Surgical orthodontic treatment of a patient with trichorhinophalangeal syndrome: A case report
Trichorhinophalangeal syndrome (TRPS) is a rare bone system disorder with autosomal dominant inheritance and characterized by distinct craniofacial and skeletal abnormalities. Bone abnormalities in TRPS result from abnormal maturation of the epiphyseal nucleus and growth plate, leading to growth ret...
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Published in | Journal of oral and maxillofacial surgery, medicine, and pathology Vol. 35; no. 2; pp. 153 - 157 |
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Main Authors | , , , , , , , |
Format | Journal Article |
Language | English |
Published |
Elsevier Ltd
01.03.2023
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Subjects | |
Online Access | Get full text |
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Summary: | Trichorhinophalangeal syndrome (TRPS) is a rare bone system disorder with autosomal dominant inheritance and characterized by distinct craniofacial and skeletal abnormalities. Bone abnormalities in TRPS result from abnormal maturation of the epiphyseal nucleus and growth plate, leading to growth retardation. Although patients with TRPS usually show mandibular hypoplasia, few reports of cases with mandibular prognathism have been documented. Herein, we describe the successful surgical orthodontic treatment of a patient with TRPS type I who presented with mandibular prognathism.
A 17-year-old female exhibited a short stature, short toes, peculiar facial features (protruding nasal apex and hypoplastic nasal wings), and hypoplastic nails. At the age of 13 years, she was diagnosed with TRPS I caused by TRPS1 gene mutation. After preoperative orthodontic treatment, a three-piece Le Fort I osteotomy from between the lateral incisor and canine on one side to that on the other side and bilateral sagittal split ramus osteotomy were performed to improve the occlusion and the maxillomandibular disharmony. One year after the surgery, the patient maintained a good occlusion and facial appearance without significant relapse. Although patients with TRPS have a bone system disorder, this patient exhibited a good prognosis after orthognathic surgery. |
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ISSN: | 2212-5558 2212-5566 |
DOI: | 10.1016/j.ajoms.2022.08.005 |