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 Table of Contents  
CASE REPORT
Year : 2020  |  Volume : 32  |  Issue : 1  |  Page : 77-80

Multifocal arteriovenous malformation of face: A rare entity


1 Department of Oral Medicine and Radiology, SVS Institute of Dental Sciences, Mahabubnagar, Telangana, India
2 Oral and Maxillofacial Surgery, SVS Institute of Dental Sciences, Mahabubnagar, Telangana, India
3 Department of Oral Pathology, SVS Institute of Dental Sciences, Mahabubnagar, Telangana, India

Date of Submission27-Nov-2019
Date of Decision06-Mar-2020
Date of Acceptance17-Mar-2020
Date of Web Publication17-Apr-2020

Correspondence Address:
Dr. Rashmitha Arutla
SVS Institute of Dental Sciences, Appannapally, Mahabubnagar, - 509 002, Telangana
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jiaomr.jiaomr_194_19

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   Abstract 


Vascular anomalies are a heterogeneous group of congenital disorders with abnormal vascular development. Vascular anomalies are classified into vascular tumors and malformations. Vascular malformations are resulted from abnormal vessel development and morphogenesis. Arteriovenous malformation (AVM) of the head and neck accounts to 0.1%, which is persistent, progressive in nature and might represent a lethal benign disease. Here, we present a case report of a 32-year-old female with AVM involving the left side of the face.

Keywords: Arteriovenous, malformations, multiple, vascular


How to cite this article:
Karankot G, Arutla R, Rajan R, Reddy S. Multifocal arteriovenous malformation of face: A rare entity. J Indian Acad Oral Med Radiol 2020;32:77-80

How to cite this URL:
Karankot G, Arutla R, Rajan R, Reddy S. Multifocal arteriovenous malformation of face: A rare entity. J Indian Acad Oral Med Radiol [serial online] 2020 [cited 2020 May 30];32:77-80. Available from: http://www.jiaomr.in/text.asp?2020/32/1/77/282613




   Introduction Top


Wardrop first recognized vascular malformation in 1818. The International Society for the Study of Vascular Anomalies recognized the classification for vascular anomalies, proposed by Mulliken and Glowacki, which were divided into tumors and malformations.[1] Arteriovenous malformation (AVM) originates due to errors in the vascular development while embryogenesis; shunting directly from the arteries to veins (without capillary bed) through a fistula or nidus (abnormal channels between feeding arteries and draining veins). AVM is considered a quiescent lesion, angiogenesis and/or vasculogenesis may be involved in AVM expansion.[2] AVM is not static they become progressive with time and recurs.[3]


   Case Report Top


A 32-year-old female patient had a history of multiple abnormal nonspecific swellings on the left cheek, upper lip, lower part of the left masseter region [Figure 1] and intraorally on the left floor of the mouth, retromolar region, buccal mucosa [Figure 2] from the past 2 years. Swellings of all regions were initially small and gradually attained the present size. Swelling on the left malar region, over the upper lip and all intraoral swellings had bluish color. All swellings were soft in consistency, non-tender, skin was pinchable, compressible and nonpulsatile. Based on this, it was provisionally diagnosed as vascular malformation on the left side of the face.
Figure 1: Swellings at the upper lip, left malar, and masseter region

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Figure 2: Intraoral sites

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Diagnostic imaging is required in order to define the origin and extent of the anomalies. A plain radiograph was taken which revealed no bony involvement. For further precise evaluation, the patient was subjected to contrast-enhanced MRI, T2-weighted images (coronal) reveals multiple hyperintense lesions in the premaxillary, retromolar, buccal, masseter, and floor of the mouth on the left side showing enhancement [Figure 3]. CT angiography reveals arterial phase showing mild enhancement in the left premaxillary region, no contrast seen in masticatory space [Figure 4]a. The venous phase shows an increase in the enhancement of premaxillary lesion and irregular enhancing area in masticatory space [Figure 4]b. The lesions depicted communication to supraorbital, labial, and facial veins on the left side [Figure 4]c and [Figure 4]d.
Figure 3: Contrast MRI (T2-weighted)

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Figure 4: CT-angiography (a) Premaxilla showing enhancement (arterial phase). (b) Enhancement in premaxillary, masticatory space (venous phase), (c and d) communication to left supraorbital, labial, and facial veins

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Surgical excision of the lesion was performed over the malar, masseter region, and on the floor of the mouth on the left side under general anesthesia [Figure 5] and specimen was sent for histopathological evaluation. Histopathology reveals highly vascularized stromal tissue containing both thick (lined by endothelial cells and elastic fibers) and thin vessels. These vascular spaces are filled with RBCs and few areas showing thrombosis suggestive of AVM [Figure 6].Based on history, clinical findings, radiographic, and histopathological features, the lesion was diagnosed as AVM on the left side of the face. Postoperatively, satisfactory healing noticed and no recurrence reported [Figure 7].
Figure 5: Intraoperative

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Figure 6: Histopathology (6a - 4x magnification, 6b - 20x magnification)

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Figure 7: Follow-up

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   Discussion Top


Vascular anomalies are congenital abnormalities resulting from abnormal vessel development and morphogenesis. The International Society for the Study of Vascular Anomalies (ISSVA) suggested a classification system, proposed by Mulliken and Glowacki [Table 1].[2] The extracranial AVM is usually locally aggressive mainly during puberty or adolescence with an expansive mass causing a cosmetic and functional disturbance, the present case also reported with facial asymmetry being a major concern.[4] The origin and pathogenesis of AVM are known about the defects in TGF-beta signaling and a genetic two-hit hypothesis are the prevailing theories to the pathogenesis and other trigger factors that can induce proliferation of the AVM which includes trauma, ischaemic event secondary to thrombosis, ectasia, hormonal changes. Sometimes puberty can also induce proliferation of the AVM and trigger the growth of the lesion.[5]
Table 1: The International Society for the Study of Vascular Anomalies (ISSAVA) classification for vascular anomalies. The updated classification was approved at the May, 2018 General Assembly in Amsterdam, the Netherlands. a)Highflow lesions; b)Defined as two or more vascular malformations found in one lesion.*

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AVM is present at birth but may not become evident until childhood, lesions may present in the 2nd, 3rd or even 5th decade with slight female predominance (1:1.5) in concordance to present case. The most common site of the extracranial AVM is the head and neck, followed by the limbs, trunk, and viscera. In the oral cavity, these can present at any site but most ordinarily occur on anterior two-thirds of the tongue, palate, gingival, and buccal mucosa.[2] AVMs clinical course can be classified according to the Schobinger clinical staging system [Table 2]. Initially, lesions are latent then evolve a warm pink-bluish skin lesion with a pulsatile thrill in adolescence. Over time, the lesion becomes expanded and leading to dystrophic skin changes, bleeding, ulceration, and tissue necrosis. If left untreated, high flow into the venous system eventually leads to volume and pressure overload within the heart and subsequent heart failure.[6]
Table 2: Schobinger clinical staging system of AVMs*

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Like other vascular malformations, imaging is a key role in characterizing AVMs and subsequent treatment planning.[6] Investigations such as Doppler ultrasonography, angiography (CT, MRI) can be done to diagnose an arteriovenous shunt.[7] Ultrasound and Doppler imaging show the presence of high systolic and diastolic flow, prominent arteriovenous shunting and arterial waveforms within venous structures suggesting pulsatile flow.[6]

On MRI, AVMs appear as a tangled mesh of dilated arteries and veins connected by linear or focal shunts. Magnetic resonance angiography (MRA) has a high temporal resolution that demonstrates arterial feeders, shunting volume, and the location and size of the nidus.[6] Unlike CT, imaging can also provide useful information in AVM assessment. Contrast-enhanced CT quantifies accurate structural assessment of arteries, veins, and the nidus, in addition to providing flow analysis important for preprocedural planning.[6]

Treatment methods for AVM can be largely divided into sclerotherapy, surgical resection, and surgical resection after embolization. Treatment timing is often individualized to the patient and the extent of the disease.[5] The management of AVMs presents a therapeutic challenge because of their hemodynamic characteristics and their modality of growth.[8] Surgical treatment consists of wide resection which is difficult and potentially hazardous due to significant blood loss during surgery.[9] Recently, angiography is useful in poorly defined cases and for the embolization of feeding vessels before surgery. It defines the flow characteristics, feeding vessels, and dangerous anastomoses.[8] The purpose of embolization is to occlude the vessels contributing to the lesion. The combination of embolization and surgery is used to control acute hemorrhage allowing for excisional surgery to be performed afterwards.[9]


   Conclusion Top


AVMs are rare, congenital, and quiescent which become progressive with time and recurs. Proper approach of AVMs can be done by complete history, precise clinical examination, using advanced imaging modalities, and management can help in preventing serious life-threatening complications.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Nair SC, Spencer NJ, Nayak KP, Balasubramaniam K. Surgical management of vascular lesions of the head and neck: A review of 115 cases. Int J Oral Maxillofac Surg 2011;40:577-83.  Back to cited text no. 1
    
2.
Lee JW, Chung HY. Vascular anomalies of the head and neck: Current overview. Arch Craniofac Surg 2018;1:243-47.  Back to cited text no. 2
    
3.
Eerola I, Boon LM, Mulliken JB, Burrows PE, Dompmartin A, Watanabe S, et al. Capillary malformation–arteriovenous malformation, a new clinical and genetic disorder caused by RASA1 mutations. Am J Hum Genet 2003;73:1240-9.  Back to cited text no. 3
    
4.
Lakkasetty YT, Malik S, Shetty A, Nakhaei K. Multiple vascular malformations in head and neck-Rare case report. J Oral Maxillofac Pathol 2014;18:137-42.  Back to cited text no. 4
[PUBMED]  [Full text]  
5.
Richter GT, Friedman AB. Hemangiomas and vascular malformations: Current theory and management. Int J Contemp Pediatr 2012;2012:1-10.  Back to cited text no. 5
    
6.
Mulligan PR, Prajapati HJ, Martin LG, Patel TH. Vascular anomalies: Classification, imaging characteristics and implications for interventional radiology treatment approaches. Br J Radiol 2014;87:1-18.  Back to cited text no. 6
    
7.
Kim JB, Lee JW, Choi KY, Yang JD, Cho BC, Lee SJ, et al. Clinical characteristics of arteriovenous malformations of the head and neck. Dermatol Surg 2017;43:526-33.  Back to cited text no. 7
    
8.
Manjunath SM, Shetty S, Moon NJ, Sharma B, Metta KK, Gupta N, et al. Arteriovenous malformation of the oral cavity. Case Rep Dent 2014;2014:1-5.  Back to cited text no. 8
    
9.
Kumar A, Mittal M, Srivastava D, Jaetli V, Chaudhary S. Arteriovenous malformation of face. Contemp Clin Dent 2017;8:482-4.  Back to cited text no. 9
[PUBMED]  [Full text]  


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]
 
 
    Tables

  [Table 1], [Table 2]



 

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