Periodontal manifestations and management of a patient with AV malformation.

Arterio-venous malformation (AVM) is an abnormal communication between an artery and a vein. The incidence of its occurrence in oral and maxillofacial region is rare, and if present, the most common sign is gingival bleeding. A 12-year-old female patient presented with an extra oral swelling in relation with upper lip. Intra oral examination showed non tender gingival swelling with spontaneous bleeding associated with maxillary arch. On initiation of phase I therapy using hand instruments, spontaneous brisk bleeding was encountered which was difficult to control. Because of severe nature of hemorrhage encountered, some type of vascular abnormality was suspected. Ultrasonography followed by angiography confirmed AVM in relation with upper lip. Embolization of lesion was followed by gingivectomy procedure and no recurrence was reported during one year of follow-up. Thus, proper recognition and therapeutic intervention is essential to avoid serious complications and potentially tragic outcome in such situations.

INTRODUCTION

A malformation is a primary anatomical defect resulting from abnormal development of an organ or a tissue. It may be isolated, occurring in an otherwise normal child, or multiple, affecting several body systems. Vascular malformations are the most common soft tissue tumors in children and young adults. Its prevalence in maxillofacial region is rare, but when present, it is associated with severe hemorrhage resulting in significant morbidity and mortality.

Arterio-venous malformation (AVM) is an abnormal communication between an artery and a vein, which can be congenital or acquired. AVMs can have a spectrum of effects on the patient ranging from disfigurement to life-threatening morbidity; therefore, it is considered a difficult clinical problem. Very little is understood about the underlying pathogenesis of these lesions, and thereby, interventions frequently involve considerable risk with suboptimal outcomes.[1] A review of fatal outcomes due to unawareness of presence AVM in dental field has shown most exsanguination results due to dental extractions, tooth eruption, or even incision. One of the most common signs of these patients, especially in the mixed dentition period, is hyper mobility of the teeth with spontaneous hemorrhage from the surrounding gingival sulcus. Abnormal bluish areas around mobile teeth can be an indication of such a lesion.[2] Bleeding is the most common sign that a vascular malformations manifests within the maxillofacial region;[3] therefore, vascular malformations may be considered in differential diagnosis of gingival bleeding with inconclusive dental radiographs.[4]

Classification

Vascular malformation can be classified as[5]

Low flow

Capillary

‘Salmon’ patch

’Port-wine’ stain

Naevus anaemicus and naevus oligaemicus

Mixed vascular malformations

Reticulate vascular naevus

Klippel–Trenaunay syndrome

Venous malformations

Blue rubber bleb naevus syndrome

Maffucci's syndrome

Zosteriform venous malformations

Gorham's disease

Other multiple vascular malformation syndromes

Lymphatic malformations

Microcystic

Macrocystic

Rapid flow (arteriovenous malformations)

Aetiology

Lisa M. Buckmiller[4] has reported that 60% of vascular malformations occur in the head and neck.

Most lesions in this region occur in the scalp and skin, and actual involvement of the mandible or maxilla is very rare. In particular, the prevalence of lesions in the maxilla is half as likely as in the mandible.

Congenital malformations are extremely rare and may be caused by environmental factors e.g.- ionizing radiation; or genetic factors e.g.- chromosomal syndromes, or combination of two. The acquired form is associated with trauma leading to broken blood vessels. On healing, an artery communicates with a vein and the normal blood flow is inverted. As a result of increase in blood pressure in the affected area, the vessels dilates, appearing to be a cavernous hemangioma or an aneurysm.[6]

CASE REPORT

A 12-year-old female patient reported to the Department of Periodontics, People's College of Dental Science and Research Centre, Bhopal, with a history of swelling in the upper lip and gums since last six years. Patient gave history of trauma during playing six years back, which resulted in laceration of upper lip, for which a local doctor was consulted. This was followed by appearance of swelling in the upper lip which increased gradually in size and was constant since the last two years. Patient also gave a history of swelling and spontaneous bleeding from the gums, because of which she refrained herself from oral hygiene maintenance.

Intra oral examination showed generalized gingival hyperplasia with maxillary arch with a typical bluish purple gingiva at the interdental areas of 11 and 21; associated with grade I mobility Also, localized gingival enlargement was present in relation with mandibular incisors and canine [Figures 1–3]. Generalized bleeding was observed at slightest provocation of gingiva. No abnormalities were detected on general systemic examination, and hematological investigations which included a coagulation profile. Orthopantomogram was also inconclusive [Figure 4].

Figure 1

Pre operative frontal view

Figure 3

Pre operative left lateral view

Figure 4

OPG

Pre operative right lateral view

Periodontal treatment which was initiated with phase I therapy using hand instruments resulted in spontaneous brisk bleeding which was difficult to control. The bleeding site was suctioned and severe hemorrhage was controlled by digital pressure which took no less than 20 to 25 minutes. Because of the severe nature of hemorrhage encountered, some type of vascular abnormality was suspected and the patient was referred to physician for opinion.

Diagnosis

Ultra sonography of the upper lip was performed which showed marked thickening of upper lip with multiple dilated vascular channels in the submucosal layer of upper lip. Dilated and enlarged arteries were seen with marked increase in systolic and diastolic flow predominantly on left side of midline. The findings were suggestive of post traumatic AVM [Figure 5].

Figure 5

Ultrasonography

Angiography is the corner stone of diagnosis of vascular lesions which helps in visualizing exact angioarchitecture of the lesion essential for treatment planning. A transfemoral angiography was performed which confirmed the diagnosis of a small high flow AV malformation measuring (approx. 16×9×12 mm) situated in the middle of upper lip [Figure 6]. The arterial feeders were from right internal maxillary artery and venous drainage via bilateral ophthalmic vein into bilateral cavernous sinuses and partly via bilateral facial vein into bilateral jugular vein.

Figure 6

Angiography report

The patient underwent a surgery in a private clinic where the mass associated with upper lip was excised using circumferential excision and the defect was closed. The patient did not turn up for further recall and reported back after two years for periodontal treatment. Intraoral examination revealed gingival enlargement was still present, and also a recurrence of the malformation of lip was noted as the central nidus of the lesion present in the midline of the upper lip was not removed. After consultation with the physician and oral surgery department, it was decided to undertake periodontal treatment after embolization of the lesion.

Periodontal management

Local anesthesia was administered to the patient and the osseous topography of the bone was determined using bone sounding. Gingivectomy and gingivoplasty were performed at the same time, so as to achieve the normal topography of gingiva. Bleeding points were marked using pocket marker so as to outline the course of incision. The incision was started using no. 15 scalpel blade from the distal most tooth apical to the bleeding points marking the course of the pocket and directed coronally to a point between the base of the pocket and crest of bone. The incision was beveled at 45 degree to the tooth surface, so as to recreate normal festooned patter of gingiva. The incised tissue was removed and the area was degranulated using gracey curettes. Taking into consideration the bleeding encountered and the extent of gingival enlargement, the surgery was performed sextant wise, the bleeding during the surgery was relatively less as compared to initial phase due to embolization of the lesion. The intrasurgical bleeding was controlled using pressure pack and postoperative bleeding was controlled by cold compression.

Clinical outcome

Post embolization of the vascular lesion present with the upper lip and following gingivectomy procedure, the patient was followed-up for a period of one year and no recurrence was noted [Figures 7–9]. The final aesthetic result was acceptable by the patient.

Figure 7

Post operative frontal view after one year

Figure 9

Post operative left lateral view after one year

Post operative right lateral view after one year

DISCUSSION

Vascular anomalies have traditionally posed diagnostic and therapeutic dilemma for the surgical fraternity. Emphasis on a comprehensive classification is still a priority in literature, and yet there remains a great deal of misinformation. A well accepted classification system was published by Mulliken, Glowacki and Finn et al., in 1982[7] based on the endothelial characteristic of these lesions.

AVM, first described by Hunter in 1757, are abnormal communications between an artery and a vein, which bypass the capillary bed. AVMs are a group of conditions that can have a spectrum of effects on the patient ranging from disfigurement to life-threatening morbidity. These are high-flow lesions of unclear aetiology, but tend to behave aggressively with unpredictable growth and tissue destruction. These malformations also seem to have a female predilection (1.5:1).[8]

Arterio-venous lesions can have a traumatic or a congenital origin. In various studies published, a traumatic origin was found in approximately 90% of cases.[910] Although AVM of the oral and maxillofacial region is a rare entity, its knowledge is imperative for the periodontist, oral surgeons, and radiologists in dental fraternity. The most common complaint by the patient is chronic intermittent bleeding, which may often occur resulting from minimal oral trauma such as eruption of a tooth or brushing. Other relevant clinical signs could be facial color and/or temperature asymmetry, audible bruit, palpable thrill, discolored mucosa, and persistent or recurrent oral infection may also be present. The symptoms can be local or general, so a periodontist can play an important role in the diagnosis of the vascular lesions, which otherwise may go undiagnosed.

Since treatment and prognosis are different for each type of anomaly, it is extremely important that these lesions be diagnosed correctly. Angiographic and ultrasound tests are widely used to provide the diagnosis and the exact location of the fistula. Angiography is currently the gold standard for the determination of location and flow characteristics of vascular lesions.