PedsQL Score Post Encephalo-duro-arterio-myo-synangiosis Procedure for Moyamoya Disease: A Single Center Experience.

BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.