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

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 dysgenes...

Full description

Bibliographic Details
Main Authors: Megan E. Falls BA, Michael P. Rabinowitz MD, Jacqueline R. Carrasco MD, Mindy R. Rabinowitz MD
Format: Article
Language:English
Published: SAGE Publishing 2018-10-01
Series:Allergy & Rhinology
Online Access:https://doi.org/10.1177/2152656718804905
_version_ 1818150339313926144
author Megan E. Falls BA
Michael P. Rabinowitz MD
Jacqueline R. Carrasco MD
Mindy R. Rabinowitz MD
author_facet Megan E. Falls BA
Michael P. Rabinowitz MD
Jacqueline R. Carrasco MD
Mindy R. Rabinowitz MD
author_sort Megan E. Falls BA
collection DOAJ
description 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.
first_indexed 2024-12-11T13:21:21Z
format Article
id doaj.art-ada5f82e2a1c4fd0b4e7c2c8ba1eb194
institution Directory Open Access Journal
issn 2152-6567
language English
last_indexed 2024-12-11T13:21:21Z
publishDate 2018-10-01
publisher SAGE Publishing
record_format Article
series Allergy & Rhinology
spelling doaj.art-ada5f82e2a1c4fd0b4e7c2c8ba1eb1942022-12-22T01:05:49ZengSAGE PublishingAllergy & Rhinology2152-65672018-10-01910.1177/2152656718804905Endoscopic Management of Lacrimal System Dysgenesis and Dacryocystoceles in Fraser Syndrome: A Case Report and Literature ReviewMegan E. Falls BAMichael P. Rabinowitz MDJacqueline R. Carrasco MDMindy R. Rabinowitz MDBackground 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.https://doi.org/10.1177/2152656718804905
spellingShingle Megan E. Falls BA
Michael P. Rabinowitz MD
Jacqueline R. Carrasco MD
Mindy R. Rabinowitz MD
Endoscopic Management of Lacrimal System Dysgenesis and Dacryocystoceles in Fraser Syndrome: A Case Report and Literature Review
Allergy & Rhinology
title Endoscopic Management of Lacrimal System Dysgenesis and Dacryocystoceles in Fraser Syndrome: A Case Report and Literature Review
title_full Endoscopic Management of Lacrimal System Dysgenesis and Dacryocystoceles in Fraser Syndrome: A Case Report and Literature Review
title_fullStr Endoscopic Management of Lacrimal System Dysgenesis and Dacryocystoceles in Fraser Syndrome: A Case Report and Literature Review
title_full_unstemmed Endoscopic Management of Lacrimal System Dysgenesis and Dacryocystoceles in Fraser Syndrome: A Case Report and Literature Review
title_short Endoscopic Management of Lacrimal System Dysgenesis and Dacryocystoceles in Fraser Syndrome: A Case Report and Literature Review
title_sort endoscopic management of lacrimal system dysgenesis and dacryocystoceles in fraser syndrome a case report and literature review
url https://doi.org/10.1177/2152656718804905
work_keys_str_mv AT meganefallsba endoscopicmanagementoflacrimalsystemdysgenesisanddacryocystocelesinfrasersyndromeacasereportandliteraturereview
AT michaelprabinowitzmd endoscopicmanagementoflacrimalsystemdysgenesisanddacryocystocelesinfrasersyndromeacasereportandliteraturereview
AT jacquelinercarrascomd endoscopicmanagementoflacrimalsystemdysgenesisanddacryocystocelesinfrasersyndromeacasereportandliteraturereview
AT mindyrrabinowitzmd endoscopicmanagementoflacrimalsystemdysgenesisanddacryocystocelesinfrasersyndromeacasereportandliteraturereview