Champagne bottle neck sign predicts intracranial hemorrhage in Moyamoya disease: A long-term follow-up study.

OBJECTIVE

Given the poor prognosis associated with hemorrhagic events in Moyamoya disease (MMD), the early identification of patients at high risk is of paramount importance for improving clinical outcomes. This study aims to explore the predictive value of champagne bottle neck sign (CBNS) at baseline for post-treatment intracranial hemorrhage (ICH) occurrence in adult MMD patients.

METHODS

This retrospective analysis included MMD patients without a history of ICH who were recruited from July 2014 to December 2020. All patients underwent preoperative digital subtraction angiography (DSA) and had a median follow-up of 6.5 years (1-10 years) to record ICH occurrence. The DSA features, including choroidal anastomosis and CBNS (ICA/CCA diameter ratio ≤ 0.5), were evaluated. Cox regression analysis was utilized to determine the associations between clinical and DSA characteristics and the occurrence of post-treatment ICH in MMD patients. Subsequently, receiver operating characteristic (ROC) curve analysis was conducted to calculate the area under the curve (AUC) of imaging characteristics in predicting ICH occurrence.

RESULTS

Among 74 recruited MMD patients (mean age: 42.5 ± 10.4 years; 35 males), 9 (12.2 %) experienced ICH over up to ten years. Patients who experienced ICH during follow-up had significantly smaller ICA/CCA diameter ratio (0.39 ± 0.10 vs. 0.51 ± 0.12, P = 0.004) and higher CBNS incidence (88.9 % vs. 46.2 %, P = 0.03) at baseline. Multivariate Cox regression analysis demonstrated that choroidal anastomosis (HR = 3.82, 95 % CI: 1.02-14.35, P = 0.047) and CBNS (HR = 9.46, 95 % CI: 1.17-76.47, P = 0.035) were independent predictors of ICH occurrence. ROC analysis revealed that in predicting post-treatment ICH, the AUC value of CBNS was 0.714 (95 %CI: 0.558 - 0.870) to distinguish MMD patients with hemorrhage from those without, P = 0.039). When combined CBNS with choroidal anastomosis, the AUC value reached 0.785 (95 %CI: 0.650 - 0.919).

CONCLUSIONS

The CBNS has independent predictive value for risk of post-treatment ICH in adult MMD patients. This study suggests that CBNS might be a useful imaging biomarker for adverse outcome of MMD.