Goltz Syndrome Associated with Omphalocele.
10.4174/jkss.2011.80.3.238
- Author:
Ho Won LEE
1
;
Chul Soo BYUN
;
Jeong HONG
Author Information
1. Department of Surgery, Pediatric Surgery, Ajou University School of Medicine, Suwon, Korea. hong@ajou.ac.kr
- Publication Type:Case Report
- Keywords:
Omphalocele;
Goltz syndrome;
Focal dermal hypoplasia
- MeSH:
Congenital, Hereditary, and Neonatal Diseases and Abnormalities;
Ectoderm;
Female;
Focal Dermal Hypoplasia*;
Foot;
Hernia, Umbilical*;
Humans;
Infant, Newborn;
Korea;
Mesoderm;
Wounds and Injuries
- From:Journal of the Korean Surgical Society
2011;80(3):238-240
- CountryRepublic of Korea
- Language:Korean
-
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.
