Arteriovenous fistula in the head and neck - a systematic review and meta-analysis of clinical presentation.

[OBJECTIVE] To identify clinical characteristics, anatomical distribution and risk factors of arteriovenous fistula in the head and neck area (hAVF).

[METHODS] A systematic review and meta-analysis on individual participant data of available literature from inception to September 2024 on extracranial and extradural hAVF was performed.

[RESULTS] The systematic search resulted in an inclusion of 869 cases with a median age of 35 years and 58.5% being male. Overall 36.5% patients with a primary hAVF and 63.5% with a secondary hAVF were observed. The most common symptom at presentation is objective sound (thrill, bruit or murmur) in 59.8% patients. The vertebral artery (32.8%), superficial temporal artery (20.4%) and brachiocephalic or subclavian artery (10.1%) were the most common affected afferent vessels in the hAVFs. The internal (31.0%) and external (5.2%) jugular and brachiocephalic or subclavian vein (9.6%) are the most common affected efferent vessels. In the secondary group 43.1% developed the hAVF after invasive treatment of which 34.0% after insertion of a central venous catheter in the internal jugular vein, 8.4% after Implantable Cardioverter Defibrillator (ICD) or pacemaker lead removal, 4.2% after a hemodialysis catheter and 7.1% after hair transplantation surgery.

[CONCLUSIONS] hAVFs are rare vascular malformations that can be differentiated into primary (congenital or spontaneous) and secondary (traumatic or iatrogenic) lesions. Patients often present with an objective sound (thrill, bruit or murmur) and symptoms that can be related to specific vessels. Secondary hAVF is almost twice as frequent compared to primary hAVF. There is a sex predilection of secondary hAVF towards males presenting more often with a traumatic hAVF. Iatrogenic risk factors such as intravenous catheter placement, hemodialysis catheter and pacemaker lead removal and hair transplantation surgery may contribute to the development of a secondary hAVF.