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Tricho-Rhino-Phalangeal Syndrome with Supernumerary Teeth

¹ Department of Pediatric Dentistry, Faculty of Dentistry, Chiang Mai University, Thailand;
² Department of Craniofacial Development, Dental Institute, Biomedical Research Centre, King’s College London, United Kingdom;
³ Institut fur Humangenetik, Universitätsklinikum, Essen, Germany;
⁴ Department of Orthodontics, Faculty of Dentistry, Chiang Mai University, Thailand,
⁵ Genetic Laboratory Rajanukul Institute, Bangkok, Thailand,
⁶ Dana-Farber Cancer Institute, Boston, MA, USA,
⁷ Department of Developmental Biology, Center for Medical Biotechnology, University Duisburg-Essen, Essen, Germany; and
⁸ Baylor College of Medicine, Houston, TX, USA

View larger version (136K): [in this window] [in a new window]   Figure 1. Expression of Trps1 during mouse tooth development and craniofacial defects observed in Trps1–/– mice. In situ hybridization for Trps1 on coronal (A-D) and sagittal ( e -H) sections of E11.5 (A,B), E13.5 (C), E14.5 (D), and P0 (e-H) first lower molar (B-D) first upper molar (e,F), and lower incisor (G,H) tooth germs. (A) In the first branchial arch, Trps1 expression is restricted to the oral mesenchyme. Note Trps1 expression in the second branchial arch at the level of the cleft (arrows). (B) Molar epithelial thickenings are indicated with arrowheads. The area boxed in white is a higher magnification of the area boxed in black, with the molar epithelial thickening outlined in black. (F) A higher magnification of the area boxed in (E). (I,J) Coronal sections of an E18.5 Trps1gt/Trps1gt head stained with hematoxylin and eosin. (I) Cleft palate is indicated with a star. A correct number of molar and incisor tooth germs was observed. (J) Higher magnification of the area boxed in (I), showing upper and lower molar tooth germs with a normal shape. Am, ameloblasts; BA1, first branchial arch; BA2, second branchial arch; Df, dental follicle; Dm, dental mesenchyme; i, incisors; m, molars; Od, odontoblasts; pOd, pre-odontoblasts. Scale bar = 100 µm.

View larger version (101K): [in this window] [in a new window]   Figure 2. TRPS clinical features of a 17-year-old Thai boy. (A) Frontal picture of the participant showing a high hairline and broad alae of nose. (B) Sagittal view of the individual. (C) Feet with very broad halluces. (D) Hand showing deviation of fingers at proximal interphalangeal joints. ( e ,F) X-rays of, respectively, foot and hand showing multiple cone-shaped epiphyses.

View larger version (124K): [in this window] [in a new window]   Figure 3. Mandibular prognathism and supernumerary teeth observed in a 17-year-old Thai male with TRPS. (A) Mandibular prognathism observed at 14 yrs old. (B) X-ray at age 17 showing a very prognathic mandible. (C) Panoramic x-ray at age 17 shows 5 non-erupted supernumerary teeth exhibiting premolar-like shape (arrows). (D) Electrophoretograms of the sequencing of PCR products. A heterozygous C>T mutation was found at nucleotide position 2756.

Journal of Dental Research, Vol. 87, No. 11, 1027-1031 (2008)
DOI: 10.1177/154405910808701102


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