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      Keratoglobus: An experience at a tertiary eye care center in India

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          Abstract

          Context:

          This study was carried out as a part of an internal audit and is the largest series of patients having keratoglobus, published in the literature. Poor visual acuity of the patients indicates the blinding nature of the disease.

          Aims:

          We report our experience with patients having keratoglobus at a tertiary eye care center in India.

          Settings and Design:

          Retrospective study.

          Materials and Methods:

          We analyzed adults and pediatric patients (<16 years) with keratoglobus, seen during 2008–2012. The age, gender, consanguinity, presenting ocular signs, ocular and systemic associations, visual acuity, corneal topography, and surgeries were documented.

          Results:

          Forty-eight patients (mean age 22 ± 15 years, 31 males) having keratoglobus were analyzed. 21 patients (42 eyes) were <16 years. Twelve eyes (16 events) had positive history of trauma. The presenting clinical signs were corneal scars/scars of tear repair (15 eyes), hydrops, healed and acute (14 eyes) and corneal or globe rupture (9 eyes). Best-corrected visual acuity was >20/40 in 6/42 (14.3%) pediatric eyes and 15/53 (28.30%) adults. Visual acuity ranging from counting of fingers to no light perception was noted in 20/53 (37.74%) adults and 21/42 (50%) pediatric patients; 13/20 (65%) with blue sclera and 8/22 eyes (36.37%) without blue sclera. Vernal keratoconjunctivitis was present in one pediatric patient. Choroidal osteoma, retinitis pigmentosa, and retinal detachment were present in adults. Surgeries performed were corneal tear repair (5 eyes), tissue adhesive application (2 eyes), descematopexy (4 eyes) and penetrating keratoplasty (PK - 8 eyes: Three had post-PK glaucoma, graft failure-one eye, 4 patients wore scleral lens - prosthetic replacement of the ocular surface ecosystem).

          Conclusions:

          About 50% of pediatric eyes (65% having blue sclera) had no functional vision. Trivial trauma was responsible for corneal rupture indicating need for protective glasses. About 50% patients had post-PK glaucoma though grafts were clear.

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          Most cited references12

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          Corneal ectasia in vernal keratoconjunctivitis.

          The type of corneal ectasia, the presence of breaks in Descemet's membrane, and the success with contact lens wear or penetrating keratoplasty were studied in 61 patients with corneal ectasia and vernal keratoconjunctivitis. There were 53 patients with keratoconus, 5 with pellucid marginal corneal degeneration, 2 with keratoglobus, and 1 with superior corneal thinning. The high rate of hydrops and the corneal ectasia itself may be related to excessive eye rubbing. Success with contact lens wear or penetrating keratoplasty in vernal keratoconjunctivitis patients with keratoconus is less than in patients with keratoconus alone.
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            Intracameral perfluoropropane gas in the treatment of acute corneal hydrops.

            This study is aimed to evaluate the role of 14% nonexpansile concentration of perfluoropropane (C(3)F(8)) gas in the management of acute hydrops in corneal ectasias. Retrospective, nonrandomized, comparative, interventional case series. The study group consisted of 62 eyes of 57 patients and the control group included 90 eyes of 82 patients with acute corneal hydrops who presented within 30 days of onset of symptoms. Patients in the control group underwent a single intracameral injection of 0.1 mL of nonexpansile concentration (14%) of C(3)F(8) gas. Patients in the control group were treated conservatively. Patients in both groups were followed regularly for 12.6±7.7 and 13.4±8.3 months in the study and control groups, respectively, and assessed clinically for complete disappearance of epithelial and stromal edema on slit-lamp biomicroscopy. The primary outcome measure was mean time to resolution of corneal edema, which was calculated both from the date of onset of hydrops and the date of initiation of therapy to the date of resolution in days. The overall time to resolution both from the date of onset of symptoms (90.5±55.8 vs 125±68.9 days; P = 0.0005) and from the date of initiation of therapy (78.7±53.2 vs 117.9±68.2 days; P = 0.0001) was significantly lower in the study group compared with the control group. However, on subgroup analysis a significant difference in the resolution time was found only in eyes with keratoconus (P<0.0001). No difference in the resolution time was seen in eyes with pellucid marginal corneal degeneration (PMCD) or keratoglobus. The main complication of this procedure was reversible pupillary block (16%; P<0.0001). There was no difference in the final visual acuity or endothelial cell counts between the 2 groups. Intracameral C(3)F(8) gas in a nonexpansile concentration is a useful modality for faster resolution of corneal edema in patients with acute corneal hydrops and keratoconus, and its role in the treatment of PMCD and keratoglobus needs further evaluation. Copyright © 2011 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.
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              Keratoglobus.

              Keratoglobus is a rare noninflammatory corneal thinning disorder characterised by generalised thinning and globular protrusion of the cornea. It was first described as a separate clinical entity by Verrey in 1947. Both congenital and acquired forms have been shown to occur, and may be associated with various other ocular and systemic syndromes including the connective tissue disorders. Similarities have been found with other noninflammatory thinning disorders like keratoconus that has given rise to hypotheses about the aetiopathogenesis. However, the exact genetics and pathogenesis are still unclear. Clinical presentation is characterised by progressive diminution resulting from irregular corneal topography with increased corneal fragility due to extreme thinning. Conservative and surgical management for visual rehabilitation and improved tectonic stability have been described, but remains challenging. In the absence of a definitive standard procedure for management of this disorder, various surgical procedures have been attempted in order to overcome the difficulties. This article reviews the aetiological factors, differential diagnosis, histopathology, and management options of keratoglobus.
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                Author and article information

                Journal
                Indian J Ophthalmol
                Indian J Ophthalmol
                IJO
                Indian Journal of Ophthalmology
                Medknow Publications & Media Pvt Ltd (India )
                0301-4738
                1998-3689
                March 2015
                : 63
                : 3
                : 233-238
                Affiliations
                [1]Cornea Services, L V Prasad Eye Institute, Kallam Anji Reddy Camus, Hyderabad, Telangana, India
                [1 ]L V Prasad Eye Institute, Kallam Anji Reddy Camus, Hyderabad, Telangana, India
                Author notes
                Correspondence to: Dr. Varsha M Rathi, L. V. Prasad Eye Institute, Kallam Anji Reddy Campus, L V Prasad Marg, Banjara Hills, Hyderabad - 500 034, India. E-mail: varsharathi@ 123456lvpei.org
                Article
                IJO-63-233
                10.4103/0301-4738.156927
                4448236
                25971168
                a229c8ba-65c5-4fb6-aba0-79e9ae7004c8
                Copyright: © Indian Journal of Ophthalmology

                This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3.0 Unported, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 12 June 2014
                : 04 December 2014
                Categories
                Original Article

                Ophthalmology & Optometry
                adults,blue sclera,keratoglobus,management,pediatric patients
                Ophthalmology & Optometry
                adults, blue sclera, keratoglobus, management, pediatric patients

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