The objective was to prepare guidelines to perform the current optimum treatment by organizing effective and efficient treatments of hemangiomas and vascular malformations, confirming the safety, and systematizing treatment, employing evidence-based medicine (EBM) techniques and aimed at improvement of the outcomes. Clinical questions (CQs) were decided based on the important clinical issues. For document retrieval, key words for literature searches were set for each CQ and literature published from 1980 to the end of September 2014 was searched in Pubmed, Cochrane Library, and Japana Centra Revuo Medicina (JCRM). The strengths of evidence and recommendations acquired by systematic reviews were determined following the Medical Information Network Distribution System (MINDS) technique. A total of 33 CQs were used to compile recommendations and the subjects included efficacy of resection, sclerotherapy/embolization, drug therapy, laser therapy, radiotherapy, and other conservative treatment, differences in appropriate treatment due to the location of lesions and among symptoms, appropriate timing of treatment and tests, and pathological diagnosis deciding the diagnosis. Thus, the Japanese Clinical Practice Guidelines for Vascular Anomalies 2017 have been prepared as the evidence-based guidelines for the management of vascular anomalies.

Objective: To evaluate the effect and safety of sclerotherapy in patients with difficult-to-resect venous malformations treated with ethanolamine oleate. Design and setting: This investigator-initiated clinical trial employed a multicenter, single-arm design and was conducted in Japan. Patients: Overall, 44 patients with difficult-to-resect venous malformations were categorized into two cohorts: 22 patients with cystic-type malformations and 22 patients with diffuse-type malformations, including children (<15 years old). Interventions: Adult patients received injections of 5% ethanolamine oleate solution, double diluted with contrast or normal saline, with a maximum dose of 0.4 mL/kg. The same method of administration was used for children (<15 years old). The maximum volume of the prepared solution in one treatment was 30 mL. Evaluation methods: Treatment effect was assessed by evaluating the difference in lesion volume using magnetic resonance imaging as a primary endpoint and differences in pain using a visual analog scale as a key secondary endpoint. Results: Among the 45 patients who consented, one was excluded owing to potential intracranial involvement of venous malformations during screening. Regarding the primary outcome, 26 of 44 patients (59.1%, 95% confidence interval: 44.41–72.31%) achieved ≥ 20% reduction in malformation volume, with 16 patients having cystic lesions (72.7%, 51.85–86.85%) and 10 patients having diffuse lesions (45.5%, 26.92–65.34%). Both cohorts showed significant improvement in self-reported pain scores associated with lesions 3 months post-sclerotherapy. No death or serious adverse events occurred. Hemoglobinuria was observed in 23 patients (52%), a known drug-related adverse event. Prompt initiation of haptoglobin therapy led to full recovery within a month for these patients. Conclusions: Ethanolamine oleate shows potential as a therapeutic sclerosing agent for patients with difficult-to-resect venous malformations.

Background/Objectives: The therapeutic efficacy of percutaneous sclerotherapy (PS) for venous malformations (VMs) based on volumetric magnetic resonance imaging (MRI) measurements and its association with early post-treatment coagulation markers remains unexplored. This study evaluates the therapeutic efficacy of 5% monoethanolamine oleate (EO)-based PS in treating difficult-to-resect VMs using volumetric MRI and investigates its association with early changes in coagulation markers. Methods: This post-hoc analysis utilized data from a prospective, open-label, multicenter clinical trial initiated on 1 January 2021. The correlation between MRI-determined volume reduction and post-sclerotherapy changes in coagulation markers was assessed. Results: Between January 2021 and April 2023, 44 patients underwent EO-based PS. Based on a ≥ 20% VM volume reduction, patients were classified into “achieved” (n = 26; 59.1%) and “non-achieved” (n = 18; 40.9%) groups. D-dimer levels significantly increased on postoperative day 1 (POD1) compared with pretreatment screening (p < 0.001), whereas fibrinogen and prothrombin international normalized ratio levels remained unchanged. In the achieved group, a significant correlation was observed between the volume reduction rate and the administered EO dose per lesion volume (mL/cm³; Spearman’s ρ = 0.43, p = 0.03). The non-achieved group showed significantly higher D-dimer elevation than the achieved group (p = 0.03). Conclusions: This is the first multicenter study to evaluate EO-based PS efficacy for VMs using volumetric MRI and explore its relationship with early post-treatment coagulation markers. Elevated D-dimer levels on POD1 were not predictive of treatment efficacy, highlighting their limited clinical utility in assessing therapeutic response.

Negative pressure wound therapy with instillation and dwell time (NPWTi‐d) and conventional NPWT are established treatments for complex wounds, yet direct comparative evidence remains limited. This retrospective observational study evaluated the clinical effectiveness of NPWTi‐d for hard‐to‐heal necrotic wounds using standardized parameters from our previous research. We included 116 patients (50 NPWTi‐d; 66 conventional NPWT). The primary endpoint was the time from therapy initiation to achieving a ‘reference wound’ suitable for definitive closure, defined in accordance with clinical trial guidance from the Japanese Pharmaceuticals and Medical Devices Agency (PMDA). The NPWTi‐d group achieved reference wound status in a median of 14 days versus 22 days for the conventional NPWT group—a statistically significant 8‐day reduction. Furthermore, whereas conventional NPWT typically requires a prolonged debridement phase prior to application, NPWTi‐d can be initiated earlier, even in the presence of residual necrotic tissue. Ultimately, NPWTi‐d provides a synergistic clinical advantage by both shortening active treatment duration and bypassing pre‐initiation delays, enabling clinicians to achieve surgical readiness significantly faster than with standard modalities.

Objective To evaluate the efficacy and safety of sclerotherapy in patients with difficult-to-resect venous malformations treated with ethanolamine oleate. Design and setting This investigator-initiated clinical trial employed a multicenter, single-arm, open-label design and was conducted in Japan. Patients Overall, 44 patients with difficult-to-resect venous malformations were categorized into two cohorts: 22 patients with cystic-type malformations and 22 patients with diffuse-type malformations. Interventions Patients received injections of 5% ethanolamine oleate solution, double diluted with contrast or normal saline, with a maximum dose of 0.4 mL/kg. The same method of administration was used for children (<15 years old). The maximum volume of the prepared solution in one treatment was 30 mL. Evaluation methods Treatment efficacy was assessed by evaluating changes in lesion volume using magnetic resonance imaging and changes in pain using a visual analog scale. Results Among the 45 patients who consented, one was excluded owing to potential intracranial involvement of venous malformations during screening. Regarding the primary outcome, 26 of 44 patients (59.1%, 95% confidence interval: 44.41–72.31%) achieved ≥ 20% reduction in malformation volume, with 16 patients having cystic lesions (72.7%, 51.85–86.85%) and 10 patients having diffuse lesions (45.5%, 26.92–65.34%). Both cohorts showed significant improvement in self-reported pain scores associated with lesions 3 months post-sclerotherapy. No death or serious adverse events occurred. Hemoglobinuria was observed in 23 patients (52%), a known drug-related adverse event. Prompt initiation of haptoglobin therapy led to full recovery within a month for these patients. Conclusions Ethanolamine oleate shows potential as a therapeutic sclerosing agent for patients with difficult-to-resect venous malformations.