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Lee, Byun, and Hong: Goltz Syndrome Associated with Omphalocele
Case Report
Journal of the Korean Surgical Society

Journal of the Korean Surgical Society 2011; 80(3): 238-240.

Published online: 15 March 2011

DOI: https://doi.org/10.4174/jkss.2011.80.3.238

Goltz Syndrome Associated with Omphalocele

Ho Won Lee, M.D., Chul Soo Byun, M.D., Jeong Hong, M.D.

Department of Surgery, Pediatric Surgery, Ajou University School of Medicine, Suwon, Korea.

Corresponding author (hong@ajou.ac.kr)

Received 18 May 2010       Accepted 14 February 2011

Copyright © 2011 The Korean Surgical Society

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.

Keywords: Omphalocele, Goltz syndrome, Focal dermal hypoplasia

Figures and Tables

Fig. 1
Omphalocele and multiple macular erythematous skin lesions in anterior and lateral aspect of trunk, both upper and lower extremities.
jkss-80-238-g001
Fig. 2
Abnormal shaped left ear.
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Fig. 3
Lobster-claw shaped anomaly of left foot.
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Fig. 4
Light microscopy of affected skin. The epidermis is normal. The dermis is replaced by adipose tissue. A thin layer of collagen is seen beneath the basal membrane. H&E stain, ×100.
jkss-80-238-g004

References

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