Arteriovenous Malformations of the Head and Neck: Natural History and Management

Abstract

This is a retrospective review of 81 patients with extracranial arteriovenous malformation of the head and neck who presented to the Vascular Anomalies Program in Boston over the last 20 years. This study focused on the natural history and effectiveness of treatment. The male to female ratio was 1:1.5. Arteriovenous malformations occur in anatomic patterns. Sixty-nine percent occurred in the midface, 14 percent in the upper third of the face, and 17 percent in the lower third. The most common sites were cheek (31 percent), ear (16 percent), nose (11 percent), and forehead (10 percent). A vascular anomaly was apparent at birth in 59 percent of patients (82 percent in men, 44 percent in women). Ten percent of patients noted onset in childhood, 10 percent in adolescence, and 21 percent in adulthood. Eight patients first noted the malformation at puberty, and six others experienced exacerbation during puberty. Fifteen women noted appearance or expansion of the malformation during pregnancy. Bony involvement occurred in 22 patients, most commonly in the maxilla and mandible. In seven patients, the bone was the primary site; in 15 other patients, the bone was involved secondarily. Arteriovenous malformations were categorized according to Schobinger clinical staging: 27 percent in stage I (quiescence), 38 percent in stage II (expansion), and 38 percent in stage III (destruction). There was a single patient with stage IV malformation (decompensation). Stage I lesions remained stable for long periods. Expansion (stage II) was usually followed by pain, bleeding, and ulceration (stage III). Once present, these symptoms and signs inevitably progressed until the malformation was resected. Resection margins were best determined intraoperatively by the bleeding pattern of the incised tissue and by Doppler. Subtotal excision or proximal ligation frequently resulted in rapid progression of the arteriovenous malformation. The overall cure rate was 60 percent, defined as radiographic absence of arteriovenous malformation. Cure rate for small malformations was 69 percent with excision only and 62 percent for extensive malformations with combined embolization-resection. The cure rate was 75 percent for stage I, 67 percent for stage II, and 48 percent for stage III malformations. Outcome was not affected significantly by age at treatment, sex, Schobinger stage, or treatment method. Mean follow-up was 4.6 years.

Somatic MAP2K1 Mutations Are Associated with Extracranial Arteriovenous Malformation

“…15 Solitary, extracranial AVM is not heritable, has similar incidence in males and females, and exhibits variable severity. These features are typical of disorders caused by a somatic mutation 16 2,17 We cannot exclude the possibility that somatic MAP2K1 mutations arise as a consequence of AVM expansion, rather than being the cause of AVM. Arguing against the former interpretation is the presence of mutations in stage I lesions ( Figure 1 and Table 2) and the absence of mutations in other enlarging vascular malformations.…”

Results of the Treatment Vascular Anomalies with the 5 ALA Induced Photodynamic Therapy

“…The success of PDT is dependent on upon the depth of necrosis being greater than the depth of the individual malformation. Thus, interstitial PDT represents is ideal, in that it allows more effective treatment of the target tissue volume [17,20]. This clinical study highlights the advantage of ultrasound, in combination with PDT.…”

“…The side effects of chemotherapy are well known and radiotherapy carries the risk of inducing new tumour. Propranolol, a known non-selective beta-blocker, has been reported to induce regression in haemangiomas in newborns [17,18]. Sclerotherapy is the most common intervention in many centers around the world.…”

Arteriovenous malformation after punch biopsy clinically mimicking a basal cell carcinoma: Case report and review of literature

“…In stage 3, destruction of the lesion or the underlying structures occurs. The final stage 4 is associated with cardiac decompensation due to high output cardiac failure 1, 2, 5, 6, 7. Traumatic AVMs are uncommon and occur in the setting of penetrating, blunt or postsurgical trauma.…”

“…AVM's and BCC's manifests with clinical overlap but can be distinguished by dermoscopy and histological assessment. An early accurate diagnosis of AVM's is important since patients receiving surgical intervention sooner will have a better outcome 5, 14…”