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This study sought to evaluate the demographic profile, clinical features, topographic features, and biomechanical and aberrometric characteristics in pediatric keratoconus (KC).Cross-sectional study.Pediatric KC cases18 years of age were evaluated at a tertiary hospital. Main outcome measurements were demographic profile, clinical features, visual acuity, corneal topography, aberrometry, and biomechanical and confocal microscopy findings.A total of 116 eyes of 62 consecutive patients were recruited with a mean ± age of 14.7 ± 2.77 years (range: 8-18 years); 46 of 62 (88%) were males; 57 of 62 cases (92%) had bilateral disease; 53 of 116 eyes (46%) had progressive KC; and 9 of 116 eyes (8%) had acute hydrops. Systemic associations were found in 6 of 62 patients (9.7%) and ocular associations in 77 of 116 eyes (66.3%); 68 of 116 eyes (58.6%) had associated vernal keratoconjunctivitis (VKC). Among eyes with VKC, 29 of 68 eyes (46%) were in stage IV KC, versus 25% of eyes with no VKC (P = .004). The mean ± SD refractive spherical equivalent was -4.72 ± 3.32 diopters (D), and refractive astigmatism was 3.69 ± 3.09 D. The mean values of maximum keratometry, thinnest pachymetry, and corneal higher-order aberrations were 60.89 ± 10.9 D, 396.05 ± 95.03 μm, and 1.18 ± 1.2 μm, respectively. Values of corneal hysteresis and corneal resistance factor correlated with the stage of KC (r = -0.26; P = .007).Pediatric KC was commonly associated with VKC in this cohort. Eyes with VKC had more severe KC than those without VKC. Nearly half of the patients presented with progressive disease. Corneal biomechanical changes correlated well with stage of KC in this pediatric age group.

Acute corneal hydrops is a pathological condition which clinically presents by marked corneal edema developing due to a break in Descemet’s membrane. Background. To analyze the results of diagnosis and treatment in patients with acute corneal hydrops. Materials and methods. 42 patients (47 eyes) suffering from acute corneal hydrops were included in the study. This condition appeared on both eyes simultaneously or sequentially in 5 patients. Mean age was 28.7 ± 10.1 years (from 19 to 54 years), 31 men, 11 women. In case of complications or inefficacy of medical therapy surgical procedures were performed: 10% gas (C 3 F 8 , SF 6 ) injection into the anterior chamber, amniotic membrane transplantation, partial lamellar keratoplasty, DALK, PKP. Results. Duration of corneal ectasia before acute hydrops occurrence was 12.6 ± 4.6 years. Disease was not diagnosed before in 11.9%. Corneal thickness varied from 692 ± 98 µm in focal hydrops to 1200 ± 220 µm in total hydrops. Area of edema, height of Descemet detachment and gap between break margins were significantly above in cases of subtotal and total hydrops compared with focal and partial hydrops (χ 2 , p < 0,001). Injection of 10% gas (C 3 F 8 , SF 6 ) in the anterior chamber allowed to significantly accelerating the resolution of this condition in cases of subtotal and total hydrops. Conclusion. Analysis of this case series showed the feasibility of a differentiated approach in the treatment of acute corneal hydrops depending on its severity.

Purpose: To compare the clinical characteristics of acute hydrops between eyes with recurrent keratoconus and eyes with treatment-naive keratoconus.Methods: Six eyes with acute hydrops caused by recurrent keratoconus (recurrent group) and 29 eyes with acute hydrops due to treatment-naive keratoconus (naive group) were included. We collected data from anterior-segment optical coherence tomography (AS-OCT), corneal topography and medical records. With AS-OCT, we investigated the location of Descemet's membrane break, the length and frequency of Descemet's membrane detachment, and the speed of corneal thinning before acute hydrops.Results: Descemet's membrane breaks were detectable in 6 cases in the recurrent group (100%) and 7 cases in the naive group (70%). Distance from the break to the central line was 2-3 mm in the recurrent group, and less than 1 mm in the naive group (p < .001). Detachment length was significantly greater in the recurrent group than in the naive group (p = .003). Steep corneal power values tended to increase before acute hydrops in the recurrent group, but the difference between groups was not significant (observation period; 6.03 ± 2.67, 7.36 ± 5.53 years, respectively). Penetrating keratoplasty was required in 5 cases (83%) in the recurrent group, and 8 cases (28%) in the naive group (p = .02).Conclusions: Acute hydrops due to recurrent keratoconus occurred around graft-host junctions and was more severe than that in eyes with treatment-naive keratoconus. Severity assessment of Descemet's membrane detachment using AS-OCT provided clues not only for diagnosis, but also for predicting prognosis.

Aim To report a case of acute hydrops with a large stromal cleft, in a case of keratoconus, managed with compression sutures and perfluoropropane (C3F8) descemetopexy and subsequent management of corneal vascularization in that eye with combination therapy of laser and anti-vascular endothelial growth factor (anti-VEGF). Background Acute hydrops in keratoconus occurs following a tear or rupture in the Descemet's membrane (DM), resulting in aqueous percolating into the stroma. The presence of large stromal clefts in acute hydrops has been described as a known risk factor for delayed resolution and persistent edema, which in turn can incite inflammation and vascularization and thereby adversely affect the chances of graft survival later. We describe such a case managed effectively with a combination of different treatment modalities. Case description A 12-year-old boy with keratoconus presented with acute hydrops in his left eye. The acute hydrops was managed with compression sutures along with C3F8 descemetopexy. Subsequent development of persistent deep corneal vascularization and recurrence of inflammation after the resolution of hydrops was managed with a combination of argon laser photocoagulation and intrastromal anti-VEGF injections, resulting in complete regression of the deep vascularization and resolution of inflammation. Conclusion In our case, a combination of management modalities to address severe acute hydrops and its subsequent complications resulted in complete regression of the superficial and deep vessels, thus heralding an optimal outcome for a future corneal graft. Clinical significance There are no definite guidelines for the management of acute hydrops in keratoconus. A customized and judicious combination of various treatment modalities described in the literature for acute hydrops and its sequelae can result in an accepted outcome, which can pave the way for an optimum result with elective treatments like keratoplasty later on. How to cite this article Kurian A, Reghunadhan I, Soman M, et al. Regression of Corneal Vascularization Occurring after Corneal Suturing in a Case of Acute Hydrops Managed with a Combination Therapy of Argon Laser Photocoagulation and Intrastromal Ranibizumab Injection: A Case Report. Int J Kerat Ect Cor Dis 2019;8(2):46–49.

Introduction: Keratoconus has been known to be associated with various ocular conditions.Objective: To describe a case of high hyperopia, keratoconus and pigmentary retinopathy.Case: An eight year old boy, whose initial refractive error was high hyperopia with short axial length of 20mm, presented as bilateral advanced keratoconus with acute hydrops in one eye. Subsequently his other eye also developed hydrops. He had nystagmus and pigmentary retinopathy from his infancy. He was managed conservatively and the hydrops resolved in both the eyes leaving paracentral scar in the cornea.Conclusion: The triad of keratoconus, high hyperopia and pigmentary retinopathy has been quite rare. There could be syndromic association between the three simultaneous ocular findings.

Purpose To compare two surgical treatment options for acute corneal hydrops in keratoconus: Mini-DMEK versus predescemetal sutures. Methods Sixteen patients were treated by either Mini-Descemet membrane endothelial keratoplasty (Mini-DMEK) (n = 7, group 1) or predescemetal sutures (n = 9, group 2) early after onset of acute hydrops. Visual acuity, maximum corneal thickness (SD-OCT) in the affected oedematous area, complications and recurrence rates were retrospectively compared between both groups. Measurements were taken immediately preoperatively as well as 1 day, 1 week and 1 month postoperatively (Table 1). Results Both groups showed reductions in corneal thickness and increased visual acuity shortly after surgery. In group 1 (average age 33 years ± 7 years), the best corrected visual acuity (BCVA) increased from logMAR 1.65 ± 0.7 before Mini-DMEK to logMAR 0.93 ± 0.6 30 days after Mini-DMEK (p = 0.046). During that period, maximum corneal thickness decreased from 1159 ± 338 µm before surgery to 531 ± 75 µm after Mini-DMEK (p = 0.046). Patients from group 2 (average age 34 ± 10 years) had a BCVA of logMAR 1.59 ± 0.8 which increased to logMAR 0.97 ± 0.5 (p = 0.014) 30 days after surgery. At the same time, the preoperative maximum corneal thickness decreased from 1439 ± 272 µm to 476 ± 162 µm (p = 0.014). There was no difference in corneal thickness or BCVA one month after the surgical intervention between both groups (p = 0.394 and p = 0.871). Conclusions Both techniques, Mini-DMEK and predescemetal sutures, are effective in the treatment of an acute hydrops in keratoconus. Mini-DMEK appears to be beneficial in large DM tears.

Summary 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.

Acute hydrops is a well-known complication of keratoconus. It usually manifests as sudden onset loss of vision. Mostly presents in the pubertal age group. Allergic conjunctivitis associated with eye rubbing is the most substantial risk factor. Primary pathology being stromal lysis, which triggers the progression of cone, causing an undue stretch on Descemet Membrane, eventually resulting in its splitting and stromal imbibition of aqueous through these ruptures. Clinical signs are circum-cillary congestion and thick/edematous cornea with obscuration of the anterior segment. Conservative therapy delays wound healing; hence early surgical intervention is recommended globally for faster resolution of stromal edema. Long-standing corneal edema mounts to corneal perforation and neovascularisation of cornea. Compressive suture, non expansile intracameral gas injection, Deep anterior lamellar keratoplasty, and mini Descemet membrane keratoplasty are various management modalities reported in literature. Acute hydrops could be well prevented with early identification of progressive keratoconus and halting its progression.

Purpose To report a case of late spontaneous detachment of Descemet membrane (DM) in a donor penetrating keratoplasty (PKP) graft and its successful management using a supra-Descemetic venting incision combined with gas descemetopexy. Methods Case report with review of literature. Results A 56-year-old man who had undergone PKP after acute hydrops in pellucid marginal degeneration 27 years before presentation experienced sudden onset vision loss. Clinical features mimicked acute graft rejection but detailed evaluation and anterior segment optical coherence tomography showed a DM detachment (DMD) of the graft. Ten weeks after the onset of DMD, he underwent descemetopexy with a supra-Descemetic stromal venting incision and sulfur hexafluoride gas tamponade. DM reattached with complete resolution of graft edema. Conclusions Late spontaneous DMD of the graft after PKP is very uncommon and must be differentiated from acute graft rejection. Anterior segment optical coherence tomography may help to diagnose this entity that can be managed successfully despite late intervention. The addition of a venting incision that drains fluid from the supra-Descemetic space can increase the chance of success of descemetopexy when compared with descemetopexy with air or gas alone.

This a case report of acute hydrops with interface fluid syndrome in a patient with post-laser in situ keratomileusis (LASIK) ectasia treated with compression sutures. A patient with post-LASIK ectasia presented with acute hydrops and fluid accumulation in the LASIK flap-stromal interface sequentially in both eyes. Full thickness compression sutures were applied to appose the edges of the Descemet tear, and this resulted in a rapid resolution of stromal edema and interface fluid. The patient had best distance visual acuities of 20/40 and 20/60 at the last follow-up in the right and left eyes, respectively. Hydrops along with fluid in the interface needed early intervention to prevent complications such as epithelial ingrowth and interface haze. Compression sutures were useful in this case to help bridge the gap between the margins of Descemet tear, hastening the resolution of stromal edema and interface fluid.

Purpose To demonstrate the occurrence of acute hydrops in the fellow eye of infants with primary congenital glaucoma (PCG) treated for glaucoma in 1 eye. Design Small, observational case series. Participants Three infants with PCG who demonstrated acute hydrops in the fellow eye after treatment of the worse eye. Methods Intraocular pressure (IOP) was lowered surgically in 2 infants and medically in 1 infant with PCG. Two infants underwent combined trabeculotomy with trabeculectomy, and goniotomy was possible in 1 infant. Main Outcome Measures Observation of the fellow eye after lowering IOP in an eye with acute hydrops. Results Two infants underwent glaucoma surgery in 1 eye and demonstrated acute hydrops in the fellow eye within 1 week. The third infant demonstrated acute hydrops after medically controlling IOP in 1 eye before the scheduled surgery. After surgical treatment, the stromal edema cleared completely in all 3 infants, leaving behind a small area of opacity where the Descemet’s tear had healed. Conclusions Our report demonstrates that the fellow eye of a patient with PCG may be at risk of sudden raised IOP resulting in acute hydrops, and early treatment may help to prevent this phenomenon in the fellow eye.

We report a rare case of late unilateral presentation of acute corneal hydrops in a 65-year-old diabetic patient. The patient was diagnosed with advanced keratoconus in the right eye; the left eye was normal. He presented with acute corneal hydrops after 8 months. An intracameral perfluoropropane injection was given; penetrating keratoplasty was performed 3 months later. This rare late unilateral presentation of acute hydrops in a diabetic individual without predisposing factors questions the concept that progression of keratoconus stabilizes as age increases and also puts into question the protective effect of diabetes on the progression of keratoconus.

A 20-year-old man with a very thin cornea developed acute hydrops with corneal thinning after accelerated transepithelial corneal crosslinking (CXL) for keratoconus. Because the corneal thinning and deformation were progressing, accelerated transepithelial CXL was performed in the right eye. However, corneal thinning still progressed after the procedure. After 3.5 years, blurred vision occurred suddenly and acute hydrops was diagnosed. With a pressure eye patch and ofloxacin ointment, the acute hydrops resolved 2 months after the onset. Corneal thickness and topographic change should be monitored carefully after accelerated transepithelial CXL, especially in patients with thin corneas.

WOS: 000477774300013, PubMed ID: 31384164, A 27-year-old male with a repetitive behavior of eyeball pressing for six months presented with decreasing visual acuity for three months. Upon arrival his best corrected visual acuity was 0.2 on the right and 0.6 on the left eyes. Scheimpflug camera system demonstrated grade 4 and grade 2 keratoconus, respectively. Psychiatric consultation revealed Tourette syndrome and treatment was started. Despite psychiatric treatment, acute hydrops occurred in both eyes decreasing visual acuity to hand motions bilaterally. Left corneal perforation due to ongoing habit of eyeball pressure was experienced which led the loss of light perception and phtisis bulbi. Although Tourette syndrome is rare, it might cause bilateral acute onset keratoconus when the repetitive movements affect periocular region. Psychiatric treatment should be considered as early as possible in these patients in order to prevent unfavorable complications.

To report a rare case of spontaneous corneal perforation after hydrops in keratoconus patients who suffer from familial Mediterranean fever and was treated systemically with Colchicine. Case report. We report a case of a 30-year-old male with keratoconus and familial Mediterranean fever treated with colchicine, presented with acute hydrops in his left eye, which progressed to a spontaneous corneal perforation. Attempts to treat the perforation with cyanoacrylate glue failed, and he underwent penetrating keratoplasty with an excellent visual outcome. Colchicine treatment may have had a role in the pathogenesis of this rare case.

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.

Keratoconus is the most common corneal ectatic disorder characterised by bilateral, often asymmetrical, progressive corneal thinning and protrusion. Depending on the disease severity, patients with...

Background Pellucid marginal corneal degeneration is a non-inflammatory disorder complicated by severe inferior corneal thinning. The central portion of the cornea, consequently, appears to protrude outwards, decreasing vision by means of an irregular stigmatism. Additionally, acute hydrops can occur in case of Descemet’s membrane rupture. Case presentation A 41-year-old Japanese woman presenting with severe visual loss in the left eye was examined and observed to have had full thickness corneal perforation as well as a Descemet membrane rupture with massive edema in the corneal stroma. Anterior segment optical coherence tomography-based corneal topography revealed a distorted crab claw sign indicating pellucid marginal corneal degeneration. The Descemet membrane rupture allowed acute hydrops to occur which was especially noteworthy given the scale of edema present within the stroma, rotating 180 degrees along the limbus, causing a smiley-face like lesion. We visualized it via a gonioscopic three-dimensional optical coherence tomography to build a three-dimensional video. Patient history revealed a previous acute hydrops in the right eye as well, which was ultimately treated with anterior lamellar keratoplasty, suggesting the pellucid marginal corneal degeneration had a classic bilateral involvement, which was also characterized with bilateral acute hydrops. Conclusion This appears to be a very rare and interesting presentation of bilateral pellucid marginal degeneration, wherein not only acute hydrops formed bilaterally, but the cavity within the cornea stroma was exceptionally large with an unusual shape. Using the gonioscopic three-dimensional optical coherence tomography imaging, we were able to easily visualize the massive intrastromal cavern, and appropriately planned the crescent-shaped anterior lamellar keratoplasty. The 3d video constructed using this data is particularly elucidative compared to 2d images. As such, we recommend utilizing 3d imaging in cases where more conventional topography is not as explanatory with respect to precise nature of deformation. Electronic supplementary material The online version of this article (10.1186/s12886-018-0757-7) contains supplementary material, which is available to authorized users.

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).

Lamellar keratoplasty is the preferred option for several indications of corneal transplantation. Deep anterior lamellar keratoplasty (DALK) by the “big bubble” (BB) technique revealed clinical clues suggesting the existence of a defined layer in the deep stroma. Simulation of DALK in donor eyes revealed different types of BB and the presence of a compact, tough stromal layer that is impervious to air and has a paucity of keratocytes. Several clinical and histological features of the layer have been elucidated. The layer was termed the pre-Descemet’s layer (Dua’s layer, DL). DL has led to three innovations in corneal surgery, namely pre-Descemet’s endothelial keratoplasty, DALK-triple and suture management of acute hydrops. It is also implicated in descemetoceles and acute hydrops. At the corneal periphery, DL continues as the collagen core of the trabecular meshwork and is populated with trabecular cells. This may have implications for glaucoma that need further investigation.

Case report A 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. Discussion Acute 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.

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 15-year-old female child with history of bilateral poor vision since childhood presented with sudden onset pain, photophobia, and diminution of vision OD for 10 days. Visual acuity was hand motion OD and 1/60 OS. Slit lamp examination revealed microcornea OU with multiple intrastromal fluid clefts OD and an irregular cornea and iridofundal coloboma OS. A clinical diagnosis of acute corneal hydrops OD was made, and the child was subjected to intraoperative optical coherence tomography guided intrastromal fluid drainage with air tamponade. The corneal edema resolved completely within 2 weeks resuming visual acuity to 3/60 allowing laser delimitation of fundal coloboma OD.