| The first neurosurgical analysis of 8 korean children with sotos syndrome. | |
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MedLine Citation:
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PMID: 19096684 Owner: NLM Status: In-Data-Review |
Abstract/OtherAbstract:
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OBJECTIVE: Sotos Syndrome is characterized by macrocephaly, overgrowth, and developmental delay, and more than 300 patients have been reported worldwide to date. The authors reviewed the clinical characteristics of 8 patients with Sotos Syndrome in Korea for a new understanding and treatment strategies. METHODS: The medical records of a total of eight Korean children with Sotos Syndrome were reviewed. All patients underwent developmental checkup, lumbar punctures for measurement of intracranial pressure (ICP), brain and spine magnetic resonance imaging and computerized tomography. RESULTS: All 8 patients showed macrocephaly and the characteristic craniofacial features of Sotos Syndrome. Other clinical characteristics shown were overgrowth (7/8), developmental delay (7/8), congenital heart defect (3/8), flat foot (8/8), scoliosis (4/8), spina bifida (8/8), hydrocephalus (4/8), cavum vergae (3/8), and increased subdural fluid collection (5/8). Mean ICP measured via lumbar puncture was 27.35+/-6.25 cm H(2)O (range 20 to 36 cm H(2)O). Two patients received ventriculo-peritoneal shunt, and 1 patient underwent subduro-peritoneal shunt with improvement. Spinal orthosis was applied to 4/5 patients with scoliosis and 4/8 children with flat foot were provided with foot orthosis. CONCLUSION: In this first Korean study of 8 Sotos Syndrome patients we demonstrated the presence of spina bifida and increased ICP, which had not been previously described. The authors therefore suggest that all patients with Sotos Syndrome should undergo examination for the presence of spina bifida, and that shunt procedures would improve development and alleviate clinical symptoms. |
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Authors:
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Jae Joon Lim; Soo Han Yoon |
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Publication Detail:
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Type: Journal Article Date: 2008-10-30 |
Journal Detail:
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Title: Journal of Korean Neurosurgical Society Volume: 44 ISSN: 2005-3711 ISO Abbreviation: J Korean Neurosurg Soc Publication Date: 2008 Oct |
Date Detail:
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Created Date: 2008-12-19 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 101467054 Medline TA: J Korean Neurosurg Soc Country: Korea (South) |
Other Details:
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Languages: eng Pagination: 240-4 Citation Subset: - |
Affiliation:
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Department of Neurosurgery, Ajou University, School of Medicine, Suwon, Korea. |
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