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Year : 2022  |  Volume : 10  |  Issue : 2  |  Page : 57-61

Mandibular arterio-venous malformation (case report)-rare life threatening condition

Kisco Diagnostic Centre, Palai, Kerala, India

Date of Submission06-Feb-2022
Date of Decision03-May-2022
Date of Acceptance06-May-2022
Date of Web Publication22-Jul-2022

Correspondence Address:
Rinesh Kochummen
KP 129 B Ayyeneth House Panayakonam, Maruthoor, Vattapara, Thiruvananthapuram - 695 028, Kerala
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jomr.jomr_2_22

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Arteriovenous malformations (AVM) are defects in the vascular system, consisting of tangles of abnormal blood vessels (nidus), in which the feeding arteries are directly connected to venous drainage without the interposition of a capillary bed. AVM can occur anywhere in the body, most common in the brain. Other common locations are hepatic, musculoskeletal AVM, spinal AVM, pulmonary, and uterine AVM. In musculoskeletal soft tissue and joints are the more common sites. Bone AVM is very rare. The mean age is 21.86 years. The patient came with complaints of swelling in the left cheek with slight redness over the skin. There was no of history of pain. On examination, no pulsation present. Clinical diagnosis of parotid swelling was given. On USG- loculated cystic lesion in the left mandible with multiple locules and floating internal echoes. On color Doppler, internal vascularity was noted with in the cystic lesion. On computed tomography (CT) scan, a loculated cystic lesion in the enlarged left mandible – enhancement noted with in this cystic lesion – suggesting vascular channels in arterial and better seen in venous phase of contrast imaging. Patient was referred to interventional radiologist where the patient has undergone angioembolization of the AVM followed by mandibulectomy and artificial teeth fixation. As biopsy of the lesion would be dangerous due to significant hemorrhagic risk. Careful attention to the Doppler uptake in USG and contrast uptake in CT angiogram relative to nearby vessels, detection of nearby enlarged abnormal vessels, and performance of dynamic angiography to document arteriovenous shunting are crucial for appropriate management.

Keywords: Arteriovenous malformation/diagnosis, auditory-verbal therapy, dilated vascular channels in Mandibular bone

How to cite this article:
Kochummen R. Mandibular arterio-venous malformation (case report)-rare life threatening condition. J Oral Maxillofac Radiol 2022;10:57-61

How to cite this URL:
Kochummen R. Mandibular arterio-venous malformation (case report)-rare life threatening condition. J Oral Maxillofac Radiol [serial online] 2022 [cited 2022 Dec 3];10:57-61. Available from: https://www.joomr.org/text.asp?2022/10/2/57/351670

  Introduction Top

Arteriovenous malformations (AVM) occur as a result of errors in vascular morphogenesis present at birth[1],[2] grows with age and manifest at any time during life due to an event such as trauma, surgery, and infection.[3]

Mandibular AVMs are uncommon and potentially life-threatening.[4],[5],[6] Young females are more affected. They are frequently high-flow vascular malformations.[7] They may present with symptoms such as deformity, gingival bleeding, dental loosening, lower lip numbness, facial deformity, malocclusion, and sometimes hemorrhagic shock, following extraction of teeth.[8],[9],[10] A review of fatal cases by Lamberg et al.[11] shows that in most instances, exsanguination is the result of dental extractions when the dentist is unaware of AVM existence.

Before the 1980s, vascular lesions were referred to as “hemangiomas.”[12] Thereafter, they were subdivided into hemangiomas and vascular malformation.[13]

In X-ray, the appearance of the lesion can be variable, ranging from small radiolucency to markedly osseous erosion of the alveolus with apparently floating teeth. Computed tomography (CT) scan and magnetic resonance imaging (MRI) are helpful imaging tools to assess the extent of the lesion into bone, soft tissue, and major vessels.


These lesions are the result of embryonic abnormality caused by the failure of differentiation in the early stages of embryogenesis.[12] They are usually extraosseous, more commonly appear in childhood and tend to regress in adults. They are rarely associated with fatal hemorrhages.[14] Vascular malformation is caused by disturbance in later stages of angiogenesis (truncal stage) and results in persistence of AVM during embryonic life-[15] can be capillary, lymphatic, venous arterial, or mixed. It develops in proportion to physical growth.[16] Local hemodynamic factors promote the increase in the size of these vascular malformations, which is asymptomatic and imperceptible at the early stage. Viscous circle of etiopathogenesis of AVM mentioned in [Figure 1], [Figure 2], [Figure 3], [Figure 4].
Figure 1: Etiopathogenesis of AVM

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Figure 2: Plain radiograph shows lytic loculated lesion in the left mandible.no relation to tooth or unerupted tooth noted

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Figure 3: (a-d) Ultrasound shows loculated cystic lesion in the left mandible, on color doppler slow flow was detected with enlarged vessels adjacent to lesion

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Figure 4: (a-b)CT angiogram showed enhancing dilated vascular lesion within the left mandible which shows contrast uptake in arterial phase itself with draining large veins noted adjacent to it

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Clinical symptoms

The most common symptom is gingival bleeding.[17] Other features are alteration of facial morphology, bruit accompanied by the thrill and neurosensory defects.[16] Vascular naevi or phlebectasias may discolor the adjacent mucosal skin.[15],[17] They can also present with nasal blockade, epistaxis, rhinitis, or diplopia.[16]

Radiological signs

Poorly defined radiolucent lesion.[16] with honeycomb/soap bubble appearance.[15],[17],[18] Root resorption has been observed, creating an appearance of teeth floating in adjacent alveolar osseous erosions.[14],[15] CT and MRI are done to know the extent of lesion, bone erosions, and involvement of vessels.[16]

Superselective arteriography is still a vital tool in the identification of vascular malformation and contributory vessels.[18] It should be done on both sides for finding collaterals and multiple anastomosis of the maxillary artery.[19]

Management strategies

Sclerosants (sodium morrhuate, water, nitrogen mustard, etc.) were used in the past, but ineffective as they are displaced by blood flow.[12] Ligation of the external carotid artery is not preferred[12],[15],[18],[20],[21],[22],[23],[24] as many anastomoses (internal carotid artery, vertebral, cervical, and contralateral external carotid artery) promote the rapid appearance of collateral circulation.

Embolization agents commonly used are polyvinyl alcohol particles,[16],[24],[25] muscle,[26] gelfoam,[17],[18],[21] cyanoacrylate,[12],[17],[23],[24],[25],[26] metal coils,[22],[26],[27] collagen;[20],[28] some clinicians use it as sole approach[23] or adjunct to excision and reconstruction surgery.[14]

If the main nutrient arteries of malformation are embolized, the blood flow is redirected to the collaterals, which is angiographically invisible due to limited perfusion, dilate as a result of the hemodynamic change and reirrigate the malformation.[11],[15],[17],[27] Therefore, recurrence follows.

Use of fluid material is more useful in obliterating distal nutrient arteries and reducing risk of recurrence.[26] Multiple embolization may be necessary and venous route may supplement conventional arterial embolization.[22],[26]

Recently, transosseous puncture of vascular bed is also done.[23],[29],[30] Core of malformation and all associated vascular pedicles are embolized.

Rodesh et al.[16] did a study suggesting a success rate of 100% in embolization management of AVM. Nine patients treated with cyanoacrylate were stable (33%) or cured (67%) without further treatment.[23]

Embolization plus surgery is the most conventional modern approach.[14],[17],[20],[21],[25],[27],[31],[32],[33],[34] It controls sudden bleeding, but does not eliminate the risk of recurrence (owing to collateral circulation). It reduces blood flow allowing excision surgery to be performed within 48 h–2 weeks.[21] Resection of the mandibular fragment containing lesions was previously considered mandatory for complete healing.[25],[27],[33] Curettage of resected fragments and reimplantation reduces morbidity.[28],[33]

  Case Report Top

A 9-year-old female patient presented at outpatient department in 2020 with asymptomatic swelling of left parotid region for 2 months. There was no history of fever or trauma. On examination, no rise of temperature and no tenderness present. Systemic examination was unremarkable. On X-ray AP view, a large expansile radiolucent lesion with soap bubble appearance in left ramus and angle of mandible. Possibility of aneurysmal bone cyst/ameloblastoma/ameloblastic fibroma was thought. On USG showed a large loculated cystic lesion with internal vascularity suggesting large torturous vessels.

Noncontrast CT shows a large expansile lytic lesion with cortical thinning and destruction of left ramus of mandible. The outer wall of lesion appears markedly thinned out. Contrast-enhanced CT show enhancing lesion in the arterial and venous phase with abnormal tuft of tortuous dilated feeding vessels at site of lesion. On contrast-enhanced CT, multiple arterial feeders to lesion were seen from external carotid artery with venous drainage into the left external jugular vein.


First feeding Artery was embolised first,particle used (355-500 micrometer),then facial artery – angiogram to assess venous “road map” allowing transvenous placement of microcatheter into venous pouch. 2 ml of 25% glue + 75% lipoidal into venous pouch with subsequent pressure over IJV. Then direct puncture of glue in remaining venous pouch with 50% of lipoidal :glue. After embolization external carotid artery angiogram was done. Post operative pulmonary radiograph showed no presence of embolic material in the lung fields.

  Discussion Top

About 51% of vascular malformation occur in head-and-neck region and male-to-female ratio is 1:1.5.[31] Arteriovenous (AV) malformations are high-flow lesion and among the most serious of vascular malformation. It is difficult to diagnose, treat, and cure. The detection rate of AV malformation in general population based on prospective date from New York Islands study is approximately 1.34/100,000 persons.[32] Death occurs in 10%–15% of patients who have hemorrhage and morbidity of various degrees occurs in approximately 50% of cases.[33] Multiple imaging modalities should be used for characterisation of lesion such as size, flow velocity, flow direction relation to surrounding structures, and lesional contents.[34] There is no specific pathognomic feature in X-ray. In X-ray, bone erosion, sclerotic changes, periosteal reactions, and cyst-like radiolucent lesions may be seen. The sunburst effect in radiography is created by spicules radiating from center.

X-ray differential diagnosis is odontogenic myxoma, central giant cell granuloma, and metastatic malignant lesion.

Before doing biopsy in children, it is better to advise a CT/MRI with contrast to rule out possibility of AV malformation to avoid death due to sudden hemorrhage. Angiography can be used as gold standard investigation for determination of location and flow characteristics of vascular lesion (blood supply of lesion-arterial feeders and venous drainage).[31] CT and MRI are done to know the extent of lesion, bone erosion, and involvement of major vessels.

Superselective arteriography and embolization of main nutrient artery is not useful. Transfemoral embolization of proximal and distal vessels is followed by surgical intervention.

Direct intralesional injection of isobutyl cyanoacrylate eliminates whole vascular lattice work feeding the lesion will promote full expression of regenerative potential of somatic growth to replace vascular anomaly.

  Conclusion Top

AVM are rare in bone. Mandibular AVM present like an asymptomatic swelling so careful radiological examination can prevent untoward incident of biopsy and massive hemorrhage.

Plain radiograph shows lytic loculated lesion in the left mandible. No relation to tooth or unerupted tooth noted.

Ultrasound shows loculated cystic lesion in the left mandible, on color Doppler slow flow was detected with enlarged vessels adjacent to lesion.

CT angiogram showed enhancing dilated vascular lesion within the left mandible which shows contrast uptake in arterial phase itself with draining large veins noted adjacent to it.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Lamberg MA, Tasanen A, Jääskeläinen J. Fatality from central hemangioma of the mandible. J Oral Surg 1979;37:578-84.  Back to cited text no. 1
Anderson JH, Grisius RJ, McKean TW. Arteriovenous malformation of the mandible. Oral Surg Oral Med Oral Pathol 1981;52:118-25.  Back to cited text no. 2
Mosnier I, Derhy S, Martin F, Princ G. Arteriovenous malformation of the mandible. Apropos of a case in a 6-year-old child. Ann Otolaryngol Chir Cervicofac 1996;113:434-9.  Back to cited text no. 3
Jackson IT, Jack CR, Aycock B, Dubin B, Irons GB. The management of intraosseous arteriovenous malformations in the head and neck area. Plast Reconstr Surg 1989;84:47-54.  Back to cited text no. 4
Holt GR, Tinsley PP Jr., Aufdemorte TB, Steed DL, Dittman WI. Arteriovenous malformation of the mandible. Otolaryngol Head Neck Surg 1983;91:573-8.  Back to cited text no. 5
Engel JD, Supancic JS, Davis LF. Arteriovenous malformation of the mandible: Life-threatening complications during tooth extraction. J Am Dent Assoc 1995;126:237-42.  Back to cited text no. 6
Kula K, Blakey G, Wright JT, Terry BC. High-flow vascular malformations: Literature review and case report. Pediatr Dent 1996;18:322-7.  Back to cited text no. 7
Glowacki J, Mulliken JB. Mast cells in hemangiomas and vascular malformations. Pediatrics 1982;70:48-51.  Back to cited text no. 8
Larsen PE, Peterson LJ. A systematic approach to management of high-flow vascular malformations of the mandible. J Oral Maxillofac Surg 1993;51:62-9.  Back to cited text no. 9
Johnson LM, Cook H, Friedlander A. Central arteriovenous malformations of the maxillofacial skeleton: Case report. J Oral Maxillofac Surg 1991;49:759-63.  Back to cited text no. 10
McKenna SJ, Roddy SC Jr. Delayed management of a mandibular vascular malformation. J Oral Maxillofac Surg 1989;47:517-22.  Back to cited text no. 11
Kelly DE, Terry BC, Small EW. Arteriovenous malformation of the mandible: Report of case. J Oral Surg 1977;35:387-93.  Back to cited text no. 12
van den Akker HP, Kuiper L, Peeters FL. Embolization of an arteriovenous malformation of the mandible. J Oral Maxillofac Surg 1987;45:255-60.  Back to cited text no. 13
Lasjaunias P, Berges O, Doyon D. Collateral circulation of the internal maxillary artery. J Neuroradiol 1979;6:197-205.  Back to cited text no. 14
Beek FJ, ten Broek FW, van Schaik JP, Mali WP. Transvenous embolisation of an arteriovenous malformation of the mandible via a femoral approach. Pediatr Radiol 1997;27:855-7.  Back to cited text no. 15
Rodesch G, Soupre V, Vazquez M, Fain J, Alvarez H, Lasjaunias P. Arteriovenous malformations in the jaws. The place of intravascular therapy. Apropos of 14 cases. Rev Stomatol Chir Maxillofac 1999;100:293-8.  Back to cited text no. 16
Kiyosue H, Mori H, Hori Y, Okahara M, Kawano K, Mizuki H. Treatment of mandibular arteriovenous malformation by transvenous embolization: A case report. Head Neck 1999;21:574-7.  Back to cited text no. 17
Mohammadi H, Said-al-Naief NA, Heffez LB. Arteriovenous malformation of the mandible: Report of a case with a note on the differential diagnosis. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1997;84:286-9.  Back to cited text no. 18
Rappaport I, Rappaport J. Congenital arteriovenous fistula of the maxillofacial region. Am J Surg 1977;134:39-48.  Back to cited text no. 19
Procházková L, Machálka M, Procházka J, Tecl F, Klimovic M. Arteriovenous malformations of the orofacial area. Acta Chir Plast 2000;42:55-9.  Back to cited text no. 20
Benndorf G, Campi A, Hell B, Hölzle F, Lund J, Bier J. Endovascular management of a bleeding mandibular arteriovenous malformation by transfemoral venous embolization with NBCA. AJNR Am J Neuroradiol 2001;22:359-62.  Back to cited text no. 21
Schneider C, Wagner A, Hollmann K. Treatment of intraosseous high flow arteriovenous malformation of the mandible by temporary segmental ostectomy for extracorporal tumour resection: A case report. J Craniomaxillofac Surg 1996;24:271-5.  Back to cited text no. 22
Nancarrow PA, Lock JE, Fellows KE. Embolization of an intraosseous arteriovenous malformation. AJR Am J Roentgenol 1986;146:785-6.  Back to cited text no. 23
Flandroy P, Pruvo JP. Treatment of mandibular arteriovenous malformations by direct transosseous puncture: Report of two cases. Cardiovasc Intervent Radiol 1994;17:222-5.  Back to cited text no. 24
Chiras J, Hassine D, Goudot P, Meder JF, Guilbert JF, Bories J. Treatment of arteriovenous malformations of the mandible by arterial and venous embolization. AJNR Am J Neuroradiol 1990;11:1191-4.  Back to cited text no. 25
Abouzgia MB, Symington JM. Recurrent arteriovenous malformation of the mandible: A case report. J Oral Maxillofac Surg 1992;50:1230-3.  Back to cited text no. 26
Behnia H, Motamedi MH. Treatment of central arteriovenous malformation of the mandible via resection and immediate replantation of the segment: A case report. J Oral Maxillofac Surg 1997;55:79-84.  Back to cited text no. 27
Seccia A, Salgarello M, Farallo E, Falappa PG. Combined radiological and surgical treatment of arteriovenous malformations of the head and neck. Ann Plast Surg 1999;43:359-66.  Back to cited text no. 28
Frame JW, Putnam G, Wake MJ, Rolfe EB. Therapeutic arterial embolisation of vascular lesions in the maxillofacial region. Br J Oral Maxillofac Surg 1987;25:181-94.  Back to cited text no. 29
Siu WW, Weill A, Gariepy JL, Moret J, Marotta T. Arteriovenous malformation of the mandible: Embolization and direct injection therapy. J Vasc Interv Radiol 2001;12:1095-8.  Back to cited text no. 30
Nekooei S, Hosseini M, Nazemi S, Talaei-Khoei M. Embolisation of arteriovenous malformation of the maxilla. Dentomaxillofac Radiol 2006;35:451-5.  Back to cited text no. 31
Stapf C, Mast H, Sciacca RR, Berenstein A, Nelson PK, Gobin YP, et al. The New York Islands AVM Study: Design, study progress, and initial results. Stroke 2003;34:e29-33.  Back to cited text no. 32
Weston-Schumacher H, Marshall R. Arteriovenous Malformations. Emedicine; 2010. Available from: http://emedicine.medscape.com/article/1160167-overview. [Last accessed on 2020 Feb 13].  Back to cited text no. 33
Hyodoh H, Hori M, Akiba H, Tamakawa M, Hyodoh K, Hareyama M. Peripheral vascular malformations: Imaging, treatment approaches, and therapeutic issues. Radiographics 2005;25 Suppl 1:S159-71.  Back to cited text no. 34


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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