Journal List > J Korean Surg Soc > v.80(3) > 1011260

Lee, Byun, and Hong: Goltz Syndrome Associated with Omphalocele


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.

Figures and Tables

Fig. 1
Omphalocele and multiple macular erythematous skin lesions in anterior and lateral aspect of trunk, both upper and lower extremities.
Fig. 2
Abnormal shaped left ear.
Fig. 3
Lobster-claw shaped anomaly of left foot.
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.


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