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Vol. 19. Issue 5.
Pages 285-289 (May 2023)
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Vol. 19. Issue 5.
Pages 285-289 (May 2023)
Case report
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
Canseda Avağa,c,
Corresponding author
cansedaavag@hotmail.com

Corresponding author.
, Selen Adiloğlua, Murat Akkocaoğlua,b
a Hacettepe University, Faculty of Dentistry, Department of Oral and Maxillofacial Surgery, Ankara, Turkey
b Private Clinic, Ankara, Turkey
c Biruni University, Faculty of Dentistry, Department of Oral and Maxillofacial Surgery, Zeytinburnu, Istanbul, Turkey
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Table 1. Number of supernumerary teeth of patients with TRPS from literature.
Abstract
Introduction 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.

Keywords:
Giedion syndrome
Impacted teeth
Supernumerary teeth
Trichorhinophalangeal syndrome
Resumen
Introducción y objetivos

El síndrome tricorrinofalángico (TRPS) es un trastorno multisistémico raro caracterizado por anomalías en el pelo (trico), la nariz (rino) y los dedos (falángico). En la literatura se han reportado una variedad de hallazgos intraorales inespecíficos, incluyendo hipodontia, demora de la erupción dental, maloclusión, paladar muy arqueado, retrognatia mandibular, hipoplasia en la sección media del rostro y múltiples dientes impactados. Además, se han detectado dientes supernumerarios en diversas personas con TRPS, en especial de tipo 1. Este informe describe las manifestaciones clínicas y el manejo dental de una paciente con TRPS 1 con múltiples dientes impactados, supernumerarios y permanentes.

Paciente

Una paciente de 15 años con historia médica conocida de TRPS 1 se presentó en nuestra clínica quejándose de laceración de la lengua causada por dientes erupcionados.

Resultados

Las imágenes radiográficas mostraron un total de 45 dientes: 2 deciduos, 32 permanentes y 11 supernumerarios. Seis dientes permanentes y 11 dientes supernumerarios de los cuadrantes posteriores resultaron impactados. Se extrajeron 4 terceros molares y dientes supernumerarios impactados, dientes deciduos retenidos y premolares maxilares impactados bajo anestesia general.

Discusión y conclusiones

Este caso evidencia que todos los pacientes con TRPS deberían someterse a exámenes orales completos a nivel clínico y radiográfico, y ser informados sobre la enfermedad y la importancia del asesoramiento dental.

Palabras clave:
Síndrome de Giedion
Dientes impactados
Dientes supernumerarios
Síndrome tricorrinofalángico

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