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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...

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Autores principales: Tehranchi, Azita, Behnia, Hossein, Nadjmi, Nasser, Yassaee, Vahid Reza, Ravesh, Zeinab, Mina, Morteza
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2016
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.
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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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