Clinical and prognostic features of venous hypertensive myelopathy from craniocervical arteriovenous fistulas: a retrospective cohort study

Abstract

OBJECTIVE Current knowledge about venous hypertensive myelopathy (VHM) is incomplete. This study was performed with the aim of clarifying the clinical features and outcomes of craniocervical VHM. METHODS This retrospective, single-center cohort study included 65 patients with craniocervical junction arteriovenous fistulas resulting in VHM treated in Xuanwu Hospital from January 1, 2002, to December 30, 2020. All patients underwent microsurgery or endovascular treatment. The primary outcome was neurological function assessment using the Japanese Orthopaedic Association (JOA) scale, modified Aminoff-Logue Scale (mALS), and Venous Hypertensive Myelopathy Scale (VHMS). The secondary outcomes were recurrences and postoperative adverse events. Pearson linear regression and receiver operating characteristic curves were used to evaluate the relationships among the three scales. Kaplan-Meier and multivariate logistic regression analyses were performed to predict outcomes. RESULTS The mean patient age was 57.4 ± 11.4 years, and 88% of patients were male. The 1-year follow-up rate was 83.1%, and the 5-year follow-up rate was 50.8%. The VHMS was correlated with the JOA (R2 = 0.6722) and mALS (R2 = 0.7399) and increased the assessment accuracy by approximately 20% when compared with the other two scales. Overall, 25.9% of patients experienced delayed neurological decline beyond the 1-year follow-up. Further logistic regression suggested that age > 65 years was an independent predictor (OR 7.831, 95% CI 1.090–56.266; p = 0.041). Embolic recanalization and new bilateral symmetry feeders were the major reasons for recurrence. Recurrence increased the risk of adverse events after the second surgery (OR 20.455, 95% CI 1.170–357.320; p = 0.039). CONCLUSIONS CCJ AVFs resulting in VHM are a rare but deadly complication, and providers should be cautious of age-related delayed neurological decline and strive for a one-time anatomical cure.

Treatment of high cervical arteriovenous fistulas in the craniocervical junction region

“…For high cervical CCJ AVFs, complications were always accompanied by open surgery or EVT, including ischemic and hemorrhagic complications, hydrocephalus, and cerebrospinal fluid leakage ( 4 , 7 , 37 , 92 ). In open surgery, the rate of complications is approximately 20%, especially in patients with massive hemorrhage or complex CCJ AVFs ( 4 , 7 , 8 , 13 , 93 ). When performing open surgery, a complete understanding of the angioarchitecture of CCJ AVFs was required to avoid injury to normal nerves and vessels.…”

“…CCJ AVFs are uniquely complex due to their inherent angioarchitecture (4)(5)(6). CCJ AVFs may present with intracranial hemorrhage due to AVF rupture or edema of the brainstem and upper cervical cord due to venous hypertension (7,8). For these CCJ AVFs, prompt treatment is needed, mainly including open surgery or endovascular treatment (EVT) (3).…”

“…Therapeutic outcomes of high cervical CCJ AVFs. CCJ AVFs(4,7,8,13,93). When performing open surgery, a complete understanding of the angioarchitecture of CCJ AVFs was required to avoid injury to normal nerves and vessels.…”

Dural arteriovenous fistulas in the falx cerebri: case series and literature review

“…1,2 DAVFs are commonly located in the cavernous sinus, sigmoid/transverse sinus, superior sagittal sinus (SSS), anterior cranial fossa, tentorium and cranial cervical junction. [1][2][3][4] However, they are extremely rare in the falx cerebri/falcine sinus, and the majority of cases are treated surgically. [5][6][7][8][9][10][11][12][13][14][15][16][17] Here, we present the largest single-center case series treated by endovascular intervention and review the relevant literature.…”