Clinical characteristics and leptomeningeal collateral status in pediatric and adult patients with ischemic moyamoya disease

Zhi-Wen Liu; Cong Han; Hui Wang; Qian Zhang; Si-Jie Li; Xiang-Yang Bao; Zheng-Shan Zhang; Lian Duan

doi:10.1111/cns.13130

Clinical characteristics and leptomeningeal collateral status in pediatric and adult patients with ischemic moyamoya disease

CNS Neurosci Ther. 2020 Jan;26(1):14-20. doi: 10.1111/cns.13130. Epub 2019 Apr 13.

Authors

Zhi-Wen Liu¹, Cong Han¹, Hui Wang¹, Qian Zhang¹, Si-Jie Li², Xiang-Yang Bao¹, Zheng-Shan Zhang¹, Lian Duan¹

Affiliations

¹ Department of Neurosurgery, The Fifth Medical Center of Chinese PLA General Hospital, The 307th Hospital of the Chinese People's Liberation Army, Academy of Military Medical Science, Beijing, China.
² Departments of Neurology and Neurosurgery, Xuanwu Hospital, Center of Stroke, Beijing Institute for Brain Disorders, Capital Medical University, Beijing, China.

Abstract

Aim: Previous studies have found significant differences in clinical characteristics between pediatric and adult moyamoya disease (MMD) patients, but few studies have focused on the factors underlying these differences. We aimed to investigate the differences in leptomeningeal collateral (LMC) status between pediatric and adult MMD patients and to analyze the effects of LMCs on clinical characteristics and therapeutic prognosis.

Methods: We retrospectively analyzed 214 MMD patients from January 2014 to January 2016. Clinical characteristics and LMC status were compared between the pediatric and adult patients. LMC status was graded as good or poor depending on the retrograde flow from the posterior cerebral artery (PCA) on digital subtraction angiography (DSA).

Results: A total of 83 pediatric and 131 adult (1:1.6) MMD patients were analyzed. Pediatric patients were more likely to experience a transient ischemic attack (81%), whereas adult patients were more likely to experience infarction (51%). Regarding the different MMD stages (the early, medium, and advanced stages corresponded to Suzuki stages 1-2, 3-4, and 5-6, respectively), the prevalence of good LMC status was higher for pediatric patients than for adult patients in the early stage (P = 0.047) and the medium stage (P = 0.001), but there were no differences between these patient groups in the advanced stage (P = 0.547). Worse postoperative angiographic outcomes (P = 0.017) were found in adult patients than in pediatric patients in the medium stage. Poor LMC status had strong correlations with infarction (P < 0.001 and P = 0.017) and poor postoperative outcomes (P = 0.003 and P = 0.043) in both pediatric and adult patients.

Conclusions: Pediatric MMD patients have greater patency and a greater ability to establish good LMC status than adult patients, and poor LMC status has a strong correlation with severe clinical symptoms and poor postoperative outcomes. LMC status may be an important factor in the differences in clinical characteristics and prognosis between pediatric and adult MMD patients.

Keywords: adult; leptomeningeal collateral; moyamoya disease; pediatric.

Publication types

Research Support, Non-U.S. Gov't

MeSH terms

Adolescent
Adult
Angiography, Digital Subtraction
Brain Infarction / epidemiology
Brain Infarction / etiology
Brain Ischemia / etiology
Brain Ischemia / physiopathology
Child
Child, Preschool
Collateral Circulation*
Humans
Ischemic Attack, Transient / epidemiology
Ischemic Attack, Transient / etiology
Meninges / blood supply*
Meninges / diagnostic imaging
Meninges / physiopathology*
Middle Aged
Moyamoya Disease / complications
Moyamoya Disease / diagnostic imaging
Moyamoya Disease / physiopathology*
Neurosurgical Procedures
Posterior Cerebral Artery / physiopathology
Retrospective Studies
Treatment Outcome