Abstract TP155: Patient Centered Outcomes In Pediatric Moyamoya Arteriopathy

Introduction: Little is known about quality of life, sleep, and mental health in children with moyamoya arteriopathy (MMA). A better understanding of patient-centered outcomes may lead to improved counseling and treatment of childhood MMA. Methods: MMA patients <26 years old (when developmentally appropriate) and their parents/guardians completed a series of validated, age-appropriate questionnaires. Measures included the Pediatric Quality of Life (PedsQL) Inventory (assessing health-related quality of life), Patient-Reported Outcomes Information System (PROMIS) sleep questionnaires (assessing sleep disturbance, defined as disturbed sleep, and sleep impairment, defined as impairment of daily activities due to poor sleep), and PROMIS depression/anxiety questionnaires. Results: Seventeen participants with MMA were enrolled, with 8 children and 17 parent proxies completing questionnaires. Median age was 7.3 years (IQR 6.6, 10.1 years). The cohort included 8 (47%) females and children with both moyamoya disease (n=9, 53%) and moyamoya syndrome (sickle cell disease n=4, neurofibromatosis n=3, hemophilia A n=1). On the PedsQL (100 = best quality of life), children with MMA scored relatively low in emotional (mean = 73.2), social (mean = 74.5), and school/work (mean = 72.1) quality of life domains compared to the physical (mean = 82.6) domain. On parent proxy PROMIS sleep questionnaires, 53% of children with MMA qualified as having sleep disturbance (41% moderate-severe) and 47% had sleep impairment (35% moderate-severe). Of 25 child and parent proxy PROMIS depression/anxiety forms, 48% indicated moderate-severe anxiety and 24% moderate-severe depressive symptoms. Conclusions: Children with MMA have high rates of poor quality of life, sleep disturbance, sleep impairment, anxiety, and depressive symptoms. Future directions include comparison of patient centered outcomes in MMA to children with other stroke etiologies as well as unaffected siblings. A better understanding of patient centered outcomes will allow for improved counseling and targeted therapies to address MMA-related sleep, emotional, social, and school-based impairment.