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

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  • 1 Department of Neurosurgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul;
  • 2 Department of Neurosurgery, Kangbuk Samsung Medical Center Hospital, Seoul; and
  • 3 Department of Neurosurgery, Seoul National University Bundang Hospital, Seongnam, Republic of Korea
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OBJECTIVE

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.

METHODS

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.

RESULTS

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.

CONCLUSIONS

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.

ABBREVIATIONS EDAMS = encephaloduroarteriomyosynangiosis; EDAS = encephaloduroarteriosynangiosis; GEE = generalized estimating equation; ICA = internal carotid artery; MMD = moyamoya disease; mRS = modified Rankin Scale; PCA = posterior cerebral artery; rCVR = regional cerebrovascular reserve; ROC = receiver operating characteristic; TFCA = transfemoral catheter angiography.

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Contributor Notes

Correspondence Seungjoo Lee: Asan Medical Center, University of Ulsan College of Medicine, Seoul, Republic of Korea. changhill@gmail.com.

INCLUDE WHEN CITING Published online May 8, 2020; DOI: 10.3171/2020.2.JNS193221.

M.K. and W.P. contributed equally to this work, and J.S.A. and S.L. contributed equally to this work.

Disclosures The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

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