|Year : 2013 | Volume
| Issue : 1 | Page : 51-56
Asymptomatic intermittent swelling on the right side of the face
Poornima Rangaiah1, Ashok Lingappa1, Rajeshwari Gangappa Annigeri2, Kirthi Kumar Rai3
1 Department of Oral Medicine and Radiology, Bapuji Dental College and Hospital, Davangere, Karnataka, India
2 Department of Oral Medicine and Radiology, Bapuji Dental College and Hospital; College of Dental Sciences, Davangere, Karnataka, India
3 Department of Oral and Maxiilofacial Surgery, Bapuji Dental College and Hospital, Davangere, Karnataka, India
|Date of Web Publication||26-Nov-2013|
Department of Oral Medicine and Radiology, Bapuji Dental College and Hospital, Davangere - 577 004, Karnataka
Source of Support: None, Conflict of Interest: None
Vascular malformations (VM) are errors of vascular morphogenesis present at lower incidence accounts approximately 7% of all benign tumors, about 1/3 rd of all VMs occur in the head and neck region, more than 50%. Arteriovenous malformations are present in the head and neck region. Clinicians who diagnose and treat oral conditions should be aware of these lesions and their impact on routine procedures. Proper recognition and therapeutic intervention can help avoid serious complications and potentially tragic outcomes.
Keywords: Arteriovenous malformation, vascular malformations, doppler color ultasonography, Hemangioma
|How to cite this article:|
Rangaiah P, Lingappa A, Annigeri RG, Rai KK. Asymptomatic intermittent swelling on the right side of the face. Int J Oral Health Sci 2013;3:51-6
| Introduction|| |
Hemangioma was the term used to describe lesions that bear a little resemblance to each other apart from being involved with vessels. What was referred to as hemangioma some years back is not the same today. The most recent and accepted concept is that hemangioma are tumors identified by rapid endothelial cell proliferation in early infancy, followed by involution over time; all other abnormalities are malformations resulting from anomalous development of vascular plexuses. The malformations have a normal endothelial cell growth cycle that affects the veins, the capillaries, or the lymphatics and they do not involute both hemangioma and vascular malformations are included in the broad and scientific term "vasoformative tumors". 
Vascular malformations (VM) are errors of vascular morphogenesis present at lower incidence accounts approximately 7% of all benign tumors, about 1/3 rd of all vascular malformations occur in the head and neck region, more than 50% arteriovenous malformations (AVM) are present in the head and neck region. , Clinicians who diagnose and treat oral conditions should be aware of the lesions and the impact they can have on routine procedures. Proper recognition and therapeutic intervention can help avoid serious complications and potentially tragic outcomes. Hereby, presenting a case report of rare vascular tumor called A-V malformation.
| Case Report|| |
A 23-year-old male patient reported to us with a chief complaint of painless swelling on the right side of the face since 6-7 months. He gave a history of swelling, which was insidious in onset not associated with pain, denied any history of trauma. He told the swelling was intermittent in nature where in it was more in the night and early mornings and which keeps on decreasing as the day progresses, i.e. "Turkey Wattle Sign" a swelling that appears only in the dependent position;  there was no history of nasal discharge, nasal discomfort or epistaxis.
Extraoral examination revealed a mild mid-facial asymmetry because of a roughly spherical swelling present on the right middle one-third of face overlying the right maxillary sinus region measured about 5 × 5 cm. Extended superoinferiorly 1 cm below the infraorbital margin up to the line joining the corner of mouth to lobule of ear. Anteroposteriorly extended from 1.5 cm lateral to ala of nose up to 3 cm anterior to the tragus of the ear. Skin over the swelling was normal; surface had irregularities due to the presence of acne scars with ill-defined borders and no visible pulsations [Figure 1] and [Figure 2].
|Figure 1: Extraoral spherical swelling of right midfacial region measuring 5 × 5 cm (color)|
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On palpation swelling was warm, non-tender, compressible, soft in consistency and palpable thrill was present. On auscultation, faint bruit was heard over the surface of swelling.
On intraoral examination inspection showed areas of reddish discoloration of the labial mucosa and buccal mucosa extending from distal aspect of 12 to the distal aspect of 17 with few superficially dilated veins [Figure 3]. On bimanual palpation, it was soft in consistency.
|Figure 3: Intraoral view to show reddish discoloration of mucosa and few dilated superficial veins-arrow indicator (color)|
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Clinical differential diagnosis included A-V malformation, Aneurysms, Hemangioma.
| Investigations|| |
Routine screening radiographs such as intraoral periapical, orthopantomograph, paranasal sinus view did not reveal any significant information. Patient was then subjected to Doppler ultrasound imaging which on gray scale imaging revealed areas of multiple dilated hypoechoic areas measuring 0.86 × 1.35 cm [Figure 4]. On color Doppler, the lesion took up the color [Figure 5]. Facial artery proximal to the lesion showed biphasic flow pattern giving an impression of A-V malformation arising from the branch of the facial artery and facial vein on the right cheek.
|Figure 4: Ultrasound gray scale imaging showing hypoechoic areas (black and white)|
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|Figure 5: Color Doppler ultrasound scan which has taken up color (color)|
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Magnetic resonance imaging (MRI) was done to know the exact extent of the lesion, which showed ill-defined signal abnormalities on the right cheek involving cutaneous and subcutaneous planes, which were hypointense on T1 weighted images [Figure 6] and hyperintense on T2 weighted images [Figure 7]. Carotid angiography was done to locate the feeder arteries, which showed prominent right external carotid artery and branches originating from the external carotid artery were supplying the lesion [Figure 8].
|Figure 6: Magnetic resonance imaging T1 weighted images (black and white)|
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|Figure 7: Magnetic resonance imaging T2 weighted images (black and white)|
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|Figure 8: Carotid angiography showing the feeding vessels (black and white)|
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| Management|| |
Patient was planned for Sclerotherapy by intralesional injection of 3% of sodium tetradecyl sulfate. The lesion was compartmentalized using nonresorbable suture prolene 2-0 Papscue's technique. In stage-I 3.5 ml of sclerosing agent was injected into the sub-dermal plane; similar procedure was carried out after a gap of 15 days as a stage-II Sclerotherapy where 4 ml of sclerosing agent was injected. Repeat Doppler ultrasound showed a significant decrease in flow pattern of lesion. The lesion which was soft in consistency has become relatively hard to palpation. Since the lesion did not resolve to a desired size and was persisting patient was undertaken for surgical excision. After 2 months of initial diagnosis surgical debulking of lesions was done by reflection of mucocutaneous flap overlying the lesion under general anesthetic. Excised specimen was measuring 4 × 2.5 × 2 cm and was sent for histopathological examination [Figure 9].
Histopathology showed many vascular channels diffusely dispersed in fibrous connective tissue with the presence of blood vessels in between the adipose tissue and the muscle fibers. Presence of dilated vessels with thickened tunica intima and lumen filled with RBC's and characteristically revealed juxta positioning of arteries and veins [Figure 10] and [Figure 11].
|Figure 10: Photomicrograph of histopathological sections (H and E) at ×5 (color)|
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|Figure 11: Photomicrograph of histopathological sections (H and E) at ×10 (color)|
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Final diagnosis of A-V malformation of right midfacial region involving right facial artery and facial vein was given. Patient was followed up for a year with no recurrence and was planned for further surgical procedures to eliminate post surgical scar.
| Discussion|| |
Review of literature
Hunter (1757) was the first person to recognize an arteriovenous (AV) fistula. Branham (1890) correlation of pulse rate on compression of AV fistula, where there was a decrease in pulse rate on compression of AV fistula called it Branham sign. Reid (1900) - gave thermocouple readings from the surface of AV malformation, he found a 2×C increase in temperature over the surface.
Biberstein and Jersner (1956) were the first to name it cirsoid aneurysm, based on the tortuous nature of it. Gomez (1955-1965) - reviewed AVM for 10 years, told majority are congenital. Pulmonary sites, extremities and head and neck rank the third site of occurrence.  Brown (1973) reported cases of AVM of orbit, middle cranial fossa and mandible. Boyel (1984) told about the high incidence of bone involvement (34%) in VMs when compared with Hemangioma (1%). Engle (1995) reported more than 100 cases maxillofacial AVMs. 
A-V malformation/A-V hemangioma of the head and neck are poorly circumscribed, superficial lesions of adulthood, composed of closely associated - structurally abnormal arteries, veins and capillaries, with vessels present that cannot be readily categorized as arteries/veins. Synonyms include arteriovenous aneurysms, central hemangioma, pulsatile hemangioma, arteriovenous shunt, arteriovenous fistula, vascular malformation, arteriovenous malformation. 
The vascular malformation represents an arrest in the development of the mesenchyme primordia in the undifferentiated capillary network stage. As differentiation progresses, primitive vessels penetrate deeper into the subcutaneous layer, the muscle, or the bone tissue. The final development stage involves the gradual replacement of the immature plexiform network by the mature vascular channels and gives rise to a vascular malformation.
A number of growth factors, including vascular endothelial growth factor, basic fibroblast growth factor, transforming growth factor β and interleukin 6 have been demonstrated as regulators of angiogenesis. 
Exact etiology is unknown. One hypothesis postulates that placental cells, such as the trophoblast may be the cell of origin for vascular malformations. Therefore, vascular malformations may arise secondary to some event in utero. The relationship between vascular malformations and placental tissues is controversial and needs further investigation.
Vascular malformations occur more commonly in whites; rarely in dark-skinned individuals and equal predilection for males and females.
Clinical differential diagnosis
Mucocele is a mucous extravasation type of cyst, presents as bluish white swelling. Common site is lower lip usually associated history of trauma. It is a non-compressible swelling and shows fluctuation.
Cavernous hemangioma is developmental vascular malformation of low flow variety. Appears as reddish to blue, soft tumors that arise above the skin level in nodular fashion. Most common sites are tongue, palate, cheek and gingiva. Size varies from few millimeters to several centimeters, do not blanch under pressure and characteristically diascopy positive.
Aneurysms are weakened areas in the vessel walls that bulge like balloons when blood flows through them. They are rarely present, pulsate and show typical paramedian location. Once they grow to a certain size, there is a risk of rupture and life-threatening bleed.
Lymphangioma is a developmental lymphatic vessel malformation. Common presentation is on the tongue, lip, buccal mucosa. Superficial lymphangioma are having multiple vesicular, cystic swelling having clear fluid inside. True lympahngiomas are rare they are many times co-existing with venous malformations.
Phlebectasias and Hematoma: Patient gives a traumatic history and disappears in few days and cannot be emptied on compression.
Angiosarcoma: Malignancy of vascular endothelium which may arise from either blood or lymphatic vessels. Oral lesions are rare if they occur then most common site is mandible and affects older age group. Appear as simple bruise and lesion continue to enlarge, which results in elevated nodular or ulcerated surface.
Larsen and Peterson - steps in diagnosing and treating VM.
Doppler ultrasound, aspiration - clinically which gives frank blood when done with a narrow bore needle. Arteriography is done to exactly delineate the feeding arteries and draining veins. Computed tomography scan is to evaluate for any bony erosions and its relationship to adjacent structures. MRI is a very useful diagnostic modality in case of intramuscular/superficial vascular malformations. Digital subtraction radiography, which permits for better visualization of lesion by removing the superimposition caused by osseous structures.
Treatment options are individualized based on tumor location, accessibility, depth of invasion, patient age and cosmetic considerations.
If the lesion in question is determined to be vascular malformation rather than a hemangioma, then its flow characteristics must be gauged. Low flow vascular malformations can be managed in numerous ways, i.e. lesions that are easily collapsible and are accessible may be managed well with Sclerotherapy, laser therapy, or cryosurgery.
High flow lesions require pre-surgical embolization followed by aggressive ablative therapy. The decision on timing of Sclerotherapy and surgical treatment needs to be individualized, depending upon the objectives of embolotherapy. Surgery should be done within 48-72 h of embolization therapy so as to prevent collateralization and recanalization of the vessels.
Various other treatment modalities are medical therapy in which steroids are used intralesionally e.g. Prednisolone 20-30 mg/d for 2 weeks to 4 months.  Triamcinolone acetonide 4 mg/ml,  use of interferon alfa-2a has also been documented. 
Embolotherapy is an adjunctive procedure in management of vascular malformations, wherein small foreign particles are injected into vascular malformation causing thrombosis thereby reducing the blood flow by promoting ischemic changes leading to fibrosis of surrounding vascular architecture. The following agents have been used:
Sclerotherapy is aimed at obliterating the lumen, with replacement of the patent vessel by fibrosis. The following are used as sclerosing agents: Sodium tetradecyl sulfate, ethanol, hypertonic saline, sodium morrhuate. Most complications are the result of extravasation of the sclerosant and allergic reactions to few sclerosants.  Ultrasound guidance is used, where the clinician can assess the extent of sclerosis as well as decreasing the risk of perivascular or unintentional intra-arterial injections.
- Absorbable material: Gelfoam, oxygel
- Non-absorbable materials:
- Particulate agents: Silastic spheres, steel pellets,
- Injectable fluids: Microfibrillar collagen, silicone rubber
- Non-particulate agents: platinum coils, detachable balloons.
Sclerotherapy is especially useful where:
Lasers are advantageous in selective photothermolysis rather than nonselective tissue destruction. The following have been used effectively: Pulsed dye laser, neodymium-doped yttrium aluminum garnet laser, argon laser,  and carbon dioxide laser. 
- Excision is not possible-too large lesion/danger of destruction of important tissue
- Patient does not want surgery
- There is a need to de-bulk the tumor before surgery/cosmesis.
Cryosurgery few studies have been done using this therapy but it has disadvantages like scarring when used on cutaneous lesions. However, it is also been reported causing minimal scar contracture, good hemostasis and little discomfort to the patient. 
Surgery is done using mucocutaneous flap reflection and debulking the lesion. Pre-auricular approaches have been used superficial skin flaps, resection in combination with superficial parotidectomy, or intraoral excision. 
Complications of vascular malformations include hemorrhage, infection, functional problems (airway, vision and hearing) and ulceration, recurrence is a common complication.
This case is of interest because it illustrates the management of a rare and potentially fatal lesion of head and neck region. It must be borne in mind, however that some vascular lesions in childhood are known to regress spontaneously whereas some does not involute rather they become more aggressive because of their unpredictable behaviors these malformations have to be treated and conservatively managed.
| Acknowledgments|| |
We are very thankful to Dr. Ahmed R. Mujib, Professor and Head, Department of Oral Pathology, Dr. Sujatha G.P and Dr. Shivaprasad S., Professors, Department of Oral Medicine and Radiology, Bapuji Dental College and Hospital, Davangere, Karnataka, for their valuable support and guidance.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10], [Figure 11]