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.

Significance: In Klippel–Trenaunay syndrome (KTS), management of a wound in the affected limb can be difficult because of the underlying vascular malformations present. This review describes the characteristics of KTS with wound complications in light of the genetic and molecular mechanisms of the disease. Recent Advances: KTS is a slow-flow combined vascular malformation characterized by the triad of capillary malformation, venous malformation with or without lymphatic malformation, and limb overgrowth. KTS is encompassed within the phosphatidylinositol-4,5-bisphosphate3-kinase catalytic subunit alpha ( PIK3CA )-related overgrowth spectrum (PROS), having recently been linked to activating mutations in the PIK3CA gene. This clearly has implications for both molecular diagnosis and potential treatment strategies for the disease. Critical Issues: KTS should be distinguished from Parkes Weber syndrome, a fast-flow-type combined vascular malformation with limb overgrowth. Individualized management is needed for KTS and should be focused on the treatment of symptoms. Future Directions: Targeted therapies that inhibit the phosphoinositide 3-kinase signaling pathway are a potential treatment option for PROS.

Background/Objectives: Vascular malformations form masses in subcutaneous and muscular tissues throughout the body and are occasionally misdiagnosed as subcutaneous nonvascular tumors. Understanding and differentiating their clinical and imaging features are crucial due to their different treatments and prognoses. This study aimed to report cases of nonvascular tumors that were initially misdiagnosed and treated as vascular malformations. Methods: In this retrospective observational study, we enrolled 14 (1.8%) patients with pathologically diagnosed nonvascular tumors from among 536 patients with 759 lesions of clinically diagnosed vascular malformations. Results: The average age at the initial visit was 41.9 years, with a male-to-female ratio of 3:11. Tumor locations included the lower limb in seven patients, the upper limb in five patients, and the trunk and head in one patient each. Ultrasound evaluation revealed 12 lesions of low-flow vascular malformations and two lesions of high-flow vascular malformations. These findings led to an initial diagnosis of venous or lymphatic malformations in 12 patients and arteriovenous malformations in two patients. Based on the clinical diagnosis, treatments administered before tumor resection included sclerotherapy in four patients and transcatheter arterial embolization in one patient. All patients underwent tumor resection. The final histopathological diagnoses included schwannoma in six patients, epidermal cyst and angiomyoma in two patients each, and other types of tumors in four patients. The average time from initial diagnosis to final histopathological diagnosis was 370 days. Conclusions: Multimodal diagnostic strategies, especially the use of ultrasound, enhance the differentiation between vascular malformations and nonvascular tumors.

Auricular arteriovenous malformation (AVM) occasionally accompanies macrotia. Here, the authors report a case of AVM with macrotia that was treated with transcatheter arterial embolization, percutaneous sclerotherapy, and subsequent otoplasty following partial resection. A 46-year-old man presented with Schobinger stage III AVM. After transcatheter arterial embolization of the feeding arteries using n-butyl-2-cyanoacrylate, 9 sessions of sclerotherapy were performed using 3% polidocanol foam. Partial resection of the AVM nidus and subsequent otoplasty for ear reduction were performed at the age of 50 years. Two years later, the remnant nidus was resected and the protruding ear was surgically corrected. No recurrence was observed, and the enlarged ear was reduced at follow-up 6 months after the final operation.

Background: Treatment of oropharyngolaryngeal venous malformations (VMs) remains challenging. This study evaluated the effectiveness and safety of fluoroscopy- and endoscopy-guided transoral sclerotherapy for oropharyngolaryngeal VMs in a hybrid operation room (OR). Methods: Patients with oropharyngolaryngeal VMs who underwent transoral sclerotherapy in a hybrid OR were enrolled. Results: Fourteen patients (six females, eight males; median age of 26 years; range, 4–71 years) were analyzed. The symptoms observed were breathing difficulties (n = 3), snoring (n = 2), sleep apnea (n = 1), and swallowing difficulties (n = 1). Lesions were extensive in the face and neck (n = 9) and limited in the oropharyngolarynx (n = 5). A permanent tracheostomy was performed on two patients, while a temporary tracheostomy was performed on five patients. The treated regions were the soft palate (n = 8), pharynx (n = 7), base of the tongue (n = 4), and epiglottis (n = 1). The median number of sclerotherapy sessions was 2.5 (range, 1–9). The median follow-up duration was 81 months (range, 6–141). Treatment outcomes were graded as excellent (n = 2), good (n = 7), or fair (n = 5). The post-treatment complication was bleeding (n = 1), resulting in an urgent tracheostomy. Conclusions: Fluoroscopy- and endoscopy-guided transoral sclerotherapy in a hybrid OR can be effective and safe for oropharyngolaryngeal VMs.