Oral Biol Res.  2019 Dec;43(4):361-364. 10.21851/obr.43.04.201912.361.

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

• Shim SH

• KMID: 2470516
• DOI: http://doi.org/10.21851/obr.43.04.201912.361

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.

Keyword

Cleft palate; Smith–Lemli–Opitz syndrome

MeSH Terms

Anesthesia, Local
Child*
Child, Preschool*
Cleft Lip
Cleft Palate
Humans
Male
Micrognathism
Nasal Mucosa
Palate, Soft
Smith-Lemli-Opitz Syndrome*
Tongue
Tooth*