Clinical Features, Prothrombotic Risk Factors, and Long-Term Follow-Up of Eight Pediatric Moyamoya Patients
2
조회
0
다운로드
0
인용
0
참고문헌
키워드
초록
Background and Purpose The aim of this study was to elucidate the clinical features, prothrombotic risk factors, and outcome of pediatric Moyamoya patients. Methods Patients diagnosed with Moyamoya disease at a tertiary center between January 2000 and December 2006 were enrolled in this study. The clinical presentations, underlying diseases, prothrombotic risk factors, family history of thrombosis, radiological findings, treatment, and outcome of the patients were reviewed retrospectively. Results Eight patients with angiographically proven Moyamoya disease were identified, one of whom had neurofibromatosis type I and one had Down syndrome. The age at diagnosis varied between 19 months and 11 years (73.4±41.8 months, mean±SD). The follow-up period after diagnosis was 52.5±14.8 months. In six patients, the initial clinical presentation was hemiparesis. None of the patients had any identifiable prothrombotic factors. Despite medical and surgical treatment, three patients had recurrences and one died. Only two patients recovered without sequelae. Conclusions The value of prothrombotic risk factor evaluation appears to be limited in Moyamoya patients; the outcome for pediatric patients remains dismal.
Abstract
Background and Purpose The aim of this study was to elucidate the clinical features, prothrombotic risk factors, and outcome of pediatric Moyamoya patients. Methods Patients diagnosed with Moyamoya disease at a tertiary center between January 2000 and December 2006 were enrolled in this study. The clinical presentations, underlying diseases, prothrombotic risk factors, family history of thrombosis, radiological findings, treatment, and outcome of the patients were reviewed retrospectively. Results Eight patients with angiographically proven Moyamoya disease were identified, one of whom had neurofibromatosis type I and one had Down syndrome. The age at diagnosis varied between 19 months and 11 years (73.4±41.8 months, mean±SD). The follow-up period after diagnosis was 52.5±14.8 months. In six patients, the initial clinical presentation was hemiparesis. None of the patients had any identifiable prothrombotic factors. Despite medical and surgical treatment, three patients had recurrences and one died. Only two patients recovered without sequelae. Conclusions The value of prothrombotic risk factor evaluation appears to be limited in Moyamoya patients; the outcome for pediatric patients remains dismal.
참고문헌 (15)
- Gosalakkal JA, "Moyamoya disease: a review", Neurol India, 50, pp.6~10 (2002)
- Ikezaki K, "A clinical comparison of definite moyamoya disease between South Korea and Japan", Stroke, 28, pp.2513~2517 (1997)
- Shetty-Alva N, "Familial moyamoya disease in Caucasians", Pediatr Neurol, 23, pp.445~447 (2000)
- Scott RM, "Moyamoya disease and moyamoya syndrome", N Engl J Med, 360, pp.1226~1237 (2009)
- deVeber G, "Prothrombotic disorders in infants and children with cerebral thromboembolism", Arch Neurol, 55, pp.1539~1543 (1998)
- Bonduel M, "Prothrombotic disorders in children with moyamoya syndrome", Stroke, 32, pp.1786~1792 (2001)
- Akgün D, "Moyamoya syndrome with protein S deficiency", Eur J Paediatr Neurol, 4, pp.185~188 (2000)
- Cheong PL, "Moyamoya syndrome with inherited proteins C and S deficiency: report of one case", Acta Paediatr Taiwan, 46, pp.31~34 (2005)
- Lutterman J, "Moyamoya syndrome associated with congenital heart disease", Pediatrics, 101, pp.57~60 (1998)
- Jea A, "Moyamoya syndrome associated with Down syndrome: outcome after surgical revascularization", Pediatrics, 116, pp.e694~e701 (2005)
- Hogan AM, "Intellectual decline in children with moyamoya and sickle cell anaemia", Dev Med Child Neurol, 47, pp.824~829 (2005)
- Scott RM, "Surgery for moyamoya syndrome? Yes", Arch Neurol, 58, pp.128~129 (2001)
- by AJ, "Direct and combined revascularization in pediatric moyamoya disease", Neurosurgery, 45, pp.50~58 (1999)
- Khan N, "Moyamoya disease and Moyamoya syndrome: experience in Europe; choice of revascularisation procedures", Acta Neurochir (Wien), 145, pp.1061~1071 (2003)
- Lanthier S, "Stroke in children: the coexistence of multiple risk factors predicts poor outcome", Neurology, 54, pp.371~378 (2000)