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Traumatic bone cyst of the mandible in Langer-Giedion syndrome: a case report
INTRODUCTION: Langer-Giedion syndrome (trichorhinophalangeal syndrome type II) is an extremely rare disorder characterized by dysmorphic facial features, multiple exostoses, mental retardation and digit deformities. We report the first case of any maxillofacial pathology in such a syndromic patient....
Autores principales: | , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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BioMed Central
2014
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4364690/ https://www.ncbi.nlm.nih.gov/pubmed/25421062 http://dx.doi.org/10.1186/1752-1947-8-387 |
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author | Nagori, Shakil Ahmed Jose, Anson Agarwal, Bhaskar Bhatt, Krushna Bhutia, Ongkila Roychoudhury, Ajoy |
author_facet | Nagori, Shakil Ahmed Jose, Anson Agarwal, Bhaskar Bhatt, Krushna Bhutia, Ongkila Roychoudhury, Ajoy |
author_sort | Nagori, Shakil Ahmed |
collection | PubMed |
description | INTRODUCTION: Langer-Giedion syndrome (trichorhinophalangeal syndrome type II) is an extremely rare disorder characterized by dysmorphic facial features, multiple exostoses, mental retardation and digit deformities. We report the first case of any maxillofacial pathology in such a syndromic patient. CASE PRESENTATION: A 22-year-old Indian woman with mild intellectual disability presented with malaligned teeth. Routine radiographic screening demonstrated a large multilocular lesion in her right mandible. She had peculiar features such as short stature, short limbs, brachydactyly, and dysmorphic facial characters, which prompted us to evaluate her further. After findings of multiple bony exostoses she was diagnosed with Langer-Giedion syndrome. On surgical exploration of her right mandibular lesion an empty cavity was found suggestive of traumatic bone cyst. The lesion healed completely after 1 year without loss of vitality of any teeth. CONCLUSIONS: Although diagnosis and management of any maxillofacial pathology can be challenging in syndromic patients, our report suggests a possible correlation between traumatic bone cyst and Langer-Giedion syndrome. Clinicians should routinely screen these patients for any undetected maxillofacial pathology. In future cases of this syndrome, one should consider the possibility of traumatic bone cyst which may not require aggressive surgical management. |
format | Online Article Text |
id | pubmed-4364690 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2014 |
publisher | BioMed Central |
record_format | MEDLINE/PubMed |
spelling | pubmed-43646902015-03-19 Traumatic bone cyst of the mandible in Langer-Giedion syndrome: a case report Nagori, Shakil Ahmed Jose, Anson Agarwal, Bhaskar Bhatt, Krushna Bhutia, Ongkila Roychoudhury, Ajoy J Med Case Rep Case Report INTRODUCTION: Langer-Giedion syndrome (trichorhinophalangeal syndrome type II) is an extremely rare disorder characterized by dysmorphic facial features, multiple exostoses, mental retardation and digit deformities. We report the first case of any maxillofacial pathology in such a syndromic patient. CASE PRESENTATION: A 22-year-old Indian woman with mild intellectual disability presented with malaligned teeth. Routine radiographic screening demonstrated a large multilocular lesion in her right mandible. She had peculiar features such as short stature, short limbs, brachydactyly, and dysmorphic facial characters, which prompted us to evaluate her further. After findings of multiple bony exostoses she was diagnosed with Langer-Giedion syndrome. On surgical exploration of her right mandibular lesion an empty cavity was found suggestive of traumatic bone cyst. The lesion healed completely after 1 year without loss of vitality of any teeth. CONCLUSIONS: Although diagnosis and management of any maxillofacial pathology can be challenging in syndromic patients, our report suggests a possible correlation between traumatic bone cyst and Langer-Giedion syndrome. Clinicians should routinely screen these patients for any undetected maxillofacial pathology. In future cases of this syndrome, one should consider the possibility of traumatic bone cyst which may not require aggressive surgical management. BioMed Central 2014-11-25 /pmc/articles/PMC4364690/ /pubmed/25421062 http://dx.doi.org/10.1186/1752-1947-8-387 Text en © Nagori et al.; licensee BioMed Central Ltd. 2014 This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly credited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated. |
spellingShingle | Case Report Nagori, Shakil Ahmed Jose, Anson Agarwal, Bhaskar Bhatt, Krushna Bhutia, Ongkila Roychoudhury, Ajoy Traumatic bone cyst of the mandible in Langer-Giedion syndrome: a case report |
title | Traumatic bone cyst of the mandible in Langer-Giedion syndrome: a case report |
title_full | Traumatic bone cyst of the mandible in Langer-Giedion syndrome: a case report |
title_fullStr | Traumatic bone cyst of the mandible in Langer-Giedion syndrome: a case report |
title_full_unstemmed | Traumatic bone cyst of the mandible in Langer-Giedion syndrome: a case report |
title_short | Traumatic bone cyst of the mandible in Langer-Giedion syndrome: a case report |
title_sort | traumatic bone cyst of the mandible in langer-giedion syndrome: a case report |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4364690/ https://www.ncbi.nlm.nih.gov/pubmed/25421062 http://dx.doi.org/10.1186/1752-1947-8-387 |
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