Acute Hydrops Research Articles

Acute Corneal Hydrops in Children with Primary Infantile Glaucoma: A Report of 31 Cases over 23 Years at the LVPEI.

PurposeRelatively little data exist regarding the outcomes of children with primary infantile glaucoma presenting with acute corneal hydrops. The aim of our study was to determine the surgical outcome of children of infantile glaucoma who presented with acute corneal hydrops.MethodsIn total, 38 eyes of 31 consecutive children of infantile glaucoma presented with acute corneal hydrops who underwent primary combined trabeculotomy-trabeculectomy (CTT) by a single surgeon from January 1990 to December 2012 at the LV Prasad Eye Institute (LVPEI), a tertiary eye care centre in Southern India were enrolled in this retrospective study. Primary outcome measures were intraocular pressure (IOP) control (IOP ≤ 16 mmHg under anaesthesia or IOP ≤ 21 mmHg without anaesthesia) and clearance of corneal edema. Secondary outcome measures were visual acuity (VA), corneal diameter, bleb appearance, intraoperative and postoperative complications.ResultsMean age at presentation was 6.4 months (range, 2–11 months) and seven eyes (23%) had bilateral affliction. At presentation, all eyes (100%) had moderate to severe degree of corneal edema with a mean preoperative IOP of 25.6 ±5.1 mmHg. Postoperatively, the IOP reduced to 12.0 ± 3.8 mmHg (difference = -13.6, 95% CI = -15.7 to -11.5, t = -13.18, p<0.0001), and the percentage reduction in IOP was 53.05%. Preoperatively 83% of the eyes were on antiglaucoma medication, and postoperatively 2 eyes (5.3%) required 1 antiglaucoma medication for control of IOP. Preoperatively, corneal edema was present in all eyes and postoperatively it cleared in all of them. Significant myopic astigmatism was present in 28 eyes (74%), the commonest being compound myopic astigmatism (75%) followed by simple myopic astigmatism (21%). Normal VA (best-corrected VA; BCVA ≥ 20/60) was achieved in 44.4% of the eyes and 22.2% eyes had low vision (BCVA, <20/60 to 20/400). Complete success (IOP control and clearance of corneal oedema) was obtained in 94.7% eyes. There were no significant intraoperative or postoperative complications. Two thirds of the patients showed low, elevated functional filtering bleb. No patient had any bleb leak, blebitis or bleb related endophthalmitis. The median follow-up was 36 months (range 2–228 months).ConclusionsPrimary CTT is safe and effective in controlling IOP, resulting in complete clearance of corneal edema with modest visual improvement in children of infantile glaucoma presenting with acute corneal hydrops. The outcome of the study will have a positive impact on counseling the parents preoperatively.

Use of a nictitating membrane flap for treatment of feline acute corneal hydrops-21 eyes.

To evaluate the effectiveness of the use of a nictitating membrane flap (NMF) as therapy in 19 cats (21 eyes) affected with feline acute corneal hydrops (FACH). Medical records from 19 cats diagnosed with FACH and treated with a NMF were retrospectively evaluated. Information was collected from multiple veterinary hospitals and included signalment, medical history, therapy, and ocular outcome. Breeds included 13 Domestic Shorthairs, 2 Exotic Shorthairs, 2 Maine Coons, 1 Persian, and 1 Domestic Medium Hair. Two cats were bilaterally affected. Median age of cats was 3.2 years (range 0.26-15 years). Eleven patients were spayed females, 6 were neutered males, and 2 were intact males. Topical steroids were previously administered in 5 (23.8%) eyes; oral steroids were previously administered in 7 cats (36.8% of patients); three patients received both oral and topical steroids. Thirteen of 21 (61.9%) eyes had a history of ocular disease including ulcerative and nonulcerative keratitis, anterior uveitis, corneal sequestrum, conjunctivitis, and glaucoma. Median duration of NMF was 15 days (range 6-30 days). Follow-up ranged from 12 to 1601 days (median 169 days). Corneal perforation occurred in 1 (4.7%) eye and was successfully repaired. One lesion (4.7%) in a diabetic patient did not resolve. Nineteen of the treated eyes (90.5%) resolved with no complications. A nictitating membrane flap successfully treated 90.5% of FACH eyes (89.5% of patients).

Deep anterior lamellar keratoplasty using the same graft after epikeratophakia for the treatment of acute corneal hydrops in keratoconus

Objective To evaluate the clinical effects of deep anterior lamellar keratoplasty (DALK) using the same graft after epikeratophakia for the treatment of acute corneal hydrops in keratoconus. Methods A consecutive 7 cases (7 eyes) with acute corneal hydrops in keratoconus in our department were retrospectively analyzed. Same corneal graft was used to perform the first stage epikeratophakia combined with C3F8 anterior chamber injection. About 6 months after the first surgery, the secondary stage deep anterior lamellar keratoplasty combined with air anterior chamber injection was performed. The best corrected visual acuity, healing status of the anterior and posterior corneal surface, anterior segment optical coherence tomography parameters, and corneal endothelial cells density were recorded. Results The central corneal thickness was (1764±256) μm before the treatment, and it was (778±147) μm, (604±23) μm, (579±20) μm and (525±19) μm at the time points of 1 d, 7 d, 1 and 6 months after the first stage surgery respectively. An average (6.1±1.3) months after the first stage surgery, the second stage DALK was performed. The descemet's membrane at the central area could be fully exposed in 3 eyes. Slow liquid exudations in the deep corneal stroma near the descemet's membrane were found in the other 4 eyes during stroma dissection. DALKs were successfully performed in all the 7 cases. Double anterior chambers were found in 2 eyes 1d after DALK. The whole corneal thicknesses were (781±57) μm, (581±49) μm and (521±19) μm at 7 d, 1 and 3 months after DALK respectively. Three months later, the thickness of the receptors' residual stroma bed was (21±11) μm at the central corneal area. At the end of the follow-up, the BCVA(LogMAR)was 0.11±0.03. Conclusion Novel curative effect was found in the treatment of acute corneal hydrops in keratoconus by deep anterior lamellar keratoplasty using the same graft after epikeratophakia. Key words: Acute corneal hydrops, keratoconus; Epikeratophakia; Keratoplasty, deep anterior lamellar; Anterior segment optical coherence tomography; Descemet's membrane

Large Penetrating Keratoplasty in the Management of Keratoglobus: A Case Report

Background: Keratoglobus is a rare noninflammatory corneal disorder characterized by diffuse corneal thinning and globular protrusion of the cornea. Surgical management of keratoglobus is challenging and the standard method has not yet been defined. Aim: To present the role of large penetrating keratoplasty (PK) in the management of keratoglobus. Case Presentation: A 29-year-old male patient with bilateral keratoglobus presented with acute corneal hydrops in his right eye following extensive Descemet’s membrane rupture, with a visual acuity in this eye limited to hand movement. Peripheral cornea was extremely thin and blue sclera was present. Acute hydrops was managed conservatively at this stage and two months later large PK was performed in the right eye using 9.5 mm diameter graft over a 9 mm patient’s cornea trephination. Minor aqueous leakage was seen on the first postoperative day, managed with 2 more interrupted 10.0 nylon sutures. No further complications were noticed and postoperative course was uneventful. Fifteen months postoperatively the graft was clear and best corrected visual acuity was 20/60. Conclusions: Large penetrating keratoplasty has an important role in the management of keratoglobus, in cases where peripheral tuck-in lamellar keratoplasty or epikeratoplasty present serious intraoperative difficulties in host lamellar dissection and in stabilizing the graft due to extensive peripheral corneal and scleral thinning.

Measurement of the Mechanical Deformation of Organ of Corti in a Model of Acute Endolymphatic Hydrops

Endolymphatic hydrops (EH) represents a histopathologic finding in which the structures surrounding the scala media are distended by increased endolymphatic volume. It is acknowledged as important substrates of Meniere’s disease (MD). This finding was initially reported independently by Hallpike and Cairnes in England and Yamakawa in Japan in the temporal bones of patients with MD. Dispensability of structures which surround the scala media, such as Reissner’s membrane, stria vascularis, the organ of Corti of the cochlea, saccule, utricle and semicircular canals of the vestiMeasurement of the Mechanical Deformation of Organ of Corti in a Model of Acute Endolymphatic Hydrops

Intraoperative outcome of deep anterior lamellar keratoplasty using femtosecond laser versus manual trephine

Purpose The aim of this study was to compare the intraoperative data of femtosecond laser-assisted deep anterior lamellar keratoplasty (F-DALK) with those of manual trephine deep anterior lamellar keratoplasty (M-DALK). Setting The study was conducted in a single private centre (Alfat’h Eye Hospital). Design This is a nonrandomized comparative retrospective, single private centre clinical study. Materials and Methods The study was conducted between February 2013 and March 2016 on 40 eyes that underwent M-DALK and 24 eyes that underwent F-DALK, including moderate-to-advanced keratoconus (KC) intolerant to contact lenses, superficial corneal opacities or degenerations, and stromal corneal dystrophies (e.g. macular dystrophies, etc.) with best spectacle corrected visual acuity (BSCVA) less than or equal to 6/60 (0.1), and excluding those with KC (with acute hydrops or posthydrops scars), corneal opacities or corneal dystrophies involving the endothelium. The intraoperative data were retrieved and analysed. Results F-DALK had fewer intraoperative complications compared with M-DALK, such as the decentred cut, which was 0% in F-DALK versus 1/40 (2.5%) in M-DALK, with a statistically significant difference in the overall intraoperative complications between the two procedures (P=0.004). Conclusion The F-DALK technique results in fewer intraoperative complications such as the inadvertent premature entry of the anterior chamber, a better centration of the cuts in patients with KC, and more success of the big bubble formation and thereby less conversion to penetrating keratoplasty or to manual dissection, which has a lower visual outcome.

An Immunohistochemical Study of Inflammatory Cell Changes and Matrix Remodeling With and Without Acute Hydrops in Keratoconus.

To determine the inflammatory cell and matrix changes in advanced keratoconus, including acute hydrops, using immunohistochemical analysis. The corneal tissue from eight subjects with keratoconus undergoing corneal transplantation (three keratoconic buttons, five buttons post acute hydrops—one of them with extensive neovascularization following hydrops) was compared with tissue from two normal corneoscleral rims (n = 10). The corneas were sectioned and analyzed with specific markers for macrophages, lymphocytes, dendritic cells, and scar associated matrix molecules laminin, fibronectin, tenascin-C, and type III collagen. Populations of cells using markers for macrophages, leucocytes and antigen presenting cells were found to be associated with the epithelium and stroma of keratoconic tissue. Populations of these cells appeared decreased in hydrops-associated keratoconus except for a large increase in leucocytes in the stroma and endothelium associated with neovascularization. Extracellular matrix deposition was found to be uniquely demonstrated in localized areas of the stroma, corresponding to the site of hydrops involvement. Immunohistochemical analysis revealed a chronic, inflammatory process with recruitment of immunoinflammatory cells and deposition of scar tissue in keratoconus. The inflammatory markers were somewhat attenuated in hydrops-associated keratoconus corneas and thus inflammation was not considered to be a major factor in the development of acute corneal hydrops.

Anatomic Predictive Factors of Acute Corneal Hydrops in Keratoconus: An Optical Coherence Tomography Study

To define the optical coherence tomography (OCT) corneal changes predisposing to acute corneal hydrops among patients with advanced keratoconus. Retrospective cohort study. A total of 191 advanced keratoconic eyes from 191 patients with advanced keratoconus cases were studied. Data collected from patients with advanced keratoconus cases were studied during a minimum period of 24 months of follow-up. High-resolution Fourier-domain corneal OCT (5 μm of axial resolution) and corneal topography were performed every 4 months during the follow-up. Several anatomic features at the keratoconus cone were analyzed with OCT, including epithelial and stromal thicknesses, the aspect of Bowman's layer, the presence of Vogt's striae, and stromal opacities. A comparative analysis between anatomic corneal features in eyes that developed corneal hydrops and those that did not develop this complication during the follow-up was performed. Evaluation of anatomic corneal changes at risk of developing a corneal hydrops on the basis of OCT findings. Eleven cases of corneal hydrops (5.8%) occurred in our series during a mean follow-up of 30 months (24-36 months). All of these patients were male and younger (23.7±5.9 years) than patients with no acute keratoconus (32.7±11.3 years). Increased epithelial thickening with stromal thinning at the conus and the presence of anterior hyperreflectives at the Bowman's layer level were significantly associated with corneal hydrops, whereas the presence of corneal scarring was a preventive factor. At the healing stage, a pan-stromal scar occurs, with a significant stromal thickening and cornea flattening. Increased epithelial thickening, stromal thinning at the keratoconus cone, anterior hyperreflectives at the Bowman's layer level, and the absence of stromal scarring are associated with a high risk of developing corneal hydrops. These aspects should be taken into account by the clinician in the evaluation of keratoconus eyes and in the planning of corneal keratoplasty.

Management of corneal hydrops using air and micro-punctures

Case reportA 22-year-old woman with an acute hydrops and Descemet membrane detachment was managed by intracameral air injection combined with multiple corneal stromal venting incisions for the drainage of intrastromal fluid. No intraoperative complications were encountered, and the intrastromal fluid was resolved over 2 weeks. DiscussionAcute corneal hydrops is a significant complication of keratoconus. If not treated, resolution usually occurs over a period of 2–4 months. The persistent edema can cause complications, such as corneal neovascularisation, infection and corneal perforation.

Acute corneal hydrops in keratoconus: a national prospective study of incidence and management.

PurposeTo determine incidence and management of acute corneal hydrops in the UK.MethodsWe used the BOSU report card system to survey cases of acute corneal hydrops in patients with keratoconus that occurred in the UK between November 2009 and December 2010. Ophthalmologists who reported a case were sent an initial questionnaire, with a follow-up questionnaire after 6 months. We collected information on the demographics, complications, changes in visual acuity, and management. The 2011 National Census was used as a source for population and ethnicity in the UK.ResultsThere were 73 incident cases of acute corneal hydrops, with a response to the initial questionnaire for 64 (88%) patients and follow-up data at 6 months for 57 (78%) patients. For the 64 confirmed cases the median (interquartile range) age of onset was 31.9 (23.2, 41.3) years and 48 (75%) of the cases occurred in males. A total of 42 (66%) patients were white, 14 (22%) were South Asian, and 7 (11%) were black. The proportion of South Asian and black patients with acute corneal hydrops was significantly higher than in the general population (P<0.001). The minimum estimated annual incidence of acute corneal hydrops in patients with keratoconus was estimated to be 1.43 (1.10, 1.83) per 1000. At 6 months following acute corneal hydrops a decision to proceed with keratoplasty had been made for 12 (20.3%) patients.ConclusionsThis is the first population-based estimate of the incidence of acute corneal hydrops in keratoconus.

Efficacy and safety of pre-Descemet's membrane sutures for the management of acute corneal hydrops in keratoconus

AimsThe aim of this study was to assess the efficacy and safety of pre-Descemet's membrane (DM) sutures associated with intracameral air injection for management of acute corneal hydrops associated with...

Acute corneal hydrops mimicking infectious keratitis as initial presentation of keratoconus in a 10-year-old child.

Purpose. To report a case of acute hydrops in a 10-year-old child with advanced keratoconus. Case Presentation. A ten-year-old boy diagnosed as having right eye (RE) infectious keratitis, not responding to antimicrobial therapy, was referred to our hospital. The diagnosis of infectious keratitis was established one month prior to his presentation following an episode of acute corneal whitening, pain, and drop in visual acuity. Topical fortified antibiotics followed by topical antiviral therapy were used with no improvement. Slit lamp examination showed significant corneal protrusion with edema surrounding a rupture in Descemet's membrane in the RE. The diagnosis of acute corneal hydrops from advanced keratoconus was highly suspected and confirmed with corneal topography. Conclusion. Although a relatively rare disease at the age of 10 years, keratoconus can be rapidly progressive in the pediatric group. Keratoconus should always be considered in the differential diagnosis of progressive vision loss in this age group.

Herpes simplex Keratitis Mimicking Acute Corneal Hydrops

Purpose: The purpose of this paper is to present a case of a Herpes simplex keratitis presenting as a case of acute corneal hydrops. Methods: Case report. Results: A 57-year-old white woman presented with acute onset of pain, photophobia and decreased vision in the left eye. In the years prior, she had been under the care of her optometrist, who had previously diagnosed her with irregular astigmatism, for which she had been using rigid gas permeable contact lenses for vision correction. At the slit-lamp biomicroscope, the left eye was found to have marked corneal edema with central endothelial folds, as well as a round, paracentral area of stromal haze and opacity. The fellow eye was shown to have inferior areas of anterior stromal scarring. Corneal topography showed markedly irregular astigmatism, with inferior steepening bilaterally. These are findings that could also be seen in acute corneal hydrops. In this case, a diagnosis of Herpes Simplex keratitis was made, and patient showed marked improvement on oral valacyclovir. Conclusions: Herpes simplex keratitis can often be a diagnostic challenge, as it can present itself in various forms. We report a case of Herpes simplex keratitis mimicking acute corneal hydrops.

Corneal Hydrops in Keratoconus

ABSTRACT Purpose The purpose of this review is to outline the etiology, clinical features, and management of acute corneal hydrops (CH) in cases of keratoconus (KC). Recent findings The advent of newer investigative modalities like ultra biomicroscopy, anterior segment optical coherence tomography and confocal microscopy has contributed toward the diagnosis, treatment planning and following the course of therapy in cases of acute hydrops. Summary Corneal hydrops is an acute complication of keratoconus which in most instances resolves spontaneously. However, prolonged corneal edema can lead to complications, such as corneal neovascularization which can jeopardise a future corneal graft. Hence, timely intervention is required in most cases to prevent such complications as well as for early visual rehabilitation. Intracameral gas injection is the most commonly performed surgical procedure for hydrops. Modifications in surgical technique can help to tackle difficult situations. How to cite this article Maharana PK, Nagpal R, Sharma N. Corneal Hydrops in Keratoconus. Int J Kerat Ect Cor Dis 2015;4(2):52-55.

Brittle Cornea Syndrome: Case Report with Novel Mutation in the PRDM5 Gene and Review of the Literature.

A 3-year-old boy presented with acute corneal hydrops on the left eye and spontaneous corneal rupture on the right eye. A diagnosis of brittle cornea syndrome was confirmed by molecular analysis. A novel mutation, the homozygous variant c.17T>G, p.V6G, was found in the gene for PR-domain-containing protein 5 (PRDM5) in exon 1. Brittle cornea syndrome is a rare connective tissue disease with typical ocular, auditory, musculoskeletal, and cutaneous disorders. Almost all patients suffer from declined vision due to corneal scarring, thinning, and rupture. The most common ophthalmologic findings include keratoconus, progressive central corneal thinning, high myopia, irregular astigmatism, retinal detachment, and high risk for spontaneous corneal or scleral rupture. In addition to describing the case with a novel mutation here we review the current literature on brittle cornea syndrome pathogenesis, clinical findings, and therapy.

Manejo del hydrops corneal mediante aire y micropunciones

Case reportA 22-year- old woman with an acute hydrops and Descemet membrane detachment was managed by intracameral air injection combined with multiple corneal stromal venting incisions for the drainage of intrastromal fluid. No intraoperative complications were encountered, and the intrastromal fluid was resolved over 2 weeks. DiscussionAcute corneal hydrops is a significant complication of keratoconus. If not treated, resolution usually occurs over a period of 2-4 months. The persistent oedema can cause complications, such as corneal neovascularisation, infection and corneal perforation.

Apport de l’OCT de segment antérieur « en face » dans le kératocône aigu

To study the corneal morphological characteristics of acute hydrops by analyzing anterior segment optical coherence tomography (AS-OCT) "en face" images. Four patients presenting with acute hydrops were examined at different stages, respectively after 1 day, 1 week, 3 weeks and 6 months. All patients had a complete ophthalmic evaluation including "en face" AS-OCT examination. In vivo confocal microscopy (IVCM) and histological analysis of the cornea obtained after penetrating keratoplasty were also performed in one patient. The "en face" AS-OCT showed that the early edema in acute hydrops corresponded to multiple epithelial microcysts associated with large intra-stromal lacunae. We also observed that, in association with the disappearance of the microcysts and lacunae, "en face" OCT demonstrated subepithelial scars in the form of weakly reflective, fine, interlaced linear opacities. These opacities subsequently increased in thickness resulting in a dense subepithelial network. These findings were consistent with the results of histological and IVCM studies. "En face" AS-OCT allows for precise study of the tissue changes occurring in acute corneal hydrops. Besides a better understanding of this rare complication of keratoconus, this new imaging technique may help clinicians to identify the corneal structural changes, which place keratoconus patients at risk of corneal hydrops.

Mechanisms for acute corneal hydrops and perforation.

Acute corneal hydrops (ACH) and perforation in corneal thinning diseases are the consequences of exposure to distending intraocular pressure (IOP) forces that are in excess of corneal resistance to them. Apart from thinning, resistance to these forces may be reduced by disease-related tissue changes, such as corneal scarring, which could lower resistance to IOP. Eye rubbing trauma has sometimes been found to be associated with ACH and perforation. This association is not surprising given that the combination of rubbing-related mechanical tissue trauma and the associated increased distending stress of higher IOP seem likely to increase the risk of complications. Many cases of ACH and perforation are described as spontaneous, but this classification may be the consequence of not considering the multiple potential mechanisms for IOP elevation such as coughing, sneezing, nose blowing, and sneeze suppression in addition to those related to eye rubbing/wiping/massaging/touching as well as changes in body orientation, strenuous exercise, and wearing swimming goggles for example. Classification of ACH or perforation as spontaneous may lead patients to assume that nothing can be done to avoid these complications. Patients with corneal thinning diseases who are counseled regarding the potential precipitating mechanisms for IOP elevation will have the opportunity of reducing exposure to them and the risk of the associated complications. In addition, when ACH or perforation occur, faster resolution of edema and wound healing may depend on reducing potentially exacerbating exposures to mechanisms for IOP elevation.

Pneumodescemetopexy with perfluoroethane (C2F6) for the treatment of acute hydrops secondary to keratoconus.

To evaluate the efficacy and safety of pneumodescemetopexy with intracameral perfluoroethane (C2F6) gas for the treatment of acute hydrops secondary to keratoconus. Retrospective, non-comparative, interventional case series. Eight eyes of eight patients who presented with acute hydrops secondary to keratoconus between July 2009 and September 2013 were consecutively recruited. All were treated with intracameral 14% isoexpansile concentration of C2F6. Preoperative and postoperative best-corrected visual acuities (BCVA), intraoperative and postoperative complications, and time taken for resolution of corneal oedema were assessed. All the patients, except for one, were followed up for 1 year. The mean age of the cohort was 29.1±13.5 years. BCVA at presentation was 6/60 or less in all patients. Improvement of BCVA was achieved postoperatively in seven (87.5%) patients, with three (37.5%) patients achieving a BCVA of 6/18. The average time between initial presentation and complete resolution of corneal oedema was 60.0±32.1 days. The C2F6 gas persisted in the anterior chamber between 6 and 8 days. All the patients required only one injection during the treatment period. There was no intraoperative or postoperative complication noted during the follow-up period. Pneumodescemetopexy with intracameral isoexpansile concentration of C2F6 gas serves as a safe and effective treatment modality for patients with acute hydrops secondary to keratoconus.

A prospective study of acute corneal hydrops by in vivo confocal microscopy in a New Zealand population with keratoconus

Background/aimsTo analyse the clinical and microstructural changes during the course of acute corneal hydrops in keratoconus.MethodsA prospective study of consecutive patients presenting with acute corneal hydrops over 12 months was...