scholarly journals Vascular Anomalies (Part II): Interventional Therapy of Peripheral Vascular Malformations

Author(s):  
René Müller-Wille ◽  
Moritz Wildgruber ◽  
Maliha Sadick ◽  
Walter Wohlgemuth

Background The International Society for the Study of Vascular Anomalies (ISSVA) categorizes vascular anomalies into vascular tumors and vascular malformations. Vascular malformations are further divided into slow-flow (venous, lymphatic, and capillary malformation) and fast-flow malformations (arteriovenous malformation and arteriovenous fistula). This interdisciplinary classification has therapeutic implications. Methods The objective of this article is to provide concise information about the current terminology and treatment strategies of peripheral vascular malformations, based on the currently available literature, with a focus on interventional therapy of venous malformations (VM), lymphatic malformations (LM), arteriovenous malformations (AVM) and arteriovenous fistulae (AVF). Results and Conclusion Accurate classification is crucial for appropriate therapy of peripheral vascular malformations. Modern imaging technologies and refined interventional treatment strategies are now central parts in the multidisciplinary management of these patients. Slow-flow and fast-flow vascular malformations can be treated successfully by percutaneous sclerotherapy and endovascular embolotherapy as first-line interventions. Key points:  Citation Format

Author(s):  
Maliha Sadick ◽  
Daniel Overhoff ◽  
Bettina Baessler ◽  
Naema von Spangenberg ◽  
Lena Krebs ◽  
...  

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:  Citation Format


2019 ◽  
Vol 3 ◽  
pp. 3
Author(s):  
Richard Brill ◽  
Moritz Guntau ◽  
Franz Stangl ◽  
Veronika Teusch ◽  
Dominik Schramm ◽  
...  

Purpose: Regarding genital lesions, the incidence of male external genitalia vascular anomalies is circa 3%, thereof one-tenth tumors and nine-tenth malformations according to the International Society for the Study of Vascular Anomalies classification. Image-guided percutaneous sclerotherapy in male external genitalia vascular malformations has rarely been described. Therefore, a retrospective analysis of sclerotherapy in a series of eight patients was conducted. Materials and Methods: The study was IRB approved. Two radiologists reviewed angiographic reports and analyzed interventionally treated male patients with external genitalia vascular malformations between February 2, 2014, and November 11, 2017, at an interdisciplinary tertiary care Vascular Anomalies Center. Inclusion criteria were a slow-flow malformation of the male external genitalia and no interventional treatment before. Operations longer than 1-year past were no exclusion criteria. Patients suffered from lymphatic and/or venous malformations and received percutaneous sclerotherapy. Malformations were treated with polidocanol, ethanol in gel form or OK-432. Patients answered a questionnaire regarding symptoms with repeat after follow-up. The initial state and post-treatment results were compared. Magnetic resonance imaging pre- and post-intervention was assessed. Complications were reported standardized. Results: Eight patients with a mean age of 21.6 years suffered from genital swelling, bleeding, thrombophlebitis, lymphorrhea, skin changes, pain, and functional genitourinary symptoms and were treated with sclerotherapy. All patients reported clinical improvement of symptoms during the average follow-up period of 30 months. No complications ensued. Conclusion: Sclerotherapy seems to be a safe and effective treatment of slow-flow malformations of the male external genitalia. Due to the low incidence of the disease, multicenter studies are necessary to assess a larger number of cases.


Author(s):  
R. BECKERS ◽  
D. GOVAERTS ◽  
M. DESMEDT ◽  
G. MALEUX ◽  
F. COMBES ◽  
...  

Extraoral vascular anomalies in the head and neck area Vascular anomalies have a prevalence of 4.5%, of which most occur in the head and neck area. In this retrospective cohort study, extraoral vascular anomalies are categorized based on the classification of the International Society for the Study of Vascular Anomalies (ISSVA). Subsequently, an analysis of the clinical presentation, treatment and complications of the 75 included patients was performed. The proportion of vascular tumors and malformations in this study was 24% (n = 18) and 76% (n = 57) respectively. Most of the extraoral vascular tumors and malformations occurred respectively in the lip (66.7%; n = 12) and cheek area (40.4%; n = 23). The most frequent complaint when patients presented with a vascular tumor, was the wish to be diagnosed (77.8%; n = 14). Swelling was the most frequent complaint when patients (35.1%; n = 20) presented with a vascular malformation. In 48.1% (n = 26) of the patients receiving interventional treatment, at least one recurrence was seen (n = 54), and almost exclusively in vascular malformations (96.2%; n = 25). Interventional therapy of vascular malformations is complex and associated with a pronounced risk of complication and recurrence, which is why multidisciplinary care for these patients is often an added value. The treatment of vascular tumors results in minimal morbidity or recurrence.


2015 ◽  
Vol 6 (3) ◽  
pp. 109-111 ◽  
Author(s):  
Jatin P Shah ◽  
Ivana Petrovic ◽  
Ben Roman

ABSTRACT Background Vascular anomalies are divided into vascular tumors, hemangiomas being the most common, and vascular malformations. Most vascular anomalies are noticed at birth or occur during infancy, and generally involve skin or subcutaneous soft tissues. Adult onset hemangiomas are rare, and intramuscular location is extremely rare. Surgical excision is recommended for hemangiomas in adults, if they are symptomatic, or manifest growth. Materials and methods We report a rare case of a 51-year-old woman, with an intramuscular hemangioma of the tongue, presenting as a submental mass. Preoperative imaging for assessment of tumor extent was followed by a successful surgical excision. Results Postoperative course was uneventful with primary healing of the wound, and with no functional deficit of tongue function. Conclusion Although a variety of treatment approaches are reported for childhood hemangiomas, surgical excision is the preferred treatment for adult onset symptomatic hemangiomas. Preoperative work up should include imaging preferably with contrast enhanced magnetic resonance imaging (MRI). Embolization may be considered for larger lesions. Intraoperative hypotension should be avoided to ensure identification of the entire lesion to ensure complete excision. How to cite this article Petrovic I, Roman B, Shah JP. Cavernous Hemangioma of the Tongue. Int J Head Neck Surg 2015;6(3):109-111.


Author(s):  
Maliha Sadick ◽  
René Müller-Wille ◽  
Moritz Wildgruber ◽  
Walter Wohlgemuth

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  Citation Format


2019 ◽  
pp. 241-260
Author(s):  
Lara Wine Lee ◽  
Cameron C. Trenor

The chapter provides a description of the main anomalies characterized by excessive proliferation of the vascular tissue and the major syndromes associated with them. The predominant types of vascular anomalies are vascular tumors and vascular malformations. Vascular tumors are further subdivided in benign, locally aggressive, and malignant, according to the clinical course of the lesions, while vascular abnormalities are classified as simple (capillary, lymphatic, venous, arteriovenous, and arteriovenous fistula) or combined. For each subgroup of vascular anomalies, clinical presentation and diagnostic procedures are discussed, as well as pathogenic mechanisms and potential comorbidities. The management of vascular anomalies may be oriented to treat the aesthetic discomfort as well as the functional impairment and may involve both pharmaceutical and surgical approaches.


Author(s):  
E. K. Donyush ◽  
Z. A. Kondrashova ◽  
Yu. A. Polyaev ◽  
R. V. Garbuzov

Vascular anomalies (VA) comprise a heterogeneous group of diseases associated with congenital angiogenesis disorder. There are no currently developed unified protocols and treatment regimens for systemic forms of VA. Numerous advantages show sirolimus, an mTOR inhibitor, as a well tolerated and effective antiproliferative and antiangiogenic therapy in patients with VA. The article presents the results of treatment of 211 patients with VA (6 patients with vascular tumors and 205 patients with vascular malformations) aged 2 months to 17 years (median – 9 years), who received sirolimus therapy for 1–86 months (median – 24 months). Sirolimus was administered at a starting dose of 0.8 mg/m2/day orally in two doses with an interval of 12 hours. The concentration of the blood preparation was maintained in the therapeutic range of 6–15 ng/ml. Since 2015, concomitant therapy with co-trimoxazole for the prevention of Pneumocystis pneumonia has been prescribed only to tracheostomy carriers. When infectious episodes occurred in patients, sirolimus therapy continued without changes in the dose of the drug and did not affect the disease, provided that the therapeutic concentration was maintained. A positive response to therapy was observed in 89.1 % of patients with VA in the form of the size of the vascular mass according to the data of visual examination and instrumental control. All patients showeda clinical response to therapy in the form of relief of painsyndrome, reduction/relief of lymphorrhea, reduction/improvement of hemostasis parameters, and an increase in functional activity and quality. For the entire observation period 2012–2020, when taking sirolimus, not a single severe adverse event occurring in post-transplant patients has been reported that would require discontinuation of the drug. The article presents two clinical cases of sirolimus use in the treatment of patients with kaposiform hemangioendothelioma and extensive venous malformation. Parents are encouraged to use the information in scientific research and publications.


Author(s):  
G. Scott Stacy

The classification of vascular tumors of the skeleton can be thought of as a spectrum of disease including both benign and malignant entities. Chapter 60 focuses on the benign entities, hemangioma and epithelioid hemangioma. The term hemangioma refers to a collection of blood vessels and vascular spaces, perhaps better classified as a slow-flow vascular malformation. Epithelioid hemangioma is a rare and controversial entity that may be confused with its malignant counterpart, the epithelioid hemangioendothelioma. This chapter will describe the clinical, pathophysiological, and imaging features of these benign vascular bone tumors, with a brief mention of treatment strategies. Familiarity with the imaging appearances and characteristics of these entities is essential in order to guide clinical management and to avoid unnecessary investigation into clearly benign lesions.


Author(s):  
Ajay K. Khanna ◽  
Akhilesh Kumar ◽  
Soumya Khanna ◽  
Amrita Kar ◽  
Puneet Kumar ◽  
...  

Abstract Vascular anomalies grouped into vascular tumors (hemangioma) (HI) and vascular malformation (VM) are benign vascular lesions that are difficult to distinguish from one another clinically and often confused with each other at histopathology. This confusing terminology leads to improper diagnosis, illogical treatment, and misdirected research. This study aimed to study GLUT 1, S-100, and nerve bundle to differentiate hemangioma and vascular malformation. Thirty two cases of vascular lesions (26 vascular malformations and 6 hemangiomas) were taken into the study. For histological evaluation and immunohistochemistry (IHC), samples of vascular lesions were collected in formalin. All the hematoxylin and eosin-stained slides were evaluated under light microscope for histology and nerve bundles. Immunohistochemical staining was performed by streptavidin–biotin method for GLUT 1 and S-100. GLUT 1 was positive in all 6 cases of hemangiomas (100%) and only in 1 case of vascular malformation. Nerve bundle was present in 24 cases of vascular malformation (92.3%) out of 26 cases but not in any cases of hemangioma and S-100 was found positive in all vascular malformation cases (100%) but not in hemangioma. So GLUT 1 expression, S-100, and presence of nerve bundle in vascular lesions can help to differentiate hemangioma and vascular malformation.


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