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J Pharm Bioallied Sci. 2012 Aug;4(Suppl 2):S171-3. doi: 10.4103/0975-7406.100262.

An unusual occurrence of Karsch-Neugebauer syndrome with orodental anomalies.

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  • 1Department of Pedodontics and Preventive Dentistry, Vivekanandha Dental College for Women, Tiruchengode, Tamil Nadu, India.

Abstract

Karsch-Neugebauer syndrome is a rare congenital anomaly characterized by split foot, split hand anomalies in association with congenital nystagmus. It is an inherited condition and often occurs in both the hands and the feet. Its pattern of inheritance is autosomal dominant, though occasionally it can skip a generation. It affects about 1 in 90,000 babies, with males and females equally being affected. This rare syndrome may be associated with additional abnormalities such as hypodontia, delayed eruption, variation in morphology and position of teeth, but it is not widely documented. In view of the low incidence of this syndrome and of the limited number of reports on this in the literature, the objective of this article is to highlight the oral findings of this syndrome.

KEYWORDS:

Split hand deformities; ectrodactylia of hands; karsch neugebauer syndrome; lobster claw malformation

PMID:
23066244
[PubMed]
PMCID:
PMC3467867
Free PMC Article
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