Congenital corneal staphyloma in 8q21.11 microdeletion syndrome.

BACKGROUND: Although 8q21.11 microdeletion syndrome (8q21.11 DS) has been reported in association with congenital corneal opacities, reports of the clinicopathological features and management are scarce. METHODS: We reviewed medical records including ophthalmic evaluations, imaging, operative reports, and pathology reports of two unrelated patients referred to the Ophthalmology Clinic of UPMC Children's Hospital of Pittsburgh with a cytogenetic diagnosis of 8q21.11 DS. RESULTS: Ophthalmological evaluation of both children revealed bilateral enlarged, staphylomatous, and cloudy corneas with neovascularization. These findings were consistent with the diagnosis of congenital corneal staphyloma (CCS). In one patient, anterior segment optical coherence tomography and high-frequency ultrasound revealed materials consistent with lens remnants embedded in the cornea; this was confirmed by histopathology. In the second patient, lens was found to be adherent to the cornea during surgery. One eye underwent enucleation for corneal perforation secondary to elevated intraocular pressure. In the other eyes, treatment consisted of penetrating keratoplasty combined with vitrectomy. Ahmed tube was subsequently placed to control intraocular pressure. CONCLUSION: 8q21.11 microdeletion syndrome can be associated with bilateral CCS, likely related to a combination of anterior segment developmental anomalies and elevated intraocular pressure. Tectonic penetrating keratoplasty is necessary to prevent corneal perforation, together with a strict control of the intraocular pressure.

Lamellar Keratoplasty Combined with Amniotic Membrane Transplantation for the Treatment of Corneal Perforations: A Clinical and In Vivo Confocal Microscopy Study.

PURPOSE: To evaluate the clinical and in vivo confocal microscopy outcome of lamellar keratoplasty combined with amniotic membrane transplantation for the treatment of corneal perforations. METHODS: In this retrospective, noncomparative, and interventional case series, 13 eyes of 13 patients with corneal perforation were included. All eyes were treated with lamellar keratoplasty combined with amniotic membrane transplantation for corneal reconstruction. Age, underlying etiology, location, size of corneal ulcer, size of corneal perforation, hospitalization days and follow-up time, and corneal confocal microscopy were investigated. Aqueous leakage, anterior chamber formation, epithelial healing time, and visual acuity (VA) were monitored after operation. RESULTS: The cause of corneal perforation (n = 13) was classified as infectious (n = 13) was classified as infectious (n = 13) was classified as infectious (. CONCLUSION: Lamellar keratoplasty combined with amniotic membrane transplantation may be an alternative, safe, and effective surgical therapy in the treatment of corneal perforations in the absence of a fresh donor cornea. We recommend this surgery to treat with the size of corneal perforation of <4 mm in diameter no matter peripheral or central corneal perforation, especially who had immune-related diseases.

Corneal melt secondary to eosinophilic granulomatosis with polyangiitis.

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare antineutrophil cytoplasmic antibody-associated vasculitis that can affect any organ system. It is most often characterised by chronic airway inflammation along with prominent peripheral blood eosinophilia, although the disease can affect the cardiovascular, gastrointestinal, renal or central nervous systems. Ocular manifestations are uncommon and when they do occur, are varied in their clinical presentations. To the best of our knowledge, this is the first case of corneal melt secondary to EGPA to have been reported.

Tectonic keratoplasty using femtosecond laser lenticule in pediatric patients with corneal perforation secondary to blepharokeratoconjunctivitis: a case report and literature review.

Blepharokeratoconjunctivitis secondary to ocular demodicosis in the pediatric population is often neglected and may result in a serious sight-threatening condition. In severe cases, it can lead to corneal perforation necessitating urgent corneal transplantation. However, the shortage and high cost of donor corneas is the foremost limitation of keratoplasty in developing countries. Small-incision lenticule extraction is an advanced flapless femtosecond laser refractive procedure in which an intrastromal corneal lenticule is detached and removed to correct myopia and myopic astigmatism. We herein describe a technique in which lenticules are used for the management of corneal perforation secondary to Demodex-induced blepharokeratoconjunctivitis. The lenticule was sutured over the site of the perforated cornea using 10-0 interrupted nylon sutures. The globe integrity was maintained with a good visual outcome. Thus, tectonic keratoplasty using small-incision lenticule extraction appears to be a safe, cost-effective, and reliable alternative method for the management of corneal perforation secondary to blepharokeratoconjunctivitis.

Treatment of perforated cornea with an autologous lamellar scleral graft: histologic findings.

We report a case of central corneal perforation treated with an autologous lamellar scleral graft and histologic findings obtained after a subsequent penetrating keratoplasty. A corneal perforation within a large Pseudomonas ulcer in a 55-year-old male rigid gas permeable contact lens wearer was sealed by a lamellar scleral graft from the same eye, followed by an uneventful penetrating keratoplasty 6 months later. Histology of the excised button revealed that the well-apposed graft, which maintained the irregular arrangement of the scleral collagen fibers, was embedded in the corneal stroma over the deep blood vessels and a rupture in Descemet's membrane. The clinical and histologic findings showed that autologous lamellar scleral grafts can be successfully used for the emergency treatment of corneal perforation when a corneal transplant is not available. The distinctive scleral structure revealed by histology and the inadequate graft transparency indicate that visual rehabilitation of eyes with a central corneal perforation can be achieved only by a subsequent optic penetrating keratoplasty.

Treatment of perforated cornea with an autologous lamellar scleral graft: histologic findings / Tratamento de córnea perfurada com enxerto autônomo de esclera lamelar: achados histológicos

ABSTRACT We report a case of central corneal perforation treated with an autologous lamellar scleral graft and histologic findings obtained after a subsequent penetrating keratoplasty. A corneal perforation within a large Pseudomonas ulcer in a 55-year-old male rigid gas permeable contact lens wearer was sealed by a lamellar scleral graft from the same eye, followed by an uneventful penetrating keratoplasty 6 months later. Histology of the excised button revealed that the well-apposed graft, which maintained the irregular arrangement of the scleral collagen fibers, was embedded in the corneal stroma over the deep blood vessels and a rupture in Descemet's membrane. The clinical and histologic findings showed that autologous lamellar scleral grafts can be successfully used for the emergency treatment of corneal perforation when a corneal transplant is not available. The distinctive scleral structure revealed by histology and the inadequate graft transparency indicate that visual rehabilitation of eyes with a central corneal perforation can be achieved only by a subsequent optic penetrating keratoplasty.

RESUMO Relatamos um caso de perfuração corneana central tratada com enxerto autólogo lamelar de esclera e os achados histológicos obtidos após ceratoplastia penetrante (CP) subsequente. Uma perfuração da córnea devido a uma grande úlcera por Pseudomonas em um usuário de lentes de contato rígidas gás permeável de 55 anos de idade foi selada por um enxerto escleral lamelar do mesmo olho, seguida de ceratoplastia penetrante, sem intercorrências, seis meses depois. A histologia do botão excisado revelou que um enxerto bem posicionado, que manteve o arranjo irregular das fibras de colágeno escleral, foi incorporado no estroma corneano sobre os vasos sanguíneos profundos e uma ruptura na membrana de Descemet. Os achados clínicos e histológicos demonstraram que o enxerto autônomo de esclerose lamelar pode ser usado com sucesso como tratamento de emergência da perfuração da córnea, quando o transplante de córnea não é possível. A estrutura escleral característica revelada pela histologia e a transparência inadequada do enxerto indicam que a reabilitação visual dos olhos com uma perfuração corneana central só pode ser alcançada através de uma ceratoplastia penetrante óptica subsequente.

Spontaneous corneal perforation in Terrien's marginal degeneration in childhood: A case report.

RATIONALE: Terrien's marginal corneal degeneration (TMD) is characterized by progressive peripheral corneal thinning. It appears primarily above the age of 40 years and is rare at younger ages. Spontaneous corneal perforation in TMD is a rare, but serious complication that may occur in childhood. PATIENT CONCERNS: This review presents the case of a 16-year-old girl presented with blurred vision in the right eye on awakening in the morning. Slit-lamp examination revealed superior corneal thinning with a corneal perforation. DIAGNOSES: The best-corrected visual acuity (BCVA) was 20/50 in the right eye and 20/20 in the left eye. Intraocular pressures were 5 and 18 mm Hg in the right and left eyes, respectively, measured using a noncontact tonometer. Slit-lamp examination revealed superior corneal thinning with superficial pannus. A 1-mm corneal perforation was observed at the 11 o'clock position. The anterior chamber of the right eye was flat and the Seidel test result was positive. The left eye showed no apparent abnormality on slit-lamp examination. Corneal topography showed 4.3 D of against-the-rule astigmatism, and anterior segment optical coherence tomography revealed superior corneal thinning. We diagnosed it as childhood onset TMD. INTERVENTIONS: Multilayered amniotic membrane transplantation was performed over the perforation site and a bandage contact lens was placed. OUTCOMES: At 1 week postoperatively, the BCVA of the right eye improved to 20/32, the amniotic membrane graft was well-attached, and the anterior chamber remained deep. At 2 months postoperatively, the BCVA was 20/25 and the anterior chamber depth was maintained. LESSONS: Spontaneous corneal perforation due to TMD is rare, but may occur in childhood. The possibility of corneal perforation should be considered even in childhood and good surgical results can be obtained with amniotic membrane transplantation.

Management of severe and refractory Mooren's ulcers with rituximab.

PURPOSE: Management of severe and refractory Mooren's ulcers is challenging as it encompasses tectonic surgical treatment and aggressive immunosuppressive therapies. Efficacy of rituximab in the management of severe Mooren's ulcers has never been reported. METHODS: Five patients (six eyes) from the Cornea and External Disorders department at the Rothschild Ophthalmologic Foundation (Paris, France) were treated for severe Mooren's ulcer unresponsive to conventional treatments between 2008 and 2016. Conventional treatment included topical steroid and ciclosporin 2%, high doses of systemic corticosteroids and/or cyclophosphamide and conjunctival resection with amniotic membrane graft. These patients received two infusions of 1000 mg of rituximab at 2 weeks interval. Epithelial healing, inflammation, additional surgery, systemic corticosteroids and rituximab-related side effects were reported. RESULTS: The mean follow-up was 46.8 months. Following rituximab treatment, we observed a complete healing of Mooren's ulcer within 2 weeks in all patients. Peripheral lamellar keratoplasty was associated when peripheral corneal perforation occurred (5/6 affected corneas). Systemic corticosteroids had been discontinued in all patients. Two recurrences occurred 13 and 53 months after the first rituximab infusion and where successfully treated with a new infusion. No rituximab-related adverse events were reported. CONCLUSIONS: Rituximab was effective in the management of severe Mooren's ulcers and could be an alternative to cyclophosphamide. Additional studies should assess the role of this biotherapy in the management of immunological corneal ulcer.

Tectonic Lamellar Keratoplasty Using Cryopreserved Cornea in a Large Descemetocele.

We describe herein a case of an impending corneal perforation with a large descemetocele in a patient with previous penetrating keratoplasty (PKP) that subsequently was treated with an emergent lamellar keratoplasty using frozen preserved cornea. A 76-year-old male patient, who had a PKP, presented with a completely whitish and edematous graft accompanied by large epithelial defects. Although antibiotics and antiviral agents were tried for three days, the corneal stroma abruptly melted, except for the Descemet's membrane and endothelium. Cryopreserved corneal tissue that was kept at -80°C was thawed and sutured on top of the remaining Descemet's membrane and endothelium. Pathological and microbiological tests were conducted using the remaining donor and recipient corneal tissues. After tectonic corneal transplantation on top of a large descemetocele, a healthy graft and relatively clear interfaces between graft-host junctions were maintained without serious adverse reactions throughout 6 month follow-up period. Microbiological evaluations of donor tissue at the time of thawing and tissue preparation were done, and the results were all negative. Tissue that was taken intraoperatively from the recipient cornea also showed negative microbiological results. In conclusion, tectonic lamellar keratoplasty, using cryopreserved corneal tissue, only onto the remaining Descemet's membrane and endothelium in an emergent condition, was a safe and effective treatment.

Tectonic Lamellar Keratoplasty Using Cryopreserved Cornea in a Large Descemetocele

We describe herein a case of an impending corneal perforation with a large descemetocele in a patient with previous penetrating keratoplasty (PKP) that subsequently was treated with an emergent lamellar keratoplasty using frozen preserved cornea. A 76-year-old male patient, who had a PKP, presented with a completely whitish and edematous graft accompanied by large epithelial defects. Although antibiotics and antiviral agents were tried for three days, the corneal stroma abruptly melted, except for the Descemet's membrane and endothelium. Cryopreserved corneal tissue that was kept at -80degrees C was thawed and sutured on top of the remaining Descemet's membrane and endothelium. Pathological and microbiological tests were conducted using the remaining donor and recipient corneal tissues. After tectonic corneal transplantation on top of a large descemetocele, a healthy graft and relatively clear interfaces between graft-host junctions were maintained without serious adverse reactions throughout 6 month follow-up period. Microbiological evaluations of donor tissue at the time of thawing and tissue preparation were done, and the results were all negative. Tissue that was taken intraoperatively from the recipient cornea also showed negative microbiological results. In conclusion, tectonic lamellar keratoplasty, using cryopreserved corneal tissue, only onto the remaining Descemet's membrane and endothelium in an emergent condition, was a safe and effective treatment.

Histopathologic findings of perforated corneas due to ferric ion infiltration.

OBJECTIVE: To report the histopathologic findings of 3 corneal buttons incised from 2 patients during penetrating keratoplasty (PKP) because of corneal perforation due to corneal siderosis. METHODS: Three eyes of 2 patients had accidental trauma to the corneas caused by iron fragments; the injuries were initially treated conservatively with antibiotics and therapeutic soft contact lenses. However, the corneal stroma melted and perforated, necessitating corneal transplantation. The corneal buttons removed during PKP were examined histopathologically, and the findings were compared with the slit-lamp biomicroscopic and anterior segment optical coherence tomographic (AS-OCT) findings. RESULTS: Slit-lamp examinations showed that the corneal epithelium was hyperplasic around the area of perforation in 2 eyes 1 month after the injury. Wide areas of the stroma had melted, and the corneas were perforated. The corneal epithelium was atrophic in the perforated corneas 4 months after injury. Histopathologically, the corneal stroma and Descemet's membrane were stained by Prussian blue, suggesting that the ferric ions had penetrated deeper than expected by the slit-lamp examinations. The areas of Prussian blue staining corresponded well with the areas altered in the AS-OCT images. CONCLUSIONS: Ferric ions penetrate much deeper into the cornea than estimated by slit-lamp biomicroscopy. The ferric ions can induce stromal melting that can lead to corneal perforation. We conclude that (i) the ferric ion-infiltrated areas can be estimated in the AS-OCT images and (ii) extensive corneal abrasion should be performed to prevent corneal melting and perforation.

Interplay of Immune Cells in Mooren Ulcer.

PURPOSE: To report the histopathological findings of the cornea with fulminant Mooren ulcer, an idiopathic autoimmune keratitis. METHODS: A 54-year-old woman was diagnosed with Mooren ulcer. Despite intensive immunosuppression, corneal perforation developed, and penetrating keratoplasty with a limbal tectonic graft was performed. The removed cornea was histopathologically evaluated for the phenotype of the cells infiltrating the lesion. RESULTS: Hematoxylin-eosin staining showed massive infiltration of inflammatory cells in the anterior corneal stroma and epithelial hyperplasia in the adjacent conjunctiva. Further analysis with immunohistochemistry indicated that the cells infiltrating the lesion included a number of CD4⁺ and CD8⁺ T lymphocytes, CD19⁺CD45⁺ B lymphocytes, and CD14⁺CD68⁺ macrophages. There were a few neutrophils and no CD56⁺ NK cells. CONCLUSIONS: Our findings suggest that multiple types of immune cells including T cells, B cells, and macrophages are involved in the pathogenesis of Mooren ulcer.

Perforating corneal injury in rat and pentadecapeptide BPC 157.

Based on its healing effects in various tissues, we hypothesized that the stable gastric pentadecapeptide BPC 157 heals corneal ulcerations in rats and effects corneal transparency. We made a penetrant linear 2-mm incision in the paralimbal region of the left cornea at the 5 o'clock position with a 20-gauge MVR incision knife at 45° under an operating microscope. Medication was BPC 157 (2 pg/mL, 2 ng/mL, and 2 µg/mL distilled water, two eye drops/left rat eye) immediately after injury induction and then every 8 h up to 120 h; controls received an equal volume of distilled water. In contrast to the poor healing response in controls, BPC 157 significantly accelerated the healing process in 2 µg and 2 ng BPC 157-treated eyes, starting 24 h after the injury, and the fluorescein and Seidel tests became negative. The epithelial defects were completely healed at 72 h (2 µg BPC 157-treated group) and at 96 h (2 ng BPC 157-treated group) after injury. Aqueous cells were absent at 96 h and 120 h after injury in the 2 µg and 2 ng BPC 157-treated groups, respectively. In conclusion, BPC 157 effects the rapid regaining of corneal transparency. Whereas controls developed new vessels that grew from the limbus to the penetrated area, BPC 157-treated rats generally had no new vessels, and those that did form in the limbus did not make contact with the penetrated area. Thus, BPC 157 eye drops successfully close perforating corneal incisions in rats.

Retained host Descemet membrane (Auto-DMET) during conversion of deep anterior lamellar keratoplasty to penetrating keratoplasty: a case report.

PURPOSE: The aim of this study was to relate a case of deep anterior lamellar keratoplasty (DALK) converted to penetrating keratoplasty in which the host central Descemet membrane (DM) with a large perforation was left attached to the host cornea. METHODS: This is a case report of a 34-year-old man who underwent an attempted DALK for keratoconus in his left eye, which became complicated with a large rupture in DM during dissection. The host DM was left in place, the donor cornea with DM intact was sutured onto the host bed, and air was injected into the anterior chamber. The patient was monitored by biomicroscopy, pachymetry, topography, anterior segment optical coherence tomography, and specular microscopy. RESULTS: The postoperative course resulted in improved uncorrected visual acuity, best spectacle-corrected visual acuity, and topography. Corneal edema was observed in the host cornea peripheral to the graft. Three months after the surgery, the endothelial cell density was reduced by 63% compared with the preoperative donor cell density. CONCLUSIONS: Leaving the host DM during conversion of DALK to penetrating keratoplasty was uneventful in this case, although corneal edema was observed in the area overlying the host cornea. It is possible that the retained DM could provide additional autologous endothelial cells to prolong graft survival.

Images in clinical medicine: Bilateral corneal perforation.

A 61-year-old woman with long-standing rheumatoid arthritis presented with the sensation of a foreign body in her right eye; she reported no symptoms in her left eye. The patient had systemic symptoms associated with her rheumatoid arthritis and was taking naproxen.

[Defect covering of a perforated corneal ulcer with bovine pericardium transplant]. / Defektdeckung eines perforierten Hornhautulkus mit einem bovinen Perikardtransplantat.

An 89-year-old patient presented in an emergency situation with a perforated corneal ulcer of neurotrophic genesis. An operative treatment with a Tutopatch® was performed. In the course of treatment a reorganization of the Tutopatch® into corneal tissue resulted and a subsequent satisfactory defect covering with maintenance of visual acuity. Tutopatch® represents a good alternative for covering perforated corneal ulcers.