International Journal of Health Sciences and Research www.ijhsr.org ISSN: 2249-9571

Case Report

A Rare Case of Submandibular and Submental Arteriovenous Malformation in a Female Patient

Kunal Shahi1, Sanjay P2, Vishal NS3, Rani Baby2

1Assistant Professor, 2Post-graduate Student, 3Senior Resident, Department of Radio-Diagnosis, BLDE University, Shri B.M.Patil Medical College, Bijapur, Karnataka, India.

Corresponding Author: Rani Baby

Received: 18/02//2014 Revised: 03/05/2014 Accepted: 12/05/2014

ABSTRACT

Vascular malformations of the submandibular and submental region can lead to varoius complications. Lesions of the submandible region are rare and potentially life threatening entities. However, the clinical course of malformations involving the mandible and the submental region remains unclear and unpredictable. Treatment may be surgical or non-surgical.

Keywords: Submandibular, submental malformation, arteriovenous, female.

INTRODUCTION The aretriovenous malformation is Arteriovenous malformations an uncommon lesion that is considered to be (AVMs) are high-flow lesions of part of the spectrum of hemangiomatous intercommunicating veins and arteries anomalies. Specifically, it represents a mass formed by abnormal vessel morphogenesis. of intercommunicating, variously sized [1] arteries and veins. [2] Most of the AVMs discussed in The AVM is classified as congenital previous review articles are congenital or acquired (traumatic). The congenital type lesions that became clinically apparent later represents vascular development arrested at in life, whereas acquired AVMs are rare and the retiform state; the acquired type results usually form post traumatically. [1] from penetrating wounds or blunt trauma. [2] Both types of AVMs may produce Lesions of the mandible are rare and symptoms of pain, swelling, or potentially life threatening entities that can discoloration, whereas some AVMs present present as innocuous episodes of gingival solely as a mass lesion. The 2 types differ in bleeding, slow-growing expansile masses, or that congenital AVMs often have multiple severe haemorrhage. A biopsy or even a communications between arteries and veins, simple tooth extraction can cause a whereas acquired lesions may be solitary catastrophic bleeding that may even lead to arteriovenous communications. [1] death.

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However, the clinical course of Here in, we report the case of a 28- malformations involving the mandible and year-old woman with an AVM of the the maxilla remains unclear and submandibular and submental region. unpredictable. [3] Vascular malformations are CASE REPORT frequently seen in the skin, but rarely affect A 28 year old female patient came the visceral organs or bones; approximately with complaints of swelling in the left 51% occur in the head and neck, with a male submandibular and submental region since female ratio of 1 to 1.5. [4] 10 years. The swelling was initially small in Treatment may be surgical or non- size and started to increase in size from past surgical. The latter includes intravascular one year. embolization with coil and/or sclerosing The swelling measures solutions. Surgical resection is reserved for approximately 5 x 2cm in size (Figure 1). lesions that are extensive and/or refractory The swelling is soft in consistency, does not to endovascular therapy. Cure is defined as change in size on swallowing or valsalva the complete eradication of disease or maneuver (Figure 2). permanent resolution of symptoms with On auscultation bruit was heard on the complete devascularisation. [4] swelling. There was no local rise of Although literature discussing the temperature in the swelling. The swelling complications of these treatments is limited, increased in size during early morning and complications that have been described the patient complains of difficulty in speech include reperfusion bleeding, ischemic in early morning due to increase in size of ulceration and necrosis, infection, numbness swelling which pushed the tongue upwards of the inferior alveolar nerve, and temporary and to the opposite side. The swelling later facial nerve paralysis. [5] subsides as day progresses. Most cases of extracranial AVMs in The patient had no other complaints. The the literature are cited in female patients patient was advised ultrasonography of the with very few cases presenting in males. submandibular region. Furthermore, only 8 cases of submandibular Ultrasound showed increased soft AVMs have been cited in the literature, with tissue echogenicity in submental and all cases presenting in females. [5] submandibular region with dilated and tortuous vessels seen bilaterally. (Figure 3 and 4)

Figure.1 Figure: 2

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Figure: 3 Figure: 4

Figure: 5 Figure: 6

Arterial phase of CT scan of the neck region The vascular lesion was fed by branches showing tuft of tortuous vascular channels arising from bilateral external carotid (L>R) in submandibular & submental arteries just after their origin, the lesions regions bilaterally (Figure 5) were draining into bilateral internal jugular Venous phase of CT scan of the neck region veins. CT features suggested arterio-venous shows the lesion draining into bilateral malformation. internal jugular veins (Figure 6). CT neck plain and contrast was performed DISCUSSION which showed tuft of tortuous vascular The first case of a submandibular channels (L>R) in submandibular & AVM was noted in the literature in 1985 and submental regions bilaterally (Figure 5 and described in a 57-year-old female patient. [2] 6). Since that time, very few cases of The lesion measured about 54 X 32mm on extraosseous submandibular AVMs have left side. been reported. [3]

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AVMs are extremely rare entities that can be sessions such as previous history of arterial life threatening if left untreated. [4] ligation or high flow characteristics of the Although CT, MRI and MRA may localize lesion or microshunts, which are invisible the arterio-venous shunt lesion, super during hyperselective catheterization and selective arteriography remains an essential therefore inaccessible to treatment. [4] tool for diagnosis and planning of treatment. Vascular malformations of the head [4] and neck can be classified into low-flow and AVMs usually present with non-specific high-flow lesions with low flow lesions symptoms including bruit, dental loosening, including hemangiomas and capillary and swelling of soft tissues, change in skin and venous malformations. High-flow lesions mucosal colour and dysesthesia of the lower such as AVMs are rarer and may not lip or chin. become clinically apparent until later in Management of AVMs is usually childhood, with the majority located in the complex and requires a multidisciplinary head and neck region, particularly in team for successful outcome. Observation intracranial locations. Extracranial lesions may be used as a temporary measure in often appear to have osseous involvement, special situations, such as extreme age, most notably in the mandibular and pregnancy or refusal of therapy. No maxillary bones. Lesions in the spontaneous regressions have been submandibular triangle that do not involve documented. On the other hand, it has been the bone are uncommon and appear to have reported that the volume of the lesion may a glandular or nodal origin. [5] gradually increase. [4] Our patient appears to be a rare Arterial ligature was used in the past reported case of an extraosseous and as a purely symptomatic treatment or before extraglandular submandibular AVM in a surgery. At present, it is well known that female patient. Furthermore, based on her ligation of external carotid artery should not CT findings, which demonstrated multiple be performed, firstly as many anastomoses arteriovenous communications within the promote the rapid appearance of a collateral lesion and her lack of a traumatic history, circulation, and secondly because future this AVM is most likely a congenital lesion. embolization would be impossible. [4] Presentation of a congenital lesion at 28 At present, superselective years of age is also very unusual because angiographic embolization is considered most lesions present by late childhood. first line treatment, alone or in combination The female predominance of reported with surgical approach to reduce submandibular lesions has not yet been intraoperative bleeding. Occlusion of the explored. Most of the documented cases lesion is obtained using movable balloon, have been in middle-aged females, coils or liquid glue. Endovascular therapy as suggesting that hormonal changes may definitive treatment for the AVMs of the affect endothelial cells and vascular mandible has been reported to have a formation. In one study, 33% of female success rate of 70%. [4] patients were noted to have exacerbation of However, serious complications after symptoms with their menstrual cycles and embolization (e.g., occlusion of pulmonary with pregnancy. This hormonal influence or cerebral vessels) or recurrence of AVMs may help explain why the AVMs seen in should be considered. In some cases, the these patients presented late and with no procedure has to be carried out in several trauma history. [5]

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Therefore, additional research is REFERENCES needed to explain the novel etiological 1. Baker LL, Dillon WP, Hieshima GB, et factors involved in the development of al. Hemangioma and vascular submandibular AVMs and their predilection malformations of the head and neck: to effect women. [5] MR characterization. Am J Neuroradiol 1993;14:307 - 14. In our case, surgical excision of 2. Burns JC, Julian J, Alexander J. AVM involving soft tissues in the Arteriovenous malformation of the submandibular space was achieved without submandibular gland. J Oral Maxillofac complications. Surg 1985;43:294 -6. 3. Fine MJ, Holliday RA, Roland JT. CONCLUSION Clinically unsuspected venous In conclusion, considering the malformations limited to the present case and literature data, we can submandibular triangle: CT findings. define the following general principles of AJNR Am J Neuroradiol 1995;16:491- treatment. 4. Arterial ligation should not be 4. R. Spreafico, L. Sordo, R. Bellotto, et al. Arterio-venous malformation of the performed, even in emergency settings. mandible. Case report and review of Super selective arterial embolization is the literature: ACTA otorhinolaryngologica treatment of choice and can be repeated in Italica:EPUB February 09, 2012. the case of relapse. 5. Heather M. Stocks, BS, Suzanne In AVMs of the mandible, surgery Sacksman, BS, Peter Thompson et al. should be reserved only for cases that are First documented case of a refractory to endovascular therapy and/or for submandibular arteriovenous therapeutic complications that are not malformation in a male patient: otherwise treatable (bone fractures and/or American Journal of Otolaryngology– necrosis). Head and Neck Medicine and Surgery In AVMs of the soft tissue, surgery 28 (2007) 275– 276. could be used if there are no significant side effects.

How to cite this article: Shahi K, Sanjay P, Vishal NS et. al. A rare case of submandibular and submental arteriovenous malformation in a female patient. Int J Health Sci Res. 2014;4(6):254-258.

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