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Vascular anomalies (VAs) are a heterogeneous group of vascular malformations and tumors that are frequently encountered in daily practice. While most are benign, malignant vascular tumors exist, as do other nonvascular soft-tissue malignancies that may masquerade as a benign VA. The subtleties of arriving at an accurate diagnosis can be intimidating, as new entities are described and names of conditions have been modified. Therefore, the goal of this article is to describe the classic imaging findings of VAs so that the interventional radiologist can confidently and competently contribute to management decisions, with special attention to ominous imaging features that should prompt biopsy or further imaging workup. [ABSTRACT FROM AUTHOR]

This observational retrospective study conducted at Hasan Sadikin General Hospital (RSHS) in Bandung from January 2019 to February 2023 aimed to characterise oral hemangiomas and vascular malformations. Out of 52 medical records analysed, infantile hemangioma was the most common type (17%), typically presenting between ages 13 to 24 years. Predominantly located around the lip, tongue, and buccal mucosa, it seldom caused bleeding or pain, with women being more affected. Treatment often involved sclerotic agent injections. Venous malformations accounted for 58% of vascular malformations, showing similar age of presentation and distribution as hemangiomas, and were also treated with sclerotic agents. Accurate diagnosis and tailored treatment are crucial due to the diverse nature of these anomalies. Early detection using imaging techniques like ultrasound and CT angiography is imperative, given the potential impact on patients, including pain, swelling, and cosmetic concerns, particularly in the head and neck area. However, treatment approaches vary based on factors such as type and size, necessitating further research to refine diagnostic and therapeutic strategies and optimise patient outcomes. [ABSTRACT FROM AUTHOR]

PHACE (posterior fossa malformations, hemangiomas, arterial anomalies, cardiac anomalies, eye anomalies) association has many recognized clinical features. A link between PHACE and non‐vascular intracranial lesions has not been well‐described. We report three pediatric patients with PHACE and non‐vascular intracranial lesions. [ABSTRACT FROM AUTHOR]

According to the International Society for the Study of Vascular Anomalies (ISSVA) classification, vascular anomalies include a diverse range of pathologies, classified as either vascular tumors or vascular malformations. This classification, last revised in 2018, aims to explain the biological basis of vascular lesions and help clinicians to manage the anomalies. In vascular tumors, there are proliferative changes of endothelial cells, while vascular malformations primarily consist of structural vascular abnormalities. Infantile hemangioma is the most common soft-tissue vascular tumor. Vascular malformations are an extensive group of malformations of the arterial, venous, and lymphatic systems, either in isolation or in combination. Radiological evaluation plays a key part in the management of pediatric patients with these entities. The understanding of sonography and magnetic resonance imaging findings entails its correlation with clinical findings at the time of scanning. [ABSTRACT FROM AUTHOR]

Background/Objectives: Ulceration is a common complication of infantile hemangioma (IH). Severe, persistent ulceration occurs in a minority of patients. This study aims to characterize the clinical features of IH with aggressive ulceration (AU). Methods: Multicenter retrospective study of clinical features of IH with AU. Results: Thirty‐five patients with AU were identified and included in the study. The majority of AU occurred in segmental IH (23/35, 65%). Segmental IH with AU were large (≥10 cm2; 16/23, 69%, p <.001) with a thin (<3 mm) superficial component (16/23, 69%, p <.001). Localized IH with AU had a thick (>3 mm) superficial component (11/12, 92%, p <.001). All diaper area IH with AU (9/35) were segmental with thin superficial component (100%, p =.02). IH with AU in the head/neck (10/35) were more commonly localized (67%) and mixed (62.5%), while segmental, thick superficial morphology was more common on trunk (9/35) and upper extremities (7/35). Conclusions: IH resulting in AU differ in clinical features by anatomic site. Those in the diaper area are nearly always segmental with thin superficial component, whereas other sites tend to be localized, mixed, with thick superficial component. These distinct phenotypes may prove useful in the clinical setting for physicians to identify patterns of IH ulceration with increased risk of aggressive, persistent ulceration. [ABSTRACT FROM AUTHOR]

Infantile haemangioma (IH) is the most common benign tumour in childhood, with an incidence of 4% to 12%. Aim: to describe the characteristics of infantile haemangioma in a sample of Romanian children <2 years old at diagnosis, types of treatment applied, recorded complications and the response to the therapeutic approach. A two-year prospective case series study (August 2019 to August 2021) was carried out. Sample: 117 patients <24 months of age diagnosed with IH at the Emergency Hospital for Children "Marie Sklodowska Curie", in Bucharest, Romania. Five therapeutic approaches were used: oral treatment with propranolol, local treatment with timolol, surgical treatment, topical treatment with steroids and no treatment ("wait and see"). Recorded factors mentioned in the literature were also present in this study population: female patients—68.4%; phototype I—58%. In 53% of cases, IHs had a head and neck location and 10% developed local complications (traumatic bleeding). The majority of patients (86%) required one type of therapy: oral propranolol (51%). A low relapse rate was recorded (4%). We consider that any child with a vascular anomaly should be referred to a highly specialised medical service for therapeutic approach. [ABSTRACT FROM AUTHOR]

Context: Congenital vascular anomalies are classically subdivided into vascular tumors and vascular malformations. The role of propranolol in the regression of infantile hemangioma (IH), a vascular tumor, is well established. Aims: This study aimed to analyze the therapeutic efficacy and complications associated with oral propranolol and adjuvant therapy in the treatment of vascular anomalies. Settings and Design: A prospective interventional study was undertaken over 10 years duration extending from 2012 to 2022 at a tertiary care teaching institute. Materials and Methods: All children with cutaneous hemangiomas and lymphatic and venous malformations under 12 years of age, except those with contraindications for administration of propranolol, were included in the study. Results: Out of 382 patients, there were 159 males and 223 females (male: female = 1:1.4). The majority (53.66%) were between ≥3 months and 1 year. There were 481 lesions in 382 patients. There were 348 patients with IH, and 11 were congenital hemangiomas (CHs). There were 23 patients with vascular malformations; lymphatic malformation (n = 19) and venous malformation (n = 4) were present. The size of the lesions ranged from 5 mm to 20 cm; 50.73% were 2-5 cm in size. Ulceration (>5 mm) was the most common complication present in 20/382 (5.24%) patients. Complications related to oral propranolol were seen in 23 (6.02%) patients. Drugs were given for a mean period of 10 months (range from 5 months to 2 years). At the end of the study, 282 (81.03%) out of 348 patients with IH showed an excellent response; 4 (36.36%) patients in the case of CH (n = 11) and 5 (21.74%) patients with vascular malformation (n = 23) showed excellent response. Conclusion: The study validates the use of propranolol hydrochloride as the first-line agent for the treatment of IHs and congenital hemangiomas. It may have an additive role in lymphatic malformations, and venous malformations, as a part of a multimodality treatment approach for vascular malformations. [ABSTRACT FROM AUTHOR]

The prevalence of hemangiomas in children reaches 45.7% of all benign childhood tumors, among newborns up to 1/4 of children are affected by the disease. The complexity of diagnosing and treating hemangiomas is exacerbated by the lack of a unified classification capable of providing mutual understanding between pediatricians, dermatologists, pediatric surgeons, and oncologists. One of the generally recognized problems in the treatment of hemangiomas is the definition of indications for the start of treatment. A serious problem is the treatment of ulcerated hemangiomas, accompanied by infection and bleeding. The tactics of managing a patient with hemangioma require an individual approach. For example, propranolol therapy and laser removal of hemangiomas are the most effective, safe, and cosmetically acceptable methods of removal. Surgical treatment and electrocoagulation show a persistent, relapse-free effect. As part of the scientific work, a statistical study of 40 case histories of children was carried out. The article presents data on the distribution of the disease by sex, and age, as well as by location and method of treatment. [ABSTRACT FROM AUTHOR]

There is a lot of ambiguity in the usage of correct terminology in the description of vascular malformations and tumors. Hemangioma and arteriovenous malformation (AVM) are the most commonly used terms and are the mostly incorrectly used as well! The aim of this review article was to lay out the correct nomenclature and describe the correct usage for the physicians and radiologists involved in diagnosing and managing these lesions. We describe the various classification systems which have been devised to define the multiple entities included under vascular anomalies. The latest classification system that should be adhered to is per the International Society for the Study of Vascular Anomalies, approved at the 20th ISSVA Workshop held in Melbourne in April 2014, last revised in May 2018. The main features of the latest revision have been highlighted. This classification, however, does not list the diagnostic clinico-radiological features for each entity. In addition, guidelines regarding the appropriate use of available imaging modalities are lacking in the literature. We, hereby, aim to address these pertinent issues in this review article. [ABSTRACT FROM AUTHOR]

Even though vascular anomalies of the hand are rare entities (7%), they are relevant regarding soft tissue mass differential diagnosis on the hand. 2 The majority of cases tend to be malformations, once denominated as deep soft tissue hemangiomas. 1,3 Due to evolving knowledge regarding vascular anomalies, the prognosis, guidelines regarding treatment and aftercare following therapy/surgery will be more accurate and detailed if the correct diagnosis is established. Retrospective data collection and analysis between 2008 and 2019, from the Orthopedic Department of Semmelweis University tumor registry. Comparison to the current literature (PubMed, Ovid). Study level of evidence III. Standardly distributed data with confidence level of 95%. N = 16, average age of 34,2. 63,5% of lesions were digital. Hemangiomas accounted for 67%. The accuracy of clinical vs. histological diagnosis was 77%. Recurrences 25%. Variable follow up period. Clinical examination, radiological evaluation, patient history and fine needle biopsy are often accurate guides. Nevertheless in our study, histological analysis of the surgical biopsy was the most effective method in establishing a definite diagnosis. Conservative and watchful waiting approach are the first line, and when complemented with appropriate imaging, should suffice until absolute or relative operative indications are present. [ABSTRACT FROM AUTHOR]

Epithelioid hemangioma is a rare, histologically benign but locally aggressive primary vascular neoplasm that can rarely arise in bone. Mainstay treatment is surgical resection or curettage with bone grafting. We report a novel multidisciplinary, joint-sparing treatment approach for an epithelioid hemangioma of bone arising in the acetabulum causing severe thinning of the subchondral bone plate. After 4 sessions of transarterial bland particle and ethanol embolization, the resultant increased ossification of the tumor allowed preservation of the articular surface during surgical resection. Imaging follow-up 14 months after surgical resection showed no evidence of recurrence and continued ossification of the portions of the lesion treated only with embolization. [ABSTRACT FROM AUTHOR]

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Vascular anomalies are classified as vascular tumors or vascular malformations according to their cellular features and biological behavior. Detailed history and clinical assessment allow for the proper clinical diagnosis of most vascular anomalies and guide the choice of imaging to evaluate them. This article discusses the general information needed from a clinical history and physical exam to formulate a diagnosis of vascular anomaly. Then, the authors review the clinical findings from the most common vascular tumors and vascular malformations. [ABSTRACT FROM AUTHOR]

OBJECTIVE: To describe and compare the histopathological characteristics of oral vascular anomalies and to classify them according to the system of the International Society for the Study of Vascular Anomalies (ISSVA). MATERIAL AND METHODS: Cross-sectional, observational and retrolective study, in which the clinicopathological characteristics of benign vascular lesions of the buccal mucosa were analyzed, from samples received between January 1980 and December 2016, at the Oral Histopathology Laboratory of the Universidad Autónoma Metropolitana, Xochimilco Unit, through a consecutive, non-probabilistic sampling. The samples were classified according to the ISSVA criteria. For the statistical analysis, the SPSS v.21 program was used; frequencies and central (mean, median) and dispersion (min-max, interquartile interval) measures were calculated. The association between variables was calculated through χ², Fisher's exact and U-Mann Whitney tests, with an alpha value < 0.05. RESULTS: From 11,034 samples, 143 (1.4%) corresponded to benign vascular abnormalities, most (60.8%) in women, with a median age of 33 (1-83, Q1-Q3:18-52) years. The frequency of hemangiomas was 54.5%, a proportion that decreased to 22.4% after applying the ISSVA criteria. Key histological differences were the relation with adjacent tissues (malformations: 28.7%, neoplasms: 7.1%) and the inflammatory infiltrate (neoplasms: 71.4%, malformations: 45.5%). CONCLUSIONS: The application of standardized systems such as ISSVA is essential for differentiating neoplastic processes of vascular malformations, and for the correct diagnosis and therapeutic management. [ABSTRACT FROM AUTHOR]

Background: Off-label drug use is associated with an increased risk of adverse drug reactions. It is common in pediatrics and in rare diseases, which are two characteristics applying to vascular anomalies (VA). Objectives: The aim of this work was to quantify off-label drug use in VA and assess its safety. Methods: A review was conducted to extract a list of drugs used in VA management. A drug was considered to have significant safety concerns if a black box warning was present or if a serious adverse drug reaction (SADR) was reported in at least 1% of the patients (SADR is defined as a noxious and unintended response to a drug that occurs at any dose and results in hospitalization, prolongation of existing hospitalization, congenital malformation, persistent or significant disability or incapacity, life-threatening condition, or death). The labelling status and safety of each drug was assessed based on the product monograph, Micromedex, and the FDA data. Results: We found that 98.9% of the inventoried drugs were used off-label or unlicensed for VA management. Only the oral solution of propranolol hydrochloride (Hemangeol®) for the treatment of infantile hemangiomas is approved. Significant safety issues concerned 73% of the drugs and were more frequent among systemic than locally delivered drugs. Conclusions: Off-label drug use in VA is the rule and not the exception. Significant safety concerns are common. It is necessary to carefully weigh risk and benefits for every patient when using systemic and local treatments carrying safety concerns. Patients should be openly informed and involved in the decision-making process. [ABSTRACT FROM AUTHOR]

Objectives: Infantile hemangiomas (IHs) are the most common benign tumors in children. Color Doppler ultrasound is a noninvasive imaging modality that can show subclinical anatomic parameters in a wide range of dermatologic conditions. The purpose of this study was to describe the ultrasound characteristics of IHs and look for subclinical features with the potential to influence the involution and therapeutic response. Methods: A review of the ultrasound database of children with clinical and ultrasound IH diagnoses was conducted. The clinically reported duration and the ultrasound assessment of the proliferative phase were compared. Descriptive and statistical analyses of qualitative and quantitative parameters of the series were performed. Significance was set at P <.05. Results: A total of 204 IHs were included. Twenty percent had arteriovenous shunts; 15% had afferent branches from main regional arteries; and almost 30% showed involvement of deep structures. Sixty‐one percent of IHs were in the head and neck. Deep hemangiomas showed significantly thicker lesional vessels. A prolonged proliferative stage was significantly associated with a higher presence of arteriovenous shunts and a higher peak systolic velocity of the arterial vessels (≥15 cm/s) within the lesions. Conclusions: Color Doppler ultrasound can support the detection of subclinical anatomic features that may potentially influence the involution and response to treatment of IHs. Some of these characteristics may serve as potential markers to predict and manage IHs in prolonged proliferative stages. [ABSTRACT FROM AUTHOR]

Infantile and congenital hemangiomas still a challenge for both the pediatric surgeon and the pediatrician. The treatment of hemangiomas must be individualized according to: the type of lesion, location, size, depth, stage of growth and evolution of the lesion. To date, none of the available therapies is considered standard therapy. Currently, personalized therapy is not widely applied in Romania, not being included in the usual therapy protocols in any of the types of hemangioma. This is a starting point for the identification of new specific therapeutic targets that preserve normal endothelial cells and determine the regression of hemangioma, especially recurrent ones and those with an increased proliferation rate. Another controversial and unexplained aspect, consequently unexploited from a therapeutic point of view is represented by the expression and role of lymphatic markers in infantile and congenital hemangiomas. [ABSTRACT FROM AUTHOR]

Congenital hemangiomas (CHs) are unusual and diverse tumors distinguished from infantile hemangiomas by being largely developed at birth and glucose transporter (GLUT1)‐negative. We describe three infants who presented in utero or at birth with segmentally distributed vascular tumors that were GLUT1‐negative, had histology compatible with congenital hemangioma, and exhibited spontaneous clinical involution. One of the three patients had high‐output cardiac failure and was found to have a mutation in GNAQ (c.626A>c, p.Gln209Pro); another had high‐output cardiac failure, heterotaxy, and transient hematologic abnormalities and was found to have a mutation in GNA11 (c.626_627delinsCC, p.Gln209Pro). In addition to describing a novel segmental pattern of congenital hemangioma variant with genetic correlations, these cases illustrate the utility of targeted genetic testing to elucidate the exact mutation and thus classification of vascular tumors. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Background: The term vascular anomalies include various vascular tumors and vascular malformations, among them infantile hemangiomas and capillary malformations are the most well-known associated diseases in early ages. Multiple drugs have been introduced for intervention, but susceptibility test in vitro were scarcely reported.Objective: To evaluate the inhibition effect of different drugs by adenosine triphosphate sensitivity assay in vitro before the treatment of infantile hemangiomas and capillary malformations.Methods: Specimens were selected from 5 cases of infantile hemangiomas and 11 cases of capillary malformations. Propranolol, rapamycin, sildenafil and itraconazole were tested for their growth inhibition effect by using the adenosine triphosphate sensitivity assay.Results: Propranolol demonstrated inhibitory effects on infantile hemangiomas cells. Rapamycin and itraconazole both showed inhibitory effects on infantile hemangiomas cells and capillary malformations cells. Sildenafil has no growth inhibitory effect on infantile hemangiomas cells or capillary malformations cells.Conclusion: Adenosine triphosphate sensitivity assay is a sensitive and useful testing method before the management of vascular anomalies, and individualized medication suggestions for the choice of therapeutic drugs were offered based on the testing result and together with a comprehensive evaluation of each infant. [ABSTRACT FROM AUTHOR]

Objective: Vascular anomalies are a diverse group of lesions, ranging from simple to complex, disfiguring anomalies. Our objective was to diagnose and provide comprehensive treatment to patients presenting with vascular anomalies, using a multi-disciplinary approach involving dermatologists, plastic surgeons, radiologists and pediatric surgeons. Methods: Patients presenting with vascular anomalies to The Indus Hospital, Karachi, from January 2017 to March 2019 were enrolled, using a pre-defined questionnaire. Assessment, diagnostic work up, management and clinical and photographic follow up was maintained to monitor outcomes. Results: One hundred eighty seven patients with a mean age of 4.6 years, (females 62%) were enrolled. Diagnoses included vascular tumors (n=89, 47.6%), lymphatic malformations (n=38, 20.3%), capillary malformations (n=19, 10%), venous malformations (n=16, 8.5%), arterio-venous malformations (n=14, 7.5%) and mixed anomalies (n=11, 5.9%). Treatment modalities, in isolation or combination, included oral propranolol, topical timolol, pulsed dye laser and intra-lesional sclerotherapy. Mean follow up was in 7.1 months, with 27 patients achieving treatment completion. 26 children were lost to follow-up. Conclusions: Vascular anomalies have mostly been managed successfully at VAC using single or multimodal treatment. Increasingly complex anomalies can be handled using a multi-disciplinary approach. Establishment of VAC has facilitated many patients who were earlier considered as diagnostic and therapeutic challenges. [ABSTRACT FROM AUTHOR]

Hemangiomas and venous malformations are frequently encountered in the head and neck region, particularly in children. The management of such lesions is especially difficult, considering the characteristics of the age group and the possible complications and sequelae regarding appearance and function. The high esthetic demands of the region, the presence of significant anatomical structures, as well as the concerns for airway maintenance make the choice of treatment extremely challenging. The local injection of a sclerosing agent represents a minimally invasive method that is often used as a first-line treatment of such lesions. The purpose of this manuscript is to review the indications, outcomes and complications of bleomycin sclerotherapy in the treatment of vascular anomalies in the maxillofacial territory. [ABSTRACT FROM AUTHOR]

Background: The 595-nm pulsed dye laser (PDL) has been used to treat vascular anomalies for about 30 years; however, there are insufficient data in Chinese patients concerning therapeutic efficacy, optimized parameters, and procedure techniques. Objective: To study the efficacy and relevant factors in PDL therapy for vascular anomalies in Chinese patients. Method: We enrolled 431 patients with 8 different vascular anomalies and no previous treatment in this retrospective study. A detailed classification of vascular anomalies and various parameters and techniques of PDL were studied. The clinical outcomes were analysed using the Investigator Global Assessment. Results: Improvements were significantly correlated with infantile haemangioma (IH) subtypes (p < 0.05). A significant correlation between efficacy and lesion colour, anatomical sites, and hypertrophic-type port-wine stain (PWS) was found (p < 0.05). There was no significant correlation between efficacy and age or sex (p > 0.05). Conclusion: PDL is an effective and safe therapeutic modality for managing vascular anomalies in Chinese patients. We determined that differentiating and identifying IH subtypes prior to treatment could be a useful parameter for predicting therapeutic results. Lesion colour, sites, and hypertrophic changes in PWS are relevant therapeutic factors. PDL parameters and techniques differ according to the various vascular anomalies to achieve optimal results. [ABSTRACT FROM AUTHOR]

Objective: To quantify complications and rates of adverse events associated with surgical excision of hemangiomas in children using a national surgical database. Data source: Pediatric American College of Surgeons (ACS) National Surgical Quality Improvement Program® (ACS NSQIP®‐pediatric), years 2012‐2016. Methods: Subjects included children under 18 years with a postoperative diagnosis of International Classification of Diseases (ICD), 9th revision code: 228.00 and 228.01, or ICD 10 codes D18.00 and D18.01—hemangioma, any site and hemangioma, of skin and subcutaneous tissue. Results: A total of 1180 patients were included. The median age was 3.2 years (interquartile range [IQR] 1.2 to 6.4 years old). No occurrences of deep incisional surgical site infection, nerve injury, mortality, sepsis, septic shock, or blood stream infections were noted. In multivariate analysis, age ≤ 3 years and advanced American Society of Anesthesiologists class were significant predictors of adverse events (P = 0.035 and 0.001, respectively). Conclusions: For children with infantile hemangioma who fail to respond or have an incomplete response to medical therapy, surgical resection may be a relatively safe treatment alternative. Children older than 3 years of age with hemangiomas had less surgical adverse events than younger children. Overall, however, the surgical complications rates were low and deemed relatively minor. [ABSTRACT FROM AUTHOR]

Introduction Gastrointestinal infantile hemangiomas (IHs) are extremely rare and represent a diagnostic challenge, especially in newborns for the limited applicability of diagnostic approaches. We report two cases of neonates with gastrointestinal bleeding from intestinal hemangioma and a literature review of gastrointestinal IHs. Case 1: Full-term baby, asymptomatic at birth. On the seventh day of life she experienced failure to thrive, weight loss and hypotonus followed by melena and vomit. The imaging showed an intestinal vascular anomaly. We performed exploratory laparotomy and intestinal resection to remove the lesion. No complication occurred. Case 2: A 20-day old girl had melena and severe anaemia. The scintigraphic evaluation excluded gastric heterotopia. We opt for explorative surgery and we performed a laparoscopy that showed a congested and oedematous intestinal segment that was resected. Post-operative course was uneventful. Discussion Gastrointestinal IHs may cause intractable bleeding, intussusception and intestinal perforations with bad prognosis. A prompt and accurate diagnosis is vital but not always easy. Colour-coded Duplex Scan are a good first-line imaging, MRI and CT give a better definition but the latter exposes to radiation. In the “era” of minimally invasive surgery, laparoscopy can have a role in the management of patients with gastrointestinal IHs. Conclusions Gastrointestinal IHs should be considered in the differential diagnosis of neonates with anemia and gastro-intestinal bleeding. In selected cases, the minimally invasive approach permits diagnosis and treatment. [ABSTRACT FROM AUTHOR]

Vascular anomalies of the head and neck region are a complex group of lesions that challenge the head and neck physicians. From the very understanding of the difference between its two distinct forms, hemangiomas and vascular malformations to its management remain confusing. The review of this anomaly attempts at comprehensively understanding the disease. Vascular anomalies are easily diagnosed by their clinical presentation, but choice of imaging and management for this spectrum of lesions is varied. The author attempts to categorize the required imaging for the lesion with suggestions on the management of both hemangiomas and vascular malformations. The available treatment options are discussed, and a comprehensive algorithm for management is suggested. Further research in developing drugs that could restrict the growth of these lesions would be the future of the management of vascular lesions. [ABSTRACT FROM AUTHOR]

Objectives To evaluate cases diagnosed as “oral hemangiomas” based on the immunohistochemical expression of human glucose transporter protein (GLUT-1) and on histopathological features, and to investigate whether the classification proposed by the ISSVA was used correctly to classify these lesions. Material and methods All cases stored in the archives of an Oral Pathology Service and diagnosed as “oral hemangiomas” were reviewed. Seventy-seven cases were analyzed regarding the expression of GLUT-1. GLUT-1(+) specimens were classified as true infantile hemangioma (IH) and GLUT-1(−) specimens were reclassified based on their histopathological features. The nomenclature of these lesions was evaluated and some cases were reclassified. Results Only 26 (33.8%) of the specimens were indeed IHs. Among the GLUT-1(−) specimens, 20 (26.0%) were reclassified as pyogenic granulomas (PGs) and 31 (40.2%) as vascular malformations. Considering the previously applied nomenclature, only 47.5% of the cases initially diagnosed as “hemangiomas” were IHs. In the group of “capillary hemangiomas”, most cases (56.2%) were PGs. Among the three “cellular hemangiomas”, two were PGs and one was IH. Most (88.8%) “cavernous hemangiomas” were vascular malformations. Conclusion Careful and parameterized review of cases of vascular anomalies is necessary using auxiliary tools such as GLUT-1, since the exclusive use of histopathological findings might be insufficient to differentiate some anomalies. Clinical relevance Accurate clinical examination and the use of biomarkers such as GLUT-1 are essential for the diagnosis. [ABSTRACT FROM AUTHOR]

Vascular anomalies present both a diagnostic and therapeutic challenge to physicians. Identification of these lesions is difficult due to their immense phenotypic variability, and naming conventions for vascular anomalies have historically been inconsistent. Terms such as "hemangioma" are informative when used correctly, but can cause confusion and miscommunication if applied indiscriminately to all vascular anomalies. Accuracy in classification is essential, as both disease course and therapeutic options differ greatly depending on the particular vascular anomaly present. In order for clinicians to properly diagnose and treat patients with these diseases, a unified nomenclature must be employed. This section provides an update on the current classification of vascular anomalies, with clinical descriptions of the most commonly encountered lesions, and clarifies the ambiguous nomenclature present in the existing literature. [ABSTRACT FROM AUTHOR]

Congenital vascular anomalies constitute a large group of lesions in children, and they differ in terms of their localization, outer appearance, as well as histology. Additionally, the nomenclature of this group of lesions has been confusing due to the indiscriminate and interchangeable use of the term haemangioma and vascular malformation in diagnosis and management. Whilst hemangiomas are true endothelial cell neoplasms, vascular malformations are localized congenital defects of vascular morphogenesis. The aim of this article is to overcome obstacles in communication when describing a vascular lesion by adhering to the correct terminology. This leads inevitably to the correct therapeutic guidelines, management and follow-up of these lesions. The therapy guidelines, management and follow-up of hemangiomas and vascular malformations differ and are beyond the scope of this article. [ABSTRACT FROM AUTHOR]

Vascular anomalies are mainly classified under two headings, i.e. under vascular tumors and vascular malformations. Hemangioma and lymphangioma are examples for such a vascular anomalies. Malformations may be seen in different combinations of vascular elements, and histologically these vessels may be filled with blood and named lymphangiohemangioma or hemangiolymphangioma according to the dominant tissue structure present. It is a rare developmental anomaly. This paper reports a case of lymphangiohemangioma in a 21-year-old female patient. [ABSTRACT FROM AUTHOR]

Objective To summarise the role of fine needle aspiration cytology in the differential diagnosis of vascular anomalies from other lesions in the head and neck region by presenting five interesting cases reported from our hospital data. Method Five patients ranging in age from 3 months to 25 years old were diagnosed with vascular anomalies based on their histories, clinical examinations, imaging examinations and ultrasonic testing. The previous diagnosis of these patients were corrected by fine needle aspiration cytology in our hospital and the proper treatment was administered. Result Two cases were wrongly treated in a local hospital. All five of the patients underwent operations after excluding vascular anomalies by fine needle aspiration cytology. Conclusion Fine needle aspiration cytology should be used in the following situations to prevent misdiagnoses and the mistreatment as vascular anomalies: when imaging examination and ultrasonic testing lead to a diagnosis of vascular malformation, but the history or clinical examination does not confirm this diagnosis; when lesions are treated as “vascular malformations” by sclerotherapy, and the hydatid fluid is not like blood or lymph fluid; and when propranolol is used to treat the proliferation stage of “haemangiomas” with without any effect. [ABSTRACT FROM AUTHOR]

Aim of the Study mTOR inhibitors are showing promising results in the management of vascular anomalies. Although current controlled trials remain to be completed, many individual experiences are being published. We present our series of children with complex vascular anomalies treated with sirolimus. Patients and Methods A retrospective review of 41 patients treated with sirolimus between January 2011 and December 2015 was performed: 15% (n = 6) had vascular tumors (4 kaposiform hemangioendotheliomas, 1 PTEN) and 85% (n = 35) had malformations (13 generalized lymphatic anomalies/Gorham-Stout diseases [GSD], 1 kaposiform lymphangiomatosis [KLA], 11 large lymphatic malformations (LMs) in critical areas, 2 lymphedemas, 4 venous malformations, and 4 aggressive arteriovenous malformations [AVM]). Several variables were collected: type of vascular anomaly, duration of treatment, dosage, response, and secondary effects. Results There was a female predominance (1.4:1). All patients received sirolimus, at initial dosage of 0.8 mg/m2/12 hour. Overall successful response rate was 80.4% of cases, presenting improvement in radiologic imaging and reduction of symptoms, at a median time of 10 weeks. Patients showing no response included four AVMs, one GSD, one LM, one KLA, and one unknown tumor. Sirolimus was well tolerated, even in neonates, with insignificant side effects. No patients had complete resolution and no patients worsened on therapy. Thirty patients remain under treatment at the present moment. Conclusion Sirolimus has become a new therapeutic option for patients with vascular anomalies that do not respond to other treatments. Unfortunately, important questions as what is the most appropriate dosage and for how long should the patient be treated remain unanswered. An international registry followed by customized controlled trials is mandatory to clarify the future of this therapy. [ABSTRACT FROM AUTHOR]

Low-flow venous malformations are congenital lesions and they are the third most common vascular anomaly in the head and neck. In this paper, the third in a series of three educational reviews, we discuss current trends in their management, and include a summary of common sclerosant agents used in their control. [ABSTRACT FROM AUTHOR]

Congenital hemangiomas are benign vascular tumors, categorized by their postnatal behavior as rapidly involuting, non‐involuting, or partially involuting. They are typically solitary, with a predilection for the head or limbs near a joint. We present two infants with small, multifocal congenital nonprogressive hemangiomas of the skin, one associated with hepatic and intracranial lesions, and another with an in utero intracranial hemorrhage and hydrocephalus. These cases further extend the differential diagnosis of congenital multifocal vascular lesions or "hemangiomatosis." [ABSTRACT FROM AUTHOR]

Las anomalías vasculares han sido tradicionalmente tratadas en conjunto aun cuando engloban patologías tan diferentes como hemangiomas y malformaciones vasculares. La clasificación dada a conocer por Mülliken y Glowacki, modificada posteriormente, no es suficientemente conocida ni usada en nuestro medio. Se revisa la clasificación actual y la correlación con imágenes de aquellas anomalías vasculares situadas en partes blandas, dada la importancia que tiene un correcto diagnóstico para la terapéutica y pronóstico de los pacientes con estas lesionesVascular anomalies include different pathologies as hemangiomas and vascular malformations, however they usually are reported together and confusion is created. Mülliken and Glowacki proposed a classification posteriorly, modificated, adapted and actually in use. Unfortunately is not sufficiently known for our radiologist. Classification and imaging correlation of soft-tissue vascular anomalies are revised, since a exact diagnosis is a great value for appropriate treatment and prognosis

Objective To study the arterial spin-labeled (ASL) imaging features of vascular anomalies in the head and neck. Materials and methods The presence or absence of ASL signal was evaluated for each vascular anomaly, and a comparison with conventional magnetic resonance (MR) sequences was performed. Results Twenty-five children were identified with vascular anomalies. All proliferating infantile hemangiomas demonstrated hyperintense ASL signal. There were eight cases that ASL imaging provided additional information when compared with conventional MR sequences, including increased lesion conspicuity and altered perfusion. Conclusions ASL imaging of cutaneous vascular anomalies shows specific signal intensity patterns and can provide additional value when compared to conventional MR sequences. [ABSTRACT FROM AUTHOR]

Aim: We identified the characteristics of an infantile haemangioma (IH) that predispose children to complications, interventions and long-term morbidity and examined perinatal risk factors for IH.Methods: We studied children with IHs admitted to Helsinki University Hospital's paediatric vascular anomaly clinic in Finland in 2004-2007 and registered perinatal records, IH characteristics, complications and interventions. These patients received a questionnaire on perinatal data and long-term morbidity resulting from IH. We analysed factors related to complications, interventions and morbidity and compared our cohort's perinatal data to the Finnish Medical Birth Register (FMBR) figures.Results: We approached 185 families, of which 136 replied to the questionnaire. Children with facial, segmental and indeterminate IHs showed more complications, interventions and higher long-term morbidity. Preterm birth predisposed infants to ulceration of IHs, with a 95% confidence interval (CI) of 1.02-5.14 and odds ratio (OR) of 2.29. In addition to earlier known risks, maternal gestational diabetes mellitus rate was higher in our IH cohort than the rate in the FMBR (95% CI 1.39-4.95, OR 2.62).Conclusion: Physicians treating IHs should consider the elevated ulceration risk in preterm infants. The association between gestational diabetes mellitus and child's risk for an IH is uncertain and requires further research. [ABSTRACT FROM AUTHOR]

PHACE syndrome is a rare neurocutaneous disorder characterized by posterior fossa malformations, hemangiomas, arterial anomalies, cardiac defects, and abnormalities of the eye. Thyroid disorders associated with PHACE syndrome have been described, although there are limited reports of this rare occurrence. We report a case of PHACE syndrome with congenital hypothyroidism in an infant, for which absent thyroid gland was diagnosed at magnetic resonance imaging. [ABSTRACT FROM AUTHOR]

Unlabelled: Vascular anomalies include a heterogeneous group of disorders that are categorized as vascular tumors or vascular malformations. Treatment options include resection, embolization, laser therapy, and sclerotherapy or medical treatment such as propranolol, steroids, interferon, and cytostatic chemotherapy. Mammalian target of rapamycin seems to play a key role in the signal pathway of angiogenesis and subsequently in the development of vascular anomalies. Recently, the successful use of sirolimus has been reported in children with lymphatic malformations and kaposiform hemangioendotheliomas. We report on six patients with different vascular anomalies (kaposiform hemangioendothelioma n = 2, combined lymphatico-venous malformation n = 2, pulmonary lymphangiectasia n = 1, and orbital lymphatic malformation n = 1) who were treated with peroral sirolimus. Three of the children initially presented with a Kasabach-Merrit phenomenon. Median duration of treatment was 10 months; two children are still on treatment. Three children each achieved complete and partial remission. Kasabach-Merrit phenomenon resolved within 1 month in all patients. Treatment with sirolimus was tolerated well; only mild reversible leukopenia was observed.Conclusion: Sirolimus proved to be effective in children with complicated lymphatic or lymphatico-venous malformations and kaposiform hemangioendotheliomas. Treatment was tolerated well with acceptable side effects. The optimum length of treatment and possible long-term side effects have to be evaluated.What Is Known: • Vascular anomalies including vascular tumors and vascular malformations may lead to life-threatening conditions.• Some patients are refractory to established treatment and/or are not available for local invasive procedures.What Is New: • We reviewed the literature focusing treatment of vascular anomalies inc hildren and adolescents.• Our data support recent studies that sirolimus is an effective treatment option in patients with complicated vascular tumors andmalformations [ABSTRACT FROM AUTHOR]

Aim: Patients with vascular anomalies are often misdiagnosed, leading to delayed or improper treatment. The aim of this study was to evaluate the impact of an interdisciplinary team on the diagnosis and treatment of paediatric patients with vascular anomalies.Methods: We reviewed the paediatric patients evaluated by our interdisciplinary team between 2002 and 2012, analysing the referral diagnosis, final diagnosis, patient age, sex, clinical history, laboratory tests, imaging studies and treatments.Results: Of the 480 patients who were evaluated, 435 (90.6%) had a vascular anomaly: 30.7% of all patients had a tumour and 55.2% had a malformation. Haemangiomas comprised 93.2% of all tumours, while malformations included capillary (9.8%), lymphatic (30.1%), venous (36.8%), arteriovenous (3.8%) and combined slow-flow (7.9%) malformations. Tumours were initially diagnosed correctly in 89.2% of the patients, but only 38.0% of the malformations were diagnosed correctly. Improper treatment was given to 1.4%, due to incorrect diagnoses.Conclusion: This study showed that haemangiomas were likely to be diagnosed correctly, but other tumours and vascular malformations were likely to be misdiagnosed. Misdiagnosis seldom led to improper treatment, but probably led to delayed treatment in many cases. The interdisciplinary approach led to improved diagnostics and treatment. [ABSTRACT FROM AUTHOR]

Vascular anomalies can be detected in utero and should be considered in the setting of solid, mixed or cystic lesions in the fetus. Evaluation of the gray-scale and color Doppler US and MRI characteristics can guide diagnosis. We present a case-based pictorial essay to illustrate the prenatal imaging characteristics in 11 pregnancies with vascular malformations (5 lymphatic malformations, 2 Klippel-Trenaunay syndrome, 1 venous-lymphatic malformation, 1 Parkes-Weber syndrome) and vascular tumors (1 congenital hemangioma, 1 kaposiform hemangioendothelioma). Concordance between prenatal and postnatal diagnoses is analyzed, with further discussion regarding potential pitfalls in identification. [ABSTRACT FROM AUTHOR]

Objective To study the demographic and clinical features of infantile hemangioma in Uzbekistan; to learn in more detail the risk factors for developing this disease; and to identify clinical characteristics associated with complications, associated risks, and the need for systemic treatment. Material and Methods A case-control study of 1,832 prospectively enrolled children and 1,832 controls matched with hemangiomas for age, sex, region, and hospital attending the maxilla facial surgery and dermatology departments between 2008 and 2012 was conducted. The patient data were collected from archives of three large hospitals in Uzbekistan Demographic and clinical presentations were summarized and compared with data from previous studies of hemangiomas. Predictive clinical factors for complications and/or treatment and potential risk factors for infantile hemangiomas were analyzed by logistic regression. Results The clinical features of this study's patients were different from those of other race/ethnicity groups reported by previous studies with regard to the morphological subtypes, complications, and predictors for complications and/or surgical and medicament treatment. After adjustment, significant risk factors for hemangiomas included lower level of maternal education (odds ratio [OR], 0.61; 95% confidence interval [CI], 0.57-0.66), mother engaged in manual labor (OR, 1.29; 95% CI, 1.12-1.48), multiple gestation (OR, 1.20; 95% CI, 1.05-1.36), maternal medication use during the periconceptional period (OR, 2.08; 95% CI, 1.88-2.31), and a positive family history of hemangiomas (OR, 1.55; 95% CI, 1.40-1.72). Conclusion Besides yielding several new findings with respect to risk factors for hemangiomas, the current study also suggests that the Uzbekistan clinical features of hemangiomas are somewhat different epidemiologically from those in the West. [ABSTRACT FROM AUTHOR]

Noninvoluting congenital hemangiomas (NICH) are rare and poorly understood vascular tumors that are present at birth, characterized by lack of growth after birth and lack of involution. We report uncharacteristic cases of NICH hypertrophy occurring later in life. This is a case series describing the clinical presentation, management, and histologic characteristics of two cases of NICH hypertrophy. Two patients with a NICH of the scalp experienced lesion hypertrophy in teenage or early adult life. Case 1 is a 14-year-old female who presented with a flat left parietal scalp lesion that at first grew slowly with the patient; however, over the span of months grew substantially resulting in an exophytic lesion. The patient had the lesion surgically excised. Case 2 is a 26-year-old female with NICH of left occipital scalp and posterior neck who noted new nodules on the inferior border of the lesion. MRA/MRI showed extension into the occipital calvarium, level V of the neck, and paraspinal musculature. The patient elected to observe given the extent of the lesion and her minimal symptoms. Although postnatal growth of NICH have been described, cases usually occur during the pre-adolescent period where growth is usually proportional to overall growth of the patient. This study describes two cases of rapid onset NICH hypertrophy occurring later in life. Knowledge of the potential for delayed hypertrophy may lead families to seek earlier intervention or opt for more definitive interventions. Additionally, recognition of these variable distinctions will contribute to a better understanding of CH and its various subtypes. • NICH may have a risk of hypertrophy later in life. • Surgical excision is an option for patients and depending on the extent of the lesion. [ABSTRACT FROM AUTHOR]

Cutaneous vascular anomalies are congenital disorders of abnormal vascular development and growth. Infantile hemangioma is a common type of vascular anomalies characterized by the abnormal growth of blood vessels in the early proliferative phase, followed by the gradual spontaneous regression of the lesion in the involuting phase. Over the past decade, significant advances have been made in our understanding of the cellular and molecular mechanisms that control the development, growth, and regression of infantile hemangioma. In this article, we present a comprehensive review of the current knowledge of the pathogenesis of hemangioma as well as promising research horizons and implications for new therapeutic advances. [ABSTRACT FROM AUTHOR]

The term "vascular anomalies" embraces a spectrum of medical conditions characterized by abnormal growth or development of blood and/or lymphatic vessels. Patient management is frequently provided in a multidisciplinary team approach, as there are many facets to evaluation and treatment. Clinical and basic research during the past decade has enhanced our knowledge, providing insight into possible etiologies, associated genetic mutations, cellular mechanisms modulating the development, and natural history of these disorders. Concomitantly, new therapeutic agents have been identified, which has transformed patient management. In this review, a brief overview of the field including an update in basic research is presented, followed by a discussion of current therapies and their purported mechanism of action. [ABSTRACT FROM AUTHOR]

Objective: The aim of this study is to assess our results of the treatment in children with vascular anomalies. Methods: We retrospectively reviewed the charts of 704 patients with 843 vascular anomalies treated in the Department of Pediatric Surgery between January 1996 and June 2012. Results: Sex ratio was females/males 1.5:1, 69% were less than one year of age at the time of admission. The average time of hospital stay was 2 days. 83.5% of vascular anomalies were hemangiomas and 83,6 % of these affected the skin and subcutaneous tissue. 64% of hemangiomas were tuberous and most were located on the head and neck. 11% affected multiple sites and/or were associated with other vascular anomalies. Conclusion: This audit allows us to improve the diagnosis, methods of treatment and improving our clinic's recording. Our results stress on the necessity for implementation of educational programs among the people living in the countryside, so that they would become aware of the importance of addressing the physician for their children's health concerns. [ABSTRACT FROM AUTHOR]