Trichodental Dysplasia: A Rare Syndrome With Distinct Dental Findings 6/11/10

Department of Pediatric Dentistry
Resident’s Name: Murphy Program: Lutheran Medical Center - Providence

Article title: Trichodental Dysplasia: A Rare Syndrome With Distinct Dental Findings
Author(s): Montalvan DMD, Ericka. Christina Mazzone, DMD. Nanci Tofsky, DMD, MM, DMD
Journal: Pediatric Dentistry
Year. Volume (number). Page #’s: 2006. 28:4. 345-349
Major topic: Presentation of Trichodental Dysplasia(TD)
Minor topic(s): Ectodermal Dysplasia and it’s MANY variants
Main Purpose: To present the clinical, radiographic, and genetic features of a 4yo child w/ TD that will add to the exsisting knowledge of this rare syndrome
Overview of method of research: Case Report

Findings: Congenital absence of 1 or more teeth w/o any anomalies is common, happening in up to 8% of the human population. A rarer cause of missing teeth is ectodermal dysplasia. With ED, hair, nails, skin, and teeth can all be affected at the same time. TD is a rare autosomal dominant variant of ED that affects only the teeth and hair. TD has been described in a number of families. Only a small handful of cases have been reported. Teeth in children with TD are described as having normal enamel, extremely thin dentin, very large pulps, and short roots. They have been described as ‘shell teeth’, or ‘Ghost teeth’. Hair in patients with TD is fine, lusterless, sparse, and slow growing. The lateral ends of the eyebrows are thin as well. Other possible signs are microcephaly, frontal bossing, and mild retardation.
In this case, a 4 yo Hispanic male presented to UMDNJ. He had thin sparse hair, frontal bossing, thin eyebrows, and a fairly large head. His mom said a CT scan was performed to assess the size/shape of his head, and that everything came back normal. The boy did not have any abnormal sweating, and no history of any fractures. His fingernails had some creasing, and mild clubbing. Intra orally, pulp chambers were visible through the lingual surfaces of his maxillary incisors, and through the occlusal of the molars. There was extensive attrition, and he had 6 abscessed teeth. Radiographically, it could be seen that his permanent teeth was of the same variety. The boy was sent to a geneticist, for evaluation, and the geneticist confirmed the original diagnosis of TD.
Treatment under GA was decided on. The 6 abscessed teeth were extracted. All of the other molars got SSC’s. The anterior teeth were left alone for fear of pulpal exposure. He was put on frequent recalls for eval, as well as daily Fl regiment.

Key points/Summary: While TD is rare, it is not uncommon to see children with ED. Treatment should include protecting the teeth best as possible with crowns, frequent recall visits.

Assessment of Article: Good case report. Nothing too Earth shattering