Nongranulomatous anterior uveitis in a patient with Usher syndrome

Alzuhairy, Sultan; Alfawaz, Abdullah M.

doi:10.1016/j.sjopt.2013.06.001

Cited by 4 publications

(3 citation statements)

References 11 publications

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“…To the best of our knowledge, this finding has not been previously reported in a patient with Usher syndrome. To date, all but one case of uveitis in Usher syndrome has been attributed to FHIC: Alzuhairy and Alfawaz described a case of non-granulomatous uveitis in a patient with Usher syndrome that did not resemble FHIC [ 3 ]. We have, however, been unable to find an observation in the literature of granulomatous uveitis in Usher syndrome.…”

Section: Discussionmentioning

confidence: 99%

“…While reports of disease prevalence vary, the condition has been estimated to occur in three in 100, 000 individuals. Moreover, Usher syndrome is the most common cause of combined deafness and blindness, accounting for nearly 50% of all cases [ 1 - 3 ].…”

Section: Introductionmentioning

confidence: 99%

“…More recently, two studies have confirmed an association between Usher syndrome and FHIC [ 10 , 11 ]. Furthermore, a recent study reported a case of non-granulomatous uveitis that was distinct from FHIC in a patient with Usher syndrome [ 3 ].…”

Section: Introductionmentioning

confidence: 99%

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Bilateral uveitis and Usher syndrome: a case report

Benson

MacDonald

2015

J Med Case Reports

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IntroductionUsher syndrome is a genetically heterogeneous condition and represents the most common cause of inherited combined vision and hearing loss. Deficits manifest as sensorineural hearing loss that typically develops at a young age and retinitis pigmentosa that can lead to peripheral vision loss and night blindness. As a result, this syndrome can have a significant impact on a patient’s quality of life.Previous studies have described an association between Usher syndrome and Fuchs’ heterochromic iridocyclitis, a form of non-granulomatous uveitis that generally presents in a unilateral manner. We present a rare finding of bilateral uveitis and, to the best of our knowledge, the first report of granulomatous uveitis as a feature in a patient with Usher syndrome.Case presentationA 45-year-old Caucasian woman with a known history of retinitis pigmentosa presented to our clinic with suspected Usher syndrome, given her report of long-standing hearing loss. Aside from a mild loss in visual acuity, our patient was otherwise asymptomatic. Visual field testing, audiology and electroretinography findings supported the diagnosis of Usher syndrome. With slit lamp examination she was found to have bilateral keratic precipitates, with large, greasy-white, mutton-fat keratic precipitates on the endothelial surface of her left eye. A thorough work-up that included blood tests and imaging was negative for an alternative cause of her uveitis.ConclusionWe present a rare finding of bilateral uveitis and what we believe to be the first reported instance of mutton-fat keratic precipitates and granulomatous uveitis as a feature in a patient with Usher syndrome. By identifying atypical presentations of the disease, we hope to contribute to the range of ophthalmic conditions that may be seen in association with Usher syndrome.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Bilateral uveitis and Usher syndrome: a case report

Benson

MacDonald

2015

J Med Case Reports

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Uveitis in Patients with Retinitis Pigmentosa: 30 Years’ Consecutive Data

Majumder

Sukhija

Roy

et al. 2017

Ocular Immunology and Inflammation

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Progression of retinal pigmentation mimicking unilateral retinitis pigmentosa after bilateral pars planitis: a case report

et al. 2018

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BackgroundTo report our findings in a young patient with unilateral retinitis pigmentosa (RP)-like appearance who developed pigmentary changes in his left retina after an episode of bilateral pars planitis.Case presentationA 17-year-old man presented with 6 months of blurry vision in both eyes. He was diagnosed with bilateral pars planitis. Progressive, intraretinal bone crepuscule pigmentation developed in his left retina during the following three months. An electroretinogram showed subnormal response only in the left eye, suggesting the diagnosis of unilateral pseudoRP.ConclusionAn inflammatory disease like pars planitis can accelerate the pigmentation of the retina and mimic a RP in young patients. Causes of pseudoRP may be considered, especially in those rare cases with unilateral affection.

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Nongranulomatous anterior uveitis in a patient with Usher syndrome

Cited by 4 publications

References 11 publications

Bilateral uveitis and Usher syndrome: a case report

Bilateral uveitis and Usher syndrome: a case report

Uveitis in Patients with Retinitis Pigmentosa: 30 Years’ Consecutive Data

Progression of retinal pigmentation mimicking unilateral retinitis pigmentosa after bilateral pars planitis: a case report

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