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KMID : 0371320110800030238
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Journal of the Korean Surgical Society
2011 Volume.80 No. 3 p.238 ~ p.240
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Goltz Syndrome Associated with Omphalocele
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Lee Ho-Won
Byun Chul-Su
Hong Jeong
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Abstract
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Goltz syndrome is a rare congenital disorder characterized by ectodermal and mesodermal dysplasia with multisystem defects. It was first reported in 1962 by Goltz as a focal dermal hypoplasia. More than 200 cases of Goltz syndrome were reported in the world literature, and 5 cases have been reported in Korea since 1994. There were few descriptions in the literature regarding surgical experience in Goltz syndrome. We report a female neonate born with omphalocele associated with Goltz syndrome that included typical cutaneous manifestations and a lobster-claw defect of the left foot. Omphalocele was successfully closed by staged reduction without any wound problems.
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KEYWORD
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Omphalocele, Goltz syndrome, Focal dermal hypoplasia
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