Posterior keratoconus and iris atrophy: a fortuitous association?

Gus, Patrı́cia Ioschpe; Araujo, Bruno Schneider de; Zelanis, Samira K.; Schmalfuss, Tiago Ribeiro; Marinho, Diane Ruschel

doi:10.5935/0004-2749.20190014

Cited by 7 publications

(4 citation statements)

References 8 publications

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“…Another demographic observation noted was that KC had a late onset, at age 30, similar to the other case reports. [6][7][8][9] Although KC commonly appears in the teens and progresses more rapidly in younger patients, there is a possibility of late onset and progression even later in life. Thus, it is prudent to follow-up these cases for a longer term than primary KC.…”

Section: Discussionmentioning

confidence: 99%

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Corneal Collagen Cross-Linking for Late Onset Progressive Keratoconus in Iridocorneal Endothelial Syndrome

Phylactou

Din

Aiello

et al. 2023

Cornea

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The aim of this study was to report the first case of successful use of corneal collagen cross-linking (CXL) to halt the progression of keratoconus in a patient with concurrent iridocorneal endothelial (ICE) syndrome.Methods: A 30-year-old White man was referred to our subspecialty corneal clinic for further investigation of right corectopia. The patient was asymptomatic and was otherwise fit. Slit-lamp examination revealed a right oval-shaped pupil decentered superiorly, a transillumination defect at the 5-o'clock position, minimal gutta-like changes in the corneal endothelium, and few inferior peripheral anterior synechiae. Dilated fundoscopy was normal in both eyes, with healthy optic discs, maculae, and peripheral retinae. Specular microscopy and slit-lamp findings suggested ICE syndrome. Subsequent investigation with corneal tomography showed progressive signs of inferior corneal ectasia with steepening, and thinning in the right eye, consistent with keratoconus. The left eye was unremarkable.Results: Epithelium-off accelerated corneal CXL was performed in the affected eye. Early post-CXL follow-up (1 week) was unremarkable, and further follow-ups were arranged at 3, 6, 12, and 24 months, respectively. In subsequent reviews, the patient's vision and corneal tomography findings were stable. Conclusions:We describe the first case of corneal CXL for progressive keratoconus with ICE syndrome. co-existing keratoconus and ICE syndrome can occur, and corneal cross-linking was used successfully in this case to halt keratoconus progression. However, further studies will need to establish the impact of epithelium-off corneal cross-linking, especially in more severely affected eyes.

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Section: Discussionmentioning

confidence: 99%

“…5 There is a paucity of cases that describe both KC and ICE syndrome variants together. [6][7][8][9] In these reports, the clinical correlation of both disease entities was described and observed. However, we report the first case of successful use of corneal CXL to halt the progression of KC in a patient with concurrent ICE syndrome.…”

mentioning

confidence: 99%

Corneal Collagen Cross-Linking for Late Onset Progressive Keratoconus in Iridocorneal Endothelial Syndrome

Phylactou

Din

Aiello

et al. 2023

Cornea

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“…described a case of unilateral posterior KC and iris atrophy that did not qualify as an ICE syndrome. [ 5 ] Chakrabarty’s case had bilateral KC with Cogan-Reese syndrome and secondary glaucoma. [ 6 ] Table 1 shows a comparison between the current case and the previous cases.…”

Section: Discussionmentioning

confidence: 99%

Unilateral keratoconus associated with iridocorneal endothelial syndrome: Case report

Kalantan

2023

Saudi J Ophthalmol

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We report a rare association of unilateral keratoconus (KC) and iridocorneal endothelial syndrome in a 34-year-old female. Slit-lamp examination showed advanced KC with faint apical scarring in her right eye. The pupil was superiorly displaced with superior peripheral anterior synechia. Specular microscopy showed abnormal endothelium with low endothelial cell count in the right eye. Corneal topography confirmed the unilateral KC diagnosis. As the patient did not tolerate hard contact lens, penetrating keratoplasty with pupilloplasty was performed with excellent outcome.

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“…A história familial varia entre 5 e 20%, com média de 12 a 13%, em grandes estudos populacionais [4][5][6][7][8][9][10][11] . O ceratocone é considerado uma condição genética heterogênea, com penetrância incompleta e expressividade variável.…”

Section: Ectasias Corneanasunclassified

Genética e doenças da córnea: abordagem diagnóstica

Silveira,

Conte,

Gus

2021

eOftalmo

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Nas últimas décadas, com o advento de uma ampla gama de ferramentas diagnósticas sofisticadas e com o desenvolvimento de terapias gênicas para diversas condições antes intratáveis, interesse crescente vem sendo voltado às doenças oculares herdadas. No atual cenário, o oftalmologista geral deve ser capaz de reconhecer a presença ou associação de uma condição genética ou hereditária no caso de algum paciente e fornecer orientação quanto às possibilidades diagnósticas e terapêuticas vigentes, porém o aconselhamento genético ao paciente e/ou família deve ser fornecido por especialistas, como geneticistas e/ou oftalmologistas com título em aconselhamento genético, portanto realizar o encaminhamento para este fim.

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Posterior keratoconus and iris atrophy: a fortuitous association?

Abstract: The authors describe an unusual association between posterior keratoconus and iris atrophy, confirmed by a complete ocular evaluation, scheimpflug imaging and pachymetric curve. A hypothesis for concomitant findings is discussed.

Cited by 7 publications

References 8 publications

Corneal Collagen Cross-Linking for Late Onset Progressive Keratoconus in Iridocorneal Endothelial Syndrome

Corneal Collagen Cross-Linking for Late Onset Progressive Keratoconus in Iridocorneal Endothelial Syndrome

Unilateral keratoconus associated with iridocorneal endothelial syndrome: Case report

Genética e doenças da córnea: abordagem diagnóstica

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