Purpose. To report the use of Descemet stripping endothelial keratoplasty (DSEK) in a patient with keratoglobus and chronic hydrops. Case Report. We describe a case of a 28-year-old man with bilateral keratoglobus and chronic hydrops in the right eye secondary to spontaneous Descemet membrane tear. The patient presented with finger counting (CF) vision, itching, foreign body sensation, and severe photophobia in the right eye. Peripheral corneal thinning with central corneal protrusion and Descemet membrane tear spanning from 4 to 7 o'clock was noted on slit lamp examination. The right eye cornea was 15？mm in the horizontal diameter. After a 5.5-month loss to follow-up, the patient presented with discomfort, photophobia, decreasing vision, and tearing in the right eye. Vision was 20/60 with pinhole. 360-degree peripheral corneal ectasia with mild neovascularization and hydrops was present. Over the next few months, the patient complained of photophobia and intermittent eye pain. His vision deteriorated to CF, he developed corneal scarring with bullae, and a DSEK was performed. Eight months postoperatively, best-corrected vision improved to 20/30, cornea was clear, and the DSEK graft was stable. Conclusions. Nonresolving hydrops secondary to Descemet membrane tear in a patient with keratoglobus may result in permanent endothelial cell damage and scar formation. This may be successfully treated with DSEK. 1. Introduction Keratoglobus is an idiopathic disorder with 360-degree peripheral corneal ectasia resulting in central corneal protrusion [1]. It is associated with ocular conditions such as orbital pseudotumor, vernal keratoconjunctivitis, chronic marginal blepharitis, and glaucoma after penetrating keratoplasty surgery; congenital conditions including Leber congenital amaurosis and blue sclera syndrome; and several connective tissue disorders, for example, Ehlers-Danlos syndrome, Marfan syndrome, and Rubinstein-Taybi syndrome [2–6]. Visual impairment in patients with keratoglobus can be profound, and may occur secondarily to corneal scarring and rupture (due to severe corneal ectasia), irregular astigmatism, and extreme myopia. Hydrops develops due to breaks in the Descemet membrane followed by aqueous infiltration of the stroma and the epithelium in up to 91% of keratoglobus eyes, and can result in corneal scar formation in severe cases [7]. Other reported serious side effects include corneal perforation, microbial keratitis, and glaucoma [8, 9]. Factors predisposing to the development of hydrops include younger age, male gender, advanced corneal
References
[1]
V. Cavara, “Keratoglobus and keratoconus. A contribution to the nosological interpretation of keratoglobus,” The British Journal of Ophthalmology, vol. 34, no. 10, pp. 621–626, 1950.
[2]
F. D. Gillespie, “Congenital amaurosis of leber,” American Journal of Ophthalmology, vol. 61, no. 5, pp. 874–880, 1966.
[3]
S. W. Hyams, H. Kar, and E. Neumann, “Blue sclerae and keratoglobus. Ocular signs of a systemic connective tissue disorder,” British Journal of Ophthalmology, vol. 53, no. 1, pp. 53–58, 1969.
[4]
J. A. Cameron, “Corneal abnormalities in Ehlers-Danlos syndrome type VI,” Cornea, vol. 12, no. 1, pp. 54–59, 1993.
[5]
S. C. Reddy, “Keratoglobus and complicated microphthalmos,” Indian Journal of Ophthalmology, vol. 26, no. 3, pp. 23–26, 1978.
[6]
M. E. Nelson and J. F. Talbot, “Keratoglobus in the Rubinstein-Taybi syndrome,” British Journal of Ophthalmology, vol. 73, no. 5, pp. 385–387, 1989.
[7]
J. A. Cameron, “Keratoglobus,” Cornea, vol. 12, no. 2, pp. 124–130, 1993.
[8]
E. D. Donnenfeld, A. Schrier, H. D. Perry et al., “Infectious keratitis with corneal perforation associated with corneal hydrops and contact lens wear in keratoconus,” British Journal of Ophthalmology, vol. 80, no. 5, pp. 409–412, 1996.
[9]
P. E. Rubsamen and W. M. McLeish, “Keratoconus with acute hydrops and perforation,” Cornea, vol. 10, no. 1, pp. 83–84, 1991.
[10]
S. J. Tuft, W. M. Gregory, and R. J. Buckley, “Acute corneal hydrops in keratoconus,” Ophthalmology, vol. 101, no. 10, pp. 1738–1744, 1994.
[11]
C. Katsoulos, V. Nick, K. Lefteris, and M. Theodore, “Fitting the post-keratoplasty cornea with hydrogel lenses,” Contact Lens and Anterior Eye, vol. 32, no. 1, pp. 22–26, 2009.
[12]
S. Riss, L. M. Heindl, B. O. Bachmann, F. E. Kruse, and C. Cursiefen, “Pentacam-based big bubble deep anterior Lamellar keratoplasty in patients with keratoconus,” Cornea, vol. 31, no. 6, pp. 627–632, 2012.
[13]
D. H. Jones and C. M. Kirkness, “A new surgical technique for keratoglobus-tectonic lamellar keratoplasty followed by secondary penetrating keratoplasty,” Cornea, vol. 20, no. 8, pp. 885–887, 2001.
[14]
R. B. Vajpayee, P. Bhartiya, and N. Sharma, “Central lamellar keratoplasty with peripheral intralamellar tuck: a new surgical technique for keratoglobus,” Cornea, vol. 21, no. 7, pp. 657–660, 2002.
[15]
S. Kaushal, V. Jhanji, N. Sharma, R. Tandon, J. S. Titiyal, and R. B. Vajpayee, “‘Tuck In’ Lamellar Keratoplasty (TILK) for corneal ectasias involving corneal periphery,” British Journal of Ophthalmology, vol. 92, no. 2, pp. 286–290, 2008.
[16]
L. Kodjikian, S. Baillif, C. Burillon, J. D. Grange, and J. G. Garweg, “Keratoglobus surgery: penetrating keratoplasty redux,” Acta Ophthalmologica Scandinavica, vol. 82, no. 5, pp. 625–627, 2004.
[17]
A. J. Kanellopoulos and L. H. Pe, “An alternative surgical procedure for the management of keratoglobus,” Cornea, vol. 24, no. 8, pp. 1024–1026, 2005.
[18]
M. A. Javadi, M. R. Kanavi, M. Ahmadi, and S. Yazdani, “Outcomes of epikeratoplasty for advanced keratoglobus,” Cornea, vol. 26, no. 2, pp. 154–157, 2007.
[19]
J. A. Cameron, J. B. Cotter, J. M. Risco, and H. Alvarez, “Epikeratoplasty for keratoglobus associated with blue sclera,” Ophthalmology, vol. 98, no. 4, pp. 446–452, 1991.
[20]
S. Grewal, P. R. Laibson, E. J. Cohen et al., “Acute hydrops in the corneal ectasias: associated factors and outcomes,” Transactions of the American Ophthalmological Society, vol. 97, pp. 187–203, 1999.
[21]
S. Kaushal, N. Sharma, and R. B. Vajpayee, “Treatment of acute corneal hydrops with intracameral C3 F8 in a patient of pellucid marginal degeneration with keratoglobus,” Clinical and Experimental Ophthalmology, vol. 35, no. 8, pp. 697–699, 2007.
[22]
C. Kiire and S. Srinivasan, “Management of bilateral acute hydrops secondary to keratoglobus with perfluoroethane (C2F6) pneumodescemetopexy,” Clinical and Experimental Ophthalmology, vol. 37, no. 9, pp. 892–894, 2009.
[23]
S. Basu, P. K. Vaddavalli, M. Ramappa, S. Shah, S. I. Murthy, and V. S. Sangwan, “Intracameral perfluoropropane gas in the treatment of acute corneal hydrops,” Ophthalmology, vol. 118, no. 5, pp. 934–939, 2011.
[24]
M. S. Gorovoy, I. R. Gorovoy, S. Ullman, and J. B. Gorovoy, “Descemet stripping automated endothelial keratoplasty for spontaneous descemet membrane detachment in a patient with osteogenesis imperfecta,” Cornea, vol. 31, no. 7, pp. 832–835, 2012.
