Long-term prognosis of 452 moyamoya disease patients with and without revascularization under perfusion-based indications

Toshihiko Hayashi; Shoko Hara; Motoki Inaji; Yukika Arai; Juri Kiyokawa; Yoji Tanaka; Tadashi Nariai; Taketoshi Maehara

doi:10.1016/j.jstrokecerebrovasdis.2023.107389

Long-term prognosis of 452 moyamoya disease patients with and without revascularization under perfusion-based indications

J Stroke Cerebrovasc Dis. 2023 Nov;32(11):107389. doi: 10.1016/j.jstrokecerebrovasdis.2023.107389. Epub 2023 Sep 29.

Authors

Toshihiko Hayashi¹, Shoko Hara², Motoki Inaji¹, Yukika Arai¹, Juri Kiyokawa¹, Yoji Tanaka¹, Tadashi Nariai¹, Taketoshi Maehara¹

Affiliations

¹ Department of Neurosurgery, Tokyo Medical and Dental University, 1-5-45 Yushima, Bunkyo-ku, Tokyo, Japan.
² Department of Neurosurgery, Tokyo Medical and Dental University, 1-5-45 Yushima, Bunkyo-ku, Tokyo, Japan. Electronic address: shara.nsrg@tmd.ac.jp.

PMID: 37778161
DOI: 10.1016/j.jstrokecerebrovasdis.2023.107389

Abstract

Objectives: To evaluate the long-term outcomes of patients treated under our perfusion-based strategy and assess whether conservative treatment without surgical treatment under our strategy is acceptable.

Materials and methods: A total of 315 adult and 137 pediatric MMD patients (follow-up period ≥ 3 years from 2001 to 2020) were included. Follow-up events in each patient group (pediatric or adult, surgically treated or conservatively treated) were evaluated and compared to each other using a log-rank test. Risk factors for stroke and nonstroke events were also investigated using a multivariate Cox proportional hazard model.

Results: In adult-onset patients, the stroke event rates (person-year %) were not different between surgically treated patients and conservatively treated patients (2.00 % vs. 1.59 %, p = 0.558); however, conservative patients showed a higher stroke rate than surgically treated hemispheres (0.34 %; p = 0.025) and hemorrhagic stroke was the major type (18/26, 69.2 %). Hemorrhagic onset was associated with increased risk of stroke in adults (hazard ratio (95 % confidence interval) = 2.43 (1.10-5.36)). In pediatric-onset patients, no conservatively treated patients experienced stroke; however, nonstroke events occurred more frequently than in surgically treated hemispheres (4.86 % vs. 1.71 %, p = 0.020 for transient ischemic attack; and 7.91 % vs. 1.31 %, p < 0.001 for asymptomatic progression on magnetic resonance angiography).

Conclusions: In adult patients, conservatively treated patients experienced stroke more frequently, especially hemorrhagic stroke. An additive strategy to prevent stroke in hemorrhagic-onset patients without hemodynamic disturbance seems to be needed. Pediatric patients with mild hemodynamic disturbance can be safely observed without initial surgical intervention, but close follow-up for disease progression is necessary.

Keywords: Adult; Cerebrovascular disorders: Long-term prognosis; Moyamoya disease; Pediatric; Perfusion imaging; Stroke.

MeSH terms

Adult
Cerebral Revascularization* / adverse effects
Child
Follow-Up Studies
Hemorrhagic Stroke* / complications
Humans
Moyamoya Disease* / complications
Moyamoya Disease* / diagnostic imaging
Moyamoya Disease* / surgery
Perfusion / adverse effects
Prognosis
Retrospective Studies
Stroke* / complications
Stroke* / etiology
Treatment Outcome

Supplementary concepts

Moyamoya disease 1