Dental treatment considerations for a pediatric patient with incontinentia pigmenti (Bloch-Sulzberger syndrome)

Amy Yi-Cheng Chen; Kevin Chen

doi:10.4103/ejd.ejd_95_17

European Journal of Dentistry, Inhaltsverzeichnis

CC BY-NC-ND 4.0 · Eur J Dent 2017; 11(02): 264-267
DOI: 10.4103/ejd.ejd_95_17

Case Report

Dental Investigation Society

Dental treatment considerations for a pediatric patient with incontinentia pigmenti (Bloch-Sulzberger syndrome)

Amy Yi-Cheng Chen

¹Pediatric Dentistry Division, Dental Department, Chang Gung Memorial Hospital, Chang Gung University, Taoyuan, Taiwan

,

Kevin Chen

²Medical Department, Medical School, China Medical University, Taichung, Taiwan

› Institutsangaben

Abstract

ABSTRACT

Incontinentia pigmenti (IP) is a uncommon gene disorder, heritage with X-linked dominant mode. IP patients have a characteristic dentition varying from marked hypodontia to delayed eruption and conical crowns on both dentitions. A 5½-year-old girl, whose mother and younger sister were also diagnosed with IP, has the whirling-like pigmented skin lesion over her trunk and four extremities. Four primary teeth and multiple permanent tooth germs were found to be congenital missing. Dental considerations of further treatment were discussed with her parents including the preservation of primary molars, possible interim prosthesis in mixed or permanent dentition, full mouth rehabilitation with orthodontic and prosthodontic combined treatment, and implant therapy in adulthood. Early and longitudinal involvement of pediatric dentist to deal with the dental complications of IP can not only solve the esthetic problem and oral function but also maintain the oral health of children with IP to adulthood.

Key words:

Congenital missing - dermal pigmented lesion - hypodontia - incontinentia pigmenti

Volltext

Referenzen

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