제대탈출이 동반된 Goltz 증후군
Goltz Syndrome Associated with Omphalocele
이호원, 변철수, 홍정,
소속 상세정보
이호원 ( Lee Ho-Won )
아주대학교 의과대학 외과학교실
변철수 ( Byun Chul-Su )
아주대학교 의과대학 외과학교실
홍정 ( Hong Jeong )
아주대학교 의과대학 외과학교실
KMID : 0371320110800030238
Abstract
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.
키워드
제대탈출; 골츠 증후군; 국한성 피부 저형성증
Omphalocele; Goltz syndrome; Focal dermal hypoplasia
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