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Multidisciplinary management of a patient with van der Woude syndrome: A case report
INTRODUCTION: Van der Woude syndrome (VWS) is the most frequent form of syndromic cleft lip and palate (SCLP) accounting for 2% of all patients with CLP. CASE PRESENTATION: We describe the orthodontic treatment of a girl diagnosed with VWS referred by her family dentist for her cosmetic concerns. DI...
Autores principales: | , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Elsevier
2016
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5192017/ https://www.ncbi.nlm.nih.gov/pubmed/28012331 http://dx.doi.org/10.1016/j.ijscr.2016.11.032 |
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author | Tehranchi, Azita Behnia, Hossein Nadjmi, Nasser Yassaee, Vahid Reza Ravesh, Zeinab Mina, Morteza |
author_facet | Tehranchi, Azita Behnia, Hossein Nadjmi, Nasser Yassaee, Vahid Reza Ravesh, Zeinab Mina, Morteza |
author_sort | Tehranchi, Azita |
collection | PubMed |
description | INTRODUCTION: Van der Woude syndrome (VWS) is the most frequent form of syndromic cleft lip and palate (SCLP) accounting for 2% of all patients with CLP. CASE PRESENTATION: We describe the orthodontic treatment of a girl diagnosed with VWS referred by her family dentist for her cosmetic concerns. DISCUSSION: Comprehensive orthodontic treatment, secondary bone graft, distraction osteogenesis (for a deficient maxilla), secondary palatoplasty and excision of lower lip pits, as well as orthodontic and prosthetic procedures may provide a satisfactory outcome. Genetic testing showed a known putative splice site mutation (c.174 + 1 G/A) as the prime cause of VWS in our patient and her family. CONCLUSION: SCLP has significant effects on facial aesthetics and the psychosocial status. Parents should be assessed and counseled appropriately. This condition is treatable in the absence of life threatening systemic anomalies. An interdisciplinary team approach is advocated. |
format | Online Article Text |
id | pubmed-5192017 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2016 |
publisher | Elsevier |
record_format | MEDLINE/PubMed |
spelling | pubmed-51920172017-01-03 Multidisciplinary management of a patient with van der Woude syndrome: A case report Tehranchi, Azita Behnia, Hossein Nadjmi, Nasser Yassaee, Vahid Reza Ravesh, Zeinab Mina, Morteza Int J Surg Case Rep Case Report INTRODUCTION: Van der Woude syndrome (VWS) is the most frequent form of syndromic cleft lip and palate (SCLP) accounting for 2% of all patients with CLP. CASE PRESENTATION: We describe the orthodontic treatment of a girl diagnosed with VWS referred by her family dentist for her cosmetic concerns. DISCUSSION: Comprehensive orthodontic treatment, secondary bone graft, distraction osteogenesis (for a deficient maxilla), secondary palatoplasty and excision of lower lip pits, as well as orthodontic and prosthetic procedures may provide a satisfactory outcome. Genetic testing showed a known putative splice site mutation (c.174 + 1 G/A) as the prime cause of VWS in our patient and her family. CONCLUSION: SCLP has significant effects on facial aesthetics and the psychosocial status. Parents should be assessed and counseled appropriately. This condition is treatable in the absence of life threatening systemic anomalies. An interdisciplinary team approach is advocated. Elsevier 2016-12-06 /pmc/articles/PMC5192017/ /pubmed/28012331 http://dx.doi.org/10.1016/j.ijscr.2016.11.032 Text en © 2016 The Author(s) http://creativecommons.org/licenses/by-nc-nd/4.0/ This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/). |
spellingShingle | Case Report Tehranchi, Azita Behnia, Hossein Nadjmi, Nasser Yassaee, Vahid Reza Ravesh, Zeinab Mina, Morteza Multidisciplinary management of a patient with van der Woude syndrome: A case report |
title | Multidisciplinary management of a patient with van der Woude syndrome: A case report |
title_full | Multidisciplinary management of a patient with van der Woude syndrome: A case report |
title_fullStr | Multidisciplinary management of a patient with van der Woude syndrome: A case report |
title_full_unstemmed | Multidisciplinary management of a patient with van der Woude syndrome: A case report |
title_short | Multidisciplinary management of a patient with van der Woude syndrome: A case report |
title_sort | multidisciplinary management of a patient with van der woude syndrome: a case report |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5192017/ https://www.ncbi.nlm.nih.gov/pubmed/28012331 http://dx.doi.org/10.1016/j.ijscr.2016.11.032 |
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