Corneal Hydrops – Aetiology and Advanced Therapeutic Strategies

Abstract

Acute hydrops refers to sudden corneal edema caused by rupture of Descemetʼs membrane (DM) – often in progressive keratectasia. It leads to a sudden decrease in visual acuity, pain, and foreign body sensation as well as an increased glare sensation. Acute hydrops usually heals with scarring within months, but complications such as corneal perforation, infectious keratitis, and corneal vascularization may occur. The prevalence in keratoconus patients is 2.6 to 2.8%. Risk factors include keratoconjunctivitis vernalis, atopic dermatitis, high keratometry, male gender, and eye rubbing. Keratoplasty should be avoided in the acute phase. The prognosis of the graft is reduced, and after scar healing of the hydrops, wearing contact lenses or glasses may be possible again. Conservative therapy alone with lubricants and hyperosmolar eye drops, prophylactic antibiotic eye drops to prevent superinfection, and topical steroids was long considered the only possible form of treatment. However, healing under conservative therapy takes an average of over 100 days. In the meantime, there are different surgical strategies that rapidly shorten the healing and thus the recovery phase of the patients to a few days. If the DM is detached without tension, a simple injection of gas into the anterior chamber can already lead to reattachment and thus to almost immediate deswelling of the cornea. If the DM is under tension, predescemetal sutures combined with a gas injection into the anterior chamber can flatten the cornea and reattach the DM. Mini-Descemet membrane endothelial keratoplasty (mini-DMEK) allows for sutureless closure of the DM defect by transplantation of a small (< 5 mm) graft. In cases of particularly large DM tears and very pronounced hydrops, suture loosening and relapse may occur after the placement of predescemetal sutures. Mini-DMEK can then lead to permanent healing, but in contrast to simple corneal sutures, it is usually performed under general anesthesia and by aid of intraoperative optical coherence tomography. The very good results with regard to the rapid healing prove that surgical therapy makes sense in the vast majority of patients with acute hydrops and should be initiated quickly.

Key words

acute hydrops - corneal edema - predescemetal sutures - mini-DMEK

Publication History

Received: 20 January 2023

Accepted: 21 February 2023

Article published online:
05 May 2023

• References/Literatur

• 1 Rychener R, Kirby D. Acute hydrops of the cornea complicating keratoconus. Arch Ophthalmol 1940; 24: 326-343
• 2 Fuentes E, Sandali O, El Sanharawi M. et al. Anatomic Predictive Factors of Acute Corneal Hydrops in Keratoconus: An Optical Coherence Tomography Study. Ophthalmology 2015; 122: 1653-1659
• 3 Donnenfeld ED, Schrier A, Perry HD. et al. Infectious keratitis with corneal perforation associated with corneal hydrops and contact lens wear in keratoconus. Br J Ophthalmol 1996; 80: 409-412
• 4 Rowson NJ, Dart JK, Buckley RJ. Corneal neovascularisation in acute hydrops. Eye (Lond) 1992; 6: 404-406
• 5 Barsam A, Petrushkin H, Brennan N. et al. Acute corneal hydrops in keratoconus: a national prospective study of incidence and management. Eye (Lond) 2015; 29: 469-474
• 6 Schiessl G, Suffo S, Milioti G. et al. [Muraine sutures accelerate healing of corneal hydrops in acute keratotorus]. Ophthalmologe 2019; 116: 665-668
• 7 Avgitidou G, Siebelmann S, Bachmann B. et al. Brittle Cornea Syndrome: Case Report with Novel Mutation in the PRDM5 Gene and Review of the Literature. Case Rep Ophthalmol Med 2015; 2015: 637084
• 8 Bachmann B, Handel A, Siebelmann S. et al. Mini-Descemet Membrane Endothelial Keratoplasty for the Early Treatment of Acute Corneal Hydrops in Keratoconus. Cornea 2019; 38: 1043-1048
• 9 Handel A, Siebelmann S, Hos D. et al. Comparison of Mini-DMEK versus predescemetal sutures as treatment of acute hydrops in keratoconus. Acta Ophthalmol 2021; 99: e1326-e1333
• 10 Handel A, Siebelmann S, Matthaei M. et al. Mini-DMEK for the Treatment of Chronic Focal Corneal Endothelial Decompensation. Cornea 2023; 42: 12-19
• 11 Simpson GV. Corneal edema. Trans Am Ophthalmol Soc 1949; 47: 692-737
• 12 Sridhar MS, Mahesh S, Bansal AK. et al. Pellucid marginal corneal degeneration. Ophthalmology 2004; 111: 1102-1107
• 13 Carter JB, Jones DB, Wilhelmus KR. Acute hydrops in pellucid marginal corneal degeneration. Am J Ophthalmol 1989; 107: 167-170
• 14 Barsam A, Brennan N, Petrushkin H. et al. Case-control study of risk factors for acute corneal hydrops in keratoconus. Br J Ophthalmol 2017; 101: 499-502
• 15 Amsler M. [Some data on the problem of keratoconus]. Bull Soc Belge Ophtalmol 1961; 129: 331-354
• 16 Tuft SJ, Gregory WM, Buckley RJ. Acute corneal hydrops in keratoconus. Ophthalmology 1994; 101: 1738-1744
• 17 Fan Gaskin JC, Good WR, Jordan CA. et al. The Auckland keratoconus study: identifying predictors of acute corneal hydrops in keratoconus. Clin Exp Optom 2013; 96: 208-213
• 18 Varshney T, Goel S, Bafna RK. et al. Rapid spontaneous resolution of corneal hydrops in post-CXL keratitis. BMJ Case Rep 2021; 14: e246141
• 19 Jovanovic V, Nikolic L, Seiler TG. et al. Acute hydrops followed by corneal perforation five years after corneal cross-linking for keratoconus. Arq Bras Oftalmol 2022;
• 20 Stock RA, Thume T, Bonamigo EL. Acute corneal hydrops during pregnancy with spontaneous resolution after corneal cross-linking for keratoconus: a case report. J Med Case Rep 2017; 11: 53
• 21 Ting DSJ, Bandyopadhyay J, Patel T. Microbial keratitis complicated by acute hydrops following corneal collagen cross-linking for keratoconus. Clin Exp Optom 2019; 102: 434-436
• 22 Monteiro T, Alfonso JF, Franqueira N. et al. Comparison of clinical outcomes between manual and femtosecond laser techniques for intrastromal corneal ring segment implantation. Eur J Ophthalmol 2020; 30: 1246-1255
• 23 Monteiro T, Alfonso JF, Freitas R. et al. Comparison of Complication Rates between Manual and Femtosecond Laser-Assisted Techniques for Intrastromal Corneal Ring Segments Implantation in Keratoconus. Curr Eye Res 2019; 44: 1291-1298
• 24 Ontiveros-Holguin A, Pacheco-Padron J. Successful Management of Corneal Hydrops and Intrastromal Corneal Ring Segment (ICRS) Migration Following ICRS Implantation for Keratoconus. Am J Case Rep 2022; 23: e936897
• 25 Ghajarnia M, Moshirfar M, Mifflin MD. Descemet detachment after femtosecond-laser-assisted placement of intrastromal ring segments in pellucid marginal degeneration. J Cataract Refract Surg 2008; 34: 2174-2176
• 26 Guell JL, Verdaguer P, Elies D. et al. Acute corneal hydrops after intrastromal corneal ring segment implantation for keratoconus. J Cataract Refract Surg 2012; 38: 2192-2195
• 27 Al Suhaibani AH, Al-Rajhi AA, Al-Motowa S. et al. Inverse relationship between age and severity and sequelae of acute corneal hydrops associated with keratoconus. Br J Ophthalmol 2007; 91: 984-985
• 28 Basu S, Vaddavalli PK, Vemuganti GK. et al. Anterior segment optical coherence tomography features of acute corneal hydrops. Cornea 2012; 31: 479-485
• 29 Basu S, Vaddavalli PK, Ramappa M. et al. Intracameral perfluoropropane gas in the treatment of acute corneal hydrops. Ophthalmology 2011; 118: 934-939
• 30 Rajaraman R, Singh S, Raghavan A. et al. Efficacy and safety of intracameral perfluoropropane (C3F8) tamponade and compression sutures for the management of acute corneal hydrops. Cornea 2009; 28: 317-320
• 31 Yahia Chérif H, Gueudry J, Afriat M. et al. Efficacy and safety of pre-Descemetʼs membrane sutures for the management of acute corneal hydrops in keratoconus. Br J Ophthalmol 2015; 99: 773-777
• 32 Seitz B, Daas L, Hamon L. et al. [Stage-appropriate treatment of keratoconus]. Ophthalmologe 2021; 118: 1069-1088
• 33 Siebelmann S, Handel A, Matthaei M. et al. Microscope-Integrated Optical Coherence Tomography-Guided Drainage of Acute Corneal Hydrops in Keratoconus Combined With Suturing and Gas-Aided Reattachment of Descemet Membrane. Cornea 2019; 38: 1058-1061
• 34 Tu EY. Descemet Membrane Endothelial Keratoplasty Patch for Persistent Corneal Hydrops. Cornea 2017; 36: 1559-1561