Sara C Tho-Calvi1, Dominic Thompson1, Dawn Saunders1, Shakti Agrawal1, Anna Basu1, Manali Chitre1, Gabriel Chow1, Frances Gibbon1, Anthony Hart1, Krishnaraya Kamath Tallur1, Fenella Kirkham1, Rachel Kneen1, Helen McCullagh1, Leena Mewasingh1, Grace Vassallo1, Kayal Vijayakumar1, Elizabeth Wraige1, Tong Hong Yeo1, Vijeya Ganesan2. 1. From the Clinical Neurosciences Section (S.C.T.-C., F.K., V.G.), UCL Great Ormond Street Institute of Child Health; Neurosurgery Department (D.T.) and Neurology Department (D.S., V.G.), Great Ormond Street Hospital for Children NHS Foundation Trust, London; Neurology Department (S.A.), Birmingham Children's Hospital NHS Foundation Trust; Institute of Neuroscience (A.B.), Newcastle University; Department of Paediatric Neurology (A.B.), Newcastle upon Tyne Hospitals NHS Foundation Trust; Department of Paediatric Neurology (M.C.), Cambridge University Hospitals NHS Foundation Trust; Department of Paediatric Neurology (G.C.), Nottingham University Hospitals NHS Foundation Trust; Department of Paediatric Neurology (F.G.), University Hospital of Wales; Neurology Department (A.H.), Sheffield Children's Hospital NHS Foundation Trust; Neurology Department (K.K.T.), Royal Hospital for Sick Children, Edinburgh; Department of Paediatric Neurology (F.K.), University Hospital Southampton NHS Foundation Trust; Neurology Department (R.K.), Alder Hey Children's NHS Foundation Trust, Liverpool; Department of Paediatric Neurology (H.M.), The Leeds Teaching Hospitals NHS Trust; Department of Paediatric Neurology (L.M.), St Mary's Hospital, Imperial College Healthcare NHS Trust, London; Neurology Department (G.V.), Royal Manchester Children's Hospital, Central Manchester University Hospitals NHS Foundation Trust; Neurology Department (K.V.), Bristol Royal Hospital for Children, University Hospitals Bristol NHS Foundation Trust; Neurology Department (E.W.), Evelina Children's Hospital, Guy's and St Thomas' NHS Foundation Trust, London, UK; and Department of Paediatrics (T.H.Y.), KK Women's and Children's Hospital, Singapore. 2. From the Clinical Neurosciences Section (S.C.T.-C., F.K., V.G.), UCL Great Ormond Street Institute of Child Health; Neurosurgery Department (D.T.) and Neurology Department (D.S., V.G.), Great Ormond Street Hospital for Children NHS Foundation Trust, London; Neurology Department (S.A.), Birmingham Children's Hospital NHS Foundation Trust; Institute of Neuroscience (A.B.), Newcastle University; Department of Paediatric Neurology (A.B.), Newcastle upon Tyne Hospitals NHS Foundation Trust; Department of Paediatric Neurology (M.C.), Cambridge University Hospitals NHS Foundation Trust; Department of Paediatric Neurology (G.C.), Nottingham University Hospitals NHS Foundation Trust; Department of Paediatric Neurology (F.G.), University Hospital of Wales; Neurology Department (A.H.), Sheffield Children's Hospital NHS Foundation Trust; Neurology Department (K.K.T.), Royal Hospital for Sick Children, Edinburgh; Department of Paediatric Neurology (F.K.), University Hospital Southampton NHS Foundation Trust; Neurology Department (R.K.), Alder Hey Children's NHS Foundation Trust, Liverpool; Department of Paediatric Neurology (H.M.), The Leeds Teaching Hospitals NHS Trust; Department of Paediatric Neurology (L.M.), St Mary's Hospital, Imperial College Healthcare NHS Trust, London; Neurology Department (G.V.), Royal Manchester Children's Hospital, Central Manchester University Hospitals NHS Foundation Trust; Neurology Department (K.V.), Bristol Royal Hospital for Children, University Hospitals Bristol NHS Foundation Trust; Neurology Department (E.W.), Evelina Children's Hospital, Guy's and St Thomas' NHS Foundation Trust, London, UK; and Department of Paediatrics (T.H.Y.), KK Women's and Children's Hospital, Singapore. v.ganesan@ucl.ac.uk.
Abstract
OBJECTIVE: To describe characteristics and course of a large UK cohort of children with moyamoya from multiple centers and examine prognostic predictors. METHODS: Retrospective review of case notes/radiology, with use of logistic regression to explore predictors of outcome. RESULTS: Eighty-eight children (median presentation age 5.1 years) were included. Thirty-six presented with arterial ischemic stroke (AIS) and 29 with TIA. Eighty had bilateral and 8 unilateral carotid circulation disease; 29 patients had posterior circulation involvement. Acute infarction was present in 36/176 hemispheres and chronic infarction in 86/176 hemispheres at the index presentation. Sixty-two of 82 with symptomatic presentation had at least one clinical recurrence. Fifty-five patients were treated surgically, with 37 experiencing fewer recurrences after surgery. Outcome was categorized as good using the Recovery and Recurrence Questionnaire in 39/85 patients. On multivariable analysis, presentation with TIA (odds ratio [OR] 0.09, 95% confidence interval [CI] 0.02-0.35), headache (OR 0.10, 95% CI 0.02-0.58), or no symptoms (OR 0.08, 95% CI 0.01-0.68) was less likely to predict poor outcome than AIS presentation. Posterior circulation involvement predicted poor outcome (OR 4.22, 95% CI 1.23-15.53). Surgical revascularization was not a significant predictor of outcome. CONCLUSIONS: Moyamoya is associated with multiple recurrences, progressive arteriopathy, and poor outcome in half of patients, especially with AIS presentation and posterior circulation involvement. Recurrent AIS is rare after surgery. Surgery was not a determinant of overall outcome, likely reflecting surgical case selection and presentation clinical status.
OBJECTIVE: To describe characteristics and course of a large UK cohort of children with moyamoya from multiple centers and examine prognostic predictors. METHODS: Retrospective review of case notes/radiology, with use of logistic regression to explore predictors of outcome. RESULTS: Eighty-eight children (median presentation age 5.1 years) were included. Thirty-six presented with arterial ischemic stroke (AIS) and 29 with TIA. Eighty had bilateral and 8 unilateral carotid circulation disease; 29 patients had posterior circulation involvement. Acute infarction was present in 36/176 hemispheres and chronic infarction in 86/176 hemispheres at the index presentation. Sixty-two of 82 with symptomatic presentation had at least one clinical recurrence. Fifty-five patients were treated surgically, with 37 experiencing fewer recurrences after surgery. Outcome was categorized as good using the Recovery and Recurrence Questionnaire in 39/85 patients. On multivariable analysis, presentation with TIA (odds ratio [OR] 0.09, 95% confidence interval [CI] 0.02-0.35), headache (OR 0.10, 95% CI 0.02-0.58), or no symptoms (OR 0.08, 95% CI 0.01-0.68) was less likely to predict poor outcome than AIS presentation. Posterior circulation involvement predicted poor outcome (OR 4.22, 95% CI 1.23-15.53). Surgical revascularization was not a significant predictor of outcome. CONCLUSIONS: Moyamoya is associated with multiple recurrences, progressive arteriopathy, and poor outcome in half of patients, especially with AIS presentation and posterior circulation involvement. Recurrent AIS is rare after surgery. Surgery was not a determinant of overall outcome, likely reflecting surgical case selection and presentation clinical status.
Authors: Annick Kronenburg; Rachel Kleinloog; Albert van der Zwan; L Jaap Kappelle; Luca Regli; Kees P J Braun; Catharina J M Klijn Journal: J Clin Med Date: 2021-04-27 Impact factor: 4.241