scholarly journals Classification, diagnosis, and treatment of vascular malformations

2021 ◽  
Vol 64 (9) ◽  
pp. 614-621
Author(s):  
Joon Seok Lee ◽  
Ho Yun Chung

Background: In the 1980s, vascular anomalies were divided into two major subgroups—vascular tumors and vascular malformations—based on the functional framework. This concept has been fundamental to the management of vascular anomalies and expanded and refined by the International Society for the Study of Vascular Anomalies. Vascular malformations are further sub-divided into several types; however, there are many cases with insufficient information about the disease.Current Concepts: Several instances have shown that the diagnosis of vascular malformations can be achieved after a careful evaluation of patient history and examination. However, recently developed imaging technology has been of great help in the diagnosis. Doppler ultrasonography, magnetic resonance imaging, computed tomography scan, and angiography are valuable tools for diagnostic and therapeutic planning. Advanced diagnostic and therapeutic technologies have also provided more accurate and safer diagnoses and appropriate treatment. Recently, an improved method of managing vascular malformations has emerged, based on a multidisciplinary approach, which is very useful for increasing the treatment efficacy. The development of gene research has enabled the investigation of disease-related genes and the development of new medications.Discussion and Conclusion: Accurate diagnosis of vascular malformations is one of the most crucial factors in determining treatment plan and prognosis, based on which it would be possible to achieve effective results through appropriate management and a multidisciplinary team approach.

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.


2017 ◽  
Vol 50 (02) ◽  
pp. 193-200 ◽  
Author(s):  
Lalit K. Makhija ◽  
Sameek Bhattacharya

ABSTRACT Introduction: Vascular anomalies are congenital lesions broadly categorised into vascular tumour (haemangiomas) and vascular dysmorphogenesis (vascular malformation). The management of these difficult problems has lately been simplified by the biological classification and multidisciplinary approach. To standardise the treatment protocol, an algorithm has been devised. The study aims to validate the algorithm in terms of its utility and presents our experience in managing vascular anomalies. Materials and Methods: The biological classification of Mulliken and Glowacki was followed. A detailed algorithm for management of vascular anomalies has been devised in the department. The protocol is being practiced by us since the past two decades. The data regarding the types of lesions and treatment modality used were maintained. Results and Conclusion: This study was conducted from 2002 to 2012. A total of 784 cases of vascular anomalies were included in the study of which 196 were haemangiomas and 588 were vascular malformations. The algorithmic approach has brought an element of much-needed objectivity in the management of vascular anomalies. This has helped us to define the management of particular lesion considering its pathology, extent and aesthetic and functional consequences of ablation to a certain extent.


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


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):  
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.


Author(s):  
Anushka Mittal ◽  
Rama Anand ◽  
Richa Gauba ◽  
Subhasis Roy Choudhury ◽  
Pooja Abbey

AbstractVascular anomalies are a common cause of soft-tissue masses in children and often referred for ultrasonographic (USG) evaluation. They are broadly classified as vascular tumors (hemangiomas, hemangioendotheliomas, and angiosarcomas) or vascular malformations (venous malformations, lymphatic malformations, and arteriovenous malformations). Findings on USG and Doppler imaging can be used to categorize vascular anomalies into high- or low-flow lesions, which forms the basis for further workup, diagnosis, and management. On careful evaluation of various sonographic features, in conjunction with clinical findings, an accurate clinicoradiological diagnosis can be made in most cases. Further imaging with magnetic resonance (MR) imaging or computed tomography (CT) helps in delineation of lesion extent, whereas MR or CT angiography is useful to map the vascular supply of high-flow lesions. We have illustrated and discussed a step-by-step approach to diagnose vascular anomalies using ultrasound and Doppler imaging.


2007 ◽  
Vol 54 (3) ◽  
pp. 81-86
Author(s):  
H. Hyodoh ◽  
K. Hyodoh

Peripheral vascular malformations are now described according to some accepted guidelines, and the principle of proper treatment (nodus ablation) is becoming clear. An appropriate classification schema for vascular anomalies and definite indications for treatment are important to successful treatment overall. Non-invasive imaging (US, CT, and MRI) in association with clinical findings is critical in establishing the diagnosis, evaluating the extent of the malformation, and planning appropriate treatment. Direct nidus phlebography is useful not only in making a correct diagnosis but also in treating the lesion by sclerotherapy. When a patient suffers clinical complications, the nidus sclerotherapy becomes mandatory. If the vascular malformation remains bloodstream to a drainage vein during nidus opacification, flow control is necessary to achieve complete nidus ablation. A multidisciplinary approach is needed in the treatment of a high-flow lesion. A dedicated team approach is necessary for appropriate management in most cases. .


Author(s):  
Yangseop Noh ◽  
Gwanghui Ryu ◽  
Hyo Yeol Kim

Arteriovenous malformation is a vascular malformation with fast-flow shunt from the artery to the vein. Extracranial arteriovenous malformation in the head and neck area is rare disease compared to other vascular malformations such as venous malformation or lymphatic malformation. Extracranial arteriovenous malformations especially on face can cause aesthetic problems, therefore surgeons should consider an importance of cosmetic outcome and choose appropriate treatment plan. We report a case of 51-year-old male who presented with enlarged nasal tip diagnosed arteriovenous malformation. This case showed successful outcome after combination therapy consisted of superselective embolization and surgical resection.


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