ISSVA Classification Research Articles

Assessment of gene–disease associations and recommendations for genetic testing for somatic variants in vascular anomalies by VASCERN-VASCA

BackgroundVascular anomalies caused by somatic (postzygotic) variants are clinically and genetically heterogeneous diseases with overlapping or distinct entities. The genetic knowledge in this field is rapidly growing, and genetic testing is now part of the diagnostic workup alongside the clinical, radiological and histopathological data. Nonetheless, access to genetic testing is still limited, and there is significant heterogeneity across the approaches used by the diagnostic laboratories, with direct consequences on test sensitivity and accuracy. The clinical utility of genetic testing is expected to increase progressively with improved theragnostics, which will be based on information about the efficacy and safety of the emerging drugs and future molecules. The aim of this study was to make recommendations for optimising and guiding the diagnostic genetic testing for somatic variants in patients with vascular malformations.ResultsPhysicians and lab specialists from 11 multidisciplinary European centres for vascular anomalies reviewed the genes identified to date as being involved in non-hereditary vascular malformations, evaluated gene–disease associations, and made recommendations about the technical aspects for identification of low-level mosaicism and variant interpretation. A core list of 24 genes were selected based on the current practices in the participating laboratories, the ISSVA classification and the literature. In total 45 gene–phenotype associations were evaluated: 16 were considered definitive, 16 strong, 3 moderate, 7 limited and 3 with no evidence.ConclusionsThis work provides a detailed evidence-based view of the gene–disease associations in the field of vascular malformations caused by somatic variants. Knowing both the gene–phenotype relationships and the strength of the associations greatly help laboratories in data interpretation and eventually in the clinical diagnosis. This study reflects the state of knowledge as of mid-2023 and will be regularly updated on the VASCERN-VASCA website (VASCERN-VASCA, https://vascern.eu/groupe/vascular-anomalies/).

Fibro-adipose vascular anomaly: A clinico-radiologic review

Fibro-adipose vascular anomaly remains in the ‘provisionally unclassified’ group of the ISSVA classification of vascular anomalies, since being first described in 2014. We present a series of 22 children diagnosed as FAVA, describing the presentation, findings on imaging and treatment strategies at a tertiary care hospital in southern India.

Emerging Trends & Techniques Related to Treatment in Patients with intra – Conal Cavernous Hemangioma of the Orbit(OCH)/orbital Cavernous Malformation (OCVM) and Treatment related Complications, A Systematic Review

Orbital Cavernous venous malformation (OCVM), formerly known as cavernous hemangioma of the orbit (OCH) is a frequent benign, slow-growing, non distensible venous malformation with ISSVA classification which classifies it into different types. This research has employed randomized controlled trials (RCTs), case reports, retrospective case studies, systematic reviews, and cohort studies to evaluate the treatment choices for OCVM and its consequences. The goal of this review is to analyze the comprehensive therapies used for OCVM. For our literature search, we looked at PubMed/MEDLINE, PubMed Central and Cochrane across six years (from 2016 to 2021). Using the Cochrane risk of bias quality assessment tool, the found studies underwent a quality check (modified Cochrane RoB 2). For the reporting of this systematic review, we adopted preferred reporting items for Systematic Reviews and Meta-Analyses (PRISMA) 2020 criteria and included a total of 10 papers. The majority of research confirmed the accuracy of OCVM detection using MRI and CT. To evaluate the effects of the intervention on OCVM more precisely, more trials with a longer follow-up time are needed. This study will explore recommendations and guide orbital surgeons, regardless. It will aid in the development of future strategies involving both invasive and noninvasive procedures to close the surgical and complication gap.

A 16-year retrospective study of vascular anomalies in the head and neck region

SummaryDepending on the diagnostic modality, the classification of vascular anomalies varies and so does the nomenclature. The ‘International Society for the Study of Vascular Anomalies’ (ISSVA) is the most widely accepted classification in the literature and is mainly based on the radiologic and clinical presentation. The aim of this article is to review the clinical practice of diagnosis and treatment of vascular anomalies in the head and neck region in a university hospital, with special focus on the nomenclature.All patients with a vascular anomaly presenting to the department of oral and maxillofacial surgery were reviewed in a retrospective manner. Nomenclature, diagnostic process, lesion characteristics, treatment and outcome were examined. The lesions were (re)classified according to the ISSVA classification.A total of 185 patients were identified, of which 12.4% (n = 23) had a congenital anomaly. After reclassification, the most common lesions were venous malformations (n = 47, 25.4%), followed by lobular capillary hemangiomas (n = 17, 9.2%). A group of 39 anomalies could not be further specified. One hundred and one patients (54,6%) received treatment, of which 93 were treated surgically (92,1% of treated patients). Endovascular treatment was considered in 41 patients but applied in only eight. This strict selection led to a low a complication rate.We provide an overview of the clinical practice in the management of vascular anomalies in a university hospital. The histology report is a source of miscommunication because clinicians use the ISSVA classification, while pathologists use the WHO classification. Every professional involved should be aware of the differences in classification and nomenclature.

A Case of Neck Avm Supplied By Arteria Lusoria

Classification of Vascular malformation is based on ISSVA classification. Here is a brief outline of vascular malformation classification.

Contemporary Interpretation of Congenital Vascular Malformations: Extratruncular or Truncular lesions

Due to the wide range of clinical presentations, degree of severity and its location, together with unpredictable clinical course and erratic response to treatment with high recurrence by its nature/pathogenesis, congenital vascular malformation (CVM) remains a most challenging and confusing diagnostic and therapeutic clinical entity. Indeed, old definition of the CVMs based on only the clinical findings and also the classification with name-based eponyms through the last century added more confusion and failed to provide proper information necessary for its advanced management. But Hamburg Classification and ISSVA Classification have been established to meet its mandate for the contemporary management through last two decades as new classifications. The role of old classification became obsolete, no longer needed for contemporary management of the CVMs since both new classifications were based on advanced knowledge of the anatomy, embryology and pathophysiology involved to the CVM pathogenesis as birth defects affecting entire vascular system: artery, vein, and lymphatics. Hamburg Classification can provide more safer clinical guide with its subclassification based on embryological characteristics of the CVM lesions depending upon the stage of embryogenesis when the developmental arrest occurs: 'extratruncular' type for the lesions originated from the 'early' classified with the potential for growth and proliferation to cause the recurrence and subsequent morbidity while 'truncular' type for the lesion from the 'late' stage with more serious hemodynamic impacts. This subclassification would give a role as the clinical histopathological classification to bridge to the future genetic classification till available. ISSVA Classification also provides a clear guideline for the differentiation between the vascular tumor, known as hemangioma, and vascular malformation which was further subgrouped based on the flow characteristics. Further, ‘updated’ ISSVA classification of 2014-2016 is a hybrid classification matching the anatomical and the genetic characteristics of many pathologies. Hence, correct understanding on these two classifications will be critical for proper management of every CVM lesion based on correct identification and confirmation of each CVM lesion on this embryological subtype.

Rare case of inguinal cystic lymphangioma diagnosed and treated in elderly patient

Cystic lymphangioma develops due to the interruption of lymphatic-venous connections during embryogenesis, leading to the formation of a cystic lesion containing lymph. These lesions fall under the ISSVA classification of vascular malformations. The first documented case dates back to 1828, with further elucidation provided by Sabin in 1909 and 1919. Cervicofacial region is the most common site, often showing early-stage symptoms. Inguinal location is rare, but if complications arise, it may present as a strangulated inguinal hernia. The tumor’s severity lies in its compression and invasion of the aerodigestive tract and adjacent organs. Diagnosis relies on imaging techniques such as ultrasound and computed tomography, which help determine the mass’s nature, boundaries, and relationship with neighboring structures. Asymptomatic lesions are generally monitored, while symptomatic ones require complete surgical excision to minimize the risk of recurrence. At Cheikh Khalifa University Hospital, we present a case highlighting our urology department’s experience in diagnosis, patient care, and surgical treatment.

Peripheral vascular lesions in adults referred to MRI/MRA: Multivariable analysis of imaging features to help differentiate benign vascular anomalies from malignancies

PurposeSymptomatic peripheral vascular lesions in adults are often clinically diagnosed as benign vascular anomalies and may receive MRI/MRA for pre-treatment vascular mapping. Malignant neoplasms are difficult to distinguish from benign vascular anomalies on MRI/MRA. This study was performed to determine if there are imaging signs that can distinguish malignancies from benign vascular anomalies in adults imaged with MRI/MRA. Materials and methodsA radiology database was retrospectively searched for ISSVA classification terms in MRI/MRA reports from 1/1/2002–1/1/2019. Adult patients (n = 50, 52 corresponding lesions) with contrast-enhanced MRI/MRA, peripheral soft tissue based lesion (s), and available pathology or long-term (>1 year) imaging follow-up were included. MRI/MRA images were reviewed by 3 readers for the following lesional characteristics: morphology (marginal lobulation, internal septations, distinct soft tissue mass), peri-articular location, T2-weighted characteristics (hyperintensity, heterogeneity, perilesional edema, and adjacent triangular T2-peaks), bulk fat, hemorrhage, enhancement pattern (peripheral, diffuse, or absent), neovascularity, low-flow venous malformation type enhancement, arterial enhancement within 6 s, enhancement curve (progressive, plateau, or washout), measured size, and multifocality. The MRI/MRA features' associated sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) were calculated. To identify factors predictive of malignancy, a two-stage multivariable analysis was performed. Results23% (12/52) of the lesions, corresponding to 22% (11/50) of the patients, were malignant neoplasms. No single imaging feature reliably predicted malignancy (PPV ≤ 60%). Absence of distinct soft tissue mass excluded malignancy (NPV 100%). Multivariate analysis derived a summary score based on the five strongest predictors of malignancy: adjacent T2 peaks, age ≥ 70 years, distinct soft tissue mass, lesion size ≥ 5 cm, and absence of septations. A score ≥ 3 resulted in sensitivity of 92% and specificity of 85%. ConclusionExtremity MRI/MRA rarely differentiates malignant from benign soft-tissue vascular tumors in adults. However, MRI/MRA can suggest malignancy when patient age and multiple imaging features are considered. Periodic clinical follow-up after the planned endovascular or operative procedure should be performed to avoid missing a malignancy.

VEGF Pathway Gene Expression Profile of Proliferating versus Involuting Infantile Hemangiomas: Preliminary Evidence and Review of the Literature.

Background. Infantile hemangiomas may have unexpected behavior. Initial regression (spontaneously or drug-induced) may be followed by unexplained recurrences. At this moment, there are no well-established criteria to predict infantile hemangioma reccurrences. Methods. We compared the VEGF pathway gene expression profile for one case of involuting infantile hemangioma versus one case of recurrent proliferative infantile hemangioma using TaqMan Array. Results. We found ten genes upregulated for both involuting and recurrent proliferative hemangiomas: ACTB, KRAS, MAP2K1, HRAS, NOS3, BAD, HSPB1, HPRT1, GUSB, and CASP9. Thirteen genes were downregulated for both involuting and proliferative hemangiomas: FIGF, ACTG1, GRB2, MAPKAPK2, ACTG2, MAP2K2, MAPK3, HSP90AA1, MAP2K6, NRAS, ACTA1, KDR, and MAPK1. Three genes showed divergent expression between proliferating and involuting hemangiomas. Proliferating hemangioma had MAPK14 and AKT1 gene upregulation and ACTA2 downregulation. Involuting infantile hemangioma was characterized by ACTA2 upregulation and AKT1 and MAPK14 downregulation. Conclusions. Three genes, AKT1, p38/MAPK14, and ACTA2, were found to have divergent expression in proliferating and involuting infantile hemangiomas. Excepting AKT1, which was mentioned in the last ISSVA classification (strictly related to Proteus Syndrome), none of the other genes were reported. An accurate gene expression profile mapping of infantile hemangiomas together with a gene expression-based hemangioma classification is stringently needed.

Reclassification and histopathological study of vascular anomalies according to ISSVA (2018 revision) classification

Background: Vascular anomalies (VAs) represent a spectrum of disorders from simple birthmarks to life-threatening entities. Consistency in the terminology of vascular anomalies and diagnosis by routine histology is necessary for the proper diagnosis and clinical management. Aim: The present study aimed to classify the vascular anomalies according to the 2018 revised classification of ISSVA and to evaluate the role of GLUT-1 (glucose transpoter protein isoform 1) immunomarker as a complementary tool to aid in differentiating vascular anomalies. Materials and methods: In this retrospective study, clinical records of 47 cases reported as vascular anomalies from January 2017 to June 2019 were retrieved from the Department of Pathology, ASRAM Medical College, Eluru. The cases were analyzed based on findings of histochemical stains such as Verhoeff-van Gieson (VVG) and classified as per ISSVA. Further immunohistochemistry studies of GLUT-1 were done. Results: Of 47 patients, the male to female ratio was found to be 1.06:1 and 46.80% of cases were vascular tumors, whereas 53.19% cases were vascular malformations in this study. Of 22 cases histologically diagnosed as vascular tumors, 06 cases were infantile hemangiomas and showed immunopositivity with GLUT-1 marker and the rest of vacular tumors and malformations were immuno negative for GLUT-1. Conclusion: ISSVA classification provides a framework for understanding the morphology, guiding the treatment plan and prognosis. In addition to this, GLUT-1 precisely differentiated infantile hemangioma from other vascular tumors and malformations. Keywords: vascular anomalies; GLUT-1, infantile hemangioma; vascular malformation; vascular tumors

DIFFERENTIAL DIAGNOSES OF ANGIOKERATOMAS

Angiokeratomas (AK) are probably the vascular lesions that induce more confusion in the literature. The most accepted classification of AK was performed by Imperial and Helwig , and they divide these lesions into five classical types: Mibelli’s AK, Fordyce’s AK, corporis diffusum AK, circumscribed naeviforme AK and solitary or multiple acquired angiokeratomas. Nevertheless, in the clinical practice, is not unusual to see AK type lesions associated to different types of vascular anomalies, and these lesions have difficult handling. In ISSVA classification AKs have been included as vascular anomalies provisionally unclassified. We perform the differential diagnoses of AKs and according to clinical picture, histopathological aspect, immunohistochemical markers and radiological findings proposed to divide AKs mainly in two groups. Primary AKs are the classical types. The new immunohistochemical findings suggest that these lesions might be included as mixed capillary-lymphatic malformations. Secondary AKs are related with different vascular anomalies, secondary to other process with lymphatic obstruction, related to drugs, or associated with no vascular lesions as lymphoid lesions. Different underlying vascular anomelies might be related with AKs including deep capillary-lymphatic malformations (CLM), venous-lymphatic malformations (VLM), capillary-lymphatic-venous malformations (CLVM) (Klippel-Trenaunauy Sd), deep lymphatic malformations (LM), venous malformations (VM) as hyperkeratotic venous malformation, cavenomatous cerebral malformations, traumatic arteriovenous fistula and eccrine angiokeratomatous hamartoma. Clinical aspect, radiological studies and histopathological examination might help to do a correct diagnosis of this heterogenous entity.

A rare case of a rapidly expanding high flow vascular anomaly

The classification of vascular lesions is constantly being updated to reflect new understanding of the biology of these lesions. However, there are still lesions that are difficult to classify. We present a case report of a 6-year-old boy who presented with a rapidly enlarging tumour in the scrotum and penis. Despite venous and arterial embolisation with coils, there was minimal improvement and he required a suprapubic catheter. Repeated MRI showed persisting marked enlargement of the penis and scrotum. The lesion had a large central draining vein with drains into the left common femoral vein with multiple feeding branches from the internal iliac arteries. The initial biopsy showed a cellular capillary vascular proliferation in the dermis. Immunohistochemistry showed no expression of GLUT-1 antigen. The biopsy was subsequently sent to Harvard Medical School and the lesion was diagnosed as an unclassified benign haemangioma. The management of this lesion has been challenging and the patient underwent an excision of the peno-scrotal AVM. However, it was difficult to completely excise the lesion due to its nature, large size and location. It was also difficult to subclassify because the specimen was obtained after embolisation. However, given the latest ISSVA classification for vascular lesions, the precise classification may provide prognostic or treatment implications which can guide further management of this case.1

2018 newly revised ISSVA classification for vascular anomalies

2018 newly revised ISSVA classification for vascular anomalies

Giant popliteal vein aneurysm in Parkes-Weber syndrome

Parkes-Weber syndrome (PWS) is a congenital vascular anomaly that is characterized by limb overgrowth, macro arterio-venous fistula, and capillary-venous malformation. 1 ISSVA Classification of Vascular Anomalies [Internet] International Society for the Study of Vascular Anomalies. www.issva.orgDate: 2014 Date accessed: December 22, 2015 Google Scholar Clinical manifestations of PWS include limb hypertrophy, cutaneous ulceration, and congestive heart failure secondary to the high-flow fistula. 2 Straface G. Biscetti F. Ghirlanda G. Flex A. Images in vascular medicine. Parkes Weber syndrome: bone involvement visualized by computed tomography angiography. Vasc Med. 2009; 14: 91-92 Crossref PubMed Scopus (7) Google Scholar

Interdisciplinary Management of Head and Neck Vascular Anomalies: Clinical Presentation, Diagnostic Findings and Minimalinvasive Therapies

ObjectivesVascular anomalies are included in the 30 000 rare diseases worldwide affecting less than 5/10 000 people. Depending on their morphology and biological properties, they can cause varied disorders with organ involvement. Almost 60% of vascular anomalies have a predilection for the head and neck region in children. Clinical and scientific effort to establish interdisciplinary management concepts for vascular anomalies is increasing worldwide. MethodsEspecially in the head and neck region, clinical impairment and organ dysfunction is associated with cosmetic issues that may represent a physical and psychological issue for the patient. Correct diagnosis, based on clinical presentation and symptoms, is a prerequisite for appropriate therapy, ranging from conservative management to a spectrum of minimally invasive treatment options. We searched PubMed for German and English language published data until December 2016 with focus on clinical studies, review articles and case reports on vascular anomalies with a focus on the head and neck region. ResultsThe last ISSVA update in 2014 has contributed to a better understanding of vascular anomalies, classifying them in vascular tumors and vascular malformations. The predominant representatives of vascular tumors are congenital and infantile hemangiomas. Infantile hemangiomas have the ability of spontaneous regression in more than 80%. Patients with symptomatic growing hemangiomas with ulcerations, bleeding complications and restriction of hearing, swallowing disorder, impairment of vision, or cosmetic dysfigurement require treatment. Therapies include oral propanolol, transcatheter embolization and surgery. Vascular malformations tend to progress with patientś age and are subdivided in slow flow and fast flow lesions. Symptomatic slow flow lesions, e.g. venous and lymphatic malformations, benefit from percutaneous sclerotherapy. Fast flow lesions, as arteriovenous malformations, are rare but undoubtedly therapeutically the most challenging vascular anomaly. Depending on location and size, they may require multiple transcatheter embolization procedures for successful occlusion of the AVM. ConclusionsThis review provides knowledge on the current ISSVA classification of vascular anomalies, their clinical presentation, diagnostic evaluation and minimally invasive therapy options to encourage the establishment of a comprehensive interdisciplinary management for head and neck vascular anomalies.

RADIOLOGICAL VISUALIZATION OF VASCULAR ANOMALIES IN CHILDREN

Background. Clinical diagnosis of vascular anomalies is often dif ficult. The informative of dif ferent instrumental evaluations isn’t the same in terms of visualization of vascular anomalies. Purpose: to establish the diagnostic value and informative of different visualization’s methods of vascular anomalies in children. Methods. The study included 194 patients with vascular anomalies in age from 1 month to 16 years. According to the updated ISSVA classification 2014, vascular anomalies divided into vascular tumors and vascular malformations (VMs). Vascular tumors are diagnosed in 95 patients, in particular, infantile hemangiomas (IHs) of soft tissues (80.0%), liver hemangiomas (5.3%), airways IHs (5.3%), IHs as a part of PHACE syndrome (2.1%). Rare vascular tumors were in 7 (7.3%) patients. In 99 patients VMs diagnosed: lymphatic malformations (LM) in 86.8%, venous malformations in 8.1%, and combined VMs in 5.1% of children. Results. Ultrasound examinations performed as a primary method and follow-up studies. The activity of His was determined by the ratio of small diameter vessels and solid tissue. CT with contrast enhanced and MRI performed in 5.3% patients with IHs of soft tissue with atypical clinical presentations and in patients with airway His (9.3%). To confirm the diagnosis of the liver hemangiomas the CT with contrast enhanced performed. In 4.4% patients with well localized cystic LM performed ultrasound only, all other patients underwent MRI. CT scans performed in 10.2% children with LM of head and neck and symptoms of airway compression as emergency diagnostic procedure. The peculiarity of VM is phleboliths formation. It can be detected as ultrasound, so X-ray examination but to determine the distribution of process is the most informative is MRI. Arteriovenous malformation in CDS scale has the same characteristic as the IHs in proliferative phase. So if these features are revealed in older children CT with contrast enhanced is necessary . Conclusions. For the diagnosis of vascular anomalies the primary investigation is ultrasound examination. The “gold standard” for diagnosis of slow-flow VM is the MRI. For the diagnosis of high flow VM informative is the CT scan with contrast enhanced.

Classification of vascular malformations

SummaryWith the term „hemangioma” different vascular anomalies are described. Here one has to discriminate the congenital vascular tumours from the vascular anomalies. But for a clear indication a definite classification is necessary. Basis is the actual ISSVA classification. In the latest version they have incorporated the embryological aspects of the Hamburg Classification. Especially in the vasculogenetic (″extratruncular″) malformations one found increasing genetic defects. Here is an overlap to the neuro-ectodermal syndromes. By this, common therapeutic strategies are in progress.

Clinicopathologic study of pediatric vascular anomalies: a report of 117 cases

To study the clinicopathologic features of pediatric vascular anomalies and application of ISSVA classification. The clinical features, histopathologic findings and immunohistochemical results were analyzed in 117 cases of pediatric vascular anomalies encountered during the period from May 2014 to May 2015. A total of 117 cases of vascular anomalies were studied. The age of patients ranged from 18 hours after birth to 11 years (mean age =34 months and median age =27 months). There were 73 male patients and 44 female patients, with the male-to-female ratio being 1.7∶1.0. Congenital skin lesions were found in 37 cases (31.6%). The common sites of involvement included head and neck region (46 cases, 39.3%), trunk (28 cases, 23.9%), extremities (14 cases, 12.0%) and internal viscera (31 cases, 26.5%). According to the new ISSVA classification, there were 74 cases of vascular malformations and 43 cases of vascular neoplasms (ratio=1.7∶1.0). The commonest vascular tumor encountered was infantile hemangioma (21 cases, 48.8%), including 17 cases in proliferative phase and 4 cases in involutive phase. Thirteen cases (23.3%) of congenital hemangioma were found, with 8 cases of rapidly involuting congenital hemangioma and 5 cases of non-involutive congenital hemangioma. Three of the congenital hemangioma occurred in liver. There were 5 cases (11.6%) of pyogenic granuloma, 3 cases (7.0%) of tufted angioma and 1 case (2.3%) of Kaposiform hemangioendothelioma. Amongst the 74 cases of vascular malformations encountered, lymphatic malformation was found in 47 cases (63.5%), venous malformation in 15 cases (20.2%), lymphatic-venous malformation in 11 cases (14.9%) and arteriovenous malformation in 1 case (1.4%). All cases of vascular anomalies were all positive for CD31 on immunostaining. Glut1 and CD15 were positive both in proliferative and involutive phases of the 21 cases of infantile hemangioma, while other vascular tumors and vascular malformations were negative. Forty-seven cases of lymphatic malformation and 11 cases of lymphatic-venous malformation showed D2-40 expression. Focal positivity for D2-40 was demonstrated in 3 cases of tufted angioma and 1 case of Kaposiform hemangioendothelioma. Vascular anomalies affecting infants and children include tumors and malformations. Accurate histopathologic diagnosis and ISSVA classification of the various types of vascular anomalies play an important role in clinical management.

脈管異常におけるISSVA 分類と静脈奇形の位置づけ

脈管異常におけるISSVA 分類と静脈奇形の位置づけ

Time-resolved contrast-enhanced MRA (TWIST) with gadofosveset trisodium in the classification of soft-tissue vascular anomalies in the head and neck in children following updated 2014 ISSVA classification: first report on systematic evaluation of MRI and TWIST in a cohort of 47 children

To evaluate the relative accuracy of contrast-enhanced time-resolved angiography with interleaved stochastic trajectories versus conventional contrast-enhanced magnetic resonance imaging (MRI) following International Society for the Study of Vascular Anomalies updated 2014-based classification of soft-tissue vascular anomalies in the head and neck in children. Time-resolved angiography with interleaved stochastic trajectories versus conventional contrast-enhanced MRI of children with diagnosis of soft-tissue vascular anomalies in the head and neck referred for MRI between 2008 and 2014 were retrospectively reviewed. Forty-seven children (0-18 years) were evaluated. Two paediatric neuroradiologists evaluated time-resolved MRA and conventional MRI in two different sessions (30 days apart). Blood-pool endovascular MRI contrast agent gadofosveset trisodium was used. The present cohort had the following diagnoses: infantile haemangioma (n=6), venous malformation (VM; n=23), lymphatic malformation (LM; n=16), arteriovenous malformation (AVM; n=2). Time-resolved MRA alone accurately classified 38/47 (81%) and conventional MRI 42/47 (89%), respectively. Although time-resolved MRA alone is slightly superior to conventional MRI alone for diagnosis of infantile haemangioma, conventional MRI is slightly better for diagnosis of venous and LMs. Neither time-resolved MRA nor conventional MRI was sufficient for accurate diagnosis of AVM in this cohort. Conventional MRI combined with time-resolved MRA accurately classified 44/47 cases (94%). Time-resolved MRA using gadofosveset trisodium can accurately classify soft-tissue vascular anomalies in the head and neck in children. The addition of time-resolved MRA to existing conventional MRI protocols provides haemodynamic information, assisting the diagnosis of vascular anomalies in the paediatric population at one-third of the dose of other MRI contrast agents.