Trichorhinophalangeal syndrome type 1 (Giedion syndrome): A case report with literature review Síndrome tricorrinofalángico tipo 1 (síndrome de Giedion): caso clínico y revisión de la literatura


Avağ C., ADİLOĞLU S., Akkocaoğlu M.

Reumatologia Clinica, 2022 (ESCI) identifier

  • Publication Type: Article / Article
  • Publication Date: 2022
  • Doi Number: 10.1016/j.reuma.2022.08.006
  • Journal Name: Reumatologia Clinica
  • Journal Indexes: Emerging Sources Citation Index (ESCI), Scopus, Academic Search Premier, EMBASE, MEDLINE, DIALNET
  • Keywords: Giedion syndrome, Impacted teeth, Supernumerary teeth, Trichorhinophalangeal syndrome
  • Hacettepe University Affiliated: Yes

Abstract

© 2022 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de ReumatologíaIntroduction and objectives: Trichorhinophalangeal syndrome (TRPS) is a rare multisystem disorder characterized by abnormalities in the hair (tricho), nose (rhino), and digits (phalangeal). A variety of nonspecific intraoral findings have been reported in the literature, including hypodontia, delayed tooth eruption, malocclusion, a high-arched palate, mandibular retrognathia, midface hypoplasia, and multiple impacted teeth. In addition, supernumerary teeth have been detected in several persons with TRPS, especially type 1. This report describes the clinical manifestations and dental management of a TRPS 1 patient with multiple impacted supernumerary and permanent teeth. Patient: A 15-year-old female patient visited our clinic with a known medical history of TRPS 1 with laceration of the tongue caused by teeth eruption in the palate. Results: Radiographic images showed a total of 45 teeth: two deciduous, 32 permanent, and 11 supernumerary teeth. Six permanent teeth and 11 supernumerary teeth in the posterior quadrants were impacted. Four impacted third molars, supernumerary teeth, retained deciduous teeth, and impacted maxillary premolars were removed under general anaesthesia. Discussion and conclusions: This case suggests that all patients with TRPS should undergo full clinical and radiographic oral examination and should be informed about the disease and the importance of dental counselling.