Arteriovenous malformation of the maxillary sinus and mandible: a case report.

Abstract

Vascular tumors are rarely encountered in the paranasal sinuses. When they do arise there, they can be life-threatening because of the risk of bleeding. Embolization alone and in combination with surgical resection has been the mainstay of treatment. We report the case of a prepubertal girl who experienced two recurrences of arteriovenous malformation, one in the maxillary sinus and the other in the mandible. To our knowledge, this is the first report in the literature of recurrences arising in these two locations.

Introduction

Hemangiomas are one of the most frequently encountered soft-tissue tumors. (1) They usually originate in the capillaries, but they sometimes arise in the venous vessels, as well. These tumors can be found in the skin, mucosa, bones, and viscera. In the sinonasal tract, the most common hemangiomatous lesions are capillary hemangiomas; angiomas and cavernous hemangiomas are extremely rare.

Arteriovenous malformations are vascular defects caused by the absence of normal capillary beds, which leads to the development of abnormal blood channels that connect the arterial circulation to the venous circulation. Although vascular tumors in the head and neck are relatively common, particularly in the jaw, arteriovenous malformations are rare. (2) These defects are usually benign, but in some cases they can be life-threatening because of their potential for intractable bleeding. Arteriovenous malformations are both congenital and acquired. Cases of acquired malformations are usually associated with a history of surgery or blunt trauma. (1) In either case, an alternate blood pathway bypasses the normal high capillary resistance region and supplies it with an excessive amount of blood; the incidence of hemorrhage is high. (3,4) The most common symptom of this condition is the presence of a slowly growing mass in the maxilla or mandible following bleeding from the involved site.

In this article, we describe a case of arteriovenous malformation in the maxillary sinus and mandible. We also discuss the important features of this condition and its treatment.

Case report

A 9-year-old girl was referred to our institution for evaluation of recurrent episodes of hemorrhage from her nose and mouth. Her right cheek was swollen and her right second maxillary molar was loose. The history revealed that these symptoms had begun 4 years earlier. She had also been diagnosed with a vascular tumor in the maxillary sinus 1 year earlier, and this had been treated by selective embolization (figure 1).

[FIGURE 1 OMITTED]

Computed tomography (CT) detected a nonhomogeneous soft-tissue mass with indistinct borders (figure 2). Angiography identified the nature of the tumor as a vascular mass that occupied the maxillary sinus. A blush was observed in the mandibular region.

[FIGURE 2 OMITTED]

Selective entholization of the feeding vessels (i.e., the internal maxillary artery, the transverse facial artery, and the lingual and facial arteries) via a femoral approach was performed with particles of different sizes. The malformation was fistulous, and venous pockets were detected in the bony tissue. Following embolization, the patient was taken to the operating room. With a gingivobuccal incision, a buccal flap was elevated and the anterior wall of the maxillary sinus was exposed. An antral window was made superior to the dental apices, and a lesion with indistinct borders was noted to be filling the maxillary sinus. The lesion was removed by curettage, and excessive bleeding was controlled with Surgicell and gauze packing. As the mass was being removed, the bleeding slowed. Following removal, the sinus was packed with absorbable sponges. Histopathologic examination of the mass revealed that it contained normal mucosa and numerous irregular arterial and venous vascular channels. We established a diagnosis of arteriovenous malformation.

The patient was discharged from the hospital after 3 days. She did well postoperatively for 4 months, but then she again began to experience bleeding from her month. Physical examination revealed active bleeding from the gingiva over the right mandibular molar. Angiography detected a mass in the mandible and a great deal of vascular feeding (figure 3). The maxillary sinuses were clear. The lesion was embolized via a femoral artery approach, and the patient again underwent surgery. The vascular lesion in the mandible was extracted transorally, and the bony defect was closed with Medpore porous polyethylene (figure 4). On continuing follow-up, the patient is free of disease and remains well.

[FIGURE 3-4 OMITTED]

Discussion

Vascular tumors occur in the nasal cavity and nasopharynx more often than they do in the paranasal sinuses. (5) Most of these tumors are capillary hemangiomas of osteogenic origin. (6) Arteriovenous malformations are not common in the maxillofacial region, but when they do occur in the maxilla or mandible, massive spontaneous bleeding or iatrogenic bleeding during surgical interventions such as tooth extraction can occur. (7) A slowly growing, expansile mass in the maxilla or mandible accompanied by spontaneous gingival bleeding near the affected site are the most common symptoms of arterioveuons malformation. Paresthesia, mobility of the involved teeth, gingival discoloration, hyperthermia over the lesion, a feeling of pulsation, and pain might also be evident. Bruits or pulsations can be detected in large hemangiomas. On the other hand, some of these tumors have no signs or symptoms. (8)

Our patient's primary symptom was spontaneous bleeding from the right second maxillary molar. Her physical examination revealed a slight discoloration of the maxillary gingiva and mobility of the right second maxillary molar. She had no history of trauma. These findings were consistent with the data in the literature. After her first operation, another vascular tumor was detected in the mandible, and this mass was successfully extracted after embolization. We could not find any other report in the literature of involvement of the maxillary sinus and mandible at approximately the same time.

Darlow et al wrote of the diagnostic importance differentiating between a vascular lesion and a highly vascular mucosa caused by an inflammatory process They recommended routine aspiration of all expansile lesions in the maxillofacial region. Direct radiography can demonstrate multilocular radiolucencies in the involved bone. Furthermore, CT can detect a highly vascular mass with multiple lobulations. Angiography is the most useful imaging modality; in fact, it is essential to confirm the diagnosis as well as to evaluate the vascular architecture. (9) In our patient, CT demonstrated that multilobular mass with indistinct borders was present in the maxillary sinus. The tumor had invaded the palatine portion of the maxilla. Massive hemorrhage from the gingival mucosa led us to the preoperative differential diagnosis of a vascular tumor. We performed angiography, which demonstrated the high degree of vascularity in the tumor.

Treating arteriovenous malformations is difficult. The primary treatment goals are to stop bleeding and to prevent its recurrence. (10) The treatment itself involves surgical excision or complete obliteration by embolization. (11) Complete resection of an arteriovenons malformation following embolization is the safest treatment, but resection can cause functional and cosmetic defects, especially in the mandibular region. (7,10) Surgical curettage following preoperative embolization or ligature has been tried; although this method is more conservative, the risk recurrence is high. (10,12) Selective embolization with particles or glue and direct transosseous puncture alone have also been reported. After the invention of steel coils for intravascular embolization in the 1970s, coil embolization was successfully used to produce permanent occlusion to arteriovenous malformations. (11) We first tried to treat the vascular lesion in our patient with embolization with particles alone, but because recurrence was detected both clinically and radiologically, we ultimately operated and completely removed the lesion from the maxillary sinus cavity. When the patient's bleeding recurred 4 months later, radiologic and angiographic investigations demonstrated a mass in the mandible. After selective erabolization, we operated again and the mass was removed by curettage.

Because recurrence following embolization for arteriovenous malformations is frequent, follow-up angiography and radiography are important. In our patient, the blush in the mandibular region was detected during the embolization prior to the first operation, but because there was no active filling, embolization and curettage were not performed. But the embolized branches revascularized quickly, and the second lesion became apparent within 4 months.

In a study of 85 paranasal vascular tumors, Fu and Perzin did not detect any case of arteriovenous malformation in the maxillary region. (5) In fact, the only such case in the literature was reported by Darlow et al in 1988. (3) Despite the infrequency of these tumors, it is important to take them into consideration in the differential diagnosis of maxillary or mandibular masses.

References

(1.) Enzinger FM, Weiss SW. Soft Tissue Tumors. 3rd ed. St Louis: Mosby, 1995:581-8.

(2.) Kelly DE, Terry BC, Small EW. Arteriovenous malformation of the mandible: Report of a case. J Oral Surg 1977;35:387-93.

(3.) Darlow LD, Murphy JB, Berrios RJ, et al. Arteriovenous malformation of the maxillary sinus: An unusual clinical presentation. Oral Surg Oral Meal Oral Pathol 1988;66:21-3.

(4.) Barnes L, ed. Surgical Pathology of the Head and Neck. New York: Dekker, 1985:728-31.

(5.) Fu YS, Perzin KH, Nnnepithelial tumors of the nasal cavity, paranasal sinuses, and nasopharynx: A elinicopathologic study. IV. Smooth muscle tumors (leiomyoma, leiomyosarcoma). Cancer 1975;35:1300-8.

(6.) Most DS. Hemangioma of the maxillary sinus. Oral Surg Oral Med Oral Pathol 1985;60:485-6.

(7.) Guibert-Tranier F, Piton J, Riche MC, et al. Vascular malformations of the mandible (intraosseous haemangiomas). The importance of preoperative embolization. A study of 9 cases. Eur J Radiol 1982;2:257-72.

(8.) Beziat JL, Marcelino JP, Bascnulergue Y, Vitrey D. Central vascular malformation of the mandible: A case report. J Oral Maxillofac Surg 1997;55:415-19.

(9.) Larsen PE, Petersoa LJ. A systematic approach to management of high-flow vascular malformations of the mandible. J Oral Maxillofac Surg 1993;51:62-9.

(10.) Chiras J, Hassine D, Goudot P, et al. Treatment of arteriovenous malformations of the mandible by arterial and venous embolization. AJNR Am J Neuroradiol 1990;11:1191-4.

(11.) Kawano K, Mizuki H, Mori H, Yanagisawa S. Mandibular arteriovenous malformation treated by transvenous coil embolization: A long-term follow-up with special reference to bone regeneration. J Oral Maxillofac Surg 2001;59:326-30.

(12.) Gallagher DM, Hilley D, Epker BN. Surgical treatment of an arteriovenous malformation of the mandible in a child. A case report. J Maxillofac Surg 1983;11:279-83.

From the Department of Otorhinolaryngology Head and Neck Surgery (Dr. Cansiz, Dr. Yener, and Dr. Kalekoglu) and the Department of Radiology and Neuroradiology (Dr. Dalkilic), Istanbul University Cerrahpasa Medical Faculty, Istanbul, Turkey.

Reprint requests: Harun Cansiz. MD, Nato Yolu Caprazli Alt Sokak, 12/7, Cengelkoy, Uskudar. 81220, Istanbul, Turkey. Phone: [phone]; fax: [phone]; e-mail: [e-mail] or [e-mail]