CASE REPORTS RUSSELL-SILVER SYNDROME

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Journal of the Korean Academy of Pediatric Dentistry 2002;29(1):51-56.
Published online February 28, 2002.

CASE REPORTS RUSSELL-SILVER SYNDROME

증례 보고 : Russell-Silver Syndrome

이진, 장기택, 김종철

서울대학교 치과대학 소아치과학교실 및 치학연구소

Abstract

Russell-Silver syndrome is a type of intrauterine growth retardation, characterized by short stature noted at birth, hemiatrophy or asymmetry, variation in sexual development and other abnormalities, including cafe-aulait pigmentation and clinodactyly. Facial features commonly associated with this syndrome are a small triangular face, decreased facial height, down-turned corners of the mouth(shark's mouth), a small mandible, and occasionally asymmetry. The major intra-oral features of the syndrome that have been reported are a high-arched palate, delayed tooth eruption, microdontia, hypodontia, and crowding. These cases were diagnosed at birth as Russell-Silver syndrome by clinical features such as prenatal growth retardation, short stature, low body weight, et al., and have been treated with growth hormone. The purpose of this paper is to report the dental findings of two patients and review the pertinent literature through the two cases.

Key Words: Growth retardation, Clinodactyly, Asymmetry, Shark's mouth, Crowding