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法伊弗综合征：基于改良分级量表的治疗算法

Pfeiffer Syndrome: A Therapeutic Algorithm Based on a Modified Grading Scale.

作者信息

Raposo-Amaral Cassio Eduardo, Denadai Rafael, Máximo Geiza, Raposo-Amaral Cesar Augusto, Ghizoni Enrico

机构信息

Institute of Plastic and Craniofacial Surgery, SOBRAPAR Hospital, Campinas, São Paulo, Brazil.

Department of Neurology, University of Campinas (UNICAMP), São Paulo, Brazil.

出版信息

Plast Reconstr Surg Glob Open. 2020 Apr 29;8(4):e2788. doi: 10.1097/GOX.0000000000002788. eCollection 2020 Apr.

DOI:10.1097/GOX.0000000000002788
PMID:32440448
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7209848/
Abstract

BACKGROUND

Pfeiffer syndrome (PS) is a very rare condition with a wide clinical spectrum. There are only a few studies that address the classification and treatment of PS and take into account the most commonly presented clinical features. Thus, the objectives of this study are to propose an algorithm for PS management based on a modified severity scale and correlate PS severity with tracheostomy placement.

METHODS

An observational retrospective study was performed on consecutive patients with PS (n = 12), who underwent surgery between 2008 and 2018. Clinical features and findings of all included patients with PS were classified as types A, B, and C, which guided treatment workflow. The Fisher test was used to correlate the severity of patients with PS with tracheostomy placement.

RESULTS

There were 12 patients, classified as type A (n = 3), type B (n = 6), and type C (n = 3). All patients who received tracheostomies (n = 6) were stratified into the severe category (n = 9; types B and C) ( < 0.05). There were 4 minor complications, and 1 major complication according to a modified Clavien-Dindo surgical complication scale.

CONCLUSION

A treatment algorithm based on the 3 different Pfeiffer types was proposed. Severity of PS statistically correlates to tracheostomy placement.

摘要

背景

法伊弗综合征（PS）是一种临床谱广泛的极为罕见的病症。仅有少数研究涉及PS的分类和治疗，并考虑到最常见的临床特征。因此，本研究的目的是基于改良的严重程度量表提出一种PS管理算法，并将PS严重程度与气管切开术的实施相关联。

方法

对2008年至2018年间接受手术的连续PS患者（n = 12）进行了一项观察性回顾性研究。所有纳入的PS患者的临床特征和检查结果被分为A、B和C型，以此指导治疗流程。采用费舍尔检验将PS患者的严重程度与气管切开术的实施相关联。

结果

共有12例患者，分为A型（n = 3）、B型（n = 6）和C型（n = 3）。所有接受气管切开术的患者（n = 6）均被分层为严重类别（n = 9；B型和C型）（P < 0.05）。根据改良的Clavien-Dindo手术并发症量表，有4例轻微并发症和1例严重并发症。

结论

提出了一种基于3种不同法伊弗类型的治疗算法。PS的严重程度与气管切开术的实施在统计学上相关。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/705b/7209848/0e7f623f2e72/gox-8-e2788-g007.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/705b/7209848/c007adecdefe/gox-8-e2788-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/705b/7209848/8015f6d04135/gox-8-e2788-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/705b/7209848/609eb99e5d30/gox-8-e2788-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/705b/7209848/5a1cbbfe5c78/gox-8-e2788-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/705b/7209848/05ac786eb297/gox-8-e2788-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/705b/7209848/813fead191d1/gox-8-e2788-g006.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/705b/7209848/0e7f623f2e72/gox-8-e2788-g007.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/705b/7209848/c007adecdefe/gox-8-e2788-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/705b/7209848/8015f6d04135/gox-8-e2788-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/705b/7209848/609eb99e5d30/gox-8-e2788-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/705b/7209848/5a1cbbfe5c78/gox-8-e2788-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/705b/7209848/05ac786eb297/gox-8-e2788-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/705b/7209848/813fead191d1/gox-8-e2788-g006.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/705b/7209848/0e7f623f2e72/gox-8-e2788-g007.jpg

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