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Pierre Robin 序列患儿的体重增加。

Weight gain in infants with Pierre Robin sequence.

机构信息

Department of Oral and Maxillofacial Surgery, Medical University of Graz, Graz, Austria.

Division of Plastic and Reconstructive Surgery, Department of Surgery, University of California, San Francisco, CA, USA.

出版信息

J Craniomaxillofac Surg. 2020 Jun;48(6):555-559. doi: 10.1016/j.jcms.2020.03.009. Epub 2020 Mar 31.

DOI:10.1016/j.jcms.2020.03.009
PMID:32317138
Abstract

OBJECTIVE

To determine weight gain during treatment with the modified palatal plate (MPP) in infants with isolated and syndromic Pierre Robin Sequence (PRS) suffering from micrognathia, upper airway obstruction (UAO), and failure to thrive (FTT), the authors conducted a retrospective study of infants treated with the MPP.

METHODS

The main outcome measure was infant weight (g) for up to three months after birth. Demographic and outcome data (associated syndromes, comorbidities, presence of cleft lip or palate, intubation attempts, tracheotomy and cleft repair) were collected.

RESULTS

14 children born January 2010 - December 2019 were included. The majority (86%) of infants showed highly significant weight gain (p < 0.001) within a 3-month period (mean pretreatment weight 3147 g with a SD of 425 g vs mean weight at three months 4435 g with a SD of 635 g). Syndromic PRS was found in 7% of infants. 43% of nonsyndromic PRS patients were found to have other congenital anomalies. Genetic testing showed normal karyotypes in 93% of infants and a microdeletion in 7% of infants. 21% of infants required tracheotomy, but no patients required mandibular distraction (MDO) or tongue-lip adhesion (TLA) to relieve UAO.

CONCLUSION

PRS infants treated with the MPP showed highly significant weight gain within a 3-month period and did not require mandibular surgery for early airway management, but faster gain of weight might have implications for strategies to perform surgery at an earlier point in time.

摘要

目的

为了确定患有小下颌、上呼吸道梗阻(UAO)和生长迟缓(FTT)的孤立型和综合征型 Pierre Robin 序列(PRS)婴儿在使用改良腭板(MPP)治疗期间的体重增加情况,作者对接受 MPP 治疗的婴儿进行了回顾性研究。

方法

主要观察指标为出生后长达三个月的婴儿体重(g)。收集人口统计学和结局数据(相关综合征、合并症、唇裂或腭裂、插管尝试、气管切开术和腭裂修复)。

结果

纳入了 2010 年 1 月至 2019 年 12 月出生的 14 名儿童。大多数(86%)婴儿在三个月内体重显著增加(p<0.001)(预处理前平均体重为 3147g,标准差为 425g,而三个月时的平均体重为 4435g,标准差为 635g)。7%的婴儿患有综合征型 PRS。43%的非综合征型 PRS 患儿存在其他先天性异常。93%的婴儿基因检测结果正常,7%的婴儿存在微缺失。21%的婴儿需要气管切开术,但没有患者需要进行下颌骨牵引术(MDO)或舌唇粘连术(TLA)来缓解 UAO。

结论

使用 MPP 治疗的 PRS 婴儿在三个月内体重显著增加,且早期气道管理不需要进行下颌骨手术,但体重增加更快可能会对更早进行手术的策略产生影响。

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引用本文的文献

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The Effect of Timing of Mandibular Distraction Osteogenesis on Weight Velocity in Infants Affected by Severe Robin Sequence.下颌骨牵张成骨时机对重度罗宾序列征患儿体重增长速度的影响
Children (Basel). 2022 Feb 28;9(3):319. doi: 10.3390/children9030319.