Bilateral Multiple Focal Choroidal Excavations in a Patient with Paroxysmal Nocturnal Hemoglobinuria (PNH)

Focal choroidal excavation (FCE) is an unusual configuration characterized by focal thinning and pitting of the choroid in the absence of staphyloma or scleral ectasia. The etiology and clinical implications of FCE are currently unknown. We report a case of bilateral multiple FCEs in a patient with...

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Main Authors: Ping Huang, Tal Ben Ami, Weiye Li
Format: Article
Language:English
Published: Wiley 2019-01-01
Series:Case Reports in Ophthalmological Medicine
Online Access:http://dx.doi.org/10.1155/2019/7925737
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author Ping Huang
Tal Ben Ami
Weiye Li
author_facet Ping Huang
Tal Ben Ami
Weiye Li
author_sort Ping Huang
collection DOAJ
description Focal choroidal excavation (FCE) is an unusual configuration characterized by focal thinning and pitting of the choroid in the absence of staphyloma or scleral ectasia. The etiology and clinical implications of FCE are currently unknown. We report a case of bilateral multiple FCEs in a patient with a long history of paroxysmal nocturnal hemoglobinuria (PNH). Focal choriocapillaris thinning and hyperreflectivity of the adjacent outer nuclear layer were revealed by spectral domain optical coherence tomography, which suggests the occlusion of the choriocapillaries and secondary degeneration of the above photoreceptors. We hypothesize that thrombophilia in the condition of PNH played an important role in the formation of FCE. Although there is no histopathological evidence to support the association between the choroid changes and PNH, this case may offer new clues as for the etiology of FCE.
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spelling doaj-art-9c59e5f9662b4d07a895b3ab7cbdede32025-02-03T01:12:34ZengWileyCase Reports in Ophthalmological Medicine2090-67222090-67302019-01-01201910.1155/2019/79257377925737Bilateral Multiple Focal Choroidal Excavations in a Patient with Paroxysmal Nocturnal Hemoglobinuria (PNH)Ping Huang0Tal Ben Ami1Weiye Li2Department of Ophthalmology, Drexel University College of Medicine, 219 N. Broad Street, Philadelphia, PA 19107, USADepartment of Ophthalmology, Drexel University College of Medicine, 219 N. Broad Street, Philadelphia, PA 19107, USADepartment of Ophthalmology, Drexel University College of Medicine, 219 N. Broad Street, Philadelphia, PA 19107, USAFocal choroidal excavation (FCE) is an unusual configuration characterized by focal thinning and pitting of the choroid in the absence of staphyloma or scleral ectasia. The etiology and clinical implications of FCE are currently unknown. We report a case of bilateral multiple FCEs in a patient with a long history of paroxysmal nocturnal hemoglobinuria (PNH). Focal choriocapillaris thinning and hyperreflectivity of the adjacent outer nuclear layer were revealed by spectral domain optical coherence tomography, which suggests the occlusion of the choriocapillaries and secondary degeneration of the above photoreceptors. We hypothesize that thrombophilia in the condition of PNH played an important role in the formation of FCE. Although there is no histopathological evidence to support the association between the choroid changes and PNH, this case may offer new clues as for the etiology of FCE.http://dx.doi.org/10.1155/2019/7925737
spellingShingle Ping Huang
Tal Ben Ami
Weiye Li
Bilateral Multiple Focal Choroidal Excavations in a Patient with Paroxysmal Nocturnal Hemoglobinuria (PNH)
Case Reports in Ophthalmological Medicine
title Bilateral Multiple Focal Choroidal Excavations in a Patient with Paroxysmal Nocturnal Hemoglobinuria (PNH)
title_full Bilateral Multiple Focal Choroidal Excavations in a Patient with Paroxysmal Nocturnal Hemoglobinuria (PNH)
title_fullStr Bilateral Multiple Focal Choroidal Excavations in a Patient with Paroxysmal Nocturnal Hemoglobinuria (PNH)
title_full_unstemmed Bilateral Multiple Focal Choroidal Excavations in a Patient with Paroxysmal Nocturnal Hemoglobinuria (PNH)
title_short Bilateral Multiple Focal Choroidal Excavations in a Patient with Paroxysmal Nocturnal Hemoglobinuria (PNH)
title_sort bilateral multiple focal choroidal excavations in a patient with paroxysmal nocturnal hemoglobinuria pnh
url http://dx.doi.org/10.1155/2019/7925737
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AT talbenami bilateralmultiplefocalchoroidalexcavationsinapatientwithparoxysmalnocturnalhemoglobinuriapnh
AT weiyeli bilateralmultiplefocalchoroidalexcavationsinapatientwithparoxysmalnocturnalhemoglobinuriapnh