A Patient with Keratoconus, Axial Hyperopia and Pigmentary Retinopathy

Bajracharya, Leena; Lama, Ang Jangmu

doi:10.3126/nepjoph.v10i1.21718

Cited by 3 publications

(1 citation statement)

References 2 publications

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“…Sammouh et al and Bajracharya et al conducted studies that proposed the possibility of a syndromic association of retinitis pigmentosa, keratoconus and hyperopia, as keratoconus is usually found in conjunction with myopia. However, our patient had an axial length of 22.8 mm, so was neither hyperopic, nor myopic [9,10].…”

Section: Discussionmentioning

confidence: 66%

Association of retinitis pigmentosa and advanced keratoconus in siblings

Zemba¹,

Zaharia²,

Dumitrescu³

2020

rjo

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Objective. The aim of the article was to present the rare association of retinitis pigmentosa and bilateral keratoconus in two brothers, one of whom developed corneal hydrops bilaterally, within a short period of time. Methods. A 29-year-old man presented to our service with corneal hydrops in the right eye, complaining of ocular pain and photophobia. He had a history of retinitis pigmentosa, having been diagnosed as an infant. He also had a younger brother carrying the same diagnosis. Slit lamp examination revealed bilateral keratoconus with corneal hydrops in the right eye, posterior subcapsular cataract, macular atrophy and the characteristic retinal signs of retinitis pigmentosa. The patient's brother was also examined, with the same findings being noted, apart from the corneal hydrops. We documented the changes using a slit lamp biomicroscope, a fundus camera, a corneal topography, Anterior Segment Optical Coherence Tomography and visual field testing. Right hydrops regressed in one month after hyperosmolar 5% sodium chloride treatment. However, 4 weeks later, the patient presented with the same corneal findings in the left eye. The same treatment was prescribed for the left eye. Results. Corneal hydrops regressed in both eyes with remaining paracentral corneal scars. However, no other treatment for keratoconus was suitable in the case of this patient. Discussion: Retinitis pigmentosa is currently not amenable to any form of treatment, from vitamin supplementation, medical therapy, gene transfer-based therapy, stem cellbased therapy to retinal implantation. However, molecular genetics may someday provide new therapeutic prospects, that could modify the course of RP. Conclusions. The association of retinitis pigmentosa with keratoconus is a fairly rare finding, worth taking into consideration. Also, presentation with keratoconus in such an advanced state is uncommon and, in our case, it was presumably due to the patient's reduced visual function since childhood, secondary to retinitis pigmentosa, that has prevented him from perceiving any visual modifications caused by keratoconus.

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Section: Discussionmentioning

confidence: 66%

Association of retinitis pigmentosa and advanced keratoconus in siblings

Zemba¹,

Zaharia²,

Dumitrescu³

2020

rjo

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The keratoconus enigma: A review with emphasis on pathogenesis

Ferrari¹,

Rama²

2020

The Ocular Surface

113

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The Prevalence of Refractive errors Among a Sample of Syrian Population with Keratoconus

Akwan

Issa

Aljaramani

et al. 2022

Preprint

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Introduction: Keratoconus is an ectatic disease of the cornea characterized by central or paracentral thinning and steepening that causes a cone-like protrusion of the cornea resulting in reduction in patient's visual acuity usually with progressive myopia and astigmatism. This study was conducted to investigate the prevalence of refractive errors and grading among a sample of Syrian patients with Keratoconus. Methods and Materials: It is a prospective cross-sectional study conducted at Damascus hospital in Syria between the period (September 2021 till the end of April 2022). The study included both newly diagnosed patients as well as patients doing checkups for their previously diagnosed KCN at the time of study. The topographical variables studied are as follows: Kmax, TL, Pachy apex, and maximum posterior and anterior elevation data measured with (BFTE) reference body within the 8-mm diameter zone. Uncorrected and best corrected distal visual acuities were determined by subjective refraction tests. A phoropter was used to subjectively define the patient's eyeglass prescription and refraction errors. All done without cycloplegic drugs Results: The sample size analyzed was 93 patients corresponding to 173 eyes. Unilateral involvement was (7.5%) and bilateral KCN was (92.5%) with a male to female distribution (44.1%) to (55.9%) respectively. Mean age was 29.7 ±10.9 within the range of [ 9 - 72 years]. The mean values for topographical variables measured were as follows: K.max (52.7 ± 6.07 D [range; 43 - 74.5]), TL (473.3 ± 43.1 µm [range; 343 - 609]), Pachy (486.05 ± 41.17 µm [range; 369 - 612]), Maximum anterior elevation (14.53 ± 10.08 µm [range; 2 - 48]) and Maximum posterior elevation (31.05 ± 19.36 µm [range; 5 - 93]). Regarding refractive status, mean spherical refraction was: -1.87 ± 3.69 [range: 10.25 to −17.00 D], mean cylindrical refraction was: -2.85 ± 1.9 [range: 0.00 to −10.00] and mean SE refraction was: -3.30 ± 3.98 [range: 8.75 to −20.50 D]. The mean values of the measured UDVA (log Mar) for 149 eyes was 0.5 ± 0.4 [range: 0 to 1.30] and of BCVA (log Mar) with spectacles was 0.14 ± 0.19 [range: 0 to 1.00]. In terms of KCN grading moderate grade was the most common (43.1%) followed by mild grade (45.1%) as for severe grade it accounted for (11.0%) in total. Compound myopic astigmatism (57.2%) constituted the majority of cases followed by Simple myopic astigmatism (23.1%) and Mixed astigmatism (9.9%). Correlation between absolute value of SE and Kmax (r = 0.669, P-value = 0.000) and with TL (r = -0.393, P-value = 0.000) Conclusion: The clinical profile of keratoconus in Syrian patients is comparable to other studies worldwide with it being more frequent in females and having Compound myopic astigmatism being the most common refractive error related to eyes with keratoconus.

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A Patient with Keratoconus, Axial Hyperopia and Pigmentary Retinopathy

Cited by 3 publications

References 2 publications

Association of retinitis pigmentosa and advanced keratoconus in siblings

Association of retinitis pigmentosa and advanced keratoconus in siblings

The keratoconus enigma: A review with emphasis on pathogenesis

The Prevalence of Refractive errors Among a Sample of Syrian Population with Keratoconus

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