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 Table of Contents  
CASE REPORT
Year : 2019  |  Volume : 9  |  Issue : 2  |  Page : 93-97

Intraoral venous malformation: A case report with review of literature and illustration of the clinical entity


Department of Oral Medicine and Radiology, KLE VK Institute of Dental Sciences, JNMC Campus, Belgaum, Karnataka, India

Date of Submission18-Apr-2018
Date of Acceptance06-Sep-2019
Date of Web Publication13-Nov-2019

Correspondence Address:
Dr. Lidiya Thomas
KLE VK Institute of Dental Sciences, JNMC Campus, Nehru Nagar, Belgaum - 590 010, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijohs.ijohs_19_18

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  Abstract 


Vascular malformations constitute embryonic abnormality of the vascular system due to abnormal development in the blood vessels, lymph vessels, veins, and/or arteries. They comprise approximately 7% of all benign tumors and can lead to serious local and systemic complications. Venous malformations (VMs) constitute a spectrum of vascular malformations with a prevalence rate of 1% and incidence of approximately 2 in 10,000. Even though rare in the oral cavity, when present, they are persistent and progressive in nature and are associated with extensive blood loss. VMs are a diagnostic challenge to clinicians because of its lethal benign nature and are often confused with other vascular malformations. Radiologists play a key role in diagnosing and advising an appropriate treatment for these lesions. Management of VMs includes use of sclerosing agents, laser therapy, and surgical excision. This case report presents a rare case of VM occurring in the right mandibular buccal vestibule in a 17-year-old male patient which was treated using a sclerosing agent, polidocanol 1%.

Keywords: Laser, sclerosing agents, venous malformations


How to cite this article:
Thomas L, Naik Z, Jirge V, Bagewadi A. Intraoral venous malformation: A case report with review of literature and illustration of the clinical entity. Int J Oral Health Sci 2019;9:93-7

How to cite this URL:
Thomas L, Naik Z, Jirge V, Bagewadi A. Intraoral venous malformation: A case report with review of literature and illustration of the clinical entity. Int J Oral Health Sci [serial online] 2019 [cited 2019 Dec 15];9:93-7. Available from: http://www.ijohsjournal.org/text.asp?2019/9/2/93/270875




  Introduction Top


Vascular malformations constitute embryonic abnormality of the vascular system due to abnormal development in the blood vessels, lymph vessels, veins, and/or arteries. They comprise approximately 7% of all benign tumors and can lead to serious local and systemic complications.[1] Venous malformations (VMs) are simple slow-flow vascular anomalies with an abnormal venous network. VMs have a prevalence rate of 1% and the incidence of approximately 2 in 10,000.[2],[3] VMs are not usually present at birth. They manifest either in childhood or adolescence and later persist throughout the life. They grow proportionately as the age of the child advances, with a sudden expansion in adulthood due to Valsalva maneuver, puberty, pregnancy, or traumatic injury.[4],[5] Even though rare in the oral cavity, when present, they are persistent and progressive in nature and are associated with extensive blood loss. VMs are a diagnostic challenge to clinicians because of its lethal benign nature and are often confused with other vascular malformations. As vascular malformations are responsive to various stimuli such as injury and surgical intervention, improper treatment often rapidly stimulates quiescent vascular malformations, making the condition worse. Radiologists play a key role in diagnosing and advising an appropriate treatment for these lesions. Doppler ultrasound (US) and magnetic resonance imaging are key imaging methods used to characterize and diagnose VMs. Treatment options include surgery, sclerotherapy, and laser therapy. Here, we report a rare case of VM occurring in the right mandibular buccal vestibule in a 17-year-old male patient which was treated using a sclerosing agent, polidocanol 1%.


  Case Report Top


A 17-year-old male patient reported to the department of oral medicine and radiology with a chief complaint of painless swelling on the right side of the face for 2–3 months. His past dental, medical, family history, and general examinations were noncontributory. Extraoral examination revealed a dome-shaped swelling of size 2 cm × 1.5 cm on the right lower-third of the face [Figure 1] and was clearly elicited on axial view [Figure 2].
Figure 1: Profile view of the patient

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Figure 2: Extraoral axial view of the patient

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Intraoral examination revealed vestibular obliteration of the right lower buccal vestibule extending from the medial aspect of 44 to distal of 46. The overlying mucosa had a bluish tint. The swelling was soft in consistency, tender, fluctuant, and compressible with no apparent thrills, bruit, or pus discharge or bleeding [Figure 3]. There was no caries, mobility, and periodontal pocket associated with premolars and molar. Based on the clinical findings, a provisional diagnosis of mucocele and differential diagnosis of vascular malformation, lipoma, and traumatic neuroma were considered.
Figure 3: Intraoral swelling with a bluish tint

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Diagnostic digital intraoral periapical radiograph [Figure 4] and OPG [Figure 5] revealed no bony involvement in the region of 44–46 and thus ultrasonography (USG) was advised for the patient. USG of the region was performed using Philips L 12-5 MHz 50 mm intraoral linear transducer. USG showed a soft-tissue lesion of size 2.5 cm × 1.1 cm along the right side close to the anterior mandibular margin. It had multiple cystic spaces with predominantly venous flow pattern without any arterial communication [Figure 6].
Figure 4: Diagnostic digital intraoral periapical radiograph showing no periapical pathology or bony changes

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Figure 5: OPG with no bone involvement

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Figure 6: Multiple cystic spaces in ultrasonography image of the swelling

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On US imaging, mucocele appears as a hypoechoic lesion without internal vascularity, whereas lipomas appear typically ellipsoid, hyperechoic without internal vascularity. The typical US appearance of traumatic neuroma is of a well-defined minimally vascular lesion, hypoechoic to surrounding subcutaneous tissue. Vascular malformations are subcategorized according to their flow dynamics as low-flow malformations (venous, VMs) and high-flow malformations (arteriovenous malformations). Any malformation with an arterial component in USG is considered high flow, while those without an arterial component are considered low flow.[6] Thus, a probable diagnosis of VM was considered.

The present case was treated by injecting a sclerosing agent, polidocanol 1%. The injections were repeated every week, up to five sessions. After 2 months postsclerotherapy, the swelling was completely disappeared [Figure 7].
Figure 7: Pre- and post-treatment images of the swelling

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  Discussion Top


VMs are part of a spectrum of vascular malformations commonly found in adults. VM is defined as a simple malformation with slow flow and an abnormal venous network. A classification scheme for vascular anomalies based on cellular features, flow characteristics, and clinical behavior was updated during the 2014 meeting of the International Society for the Study of Vascular Anomalies [Figure 8].[7]
Figure 8: Classification of vascular malformation

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The present case was managed by injecting a sclerosing agent, polidocanol 1%. Polidocanol is a popular surfactant agent that is relatively effective, painless, and incites minimal endothelial damage. The exact mechanism by which polidocanol acts as an in vivo sclerosant is by denudation of the endothelial cell from the internal elastic lamina of the vascular wall. Platelet aggregation on the denuded surface then embolizes the vessel from the periphery to the center.[8],[9],[10] Polidocanol injection, 0.2 ml, was given along the circumference of the swelling with tuberculin syringe. Postsclerotherapy, antibiotics and analgesics were given along with ice-pack application. The injections were repeated every week, up to five sessions. After 2 months postsclerotherapy, the swelling was completely disappeared. Reversible cardiac arrest has been reported in cases where polidocanol was used to treat VM of legs. Hence, care should be taken when using in cardiac patients.[11]


  Review of Literature Top


Clinical features

VMs are clinically characterized as a soft, compressible, nonpulsatile tissue mass. The overlying skin usually has a bluish tint. The common sites affected are the head and neck (40% of cases), extremities (40%), and trunk (20%). Intraoral VMs can bleed, distort dentition, cause speech problems, and obstruct the upper airway and pharynx. Thrombosis (40%) and pain are also commonly seen in VMs.[12]

Coagulopathic features

A coagulation profile should be performed for any patient with an extensive VM, especially if there is a history of easy bruising or bleeding. Stagnation within a VM can cause localized intravascular coagulopathy.

Genetic features

Venous anomalies usually occur sporadically, but families with dominant inheritance have been identified. In these cases, mutation in chromosome 9p21 has been established. This mutation causes ligand-independent activation of an endothelial cell-specific receptor tyrosine kinase.[13] Consequently, venous anomalies could be caused by gene mutations that regulate angiogenesis.

Imaging

VMs can be diagnosed by various imaging modalities such as Doppler US, computed tomography, MRI, and direct phlebography. Interventional radiologic techniques also play an important role in the diagnosis of VMs. Absence of flow or low-velocity venous flow is observed at Doppler US. MRI is very useful in assessing the extension of VMs. These lesions are usually hypointense on T1-weighted images and markedly hyperintense on T2-weighted images. Direct phlebography is useful in confirming the diagnosis and excluding other soft-tissue tumors.

Treatment

Medical treatment

Lower-extremity VMs should first be treated with elastic stockings. Low doses of aspirin seem to minimize phlebothromboses. Preoperative control of intravascular coagulopathy with heparin should be considered before the resection of large VMs.

Sclerotherapy

In cases involving very superficial cutaneous or oromucosal lesions, sclerotherapy is preferred. Sclerotherapy should be performed under fluoroscopic control by a skilled interventional radiologist. The amount of sclerosing agent required is evaluated with percutaneous phlebography. It is important to avoid spillage of the drainage veins with the sclerosing agent. A tourniquet or manual compression is useful in minimizing passage of the sclerosing agent into the systemic circulation. The main complications of sclerotherapy are cutaneous necrosis and neural toxicity. Systemic complications are rare and are related to the passage of sclerosing agent into the systemic circulation. These include hemolysis with potential renal toxicity and cardiac arrest. Sclerotherapy induces an inflammatory reaction that will worsen the symptoms during the week following intervention. Analgesics and anti-inflammatory agents (nonsteroidal anti-inflammatory agents or corticosteroids) must be given to minimize the symptoms. Venous anomalies have a propensity for recanalization and recurrence.

Surgical resection

Surgery is generally contemplated after sclerotherapy when treatment is incomplete or when an aesthetic prejudice requires correction.

Laser therapy

Laser therapy can be useful in very superficial forms of VMs and in oromucosal lesions. Photocoagulation can be performed with argon, yellow dye, or a neodymium yttrium aluminum garnet laser.[14] For deeper lesions, laser probes can be inserted subcutaneously. Satisfactory results with minimal scarring have been reported, but recurrences and repeated treatments are common.


  Conclusion Top


VMs are relatively challenging to diagnose. However, radiologists play a key role in diagnosing and avoiding inappropriate treatment of these lesions. Conservative treatment is recommended in most cases. In cases of functional impairment or significant esthetic prejudice, sclerotherapy with or without surgery is useful even if recurrences are frequent.

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 b'e 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.
Vikkula M, Boon LM, Mulliken JB. Molecular genetics of vascular malformations. Matrix Biol 2001;20:327-35.  Back to cited text no. 1
    
2.
Eifert S, Villavicencio JL, Kao TC, Taute BM, Rich NM. Prevalence of deep venous anomalies in congenital vascular malformations of venous predominance. J Vasc Surg 2000;31:462-71.  Back to cited text no. 2
    
3.
Monteiro LS, Azevedo A, Cadilhe S, Sousa D, Faria C, Martins M. Laser treatment of vascular anomalies of oral cavity. Rev Port Stomatol Med Dent Maxillofac Surg. 2013;54:171-5.  Back to cited text no. 3
    
4.
Behravesh S, Yakes W, Gupta N, Naidu S, Chong BW, Khademhosseini A, et al. Venous malformations: Clinical diagnosis and treatment. Cardiovasc Diagn Ther 2016;6:557-69.  Back to cited text no. 4
    
5.
Kim B, Kim K, Jeon P, Kim S, Kim H, Byun H, et al. Long-term results of ethanol sclerotherapy with or without adjunctive surgery for head and neck arteriovenous malformations. Neuroradiology 2015;57:377-86.  Back to cited text no. 5
    
6.
Albanese G, Kondo KL. Pharmacology of sclerotherapy. Semin Intervent Radiol 2010;27:391-9.  Back to cited text no. 6
    
7.
Winter H, Drager E, Sterry W. Sclerotherapy for treatment of hemangiomas. Dermatol Surg 2000; 26:105–108.  Back to cited text no. 7
    
8.
Yamaki T, Nozaki M, Sasaki K. Color duplex-guided sclerotherapy for the treatment of venous malformations. Dermatol Surg 2000;26:323-8.  Back to cited text no. 8
    
9.
Jain R, Bandhu S, Sawhney S, Mittal R. Sonographically guided percutaneous sclerosis using 1% polidocanol in the treatment of vascular malformations. J Clin Ultrasound 2002;30:416-23.  Back to cited text no. 9
    
10.
Dasgupta R, Fishman SJ. ISSVA classification. Semin Pediatr Surg 2014;23:158-61.  Back to cited text no. 10
    
11.
Dompmartin A, Vikkula M, Boon LM. Venous malformation: Update on aetiopathogenesis, diagnosis and management. Phlebology2010;25:224-35. 10.1258/phleb. 2009.009041  Back to cited text no. 11
    
12.
Boon LM, Mulliken JB, Vikkula M, Watkins H, Seidman J, Olsen BR, et al. Assignment of a locus for dominantly inherited venous malformations to chromosome 9p. Hum Mol Genet 1994;3:1583-7.  Back to cited text no. 12
    
13.
Pavlov KA, Dubova EA, Shchyogolev AI, Mishnyov OD. Expression of growth factors in endotheliocytes in vascular malformations. Bull Exp Biol Med 2009;147:366-70.  Back to cited text no. 13
    
14.
Duyka LJ, Fan CY, Coviello-Malle JM, Buckmiller L, Suen JY. Progesterone receptors identified in vascular malformations of the head and neck. Otolaryngol Head Neck Surg 2009;141:491-5.  Back to cited text no. 14
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8]



 

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  Case Report
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