Development and validation of a risk scoring model for postoperative adult moyamoya disease

Abstract

<jats:sec><jats:title>OBJECTIVE</jats:title><jats:p>The current grading system for moyamoya disease (MMD) is focused on angiographic studies with limited clinical application. The authors aimed to determine relevant factors that may impact postoperative outcome and establish a scoring system to predict the functional outcome.</jats:p></jats:sec><jats:sec><jats:title>METHODS</jats:title><jats:p>Adult patients with MMD who underwent treatment between 1998 and 2016 were included. Factors such as age, sex, comorbidity, smoking, MMD family history, initial presentation, multimodal imaging modalities, and types of surgical revascularization were thoroughly reviewed. These factors were analyzed to determine possible risk factors related to unfavorable 6-month postoperative outcomes using the modified Rankin Scale (mRS) (unfavorable: mRS score ≥ 3). A scoring system was developed using these independent risk factors to predict the outcome and validated using prospectively collected data from multiple centers between 2017 and 2018.</jats:p></jats:sec><jats:sec><jats:title>RESULTS</jats:title><jats:p>Of 302 patients for whom applications were submitted, 260 patients (321 hemispheres) met the diagnostic criteria. In multivariate analysis, hyperlipidemia, smoking, cerebral infarction on preoperative CT or MRI, and moderately to severely reduced regional cerebrovascular reserve results from Diamox SPECT were significantly related to unfavorable outcome. The authors developed a scoring system and stratified patients into risk groups according to their scores: low-risk (score 0–3), intermediate-risk (score 4–6), and high-risk (score 7–9) groups. This model demonstrated both good discrimination and calibration using C-statistics and the Hosmer-Lemeshow goodness-of-fit test showing 0.812 (95% CI 0.743–0.881) (p = 0.568) for the development and 0.954 (95% CI 0.896–1) (p = 0.097) for the temporal and external validation cohort.</jats:p></jats:sec><jats:sec><jats:title>CONCLUSIONS</jats:title><jats:p>The authors’ scoring system is readily adoptable to predict the postoperative outcome for MMD. Their data revealed the importance of smoking and hyperlipidemia, which were the only modifiable factors included in the scoring system. The authors validated their scoring system both internally and externally and maintained good performance, highlighting the system’s generalizability and reliability.</jats:p></jats:sec>

Preoperative Brain Functional Connectivity Improve Predictive Accuracy of Outcomes After Revascularization in Moyamoya Disease

“…31 Other risk factors include higher mRS scores at admission, advanced Suzuki stages, and PCA involvement. 4,6 It has been reported that steno-occlusion in PCA is a hallmark of MMD progression and is significantly associated with an increased risk of recurrent ischemic stroke. 7,32 It remains true after we extend follow-up that PCA involvement accounted for the greatest discriminant weight in model 1 but is less significant than functional connectivity in model 2.…”

“…5 We included BMI in the prediction model because smoking, hyperlipidemia, hypertension, and diabetes are prevalent comorbidities and contribute to poor outcomes in patients with MMD. 6,20,21 These comorbidities are strongly correlated with BMI. 31 Other risk factors include higher mRS scores at admission, advanced Suzuki stages, and PCA involvement.…”

“…In this study, discriminant function analysis (DFA) was used to determine whether a combination of variables could predict different outcomes (SPSS v.25, IBM). According to prior literature, [4][5][6]20,21 patients' age, body mass index (BMI), mRS at admission, Suzuki stage, and PCA involvement as well as the extracted functional connectivity were chosen to be tested. Multicollinearity analysis was conducted by calculating the variance inflation factor values of all variables, which were less than 5, verifying independent relationship among them (Supplementary Table 1, http://links.lww.com/NEU/D428).…”

Structural equation model based on salutogenesis theory for evaluating factors affecting health-related quality of life in adolescents with moyamoya disease

“…Moyamoya disease, a rare and incurable disease, is a primary cause of strokes or transient ischemic attacks in adolescents 1 . The prevalence of moyamoya disease is reported to be 3.16 per 100,000 people, and the incidence rate is reported to be 0.5 per 100,000 people per year 2 . The highest incidence rates are among people aged 5–15 and 30–49 years.…”

“…Moyamoya disease can easily cause cerebral vascular hypo circulation-related symptoms even with minor changes in blood flow. It can also cause transient cerebral ischemic attacks in stressful situations 7 ; therefore, stress management is required 2 , 8 . The health issues and symptoms experienced by adolescents with moyamoya disease are often stress-related 9 .…”