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Purpose:  Analysis of clinical and diagnostic findings in rare urogenital and perineal vascular malformations only occurring in 2-3 % of vascular anomalies with regard to clinical symptoms and treatment decisions., Materials and Methods:  All 25 out of 537 patients presenting with congenital urogenital and perineal vascular malformations at our institution from 2014 to 2021 were included. Vascular anomaly classification, anatomical location, clinical symptoms at presentation, diagnostic imaging, and pain intensity were retrospectively assessed from the patient record and therapy management was evaluated., Results:  In total, 25 patients (10 females (40 %), 15 males (60 %)), aged 6 to 77 years were included. Diagnoses were: 10 (40 %) venous malformations (VMs), 5 (20 %) lymphatic malformations (LMs) and 10 (40 %) arteriovenous malformations (AVMs). Malformation manifestations were: 12 (32 %) lesser pelvis, 12 (32 %) external genitalia, and 13 (34 %) perineal/gluteal region. One AVM was located in the kidney. The leading clinical symptom was pain. The mean intensity was 6.0/10 for LM, 5.7/10 for VM, and 4.5/10 for AVM. Further major symptoms included physical impairment, local swelling, and skin discoloration. Bleeding complications or sexual dysfunction were rare findings. Patients with VM reported significantly more symptoms than patients with AVM (p = 0.0129). In 13 patients (52 %) minimally invasive therapy was indicated: 10 (77 %) sclerotherapies and 3 (23 %) transcatheter embolization procedures. Complete symptomatic remission was achieved in 9 (69 %) patients, partial response in 3 (23 %) patients, and 1 patient showed no clinical response to therapy. Follow-up appointments without the need for immediate minimally invasive therapy were significantly more common in patients with AVMs than in patients with VMs (p = 0.0198)., Conclusion:  To create a higher awareness of congenital urogenital and perineal vascular malformations. Awareness of this rare condition avoids misdiagnosis. Therapy decisions should be symptom-oriented. Emergency intervention is rarely required, even in fast-flow vascular malformations., Key Points:   · Venous malformations cause more symptoms with higher pain intensity than arteriovenous malformations.. · Diagnosis and adequate treatment can be hampered by a lack of awareness of the clinical presentation.. · Bleeding complications are rare, even in high-flow vascular malformations.. · Pain and physical impairment are the most commonly observed symptoms in these patients.., Competing Interests: The authors declare that they have no conflict of interest., (Thieme. All rights reserved.)

Background: Vascular malformations are rare diseases that should be treated in dedicated vascular anomaly centers (VAC). There is only a small amount of data on the diagnostic and therapeutic handling of these patients, before they are referred to a VAC., Purpose: To demonstrate the disease-specific patient characteristics in a German VAC, which are required to determine diagnostic and therapeutic steps., Material and Methods: In a retrospective study, all patients who were treated in the VAC from April 2014 until August 2021 were identified. In total, 593 patients were included in this study., Results: Almost all patients had previously consulted a physician (591/593, 99.7%). A mean of two different physicians had been consulted (range 0-10). Patients with more complex, syndromal vascular malformations had significantly more previous appointments ( P  = 0.0018). In only 44% (261/593) of patients, the referral diagnosis was made correctly. Most patients had been previously treated for their vascular anomaly: pharmacotherapy (n = 130; 21.9%), compression garments (n = 141; 23.8%), surgical resection (n = 80; 17.3%) and sclerotherapy (n = 68; 11.5%). Fifty-two patients who had been falsely diagnosed had also received therapy prior to their referral to the VAC (8.8%). Most patients received an ultrasound examination in the VAC (n = 464; 78.2%). Most frequently, compression therapy was prescribed (n = 256; 43.2%), followed by sclerotherapy (n = 175, 29.5%) and pharmacotherapy (n = 55; 9.3%)., Conclusion: Patients suffering from vascular anomalies often go through a complicated scheduling with numerous outpatient appointments and have a high risk of misdiagnosis and mistreatment prolonging the medical condition. Therefore, patients with vascular anomalies should be treated in a dedicated vascular anomaly center., Competing Interests: Declaration of conflicting interestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Background: Vascular malformations are rare diseases that are best managed in multidisciplinary vascular anomaly centers (VAC). So far, there are few published data on the logistic structure, patient allotment, and internal workflow in an interdisciplinary VAC., Purpose: To demonstrate the referral structure and patient allotment in a European VAC., Material and Methods: A retrospective cohort study was conducted. All patients treated in the VAC since its establishment in April 2014 until June 2020 were identified. A total of 461 patients were included in this study., Results: Most often, a venous malformation was diagnosed (49.9%), followed by arteriovenous malformations (8.7%) and syndromal vascular malformations (8.2%). Only 45.1% of referral diagnoses were correct. Of referrals, 35.1% were internal on-campus references, 28.0% were external references through physicians in private practice, and 19.7% came via external cooperating hospitals. Of the patients, 17.1% were self-admissions without a referral., Conclusion: The most important referring clinics are internal medicine, pediatric, and vascular surgery. A substantial proportion of referrals came from private practices. Recruiting these cooperation partners should be considered a high priority when establishing a new VAC. The rate of misdiagnosis is high, and more education of fellow clinicians is urgently needed. Therefore, patients with vascular malformations should be referred to and treated in dedicated centers for vascular anomalies.

Arteriovenous malformations (AVMs) are a rare congenital vascular disorder. They represent a fast-flow vascular malformation. Clinically, AVMs present a heterogenous expression and can affect every part of the body. Here, we will solely focus on extracranial AVMs. Generally, AVMs progress with the patient's age. Patients often suffer from pulsation, skin discoloration, pain, ulceration, bleeding, and disfigurement. Diagnostic tools include color - coded duplex sonography, MRI and CT imaging, as well as the clinical examination. 4D dynamic perfusion - computed tomography may help in the interventional planning. Digital subtraction angiography is required during interventional therapy. AVMs pose a great challenge to the treating physician. The therapy of this rare disease should be managed in an interdisciplinary center for vascular malformations. It consists of conservative measures, such as compression garments and pain medication, transcatheter or, more rarely, percutanous embolization, and surgical resection. In smaller, localized lesions, resection with primary wound closure may be feasible, whereas extensive AVMs regularly require the reconstruction of the resulting soft tissue defect and possibly affected functional structures by means of free tissue transfer. In the interdisciplinary setting required for an appropriate treatment of AVMs, extensive knowledge of the various therapies, including those from different specialties, is necessary. Therefore, this article aims to provide an overview over both the interventional and surgical therapeutic options.

Abstract: Alomari and colleagues described in 2014 for the first time a distinct combination of vascular malformation, fibrofatty muscular infiltration and contracture which was termed fibroadipose vascular anomaly (FAVA) (J Pediatr Orthoped 34, 109-117 (2014). So far only few publications (J Pediatr Orthoped (2014) 34, 109-117; J Hand Surg (2020). 45, 68.e1, 68.e13; Ann Vasc Dis (2014) 7, 316-319; Pediatr Radiol 46, 1179-1186 (2016)) concerning this newly described disease have been published, covering only a limited number of cases. We present a case of a 19-year-old male patient suffering from a FAVA of the proximal forearm with a severe contracture of the infiltrated flexor musculature. Upon surgery, we observed infiltration of the ulnar nerve. We were able to successfully resect the vascular malformation. Secondary tendon transfer was performed after extensive resection of the flexor musculature.FAVA presents a differential diagnosis in patients with solid growth of the upper or lower extremity and contracture of the involved extremity. We conclude that patients suffering from FAVA of the upper extremity should be referred to a center specialized in oncologic extremity surgery and reconstructive hand and microsurgery., Competing Interests: Conflict of interest and sources of funding: none declared., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Vascular anomalies represent a rare congenital disease with manifestation at diverse anatomical sights and presenting with heterogenous symptoms. Undetected, they can progress and create acute and chronic complications with functional impairment. The manifestation in the female and male pelvis and the urogenital tract represents a multidisciplinary challenge for physicians. Especially outpatient management in gynaecology and urology is affected. Diagnostic Radiology holds an important supportive role in early diagnosis of the underlying urogenital vascular anomaly and referral to interventional radiology, either for minimal invasive treatment, or to surgery for further assessment. This pictorial review creates awareness for the spectrum of vascular anomalies of the gynaecological and urogenital tract, their characteristic imaging findings and dedicated interventional treatment options. The individual description of vascular anomalies, based on an appropriate nomenclature and classification standard, is a guide for radiologists to distinguish the underlying vascular anomaly from other vascular disorders and to accelerate diagnosis as well as therapeutic proceedings. In consequence, interdisciplinary management of patients with vascular anomalies of the female and male pelvis will benefit.

Background:  Peripheral vascular anomalies represent a rare disease with an underlying congenital mesenchymal and angiogenetic disorder. Vascular anomalies are subdivided into vascular tumors and vascular malformations. Both entities include characteristic features and flow dynamics. Symptoms can occur in infancy and adulthood. Vascular anomalies may be accompanied by characteristic clinical findings which facilitate disease classification. The role of periinterventional imaging is to confirm the clinically suspected diagnosis, taking into account the extent and location of the vascular anomaly for the purpose of treatment planning., Method:  In accordance with the International Society for the Study of Vascular Anomalies (ISSVA), vascular anomalies are mainly categorized as slow-flow and fast-flow lesions. Based on the diagnosis and flow dynamics of the vascular anomaly, the recommended periinterventional imaging is described, ranging from ultrasonography and plain radiography to dedicated ultrafast CT and MRI protocols, percutaneous phlebography and transcatheter angiography. Each vascular anomaly requires dedicated imaging. Differentiation between slow-flow and fast-flow vascular anomalies facilitates selection of the appropriate imaging modality or a combination of diagnostic tools., Results:  Slow-flow congenital vascular anomalies mainly include venous and lymphatic or combined malformations. Ultrasound and MRI and especially MR-venography are essential for periinterventional imaging. Arteriovenous malformations are fast-flow vascular anomalies. They should be imaged with dedicated MR protocols, especially when extensive. CT with 4D perfusion imaging as well as time-resolved 3D MR-A allow multiplanar perfusion-based assessment of the multiple arterial inflow and venous drainage vessels of arterio-venous malformations. These imaging tools should be subject to intervention planning, as they can reduce procedure time significantly. Fast-flow vascular tumors like hemangiomas should be worked up with ultrasound, including color-coded duplex sonography, MRI and transcatheter angiography in case of a therapeutic approach. In combined malformation syndromes, radiological imaging has to be adapted according to the dominant underlying vessels and their flow dynamics., Conclusion:  Guide to evaluation of flow dynamics in peripheral vascular anomalies, involving vascular malformations and vascular tumors with the intention to facilitate selection of periinterventional imaging modalities and diagnostic and therapeutic approach to vascular anomalies., Key Points:   · Peripheral vascular anomalies include vascular malformations and vascular tumors. Both entities represent a rare disease with an underlying congenital mesenchymal or angiogenetic disorder. · The role of periinterventional imaging is confirmation of the diagnosis by assessing the flow dynamics of the vascular anomaly. · Slow-flow congenital vascular anomalies include venous, lymphatic and venolymphatic malformations. Arteriovenous malformations are fast-flow vascular anomalies, whereas hemangiomas are fast-flow vascular tumors that are frequently associated with fast-flow arteriovenous shunts. The periinterventional imaging modalities of choice include dedicated MR protocols and CT with 4D perfusion imaging as well as invasive transcatheter angiography.., Citation Format: · Sadick M, Overhoff D, Baessler B et al. Peripheral Vascular Anomalies - Essentials in Periinterventional Imaging. Fortschr Röntgenstr 2020; 192: 150 - 162., Competing Interests: The authors declare that they have no conflict of interest., (© Georg Thieme Verlag KG Stuttgart · New York.)

Background:  Vascular anomalies are a diagnostic and therapeutic challenge. They require dedicated interdisciplinary management. Optimal patient care relies on integral medical evaluation and a classification system established by experts in the field, to provide a better understanding of these complex vascular entities., Method:  A dedicated classification system according to the International Society for the Study of Vascular Anomalies (ISSVA) and the German Interdisciplinary Society of Vascular Anomalies (DiGGefA) is presented. The vast spectrum of diagnostic modalities, ranging from ultrasound with color Doppler, conventional X-ray, CT with 4 D imaging and MRI as well as catheter angiography for appropriate assessment is discussed., Results:  Congenital vascular anomalies are comprised of vascular tumors, based on endothelial cell proliferation and vascular malformations with underlying mesenchymal and angiogenetic disorder. Vascular tumors tend to regress with patient's age, vascular malformations increase in size and are subdivided into capillary, venous, lymphatic, arterio-venous and combined malformations, depending on their dominant vasculature. According to their appearance, venous malformations are the most common representative of vascular anomalies (70 %), followed by lymphatic malformations (12 %), arterio-venous malformations (8 %), combined malformation syndromes (6 %) and capillary malformations (4 %)., Conclusion:  The aim is to provide an overview of the current classification system and diagnostic characterization of vascular anomalies in order to facilitate interdisciplinary management of vascular anomalies., Key Points:   · Vascular anomalies are comprised of vascular tumors and vascular malformations, both considered to be rare diseases.. · Appropriate treatment depends on correct classification and diagnosis of vascular anomalies, which is based on established national and international classification systems, recommendations and guidelines.. · In the classification, diagnosis and treatment of congenital vascular anomalies, radiology plays an integral part in patient management.., Citation Format: · Sadick M, Müller-Wille R, Wildgruber M et al. Vascular Anomalies (Part I): Classification and Diagnostics of Vascular Anomalies. Fortschr Röntgenstr 2018; 190: 825 - 835., Competing Interests: Disclosure The authors report no conflicts of interest in this work., (© Georg Thieme Verlag KG Stuttgart · New York.)

Background  Radiology is an interdisciplinary field dedicated to the diagnosis and treatment of numerous diseases and is involved in the development of multimodal treatment concepts. Method  Interdisciplinary case management, a broad spectrum of diagnostic imaging facilities and dedicated endovascular radiological treatment options are valuable tools that allow radiology to set up an interdisciplinary center for vascular anomalies. Results  Image-based diagnosis combined with endovascular treatment options is an essential tool for the treatment of patients with highly complex vascular diseases. These vascular anomalies can affect numerous parts of the body so that a multidisciplinary treatment approach is required for optimal patient care. Conclusion  This paper discusses the possibilities and challenges regarding effective and efficient patient management in connection with the formation of an interdisciplinary center for vascular anomalies with strengthening of the clinical role of radiologists. Key points   · Vascular anomalies, which include vascular tumors and malformations, are complex to diagnose and treat.. · There are far more patients with vascular anomalies requiring therapy than interdisciplinary centers for vascular anomalies - there is currently a shortage of dedicated interdisciplinary centers for vascular anomalies in Germany that can provide dedicated care for affected patients.. · Radiology includes a broad spectrum of diagnostic and minimally invasive therapeutic tools which allow the formation of an interdisciplinary center for vascular anomalies for effective, efficient and comprehensive patient management.. Citation Format · Sadick M, Dally FJ, Schönberg SO et al. Strategies in Interventional Radiology: Formation of an Interdisciplinary Center of Vascular Anomalies - Chances and Challenges for Effective and Efficient Patient Management. Fortschr Röntgenstr 2017; 189: 957 - 966., Competing Interests: Disclosure The authors report no conflicts of interest in this work., (© Georg Thieme Verlag KG Stuttgart · New York.)