Introduction: The hemorrhage risk is essential to make treatment strategies for sporadic cerebral cavernous malformations (CCMs). This study aimed to develop and validate a nomogram model for predicting the 5-year hemorrhage risk of sporadic CCMs.
Methods: Patients with sporadic CCMs in a referring hospital from January 2008 to December 2018 were prospectively included as the training cohort. A Cox regression model was employed to investigate the factors related to the 5-year hemorrhage risk. A nomogram to predict the 5-year hemorrhage risk was formulated based on the result of a multivariable Cox regression model within the training cohort. Patients with sporadic CCMs were included from an independent cohort as the external validation cohort to validate the performance of the nomogram for hemorrhage risk. The model performance in terms of 5-year hemorrhage risk was assessed using the C-index.
Results: The training cohort included 337 patients with sporadic CCMs (158 males with a mean age of 39.1 years). Hemorrhage events occurred among 42 patients (12.7%) within a 5-year follow-up. A multivariate Cox regression analysis demonstrated their previous history of CCM hemorrhage (HR, 2.66; 95%CI, 1.4-5.06, P=0.003), CCM lesions >1.5cm (HR, 2.89; 95% CI, 1.45-5.79; P=0.003), developmental venous anomalies (HR, 2.24; 95% CI, 1.2-4.19; P=0.012), brainstem CCMs (HR, 4.04; 95% CI, 2.00-8.16; P< 0.001) and Zabramski type I (HR, 3.65; 95% CI, 1.32-10.04; P=0.012) as risk factors related to the 5-year hemorrhage of sporadic CCMs. The nomogram integrating the above factors performed well in predicting the hemorrhage risk of sporadic CCMs, with a C-index of 0.81 (95% CI, 0.75-0.87) for the training cohort and 0.87 (95% CI, 0.78-0.95) for the external validation cohort (26 males, mean age 39.2 years, with 14 hemorrhage events).
Conclusion : Previous CCM hemorrhage history, CCM lesion >1.5cm, developmental venous anomalies, brainstem CCMs and Zabramski type I were risk factors related to the 5-year hemorrhage of sporadic CCMs. This study provided a highly accurate nomogram for the 5-year hemorrhage risk of sporadic CCMs, which might help make treatment strategies.
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