皮埃尔·罗宾综合征腭裂7例临床分析

A.D.C. Opango, M.S.K. Hattab, Y. Bennaoui, D. Ndélafei, Z. Aziz, N. Mansouri-Hattab
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引用次数: 0

摘要

引言Pierre-Robin综合征是一种畸形序列,与颚后裂、舌下垂和中位腭裂有关,中位腭裂通常较大。这就提出了其治疗管理的问题。目的介绍穆罕默德六世大学医院口腔颌面外科在Pierre Robin综合征腭裂治疗中的方法。这是一项为期5年的回顾性研究,我们纳入了7例Pierre-Robin综合征腭裂病例。我们的研究参数包括流行病学、临床、治疗和进化。结果我们的患者在第一次会诊时的平均年龄为4个月。男性略占优势,性别比为1.3。57.1%的患者出现U型裂隙,42.9%的患者出现V型裂隙。三名患者采用Von Langenbeck技术进行了一期腭发育成形术。四名患者接受了两阶段闭合术,即第一阶段的血管内发育成形术,第二阶段的残余腭裂闭合术。结果令人满意。42.8%的病例出现腭瘘,二次治疗。结论Pierre-Robin综合征腭裂的治疗对颌面外科医生来说是一个真正的挑战。
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Cleft palate in Pierre Robin syndrome: A Review of 7 cases

Introduction

Pierre Robin syndrome is a malformative sequence associating retrognathism, glossoptosis and a median cleft palate which is usually large. This poses the problem of its therapeutic management.

Purpose

To show the approach of the Stomatology and Maxillofacial Surgery Department of the Mohammed VI University Hospital in the management of cleft palate in Pierre Robin syndrome.

This was a retrospective study, covering 5 years, which allowed us to include 7 cases of cleft palate in Pierre Robin syndrome. Our study parameters were epidemiological, clinical, therapeutic and evolutionary.

Results

The average age of our patients, at the time of the 1st consultation, was 4 months. There was a slight male predominance with a sex ratio = 1.3. The patients presented a U-shaped cleft in 57.1% of cases, and a V-shaped cleft in 42.9% of cases. Three patients underwent a one-stage closure by velopalatoplasty using the Von Langenbeck technique. Four patients underwent a 2-stage closure, i.e., intravascular veloplasty in the first stage, followed by closure of the residual cleft palate in the second stage. The results were satisfactory. A palatal fistula was noted in 42.8% of cases, which was managed secondarily.

Conclusion

The management of cleft palate in Pierre Robin syndrome is a real challenge for the maxillofacial surgeon.

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