Dural Arteriovenous Fistulas at the Craniocervical Junction: A Series Case Report

Craniocervical junction dural arteriovenous fistulas (CJDAVFs) are rare vascular malformations with unclear clinical characteristics. This study investigated the clinical characteristics and outcomes of patients with CJDAVFs.

Thirty-eight patients with CJDAVFs who had undergone either conservative or surgical treatment were retrospectively analyzed.

Eleven (28.9%) patients were women and 27 (71.1%) were men (median age, 52.5 years). Two (5.3%) had myelopathy, and 36 (94.7%) had subarachnoid hemorrhage (SAH). Three patients had SAH recurrence before treatment. Five patients (13.9%) with SAH initially had negative results on angiography, which may have been due to a low-flow fistula without varicose veins (P = 0.034) and acute hydrocephalus (P = 0.084). Coincidental vascular lesions were noted in 5 patients (13.2%). Caudal drainage was mainly found in patients with myelopathy, whereas superolateral drainage was frequently observed in patients with SAH (P = 0.021). Thirty-six (94.7%) patients underwent microsurgery; of these, 33 (91.7%) had favorable outcomes and 3 (8.3%) had unfavorable outcomes. The main neurosurgical complications included acute hydrocephalus in 4 (10.5%) and new-onset mild persistent myelopathy in 6 (15.7%). According to the univariate analysis, the presence of myelopathy predicted poor outcomes, whereas SAH predicted favorable outcomes (P = 0.004). However, the multivariate analysis did not show statistical significance.

SAH is a common presenting sign of CJDAVF that may be overlooked on initial cerebral angiography, especially in patients with acute hydrocephalus and a low-flow fistula without varicose veins. Microsurgery involving disconnecting the draining vein is effective and beneficial. Further studies should be performed to investigate predictive factors influencing the prognosis.