| Title:
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Bilateral Keratoconus in a Patient with Isolated Foveal Hypoplasia
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| Author(s):
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Kiana Hassanpour, Ramin Nourinia, Nazanin Behnaz, Danial Roshandel, Mohsen Azarmina
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| Presentation Type:
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Poster
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| Subject:
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Cornea and Refractive
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| Others:
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Presenting Author:
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| Name:
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Kiana Hassanpoor
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| Affiliation :(optional)
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Ophthalmic Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran
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| E mail:
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kiana.hassanpour@gmail.com
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| Phone:
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Mobile: |
09123019872
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Abstract (Max 200 words)
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| Purpose:
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Foveal hypoplasia (FH) in the absence of other developmental ocular abnormalities (i.e. isolated foveal hypoplasia; IFH) is an extremely rare entity which presents with low vision and nystagmus in the early childhood. KC is known to be a basement membrane disorder of the cornea in which cell-substrate interactions between epithelium and basement membrane are altered. Herein, we report a case of isolated foveal hypoplasia in a patient with bilateral keratoconus for the first time.
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| Methods:
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The patient was a 29-year old lady complaining of decreased visual acuity since childhood which had deteriorated in the past 6 months. She had a history of ocular rubbing since her childhood. In ophthalmic examination, a low amplitude jerky horizontal nystagmus, worsened in end-gazes, was detected in both eyes. Best corrected visual acuity (BCVA) was 20/70 and 20/50 in both eyes using spectacles and RGP, respectively
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| Results:
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On slit lamp examination, mild paracentral corneal thinning and bulging, Fleischer’s ring and Vogt’s striae were present. Dilated fundus examination revealed generalized chorioretinal atrophy with lack of foveal reflex and macular
yellow hue. Retinal capillaries at the macular area showed mild irregularity. Findings in placido-based corneal topography and Orbscan II were consistent with KC in both eyes. Optical coherence tomography and fluorescein angiography revealed typical findings of FH including fovea plana and absence of foveal avascular zone. ERG and EOG were normal. There was no sign of cutaneous and/or ocular albinism, aniridia, achromatopsia, microphthalmos and other ocular abnormalities so that the case was considered as IFH.
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| Conclusion:
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Although several studies have demonstrated strong association between KC and other diseases like Down’s syndrome, atopic keratoconjunctivitis and Leber’s congenital amaurosis, this is the first case of co-incidence of IFH and KC. We have previously proposed that KC might be a generalized disease rather than only a localized corneal abnormality, at least in some cases. This case report might be the first evidence of a third type of KC pathogenesis as a developmental disease in which homeobox gene mutations might result in such coincidence
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| Attachment:
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17Iravo.pptx
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