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范德伍德综合征患者的多学科管理:一例报告

Multidisciplinary management of a patient with van der Woude syndrome: A case report.

作者信息

Tehranchi Azita, Behnia Hossein, Nadjmi Nasser, Yassaee Vahid Reza, Ravesh Zeinab, Mina Morteza

机构信息

Preventive Research Center, Research Institute of Dental Sciences, Shahid Beheshti University of Medical Sciences, Tehran, Iran; Orthodontic Department, Dental School, Tehran, Iran.

Dentofacial Deformities Research Center, Research Institute of Dental Sciences, Shahid Beheshti University of Medical Sciences, Tehran, Iran; Dental School, Tehran, Iran.

出版信息

Int J Surg Case Rep. 2017;30:142-147. doi: 10.1016/j.ijscr.2016.11.032. Epub 2016 Dec 6.

Abstract

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+1G/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.

摘要

引言

范德伍德综合征(VWS)是综合征性唇腭裂(SCLP)最常见的形式,占所有唇腭裂患者的2%。

病例报告

我们描述了一名被诊断为VWS的女孩的正畸治疗情况,她因美容问题被家庭牙医转诊。

讨论

综合正畸治疗、二期植骨、牵张成骨(用于上颌骨发育不全)、二期腭裂修复术和下唇凹陷切除术,以及正畸和修复程序可能会带来令人满意的结果。基因检测显示,一个已知的推定剪接位点突变(c.174+1G/A)是我们患者及其家族中VWS的主要病因。

结论

SCLP对面部美学和心理社会状况有重大影响。应对父母进行适当的评估和咨询。在没有危及生命的全身异常的情况下,这种疾病是可治疗的。提倡采用多学科团队方法。

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