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Intranasal teeth in a 3-year-old child with Smith-Lemli-Opitz syndrome

심수현
치의학연구원 (The Institute of Dental Science, Chosun University)|2019.12|Oral Biology Research|제43권 제4호|pp. 361-364|DOI https://doi.org/10.21851/obr.43.04.201912.361

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참고문헌

키워드

Cleft palateSmith–Lemli–Opitz syndrome
주제분류치의학

초록

Smith–Lemli–Opitz syndrome (SLOS) is a rare autosomal recessive genetic disorder accompanied with congenital anomalies and mental impairment. Clefts of the hard and/or soft palate, small tongue, broad alveolar ridges, and micrognathia have been reported frequently. However, intranasal teeth associated with SLOS have never been described. Here, a case of intranasal teeth in a 3-year-old boy, who was treated operatively for a cleft lip and alveolus at 7 months of age, is reported. Two intranasal teeth were asymptomatic. The teeth were extracted under local anesthesia when they were found to be very loosely attached to the nasal mucosa. Unusual features of this case are highlighted.

Abstract

Smith–Lemli–Opitz syndrome (SLOS) is a rare autosomal recessive genetic disorder accompanied with congenital anomalies and mental impairment. Clefts of the hard and/or soft palate, small tongue, broad alveolar ridges, and micrognathia have been reported frequently. However, intranasal teeth associated with SLOS have never been described. Here, a case of intranasal teeth in a 3-year-old boy, who was treated operatively for a cleft lip and alveolus at 7 months of age, is reported. Two intranasal teeth were asymptomatic. The teeth were extracted under local anesthesia when they were found to be very loosely attached to the nasal mucosa. Unusual features of this case are highlighted.

참고문헌 (30)

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