Krylova Marharyta, Hauck Erik F
Department of Neurosurgery, Duke University, Durham, North Carolina.
J Neurosurg Case Lessons. 2025 Jun 9;9(23). doi: 10.3171/CASE24857.
Intraorbital arteriovenous fistulas (ioAVFs) represent a rare but distinct entity of arteriovenous shunts, separate from the more common carotid-cavernous fistulas (CCFs). There is currently no consensus on the optimal treatment approach for ioAVFs, necessitating analysis of available cases to guide clinical decision-making and establish effective management strategies.
A systematic literature review revealed a total of 28 ioAVF cases reported since 1978. An additional illustrative case is included. Intraorbital AVFs present with similar symptoms as the CCFs such as skin changes (chemosis, ecchymosis, erythema; 86.2%), proptosis (72.4%), and headaches (34.5%). Visual impairment was observed in 12 cases (41.4%); ophthalmoplegia occurred in 8 cases (27.6%). Five patients (17.2%) presented with an afferent pupillary defect. All patients underwent catheter angiography for their diagnostic workup. The ophthalmic artery (OA) was a direct feeder to the fistula in almost all patients (96.6%). Additional feeding vessels included the facial artery, sphenopalatine artery, middle meningeal artery, internal maxillary artery, and superficial temporal artery. Venous drainage was mainly via the superior ophthalmic vein (SOV). Other draining veins involved the inferior ophthalmic vein, facial vein, superior temporal vein, basal vein of Rosenthal, and superior sagittal sinus. Spontaneous resolution occurred in 5 cases (< 20%). Treatment was reported in 21 cases and included embolization (66.7%), resection (14.3%), or combined techniques (19.0%). Embolization was performed most often transvenously (14 cases). Three cases were embolized via direct puncture. A transarterial approach was selected in 6 cases. The OA was embolized directly (distal to the central retinal artery) in 2 cases, including the illustrative case. The average follow-up was 7.8 months (range 3 days-3 years). Overall, treatment was successful with a high cure rate (85.7%). Complications included extensive hemorrhage during transvenous embolization in 1 case and profound thrombosis of the SOV in 2 cases.
Intraorbital AVFs are characterized by direct inflow via the OA. Treatment can be challenging. A multimodal approach, including observation, can result in high cure rates. Transvenous embolization is the most commonly performed intervention. Embolization via the OA is an option, but carries the risk of permanent visual deficit. https://thejns.org/doi/10.3171/CASE24857.
眶内动静脉瘘（ioAVF）是一种罕见但独特的动静脉分流病变，与更为常见的颈动脉海绵窦瘘（CCF）不同。目前对于ioAVF的最佳治疗方法尚无共识，因此有必要分析现有病例以指导临床决策并制定有效的管理策略。
一项系统的文献综述显示，自1978年以来共报告了28例ioAVF病例。本文还纳入了一个典型病例。眶内动静脉瘘的症状与颈动脉海绵窦瘘相似，如皮肤改变（结膜水肿、瘀斑、红斑；86.2%）、眼球突出（72.4%）和头痛（34.5%）。12例（41.4%）出现视力障碍；8例（27.6%）发生眼肌麻痹。5例患者（17.2%）出现传入性瞳孔障碍。所有患者均接受了导管血管造影以进行诊断检查。几乎所有患者（96.6%）的眼动脉（OA）都是瘘的直接供血动脉。其他供血血管包括面动脉、蝶腭动脉、脑膜中动脉、上颌内动脉和颞浅动脉。静脉引流主要通过眼上静脉（SOV）。其他引流静脉包括眼下静脉、面静脉、颞上静脉（Rosenthal基底静脉）和上矢状窦。5例（<20%）出现自发缓解。21例报告了治疗情况，包括栓塞（66.7%）、切除术（14.3%）或联合技术（19.0%）。栓塞最常通过静脉进行（14例）。3例通过直接穿刺进行栓塞。6例选择经动脉途径。2例直接栓塞眼动脉（在视网膜中央动脉远端），包括典型病例。平均随访时间为7.8个月（范围3天至3年）。总体而言，治疗成功率高，治愈率为85.7%。并发症包括1例经静脉栓塞期间的广泛出血和2例眼上静脉的严重血栓形成。
眶内动静脉瘘的特点是通过眼动脉直接流入。治疗可能具有挑战性。包括观察在内的多模式方法可导致高治愈率。经静脉栓塞是最常进行的干预措施。通过眼动脉进行栓塞是一种选择，但有导致永久性视力缺陷的风险。https://thejns.org/doi/10.3171/CASE24857 。
