choice varied by lesion type and scenario. The risks most frequently communicated were skin necrosis (92% for lymphatic malformations and 94% for venous malformations, respectively), pigmentary changes (70% and 75%), and nerve injury (68% and 75%), whereas systemic complications such as cardiovascular collapse (16% and 22%) or death (14% and 22%) were less often discussed. In children, general anesthesia was preferred (90% and 86%), whereas adults received local (49% and 41%) or general anesthesia (47% and 43%). Preprocedural coagulation testing and anticoagulation strategies showed marked heterogeneity, especially for venous malformations. CONCLUSIONS This survey demonstrates substantial variability in SFVM management, particularly regarding sclerosant selection, anesthesia, coagulation, periprocedural care, and risk communication, and identifies areas where future prospective and collaborative research will be needed before evidence-based recommendations can be developed.
Background: Osteogenesis imperfecta (OI) is a congenital bone fragility disorder, in which 90% of patients have abnormalities in type 1 collagen. Cardiovascular complications, including valvular heart disease and vascular aneurysms, are reportedly comorbid with this disease. Methods: Forty-three adult Japanese patients clinically diagnosed with OI were recruited. Participants underwent echocardiography, brain magnetic resonance imaging (MRI), and magnetic resonance angiography. We compared the prevalence of cardiovascular comorbidities with that of a healthy Japanese population using direct age-standardization and a one-sample binomial test. Results: The median age was 42.5 (IQR 37.4–53.5) years. OI severity was mild in 11, moderate in 11, and severe in 21 patients. Two patients had ⩾ moderate aortic regurgitation (moderate OI: 1; severe OI: 1), one patient had severe mitral regurgitation (moderate OI: 1), and another had previously received valvuloplasty for mitral regurgitation (mild OI: 1). The prevalence of valvular heart disease (9.3%) was significantly higher than that of the healthy Japanese population (0.8%) ( p < 0.001). Five patients (11.6%) had unruptured intracranial aneurysms (UIAs) (mild OI: 2; severe OI: 3), and the prevalence was significantly higher than that of the healthy Japanese population (2.5%) ( p < 0.005). Notably, two patients presented with both valvular heart disease and a UIA. Additionally, 27 patients (62.8%) had tortuosity in the intracranial arteries, and seven patients (16.3%) had significant cortical atrophy. Conclusion: This study revealed an increased prevalence of valvular heart disease, UIAs, and possibly intracranial arterial tortuosity and cortical atrophy among adults with clinically diagnosed OI. Routine echocardiography, and brain MRI and MRA are recommended regardless of disease severity. Trial registration: UMIN-CTR; UMIN000041901 .
Background: In popliteal artery aneurysms (PAAs), computed tomography angiography (CTA)-derived intraluminal thrombus burden and distal runoff vessel patency remain insufficiently characterized. We examined this relationship and the added value of thrombus burden assessment in CTA-based radiologic evaluation of PAAs. Methods: This retrospective, single-center study included PAA cases. Aneurysm size, anatomical extent, percentage-based intraluminal thrombus burden, and coexisting aneurysms were assessed. Distal runoff was evaluated using a tibial runoff score based on the number of patent tibial arteries. Associations between runoff, clinical variables, and CTA variables were analyzed. Results: Sixty-eight patients (median age: 73 years) with 101 PAA-affected limbs were included (median aneurysm diameter: 23 mm). Thrombus was present in 73% of affected limbs and was associated with larger aneurysms ( p = 0.001). Multisegmental aneurysms were significantly larger than monosegmental aneurysms ( p < 0.001). In univariable analysis, increasing thrombus burden was associated with impaired distal runoff vessel patency (odds ratio [OR] per 10% increase: 0.85; 95% CI: 0.76–0.94; p = 0.001). Thrombus burden remained independently associated with impaired runoff after adjustment for age, sex, aneurysm diameter, and concomitant abdominal aortic aneurysm (adjusted OR per 10% increase: 0.84; 95% CI: 0.76–0.94; p = 0.001), whereas maximum aneurysm diameter was not. Concomitant abdominal aortic aneurysm was independently associated with preserved runoff vessel patency (adjusted OR: 2.34; 95% CI: 1.01–5.43; p = 0.046). Conclusion: CTA-derived intraluminal thrombus burden is independently associated with impaired distal runoff vessel patency in PAAs. Assessment of thrombus burden can be readily incorporated into routine CTA interpretation without additional imaging or postprocessing.
