Leeds Children's Hospital, Leeds Teaching Hospitals, Leeds, United Kingdom.
Leeds Children's Hospital, Leeds Teaching Hospitals, Leeds, United Kingdom.
World Neurosurg. 2020 Dec;144:e674-e678. doi: 10.1016/j.wneu.2020.09.043. Epub 2020 Sep 12.
Moyamoya disease is a progressive, occlusive arteriopathy of the intracranial vessels causing an increased risk of stroke. It often results in functional impairment and decrease in quality of life, both in the presence and absence of stroke. Revascularization is the accepted treatment for patients with symptomatic moyamoya disease, preventing further stroke. At Leeds Children's Hospital we use the encephalo-duro-arterio-myo-synangiosis (EDAMS) technique to facilitate revascularization. We aim to assess the quality of life outcomes of patients who have undergone operative intervention at our unit for moyamoya disease.
Pediatric patients with operated moyamoya disease from Leeds Children Hospital between February 2009 and January 2019 were included. Patients awaiting primary surgery were excluded. Patients were contacted via telephone and the Pediatric Quality of Life Inventory 4.0 (PedsQL) questionnaire administered via parent proxy. Quality of life outcomes were assessed using the PedsQL questionnaire score, which measures physical, emotional social and school functioning domains. This score was then converted to a health-related quality of life score. Scores in each domain and mean scores were compared with normative data.
This study included 11 children (5 boys), median age 6.8 years (range: 22 months to 15.5 years), and 9 children underwent bilateral operations. Mean parent proxy PedsQL score was 66 (range: 12.5-98.4), with a mean score of 61.9 in physical function, 88.9 in emotional function, 70.9 in social function, and 58.7 in school function. This was lower than healthy controls overall and in each individual domain except emotional function, which was similar to normative data.
Children with moyamoya disease have a lower quality of life than healthy controls within this series. This suggests that children with moyamoya should be offered additional psychosocial support within the community.
烟雾病是一种颅内血管进行性闭塞性疾病，会增加中风的风险。它经常导致功能障碍和生活质量下降，无论是在存在还是不存在中风的情况下。血管重建术是治疗有症状烟雾病患者的公认方法，可以预防进一步的中风。在利兹儿童医院，我们使用脑硬膜动脉血管融通术（EDAMS）技术来促进血管重建。我们旨在评估在我们的单位接受手术干预治疗烟雾病的患者的生活质量结果。
纳入 2009 年 2 月至 2019 年 1 月期间在利兹儿童医院接受手术治疗的烟雾病患儿。排除等待初次手术的患儿。通过电话联系患儿，由患儿家长作为代理人使用儿童生活质量量表 4.0（PedsQL）问卷进行调查。使用 PedsQL 问卷评分评估生活质量结果，该评分测量身体、情感、社会和学校功能领域。然后将该评分转换为健康相关生活质量评分。比较每个领域和平均评分与参考数据。
本研究纳入 11 名儿童（5 名男孩），中位年龄 6.8 岁（范围：22 个月至 15.5 岁），9 名儿童接受了双侧手术。家长代理人 PedsQL 平均评分 66（范围：12.5-98.4），身体功能平均评分为 61.9，情感功能为 88.9，社会功能为 70.9，学校功能为 58.7。这一评分均低于整体健康对照组和各个单项评分，除情感功能外，均与参考数据相似。
在本系列中，烟雾病患儿的生活质量低于健康对照组。这表明烟雾病患儿应在社区内获得额外的社会心理支持。
