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CASE REPORT
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
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jomr.jomr_2_22

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  Abstract 


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 Aug 11];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.

Etiopathogenesis

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.

Technique

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

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

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    Figures

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



 

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