Endoscopic Management of Lacrimal System Dysgenesis and Dacryocystoceles in Fraser Syndrome: A Case Report and Literature Review.
Allergy Rhinol (Providence). 2018 Jan-Dec;9:2152656718804905
Authors: Falls ME, Rabinowitz MP, Carrasco JR, Rabinowitz MR
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.
PMID: 30364810 [PubMed]
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