Endoscopic Management of Lacrimal System Dysgenesis and Dacryocystoceles in Fraser Syndrome: A Case Report and Literature Review.

IF 2.3 Q1 OTORHINOLARYNGOLOGY

Allergy & Rhinology Pub Date : 2018-10-22 eCollection Date: 2018-01-01 DOI:10.1177/2152656718804905

Megan E Falls, Michael P Rabinowitz, Jacqueline R Carrasco, Mindy R Rabinowitz

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引用次数: 3

Abstract

Background: Fraser syndrome is an autosomal recessive disorder characterized primarily by syndactyly, cryptophthalmos, urinary tract anomalies, ambiguous genitalia, and laryngeal anomalies. A 28-year-old man with Fraser syndrome presented with cryptophthalmos, microphthalmia, lacrimal system dysgenesis, and chronic sinusitis.

Objective: The patients' clinical condition and surgical treatment are described. A literature review was conducted, and articles relevant to the case are presented.

Methods: Case report.

Results: To our knowledge, this is the first published case report of endonasal management of dacryocystoceles in a Fraser syndrome patient. The patient was treated via endoscopic endonasal marsupialization and drainage.

Conclusion: Fraser syndrome patients may initially present to many different specialties as the spectrum of clinical manifestations is broad. Physicians treating these patients should take a collaborative approach to surgical and medical management.

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弗雷泽综合征中泪系统发育不良及泪囊囊肿的内镜治疗:1例报告及文献复习。

背景:Fraser综合征是一种常染色体隐性遗传病，主要表现为并指畸形、隐眼、尿路异常、生殖器模糊和喉部异常。28岁男性弗雷泽综合征表现为隐眼，小眼，泪系统发育不良，慢性鼻窦炎。目的:描述患者的临床情况及手术治疗。进行了文献综述，并提出了与该病例相关的文章。方法:病例报告。结果:据我们所知，这是首次发表的弗雷泽综合征患者鼻腔内处理泪囊囊肿的病例报告。患者经鼻内窥镜造袋引流术治疗。结论:弗雷泽综合征患者最初可能表现为许多不同的专科，因为临床表现广泛。治疗这些患者的医生应该采取外科和医学管理的合作方法。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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