Acute Corneal Hydrops In Keratoconus Research Articles

Acute Corneal Hydrops in Keratoconus Coinciding With COVID-19 Infection.

To the Editor: Acute corneal hydrops (AH), characterized by corneal stromal edema after a Descemet membrane rupture that allows aqueous humor entry, is observed in cases of keratoconus. Patients typically present with rapidly deteriorating visual acuity, photophobia, and pain. A timely diagnosis and management are essential to ruling out other vision-threatening pathologies such as corneal edema, infectious keratitis, uveitis, Fuchs endothelial dystrophy, postoperative edema, and acute transplant rejection. Here we describe a case of AH in a patient with keratoconus coinciding with coronavirus disease 2019 (COVID-19) infection and emphasize the importance of patient education in the context-restricted face-to-face consultations. A 20-year-old healthy man was evaluated regularly every 3 months for keratoconus using visual acuity and refraction assessments and anterior segment optical coherence tomography imaging. He wore corneal rigid gas-permeable contact lenses in both eyes. He also had a mild ocular allergy but no atopy or habit of eye-rubbing. There was no family history of keratoconus. A year earlier, he had an episode of AH in his left eye. While his left eye stabilized after pressure patching, the keratoconus progressed in his right eye. Examination findings at his last visit before the COVID-19 diagnosis included: corrected distance visual acuity with a gas-permeable contact lens of 20/30 (right) and 20/16 (left), anterior keratometry of 66.4 D (right) and 43.5 D (left), the thinnest corneal thickness of 264 mm (right) and 468 mm (left), and posterior “best-fit sphere” 9-mm radius values of 4.88 (right) and 6.46 mm (left). Approximately 2 months after the last follow-up, he developed flu-like symptoms (sore throat and fever) and was diagnosed the next day with COVID-19 on polymerase chain reaction. Two days after symptom onset, he noticed sudden deterioration of vision in his right eye and called our hospital. The cornea specialist in the ophthalmology department made a tentative phone diagnosis of AH based on his ocular history and instructed him to apply a pressure patch with ophthalmic ointment. He was examined at our hospital after 10 days of quarantine. The corrected distance visual acuity of the right eye was 20/2000, while a slitlamp examination revealed corneal protrusion and marked corneal epithelial as well as stromal edema in the right eye. Fluorescein corneal observation showed no epithelial abnormality, and a definitive AH diagnosis was made. Optical coherence tomography imaging revealed a break in Descemet membrane with overlying corneal edema (Supplemental Digital Content Fig. 1, https://links.lww.com/APJO/A179). No abnormality was noted in the left eye. He had received the second COVID-19 vaccine dose 1 month before the viral infection and reported experiencing no vaccine-related complications. On examination a month later, the AH in the right eye had remarkably improved. Various ophthalmic management and care delivery experiences have been reported during the COVID-19 pandemic. Perspectives in corneal crosslinking were reported relating to keratoconus in particular.1,2 To the best of our knowledge, our case is the first of AH with concomitant keratoconus and COVID-19 infection, although it was not possible to demonstrate a clear association among them. A previous history of AH in either eye is a predisposing risk factor for AH.3 This case underscores the importance of informing patients of their disease status and the need to undergo regular examinations. However, patients with COVID-19 may not be able to receive a timely ophthalmic examination. This patient was distressed until the postquarantine ophthalmic examination was performed; however, his previous experience with AH including successful pressure patching was helpful for the clinician and patient, who was better able to understand the extent of his condition and follow the provided instructions. In the absence of a clinical history of keratoconus, effectively managing AH before a face-to-face examination could be performed would have been challenging. Here are summary points of consideration when educating a patient with an acute event during a pandemic setting: Recognize the patient’s distress due to being in isolation during the acute event. Provide clear information about the disease and instructions for its independent management during quarantine. Strict hand hygiene should be thoroughly practiced while applying the pressure patch and patients should keep unwashed hands away from their face at times. Remote management may require conservative management methods. This requires careful consideration, particularly in acute ophthalmic cases. Although keratoconus was previously considered a noninflammatory ectatic corneal disease, recent evidence shows a relationship between its pathogenesis and chronic inflammatory events.4,5 Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection is known to cause a proinflammatory state.6 There are several reports of corneal graft rejection in patients infected with COVID-19.6–8 Large population-based case-control studies are needed to investigate the possible association between AH in keratoconus and the SARS-CoV-2 cytokine response. Shizuka Koh, MD, PhD*† Takeshi Soma, MD, PhD† Vishal Jhanji, MD‡ Kohji Nishida, MD, PhD†

The pre-Descemet's layer (Dua's layer, also known as the Dua-Fine layer and the pre-posterior limiting lamina layer): Discovery, characterisation, clinical and surgical applications, and the controversy.

The pre-Descemet's layer/Dua's layer, also termed the Dua-Fine layer and the pre-posterior limiting lamina layer, lies anterior to the Descemet's membrane in the cornea, is 10 μm (range 6-16) thick, made predominantly of type I and some type VI collagen with abundant elastin, more than any other layer of the cornea. It has high tensile strength (bursting pressure up to 700mm of Hg), is impervious to air and almost acellular. At the periphery it demonstrates fenestrations and ramifies to become the core of the trabecular meshwork, with implications for intraocular pressure and glaucoma. It has been demonstrated in some species of animals. The layer has assumed considerable importance in anterior and posterior lamellar corneal transplant surgery by improving our understanding of the behaviour of corneal tissue during these procedures, improved techniques and made the surgery safer with better outcomes. It has led to the innovation of new surgical procedures namely, pre-Descemet's endothelial keratoplasty, suture management of acute hydrops, DALK-triple and Fogla's mini DALK. The discovery and knowledge of the layer has introduced paradigm shifts in our age old concepts of Descemet's membrane detachment, acute corneal hydrops in keratoconus and Descemetoceles, with impact on management approaches. It has been shown to contribute to the pathology and clinical signs observed in corneal infections and some corneal dystrophies. Early evidence suggests that it may have a role in the pathogenesis of keratoconus in relation to its elastin content. Its contribution to corneal biomechanics and glaucoma are subjects of current investigations.

Different compression sutures combined with intracameral air injection for acute corneal hydrops.

To evaluate the efficacy and safety of full-thickness sutures combined with intracameral air injection (FTS-AI) versus pre-Descemet's membrane sutures combined with intracameral air injection (PDS-AI) in the management of acute corneal hydrops in keratoconus. The research included 8 patients (8 eyes) suffering from acute corneal hydrops caused by keratoconus. Four patients were randomly assigned to FTS-AI. And the other four were randomly assigned to PDS-AI. Corneal oedema, visual acuity, corneal thickness were assessed during follow-up. The demographics, preoperative duration of symptoms and severity of corneal hydrops between the two groups were not significantly different. The mean corneal oedema resolution time after FTS-AI and PDS-AI were 11±1.15 and 15±1.41d, respectively (P=0.005). The maximum corneal thickness of the scarred region decreased in both groups at one week postoperatively (P<0.05). No obvious difference was found in the mean maximal corneal thickness between the two groups postoperatively. The BCVA improved significantly after FTS-AI and PDS-AI at three months postoperatively. No obvious difference was found in the BCVA after FTS-AI and PDS-AI at three months postoperatively. FTS-AI and PDS-AI are safe and effective therapies to accelerate the resolution of corneal oedema in acute corneal hydrops secondary to keratoconus. Despite faster resolution of corneal oedema in the FTS-AI group, we recommend PDS-AI to avoid potential endothelium cell damage.

One-step thermokeratoplasty for pain alleviating and pre-treatment of severe acute corneal hydrops in keratoconus.

To observe the efficacy of simplified one-step thermokeratoplasty for the treatment of severe acute corneal hydrops in keratoconus. In this retrospective, noncomparative clinical study, 10 patients (10 eyes) with acute hydrops in keratoconus were treated with simplified one-step thermokeratoplasty. Pain, changes in the corneal curvature, thickness, and size and morphology of the Descemet membrane breaks were detected before and after surgery. Eight patients were successfully treated using modified deep anterior lamellar keratoplasty (DALK). Graft transparency, visual acuity, and immunological rejection were evaluated for 6 to 12mo. Pain and corneal oedema were promptly alleviated, and the intrastromal ruptures diminished within 3 to 6wk after thermokeratoplasty. At 3 to 6wk after corneal oedema was faded, Descemet membrane breaks and intrastromal ruptures were healed. Two patients did not undergo subsequent corneal transplantation after thermokeratoplasty. Eight patients underwent DALK successfully and safely after thermokeratoplasty, without corneal perforation. Central corneal opacity faded or disappeared within 6mo. The mean best-corrected visual acuity was increased to 20/30 at 12 mo after DALK. No one was observed with any immune rejection. One-step thermokeratoplasty can successfully and efficiently accelerate the absorption of prominent corneal oedema in severe acute hydrops patients. This simple procedure with no complications can be performed in the emergency department by residents. This method can improve the safety of DALK and obtain good postoperative vision. Long-term management of acute corneal hydrops using simplified one-step thermokeratoplasty seems promising.

Compression sutures combined with intracameral air injection versus thermokeratoplasty for acute corneal hydrops: a prospective-randomised trial

AimsTo compare the efficacy of compression sutures combined with intracameral air injection (CSAI) and thermokeratoplasty (TKP) for the management of acute corneal hydrops in keratoconus.MethodsIn this multi-centre randomised clinical trial,...

Acute Corneal Hydrops in Keratoconus With Atopic Dermatitis

Acute Corneal Hydrops in Keratoconus With Atopic Dermatitis

Mini-Descemet Membrane Endothelial Keratoplasty for the Early Treatment of Acute Corneal Hydrops in Keratoconus.

To describe a new surgical option for the treatment of acute corneal hydrops in keratoconus and to present the first results. This is a retrospective analysis of 3 patients who presented to our clinic with a massive corneal hydrops in acute keratoconus and were treated by mini-Descemet membrane endothelial keratoplasty (DMEK). According to the size and the shape of the gap in the patient's Descemet membrane (DM), 1 DMEK graft was trephined with a round 5-mm punch. The other grafts were trimmed with a razor blade to a width of about 3 mm and a length adjusted to the length of the defect of the recipients' DM. The graft was inserted with a regular intraocular lens shooter. Correct unfolding of the graft was controlled by using intraoperative optical coherence tomography. At the end of the surgery, the graft was attached to the posterior corneal surface by a small air bubble. Thereafter, the complete anterior chamber was filled with 20% SF6 gas. All 3 patients (age 32 ± 3 years on average) showed a rapid increase in uncorrected visual acuity from the logarithm of the minimum angle of resolution (LogMAR) 1.66 (±0.46) before mini-DMEK to the LogMAR 1.2 (±0.3) within 6 to 8 weeks after mini-DMEK. The thickest corneal point within the edematous cornea decreased in all 3 patients (1088 ± 280 μm before surgery vs. 630 ± 38 μm 1 week after surgery). One mini-DMEK failed in a first attempt. In this patient, the recipient DM was under strong tension and showed a pronounced dehiscence. Therefore, a small part of the recipient's DM around the preexisting gap in DM was removed before a second mini-DMEK graft was placed successfully. The other 2 patients developed partial graft detachment within 1 to 2 weeks after surgery. However, the corneas of these patients were dehydrated to physiological levels after mini-DMEK, and despite partial detachment, there was no relapse of the hydrops. Mini-DMEK could be helpful in patients with larger defects and detachments of DM in very ectatic corneas in the acute phase of corneal hydrops in acute keratoconus. These patients may not be successfully treated by intracameral gas application alone or in combination with pre-Descemetal sutures. Further investigations are needed to identify factors helping to decide on the best surgical approach in hydrops in acute keratoconus.

Keratokonusta Akut Korneal Hidropsun Cerrahi Tedavisi

Keratokonusta Akut Korneal Hidropsun Cerrahi Tedavisi

Management of acute corneal hydrops in keratoconus with pre‐Descemet's membrane sutures

PurposeTo report three cases of corneal hydrops in patients with keratoconus that were managed with pre‐descemet's membrane (DM) sutures associated with intracameral air injection assisted with the ZEISS RESCAN 700 surgical microscope under general anesthesia to shorten the period of corneal edema, to achieve corneal stability and to reduce the duration of the use of carbonic anhydrase inhibitor.MethodsThe first patient, a 27‐year‐old man affected by a Down's syndrome associated with a bilateral keratoconus presented with corneal hydrops in his right eye resulting from a central vertical tear in DM. Three horizontal pre‐DM sutures were performed. The second patient, a 26‐year‐old man affected by a Down's syndrome associated with a bilateral keratoconus presented with corneal hydrops in his left eye resulting from a central horizontal tear in DM. Five vertical pre‐DM sutures were achieved. The third patient, a 43‐year‐old woman with bilateral keratoconus presented with corneal hydrops in her left eye resulting from a central vertical tear in DM. Four horizontal pre‐DM sutures were achieved.ResultsOne month after the pre‐DM sutures, the edema resolved clinically and the pachymetry dramatically decreased on anterior segment OCT. The visual acuity of the Two first patients, while subjectively improved, were not measurable, the third patient visual acuity improved from counting fingers at 1 foot to 1,4logMAR at 20 foots from day one to month six.ConclusionsIntrastromal pre‐DM sutures and intracameral air injection could promptly restore imperviousness of posterior stroma. This technique seems to be a useful procedure to shorten acute corneal hydrops. This impressive clinical response to stromal sutures led us to hypothesize that the pathogenesis of corneal hydrops may correspond to an acute ‘fracture’ of the corneal stroma secondarily leading to DM tear.

Case-control study of risk factors for acute corneal hydrops in keratoconus

PurposeTo determine risk factors for the development of acute corneal hydrops in keratoconus in the UK in a case-controlled study.MethodsBetween November 2009 and December 2010, we prospectively identified 73 individuals...

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

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 in Keratoconus—New Perspectives

To summarize the current concepts and recent literature regarding the epidemiology, pathogenesis, imaging modalities, and treatment of acute hydrops in keratoconus. Perspective. Review and synthesis of selected literature, with interpretation and perspective. Acute corneal hydrops is an incompletely understood complication of keratoconus, characterized by marked corneal edema caused by a break in Descemet membrane, allowing aqueous to enter the corneal stroma and epithelium. Although usually self-limiting, with clinical signs of edema typically resolving after 3months, it often leaves a vision-impairing scar, necessitating and expediting the need for corneal transplantation. Studies have identified risk factors for developing acute hydrops. Modern imaging modalities such as ultrasound biomicroscopy, anterior segment optical coherence tomography, and invivo confocal microscopy have enlightened us to the microstructural changes that take place during acute hydrops, the factors that influence its duration, and sequelae. Newer treatment regimens have seen a reduction in the duration of corneal edema during acute hydrops, and have improved the survival of corneal grafts after transplantation for resolved hydrops. Effective management of acute corneal hydrops in keratoconus is based on recognizing and addressing the risk factors, treating the acute event effectively and promptly to reduce the duration of edema and its complications, and, ultimately, successful corneal transplantation with acceptable long-term graft survival rates. Improved invivo imaging of the cornea during acute hydrops has led to an enhanced understanding of the pathogenesis and ultrastructural changes of the condition, and in turn has resulted in improved management of the disease.

The Auckland keratoconus study: Identifying predictors of acute corneal hydrops in keratoconus

BackgroundThe aim was to identify potential factors associated with acute corneal hydrops in a New Zealand population with keratoconus referred to a hospital eye service.MethodsIn a single hospital centre, in a retrospective review, demographic and clinical features of subjects with keratoconus and corneal hydrops over a 17‐year period were compared with an age‐ and gender‐matched control group of subjects with keratoconus but no history of corneal hydrops.ResultsOne hundred and one eyes of 101 subjects (mean age 24.6 ± 8.4 years) were identified with keratoconus‐related corneal hydrops. Subjects were more likely to be of Pacific but less likely to be of New Zealand European ethnicity than control subjects (n = 101). In comparison, Maori ethnicity was not found to have a significantly positive or negative association with hydrops. The pre‐hydrops visual acuity (VA) of affected eyes was poorer than that of controls (p < 0.001) at first presentation to our tertiary referral corneal and contact lens service. Hydrops typically developed approximately four years after diagnosis of keratoconus. Subjects with hydrops were more likely to have a history of eye‐rubbing (p = 0.011) but less likely to have a family history of keratoconus (p = 0.05). In 31 cases, the acute hydrops event was their first optometric/ophthalmologic contact. There were no statistically significant differences in the prevalence of atopic disease, contact lens wear or overall corneal transplantation rate between the two groups.ConclusionsPacific ethnicity, history of eye‐rubbing, poor VA at first hospital presentation and lack of family history were statistically associated with developing acute corneal hydrops in keratoconus in a New Zealand population. Greater understanding of such predisposing risk factors may help develop early management strategies to delay or prevent progression of this disease.

Acute corneal hydrops in keratoconus

Acute corneal hydrops is a condition characterized by stromal edema due to leakage of aqueous through a tear in descemet membrane. The patient presents with sudden onset decrease in vision, photophobia, and pain. Corneal thinning and ectasias combined with trivial trauma to the eye mostly by eye rubbing is considered as the underlying cause. With conservative approach self-resolution takes around 2 to 3 months. Surgical intervention is required in cases of non-resolution of corneal edema to avoid complications and for early visual rehabilitation. Intracameral injection of air or gas such as perflouropropane is the most common surgical procedure done. Recent investigative modality such as anterior segment optical coherence tomography is an extremely useful tool for diagnosis, surgical planning, and postoperative follow up. Resolution of hydrops may improve the contact lens tolerance and visual acuity but most cases require keratoplasty for visual rehabilitation.

Acute Corneal Hydrops in Keratoconus

To determine the clinical factors associated with the development of acute corneal hydrops and its subsequent outcome. The authors identified 147 eyes (124 patients) with acute hydrops from a database of 5242 eyes (2723 patients) with keratoconus. They compared the clinical features of patients in whom hydrops developed with unaffected patients and assessed the progression to penetrating keratoplasty by actuarial methods. Patients in whom acute hydrops developed tended to be younger males who had advanced corneal ectasia and a poor corrected Snellen visual acuity at the diagnosis of their keratoconus (P < 0.001). Acute hydrops also was more common in the presence of severe allergic eye disease (P < 0.001). The development of hydrops was a very significant risk factor for subsequently receiving a penetrating keratoplasty (P < 0.00001) and at the end of the study period 87 (59%) of the 147 eyes had surgery for visual rehabilitation. These eyes had a greater rate of graft rejection than eyes grafted without hydrops (P = 0.02). After resolution of the hydrops, 46 of the 60 unoperated eyes had been refitted with contact lenses, of which 28 (61%) achieved a Snellen visual acuity of 20/40 or better, although a better visual acuity often was present in the contralateral eye. Microbial keratitis developed in two of these eyes after refitting. Although a penetrating keratoplasty often is indicated for visual rehabilitation after acute corneal hydrops, there is an increased rate of rejection. Only a minority of eyes were re-established in contact lenses after resolution of hydrops, but some patients achieved a functional level of visual acuity such that the procedure could be delayed or avoided.