Simultaneous Muraine Sutures and Excimer Laser-Assisted Penetrating Keratoplasty for Acute Keratoconus.

Background: Acute keratoconus (acute KC), which affects approximately 1.6-2.8% of keratoconus (KC) patients, is a pathological condition of the cornea characterized by stromal edema due to entry of aqueous humor through a tear in Descemet's membrane. Methods: We present a novel combination of surgical procedures that allows swifter visual recovery in a consecutive, retrospective case series. The new surgical procedure for acute KC consists of a combination of Muraine corneal sutures to smooth the corneal curvature and Excimer laser-assisted penetrating keratoplasty and was performed in six acute KC patients from 2019 to 2022 at the Department of Ophthalmology, University Hospital of Martin-Luther-University Halle-Wittenberg (UMH), Germany. We monitored data on preoperative status, operative details, intraoperative and postoperative complications and visual outcomes were analyzed. Results: The mean age was 41.5 ± 13.5 years (3 OD, 3 OS). Neurodermatitis was present in 3 patients (50%). All patients received significant visual benefits from the procedure. Preoperative BCVA was hand motion (logMAR 3.0) in all patients; postoperatively, BCVA improved significantly logMAR 0.03 ± 0.09 [range: 0.2-0.4; p < 0.001, FUP 20+/-10 months). Visual acuity remained stable throughout the roughly biannual follow-ups. One patient developed endothelial graft rejection after 2 years. During the last examination, all eyes had clear grafts and stable curvatures, K1 and K2 being 42.43 ± 4.17 D and 44.95 ± 4.07 D, respectively, and mean corneal astigmatism was 2.61 ± 1.74 D. The thinnest corneal thickness was 519 ± 31 µm. A graft size of 8.0 × 8.1 mm was the most beneficial. Conclusions: in patients with acute KC and hydrops, a penetrating keratoplasty with Muraine corneal sutures is successful in terms of graft clarity and visual outcome. Combining the procedures allows quicker visual recovery. Patients with a history of neurodermatitis should have preoperative and postoperative dermatologic treatment and close follow-up for possible complications.

Corneal ectasia, cerulean (blue dot) cataract with acute hydrops in a child with Down's syndrome and hypothyroidism - a rare presentation.

Down's syndrome or trisomy 21 is a genetic disorder caused by presence of all or a part of a third copy of chromosome 21. Keratoconus occurs in up to 15% of the adult population with Down's syndrome. There is a close consortium between trisomy 21 and keratoconus (a conical ectatic protrusion of the cornea), and children with Down's syndrome are also susceptible to developing thyroid disease, including hypothyroidism and thyrotoxicosis with exophthalmos. The authors describe a case report on acute corneal hydrops with congenital cerulean cataract in a patient with Down's syndrome with hypothyroidism having bilateral advanced keratoconus. As per the detailed literature review, this is the first case of Down's syndrome with hypothyroidism presenting with acute corneal hydrops.

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 700 mm 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.

Partial-thickness compression sutures without descemetopexy for management of acute hydrops in keratoconus: A novel surgical technique.

This article reports a novel surgical technique of partial-thickness compression sutures without descemetopexy with air or gas for the management of acute hydrops in keratoconus. Two patients presented with localized corneal edema with a Descemet membrane (DM) tear in the left eye. Tomography of the right eye revealed localized steepening with increased maximum keratometry and decreased central pachymetry. They were diagnosed with keratoconus in the right eye and acute corneal hydrops (ACH) in the left eye. Compression sutures were passed through the stroma without touching the DM. The anterior chamber was not entered at all at any point during the surgery. Resolution of edema was noted intraoperatively itself. Further resolution of edema was noted from the first postoperative day which markedly reduced within the first week. A corneal scar with no edema was seen at six weeks. In both the patients, vision at presentation was counting fingers close to face which improved to 20/60 and 20/50, respectively, at the last visit.

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

AIM: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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.

Management of acute hydrops with pre-Descemet's deep anterior lamellar keratoplasty.

This case report describes a 23-year-old female patient who had bilateral keratoconus and a history of right eye penetrating keratoplasty who presented with acute hydrops in the left eye which did not respond to conservative management. Pre-Descemet's deep anterior lamellar keratoplasty was performed in the acute stage for management of the impending perforation with good visual outcomes.

Management of acute corneal hydrops with compression sutures and air tamponade.

Background: Acute corneal hydrops is a vision threatening complication of corneal ectasia like keratoconus, keratoconus, keratoglobus, Pellucid marginal degeneration, Terrien's marginal degeneration and post refractive surgery keratectasia. The associated risk factors for development of corneal hydrops (CH) are early onset of keratoconus, microtrauma associated with contact lens use, eye rubbing, allergic conjunctivitis, atopy, and Down's syndrome. With the conservative approach of management of CH, it takes longer time (in months) for corneal oedema to get resolved and there is development of vascularization and scarring. This video presents the simple technique of using compression sutures along with pneumodescemetopexy by intracameral air injection for management of CH. It led to rapid resolution of corneal oedema. It is a simple technique, with no need of special gases like C3F8 or SF6 and can be easily performed at a very basic set up. Purpose: To highlight the efficacy of simple technique of applying compression sutures and air tamponade in management of CH and to demonstrate the efficacy of anterior segment OCT in diagnosis and to assess the prognosis of a case of CH. Synopsis: A 9-year-old boy presented with CH, with anterior segment OCT showing torn descemet's membrane and fluid pockets in corneal stroma. Four full-thickness compression sutures were applied and intracameral sterile air was used for pneumodescetopexy. The serial post operative clinical and OCT picture showed rapid resolution of corneal oedema. Highlights: This video highlights the use of OCT imaging in the diagnosis of CH and full-thickness compression sutures as the safe and effective technique in the management of acute CH. Video link: https://youtu.be/54C3hJB_WTM.

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

AIM: To observe the efficacy of simplified one-step thermokeratoplasty for the treatment of severe acute corneal hydrops in keratoconus. METHODS: 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. RESULTS: 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. CONCLUSION: 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.

Deep anterior lamellar keratoplasty following thermokeratoplasty assisted epikeratophkia: A novel two-stage one-graft method to treat acute corneal hydrops.

Purpose: To evaluate the clinical effects of deep anterior lamellar keratoplasty (DALK) using a single graft after thermokeratoplasty assisted epikeratophakia for the treatment of acute corneal hydrops. Methods: This novel surgical procedure was performed on seven eyes of seven patients between 2019 and 2020. The procedure combines a first-stage surgery of thermokeratoplasty assisted epikeratophkia with intracameral sterile air injection and a second-stage surgery of DALK using the same corneal graft for both procedures. Main outcome measures included pre- and postoperative corrected distance visual acuity (CDVA) and anterior segment optical coherence tomography (AS-OCT) parameters. Corneal transparency, epithelization, and the presence of neovascularization, were evaluated at the 1-year follow-up visit. Results: Corneal edema resolved rapidly in six of the seven cases. The mean central corneal thickness was significantly reduced from baseline to 1 day, 1 week, 1 month, and 2 months after the first-stage surgery (P < 0.0001). At a mean of 2.1 ± 0.7 months after the first-stage surgery, DALK was successfully performed in all cases. Six months later, the mean central corneal thickness was 611 ± 31 µm and the mean thickness of the recipient's residual stroma bed was 20 ± 6 µm at the central corneal area. Mean LogMAR CDVA improved from 1.74 ± 0.34 at baseline to 0.20 ± 0.11 after DALK (P < 0.0001). No postoperative complications appeared in our case series during the 1-year observation period. Conclusion: Very good visual results were obtained with a novel technique (thermokeratoplasty assisted epikeratophakia followed by DALK using the same corneal graft) in the treatment of acute corneal hydrops.

Treatment strategies for the management of acute hydrops.

Acute corneal hydrops is a condition characterized by marked stromal edema attributable to leakage of aqueous humor through a rupture in Descemet's membrane. In addition to the clinical examination, imaging options include ultrasound biomicroscopy, anterior segment optical coherence tomography and in vivo confocal microscopy. While it is a self-limiting condition, the duration of acute hydrops is an important factor linked to complications such as neovascularization. Therapeutic options may relieve symptoms and/or reduce healing time; these include conservative, medical and/or surgical therapies. Several new medical and surgical management options have been reported in the past few years and will be discussed in this article.

Primary surgical options for acute corneal hydrops: A review.

Acute corneal hydrops usually resolves alone or with medical therapy along the first 4-6 weeks. However, depending on the severity of the corneal edema or the size of the Descemet break, self-healing might be difficult. Years ago, those patients had no more options than corneal transplantation, but surgical alternatives are on the rise in this century. These surgeries are becoming more popular with a variety of techniques relegating penetrating keratoplasty to a residual role. These techniques aim to accelerate corneal healing, reduce the edema, improve visual acuity and prevent from severe complications that may appear if corneal hydrops is not treated rapidly. Feasibility and safety are the favorable aspects of these techniques since the complications rates described remain low. In this review, we emphasize the recently published studies that describe both the techniques and their results.

Management of acute corneal hydrops with intracameral gas injection.

PURPOSE: To report two cases of severe acute corneal hydrops that were resolved by intracameral gas injection alone. OBSERVATIONS: Case 1 is a 27-year-old woman with bilateral severe keratoconus who developed sequential acute corneal hydrops in the right eye followed by the left eye that were each successfully treated using intracameral 20% sulfur hexafluoride gas injection. Case 2 is a 62-year-old man that developed a large fluid cleft beneath a pre-existing LASIK flap, which resolved with intracameral 20% sulfur hexafluoride gas injection without the need for corneal transplantation. CONCLUSIONS AND IMPORTANCE: In acute corneal hydrops, intracameral gas injection to tamponade Descemet's membrane tears with decompression of stromal fluid can be an effective intervention to delay or avoid keratoplasty in individuals whose corneal hydrops does not improve with conventional medical management.

Pneumodesmetopexy with perfluoropropane for acute corneal hydrops in keratoconus.

Acute corneal hydrops (CH) occurs when there is a tear in descemets membrane causing rolling of its edges, creating a gap through which aqueous from anterior chamber (AC) percolates into corneal stroma. Acute CH occurs in approximately 2.5-3% of eyes with KC. Conventional therapy is patching or bandage contact lens application with topical cycloplegics and topical hypertonic sodium chloride. Here, we report a case of 9-year-old boy with bilateral keratoconus with left eye acute hydrops who was successfully treated with pneumodesmetopexy with perflouropropane (C3F8).

Unusual Case of Acute Corneal Hydrops in Mooren's Ulcer Treated with Intracameral Injection of Perfluoropropane.

A 15-year-old boy was diagnosed with Mooren's ulcer in the right eye at our cornea clinic. On treatment, he developed acute corneal hydrops, and the vision in the right eye dropped to perception of light. The patient was treated with intracameral injection of perfluoropropane (C3F8) gas which resulted in complete resolution of corneal edema. Contact lens fitting yielded a good visual outcome with a best-corrected visual acuity of 20/80. To the best of our knowledge, this is the only case reporting the occurrence of acute corneal hydrops in a child with Mooren's ulcer.

Acute Corneal Hydrops 3 Years after Intra-corneal Ring Segments and Corneal Collagen Cross-linking.

This case report describes a 15-year-old male with allergic conjunctivitis and keratoconus, who underwent uneventful intra-corneal ring segment (ICRS) implantation and corneal collagen cross-linking (CXL) in the right eye. During the follow-up periods, the patient was noted to have several episodes of allergic conjunctivitis that were treated accordingly. At the 2 years postoperatively, he presented with another episode of allergic conjunctivitis and progression of keratoconus was suspected on topography. However, the patient was lost to follow-up, until he presented with acute hydrops at 3 years postoperatively. There are no reported cases of acute corneal hydrops in cross-linked corneas. We suspect the young age, allergic conjunctivitis and eye rubbing may be a risk factors associated with possible progression of keratoconus after CXL. Prolonged follow-up and aggressive control of the allergy might be necessary in similar cases.

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

OBJECTIVE: 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). METHODS: 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. RESULTS: 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. CONCLUSIONS: A nictitating membrane flap successfully treated 90.5% of FACH eyes (89.5% of patients).