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皮埃尔·罗宾序列:表现与创新治疗方案 25 年的经验。

Pierre Robin sequence: appearances and 25 years of experience with an innovative treatment protocol.

机构信息

Clinic for Facial Cleft Deformities, Department of Maxillofacial and Surgery, University of Pretoria, PO Box 1266, Pretoria 0001, South Africa.

出版信息

J Pediatr Surg. 2009 Nov;44(11):2112-8. doi: 10.1016/j.jpedsurg.2009.04.018.

DOI:10.1016/j.jpedsurg.2009.04.018
PMID:19944218
Abstract

PURPOSE

The aim of the study was to evaluate the largest number of Pierre Robin sequence (PRS) cases to date and its treatment outcome.

DESIGN

This is a retrospective study.

SETTINGS

The study was conducted in a tertiary care university hospital.

PATIENTS

One hundred eighty-eight patients with PRS have been subjected to analysis, defined by the clinical triad of glossoptosis, retro/micrognathia, and cleft or agenesis of the palate, for incidence, risk factors, associated syndromes, other concomitant abnormalities, airway and feeding difficulties, and outcome regarding the neonatal and perinatal surgical and nonsurgical management.

RESULTS

The incidence of PRS was 6.02%. Risk factors were found in 41.5% of mothers with children with PRS. Eleven other syndromes/appearances were associated. Feeding difficulties were seen in 51.8% of the patients. Glossopexy (6.9%) and tracheotomy (2.13%) for airway management were performed very seldom. The suction and drinking plate as well as the surgical treatment protocol may be considered to be the reasons for reduced airway (up to 91.0%), feeding problems (up to 79.9%), and mortality rate (2.1%).

CONCLUSION

The Pierre Robin sequence, as seen as a heterogeneous group, presents with variation of the cleft palate defects with glossoptosis and concomitant micrognathic mandible. The surgical management and certain prepalatal intervention as nonsurgical management with an innovative treatment strategy were evaluated.

摘要

目的

本研究旨在评估迄今为止最大数量的 Pierre Robin 序列(PRS)病例及其治疗结果。

设计

这是一项回顾性研究。

设置

该研究在一家三级护理大学医院进行。

患者

188 例 PRS 患者接受了分析,PRS 的临床三联征为舌下垂、后/小下颌和腭裂或发育不全,分析了发病率、危险因素、相关综合征、其他伴随异常、气道和喂养困难以及新生儿和围产期手术和非手术管理的结果。

结果

PRS 的发病率为 6.02%。在有 PRS 患儿的母亲中发现了 41.5%的危险因素。还发现了 11 种其他综合征/表现。51.8%的患者存在喂养困难。气道管理很少进行颏舌固定术(6.9%)和气管切开术(2.13%)。抽吸和饮水板以及手术治疗方案可能是减少气道(高达 91.0%)、喂养问题(高达 79.9%)和死亡率(2.1%)的原因。

结论

Pierre Robin 序列是一种异质性群体,表现为腭裂缺陷伴舌下垂和伴发的小下颌。对手术管理和某些腭前干预(非手术管理)以及创新的治疗策略进行了评估。

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