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A young a presented with painless, progressive diminution of vision in both eyes (BE). Slit lamp examination revealed the presence of a single central corneal opacity in the right eye and multiple corneal opacities of varying sizes in the left eye (LE), limited to the anterior-mid corneal stroma. Microcornea with reduced central corneal thickness and complete inferonasal iris coloboma along with inferior fundal coloboma, sparing both the disc and macula, were noted in BE. A diagnosis of BE macular corneal dystrophy (MCD) and iridofundal coloboma (IFC) was made. The patient underwent LE sutureless anterior lamellar therapeutic keratoplasty. On histopathological examination, the excised corneal tissue revealed stromal lamellar disarray with positive colloidal iron staining, strongly suggestive of MCD. Whole-exome sequencing revealed the presence of a likely pathogenic carbohydrate sulfotransferase 6 ( CHST6 ) mutation, confirming the diagnosis of MCD. This concurrent presence of IFC with a corneal stromal dystrophy is previously unreported in the literature, to the best of our knowledge., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Purpose: To report the outcomes of manual small incision cataract surgery (MSICS) in eyes with corneal opacity., Setting: Tertiary care ophthalmic hospital., Design: Retrospective study., Methods: This retrospective study included 286 eyes of 286 patients having cataract with a pre-existing corneal opacity who underwent manual small incision cataract surgery (MSICS) at a tertiary eye institute between January 2020 and January 2022. Data were retrieved from electronic medical records, and we documented demographics, history, detailed anterior and posterior segment examination, cataract grading, pre- and post-operative vision, intra-operative complications and its management, and post-operative course. All these parameters were recorded at the baseline visit, day 1 and at 1 month post-operatively., Results: Two hundred eighty-six eyes having cataract with a pre-existing corneal opacity which underwent MSICS were evaluated. Corneal opacity was graded as nebular, nebulo-macular, macular and leucomatous types; nebular opacity being the most common. Trauma was the most common cause of opacity followed by infective keratitis. Intra-operative complication rate was 4.89%; which included-7 posterior capsular rent with vitreous disturbance, 2 zonular dialysis, 2 iridodialysis, 2 eyes with aphakia and 1 with Descemet membrane detachment. On follow-up, 6 patients had decentered intraocular lens and 10 had residual cortex. Median logMAR vision improved significantly (p < 0.001) from 1.08 (5/60) pre-operatively to 0.3 (6/12) post-operatively., Conclusion: MSCIS is efficient in providing favorable visual outcomes in patients where corneal opacity makes it difficult for the surgeon to perform a phacoemulsification surgery., (© 2023. The Author(s), under exclusive licence to Springer Nature B.V.)

Rationale: Corneal opacity can be caused by various disease. Generally, the opacity gradually increases as the disease progresses. Sudden corneal opacity is mainly caused by corneal trauma, toxic drugs entering the cornea, or acute edema of the keratoconus. However, sudden corneal opacity caused by diabetes has not been reported., Patient Concerns: A 60-year-old man reported blurred vision and the black eye became white in appearance in the left eye for 5 days. The patient had a history of diabetes which had not been treated., Diagnoses: He underwent slit-lamp examination, anterior segment optical coherence tomography, ultrasound bio microscopy, B-mode ultrasound, corneal endothelial examination, random blood glucose testing, and other examinations. The diagnosis of Diabetic Keratopathy was made., Interventions: Topical glucocorticoids and dilating eye drops were administered and undergo blood sugar control treatment., Outcomes: The corneal of the patient was completely transparent in a few days, and the flocculent exudation in the anterior chamber disappeared., Lessons: Although diabetes generally causes chronic corneal edema, acute corneal edema may also occur when blood sugar is poorly controlled. Therefore, when we see sudden corneal opacity without obvious incentives, we must consider systemic diseases, especially diabetes., Competing Interests: The authors have no funding and conflicts of interest to disclose., (Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc.)

Background: High-intensity focused ultrasound (HIFU) is a cosmetic procedure that aims to tone the skin through thermal collagen coagulation. The energy is delivered in the deep layers of the skin, and because of these characteristics, the risks of severe damage to adjacent tissue and the ocular surface may be underestimated. Previous reports have demonstrated superficial corneal opacities, cataracts, increased intraocular pressure, or ocular refractive changes in different patients following HIFU. In this case, we report deep stromal opacities associated with anterior uveitis, iris atrophy and lens opacity formation following a single HIFU superior eyelid application., Case Presentation: A 47-year-old female presented to the ophthalmic emergency department complaining of pain, hyperemia and photophobia in the right eye following a HIFU application to the superior right eyelid. A slit lamp examination showed three temporal-inferior corneal infiltrates with edema and severe anterior uveitis. The patient was treated with topical corticosteroids, and six months later, there was residual corneal opacity, iris atrophy and peripherical cataract formation. No surgical procedure was needed, and the final vision was Snellen 20/20 (1.0)., Conclusion: The risk of severe impairment to the ocular surface and ocular tissues may be underestimated. Cosmetic surgeons and ophthalmologists must be aware of the complications, and the long-term follow-up of these changes needs further investigation and discussion. Safety protocols of the HIFU intensity threshold for thermal lesions in the eye and the use of protective eye devices should be better evaluated., (© 2023. The Author(s).)

Alkali burn-induced corneal inflammation and subsequent corneal neovascularization (CNV) are major causes of corneal opacity and vision loss. M1 macrophages play a central role in inflammation and CNV. Therefore, modulation of M1 macrophage polarization is a promising strategy for corneal alkali burns. Here, we illustrate the effect and underlying mechanisms of upadacitinib on corneal inflammation and CNV induced by alkali burns in mice. The corneas of BALB/c mice were administered with 1 M NaOH for 30 s and randomly assigned to the vehicle group and the upadacitinib-treated group. Corneal opacity and corneal epithelial defects were assessed clinically. Quantitative real-time PCR (qRT-PCR), immunohistochemistry, and western blot analysis were performed to detect M1 macrophage polarization and CD31 + corneal blood vessels. The results showed that upadacitinib notably decreased corneal opacity, and promoted corneal wound healing. On day 7 and 14 after alkali burns, upadacitinib significantly suppressed CNV. Corneal alkali injury caused M1 macrophage recruitment in the cornea. In contrast to the vehicle, upadacitinib suppressed M1 macrophage infiltration and decreased the mRNA expression levels of inducible nitric oxide synthase (iNOS), monocyte chemotactic protein-1 (MCP-1), tumor necrosis factor-alpha (TNF-α), interleukin (IL)-6, IL-1β, and vascular endothelial growth factor A (VEGF-A) in alkali-injured corneas. Moreover, upadacitinib dose-dependently inhibited M1 macrophage polarization by suppressing interferon (IFN)-γ-/lipopolysaccharide-stimulated STAT1 activation in vitro. Our findings reveal that upadacitinib can efficiently alleviate alkali-induced corneal inflammation and neovascularization by inhibiting M1 macrophage infiltration. These data demonstrate that upadacitinib is an effective drug for the treatment of corneal alkali burns., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2023. Published by Elsevier B.V.)

Background: Axenfeld-Rieger syndrome (ARS) is a rare kind of anterior segment dysgenesis (ASD). The most common ocular features of ARS are posterior embryotoxon and iris hypoplasia, while some patients may manifest as corneal opacity and edema. However, the current understanding of how ARS affects the cornea is still incomplete. This study reports a novel histopathological finding of ARS, complicating corneal abnormalities, including congenital corneal opacity and irreversible endothelial decompensation., Methods: This retrospective study included 6 eyes of 3 ARS patients, 5 of which underwent keratoplasty for irreversible endothelial decompensation from May 2016 to January 2019. No eye had a history of surgery. We reviewed the data of epidemiology, clinical manifestations and histopathologic examinations., Results: Five eyes developed irreversible endothelial decompensation, among which 4 were born with corneal opacity. One eye exhibited transparent cornea but showed a continuous loss of endothelial cells in the absence of surgery and elevated intraocular pressure thereafter. Anterior segment optical coherence tomography photographs showed that anterior synechia existed in the area with corneal opacities, where we found the interlayer splitting of the Descemet membrane inserted by hypoplastic iris and a basement membrane-like structure under a light microscope., Conclusion: Anterior synechia might be associated with corneal abnormalities in ARS patients. The novel histopathologic finding revealed the internal relation between anterior segment dysgenesis and would help explore the inner mechanism of corneal abnormalities in ARS., (© 2022. The Author(s).)

Competing Interests: Competing interests: None declared.

Background: Respiratory and gastrointestinal manifestations are the main causes of mortality and morbidity in cystic fibrosis. Although these symptoms are well recognized, ophthalmic involvement of cystic fibrosis secondary to vitamin A deficiency is uncommon and has been reported very rarely in the medical literature., Case Presentation: Here, we report a 2.5-year-old Iranian boy who presented with bilateral corneal xerosis and corneal opacity secondary to vitamin A deficiency related to cystic fibrosis malabsorption., Conclusion: Malabsorption of fat-soluble vitamins is a common presentation in cystic fibrosis, but corneal opacity secondary to vitamin A deficiency as the initial presentation of cystic fibrosis is a very rare manifestation of fat malabsorption. This highlights the importance of complete systemic examination besides ophthalmic examination in approaching a child with ophthalmic complaint., (© 2022. The Author(s).)

Background: Cataract and corneal blindness continue to be leading causes of reversible blindness in India. These can co-exist in a multitude of pathologies such as trauma, healed keratitis (old herpetic scar), chronic degenerative changes such as labrador keratopathy, bullous keratopathy, corneal dystrophies etc. Phacoemulsification in such eyes is rewarding to the patient in terms of minimal intervention, less risk of complications owing to reduced open sky time (as in case of combined keratoplasty), and better predictable visual outcomes. Approach to such eyes with poor visualisation is highly challenging., Purpose: We illustrate a modified surgical technique of chandelier illumination through pars plana for cataract surgery in eyes with corneal opacity of varying grades., Synopsis: Five patients with dense cataract and small pupils, associated with corneal opacity (leucomatous and macular grade) are described. Closed chamber phacoemulsification with intraocular lens with or without pupil expanders was performed assisted by 23 or 25 gauge pars plana chandelier illumination introduced in the vitreous cavity through a sclerotomy wound made prior to phacoemulsification in the inferotemporal quadrant., Highlights: Chandelier illumination aids in reducing the light scatter that occurs due to corneal opacity. Ease of visualisation of lens structures and of performing cataract surgery was noticed. One case was combined with penetrating keratoplasty with reduced open sky time. This assisted technique has advantages such as enhancing visualisation intraoperatively and allowing working in closed chamber. Its self-retaining nature aids bimanual manipulation. No complications were encountered. The video highlights the utility, advantages and practicality of chandelier retroillumination in patients with corneal opacities of varying degree undergoing phacoemulsification., Video Link: https://youtu.be/I3z6QG-&#95;wD8., Competing Interests: None

Peter's anomaly is a rare form of congenital anterior segment dysgenesis of the eye. Varying degrees of central corneal opacity and lenticulo-corneal or irido-corneal synechiae are the key hallmarks. The association of Peter's anomaly along with short stature, rhizomelia, broad short hands or brachydactyly, with facial dysmorphism, cleft lip, cleft palate, genitourinary and cardiovascular anomalies is a distinct and is often termed Peter's plus syndrome. Early detection is imperative to prevent sensory deprivation amblyopia. Glaucoma can be present at initial diagnosis or at any stage later, but treatment can be difficult. For the dense leukoma, corneal graft may be needed but visual prognosis is poor. Research focussing on gene editing and regenerative medicine using native corneal endothelial cells is ongoing., (© 2022 Foundation Acta Paediatrica. Published by John Wiley & Sons Ltd.)

Performing phacoemulsification in eyes with corneal opacities is challenging even with expert surgeons. Several techniques have been described to improve intraoperative visualization through opacified corneas. This retrospective interventional case series included 10 eyes of 10 patients with coexisting senile cataract and corneal opacity who underwent phacoemulsification with intraocular lens (IOL) implantation under slit illumination of the surgical microscope. Uneventful phacoemulsification with IOL implantation was achieved in all eyes without intraoperative complications. Slit illumination reduced the light scattering and reflection from the corneal opacity, enhanced the red reflex, and improved depth perception simultaneously in different steps of phacoemulsification. Phacoemulsification was safely performed in eyes with corneal opacity under slit illumination of the surgical microscope. It can be considered as a viable option for improving intraoperative visualization in patients with corneal opacities without additional instrumentation., (Copyright © 2021 Published by Wolters Kluwer on behalf of ASCRS and ESCRS.)

Introduction: Peters' anomaly (PA) and Axenfeld-Rieger syndrome (ARS) are typical classifications of anterior segment dysgenesis (ASD) and ascribed to congenital eye diseases that encompass developmental defects in anterior segment structures. The aim of this study is to discuss the unusual association between PA and ARS and to determine the results of penetrating keratoplasty combined with extracapsular cataract extraction and anterior vitrectomy for this unusual ophthalmic phenotype., Patient Concerns: A 72-year-old female was referred to Changzhou No. 2 People's Hospital for a progressive decrease in visual acuity in both eyes in the past few decades., Diagnoses: The patient was diagnosed with PA with cone-shaped polar cataracts in the left eye based on a series of ophthalmic examinations. ARS with retinal detachment was diagnosed in the right eye 2 years prior., Interventions: Penetrating keratoplasty combined with extracapsular cataract extraction and anterior vitrectomy were performed to manage PA with cataracts in the left eye., Outcomes: Her best corrected visual acuity did not improve significantly after the operation. Patients with ARS and PA should be treated cautiously because of fundus lesions., Conclusion: This study revealed that cases with PA accompanied by iridocorneal adhesions, or other ocular anomalies, need to be treated cautiously for a very low success rate. It is of reference value for the evaluation of treatment prognosis for this joint occurrence of ophthalmic phenotypes., Competing Interests: The authors have no conflicts of interests to disclose., (Copyright © 2022 the Author(s). Published by Wolters Kluwer Health, Inc.)

Purpose: The purpose of this study was to report a novel clinical entity characterized by bilateral calcium deposits in the flap interface after uncomplicated laser in situ keratomileusis (LASIK)., Methods: Slit-lamp examination, anterior segment optical coherence tomography imaging, and histopathologic analysis of an interface opacity were performed to characterize and identify the origin of the interface opacities., Results: Two unrelated healthy young men who underwent LASIK in both eyes at 20 (case 1) and 44 (case 2) years of age were diagnosed with bilateral, white anterior stromal opacities 5 years after LASIK surgery. Slit-lamp examination and anterior segment optical coherence tomography imaging demonstrated that the opacities were located at the level of the LASIK interface in both eyes of both cases, with most of the opacities located at the temporal edge of the flap in each eye of case 2. An opacity from case 2 demonstrated birefringence using polarization microscopy and staining with Alizarin red, indicative of calcium deposition. The serum calcium level was borderline elevated in case 1 and within normal limits in case 2., Conclusions: Intrastromal calcium deposition can occur after LASIK surgery, with the deposits resembling dystrophic deposits located in the LASIK flap interface in individuals with granular corneal dystrophy type 2. Because the etiology and management of calcific and dystrophic interface deposition after LASIK are distinct, it is important for clinicians to differentiate the 2 entities based on the examination, diagnostic imaging, and, if necessary, molecular genetic analysis., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Purpose: To evaluate changes of anterior and posterior corneal astigmatism after superficial keratectomy in peripheral hypertrophic subepithelial corneal opacification (PHSCO)., Methods: Patients with PHSCO, who had received superficial keratectomy with mitomycin C 0.02%, were included in this retrospective study. Scheimpflug imaging of the cornea (Pentacam®, Oculus, Wetzlar, Germany), best-corrected visual acuity (BCVA) and objective refraction were determined preoperatively and 3 months after superficial keratectomy., Results: Fifteen eyes of 15 patients (age: 55 ± 16 years; range: 36-82 years) were included. The mean preoperative BCVA was logMAR 0.4 ± 0.2 and improved to logMAR 0.21 ± 0.3 ( p < .01) postoperatively. The median preoperative astigmatism of the anterior corneal surface was 4.67 ± 2.4 D (range: 0.9-13.2 D) and decreased to 1.4 ± 0.4 D (range: 0.8-2.3 D) 3 months after surgery. The median astigmatism of the posterior corneal surface was 0.6 ± 0.5 D (range: 0.1-2.2 D) before surgery and decreased to 0.3 ± 0.2 D (range: 0-0.7 D) 3 months after surgery., Conclusion: Superficial keratectomy reduces anterior corneal astigmatism more than posterior corneal astigmatism in patients with PHSCO. Furthermore, a myopic shift and corneal steepening in the peripheral and mid-peripheral cornea was observed after removal of the subepithelial corneal opacification spots.

Background: Peters anomaly is a rare form of anterior segment ocular dysgenesis, the antenatal image of Peters anomaly had not been reported. We herein showcased a discordant finding of Peters anomaly in a monozygotic twin complicated with twin-twin transfusion syndrome (TTTS) and exhibited its antenatal sonographic images, CASE PRESENTATION: A 38-year-old gravida 2 para 1 pregnant woman visited our clinic at the gestational age of 18 weeks where TTTS stage III was diagnosed and the following laser therapy was done successfully. Ten days after the surgery, the follow-up ultrasound detected the opacity of both fetal eyeballs in the donor twin and thus congenital cataract was suspected initially. Then magnetic resonance imaging (MRI) examination was arranged at the gestational age of 23 weeks, and no central nervous system or other anomaly was found. At the 29 weeks of gestation, the opacity of both fetal eyeballs of the donor twin did not clear. The pregnancy resulted in cesarean section at the gestational age of 37 weeks indicated by malpresentation where two male live births were born. Examination under anesthesia was arranged for donor twin after delivery and Peters anomaly was diagnosed based on central corneal opacity with iridocorneal and corneolenticular adhesions., Conclusions: The prenatal image of Peters anomaly may present as the opacity of the fetal eyeballs similar to congenital cataract. Some cases of the Peters anomaly had been reported with a genetic abnormality, but since our case presented discordant presentation in monozygotic twin pregnancy where both twins are supposed to share the same genetic make-up, therefore other factors that are epigenetic may be held accountable. Nevertheless, a genetic origin of the anomaly in our case cannot be excluded.

Purpose: To determine the visual outcome and safety of cataract surgery alone and compare with the long-term visual outcome of triple procedure in one-eyed patients., Design: Retrospective study., Methods: This study reviewed 44 one-eyed patients with cataract coexistent with corneal opacity. The patients who underwent cataract surgery alone (group A, n = 25) were compared with patients managed by triple procedure (group B, n = 19). Outcome measures were the improvement of best corrected visual acuity (BCVA) and period of maintained ambulatory vision. Institutional ethics committee approval was obtained., Results: The most common etiology of corneal opacity was fungal and the commonest cause of permanent visual loss in other eye was phthisis bulbi. Mean age was 61.2 ± 8.1 years and 62.5 ± 6.9 years in groups A and B, respectively. At each follow-up, the mean postoperative BCVA was found significantly better than the preoperative vision in both groups and at the end of 3 years, mean postoperative vision of group A was better than that of group B (P = 0.012). Group A had longer (33.36 ± 11.97 months) mean period of maintained ambulatory vision than that of group B (26.5 ± 13.5 months) (P = 0.245) and showed less risk of losing ambulatory vision. Limited visual outcome was due to continuing presence of corneal opacity in group A, and graft infection and graft rejection in group B., Conclusions: Cataract surgery with intraocular lens (IOL) implantation alone can be considered as an alternative or temporary option to provide stable ambulatory vision in one-eyed patients.

Background: Visual impairment (VI) increases with age and has been reported to be more prevalent among older adults living in old age homes than in the general population., Aim: To determine the prevalence and causes of VI among older adults living in low-income old age homes in Durban, South Africa., Setting: This study was conducted at low-income old age homes in Durban., Methods: This cross-sectional study of 118 residents aged 60 years and older, collected socio-demographic data, presenting visual acuities (VAs) for each eye, and binocularly. Anterior segment eye examinations were conducted with a penlight torch and a portable slit-lamp, while posterior segment evaluation was conducted with direct and indirect ophthalmoscopy. Objective and subjective refractions were performed, and the best-corrected distance and near VAs were measured in each eye. VI was defined as presenting VA 6/18 and included moderate VI ( 6/18-6/60), severe VI ( 6/60 -3/60) and blindness ( 6/120)., Results: The mean age of the participants was 73.3 years and included 80.5% females and 19.5% males. The prevalence of VI and blindness was 63.6%. Optical correction significantly reduced the prevalence of VI and blindness by 19.5% (p 0.05). The main causes of non-refractive VI and blindness were cataract (54.5%), posterior segment disorders (25.5%) and corneal opacities (20%)., Conclusion: The prevalence of VI and blindness is high among residents in low-income old age homes living in Durban. Refractive correction and surgical cataract intervention can significantly reduce the burden of VI and blindness among the elderly residents.

Purpose : Reliable data on eye care needs in Kyrgyzstan are not readily available. The purpose of this study was to determine the prevalence and causes of blindness and visual impairment in persons aged 50 and above in the southwest of Kyrgyzstan and to support the Ministry of Health (MoH) in the planning of eye care in the region. Methods : A population-based survey was conducted in three states (Oblast) in the southwest region of Kyrgyzstan. Sixty clusters of 50 people aged 50 years and older were selected by probability proportionate to size sampling. Ethical approval was obtained from the MoH, consent was obtained from each participant. Results : A total number of 3,000 persons aged 50 and older were sampled. Among these 2,897 (95.9%) were examined. The prevalence of bilateral blindness was 1.7% [95%CI: 1.1-2.4]. Cataract (43.3%) was the main cause of blindness, followed by glaucoma (30%), age-related macular degeneration (ARMD) (8.3%), other posterior segment diseases (6.7%) and non-trachomatous corneal opacities (5%). The prevalence of blindness and visual impairment increased strongly with age. The cataract surgical coverage in blind persons was 59%. Conclusion : Cataract and glaucoma were the major causes of blindness and visual impairment in persons 50 and above. The majority of the causes (85%) were avoidable, with 45% (cataract and uncorrected aphakia) treatable, 6.7% (corneal opacity and phthisis) preventable by primary health care/eye care services and 33.3% (cataract surgical complications, glaucoma) preventable by specialized ophthalmic services. The data suggest that an expansion of eye care services to reduce avoidable blindness is needed, as ageing will lead to an increase in older people at risk and a higher demand for eye care in the future.

Background: To evaluate the effect of toric intraocular lens implantation in cataract patient with corneal opacity and high astigmatism., Methods: Thirty-one eyes of 31 patients who underwent cataract surgery with toric intraocular lens implantation were included. All patients had corneal opacity with astigmatism. Preoperative total corneal astigmatism was determined considering posterior astigmatism using a rotating Scheimpflug camera (Pentacam®: Oculus, Wetzlar, Germany). At 2 months after toric intraocular lens implantation, we evaluated residual astigmatism, uncorrected visual acuity (UCVA) and best corrected visual acuity (BCVA)., Results: Postoperative UCVA and BCVA (0.30 ± 0.17, 0.22 ± 0.16LogMAR) were statistically improved compared to preoperative UCVA and BCVA (1.2 ± 0.34, 1.1 ± 0.30LogMAR, respectively) (P < 0.01). Postoperative residual refractive astigmatism (1.2 ± 0.35D) was statistically reduced compared to preoperative refractive astigmatism (2.4 ± 0.65D) (P < 0.05). Preoperative and postoperative total corneal astigmatism values were not statistically different. All eyes achieved postoperative visual acuity as good as or better than preoperative one. The size of corneal opacity covering pupil had significant negative correlation with postoperative UCVA and BCVA (logMAR) (R = 0.91 P < 0.05 and R = 0.92 P < 0.05, respectively)., Conclusion: Toric intraocular lens implantation can improve UCVA, BCVA, and refractive astigmatism in cataract patient with corneal opacity. The size of corneal opacity covering pupil is the major prognostic factor for postoperative visual improvement. Therefore, toric intraocular lens implantation should be considered for cataract patients who have corneal opacity with high astigmatism.

Purpose: To evaluate causes and management of congenital corneal opacities (CCO) diagnosed in a tertiary care eye center and to compare the data with a previous study at the same institution., Methods: Computerized medical records in all patients with congenital corneal opacities diagnosed in the Cornea Service at Wills Eye Hospital (Philadelphia, PA) between January 1, 2007, and December 31, 2015, were retrospectively reviewed. Children aged 12 years and younger at the first visit were included in the study. Patients' demographics, ocular diagnosis, laterality, associated ocular abnormalities, other ocular surgery performed prior or subsequent to the first visit, and their treatment were extracted from the medical records., Results: A total of 77 eyes in 56 patients were examined. The mean age at presentation was 32.8 ± 44.2 months, with the mean follow-up period of 26.7 ± 30.1 months. The most frequent diagnosis was Peters anomaly (53.2%), followed by limbal dermoid (13.0%), aniridia with glaucoma and microphthalmos (6.5%), sclerocornea and congenital glaucoma (5.2%), idiopathic (3.9%), Axenfeld-Rieger anomaly and Hurler syndrome (2.6%), and microcornea (1.3%). Primary keratoplasty was performed in 26 eyes, with the outcome rate in the clear cornea of 76.0% during the follow-up., Conclusion: Peters anomaly is the most common cause of congenital corneal opacities encountered at our institution. Penetrating keratoplasty is the most frequent choice of corneal surgery to treat congenital corneal opacities. Additional interventions during penetrating keratoplasty were moderately positively correlated with graft failure. This study also shows the rates of some etiologies of that changed over the recent decades in our tertiary care Cornea Service. Although Peters anomaly remains the most common presenting reason for congenital corneal opacities, its rate appears to be increasing over the recent decade. Congenital corneal opacities due to birth trauma, which is one of the preventable causes, were observed in a previous study in our clinic; however, no new cases were noted in this study.

While phacoemulsification cataract surgery is a routine and safe procedure, clear visualization of the anterior segment is challenging in patients with corneal opacity. Illumination from the operating microscope can cause scattering and light reflection in a patient with corneal opacity. A frequent approach for these cases is cataract surgery with sequential or simultaneous corneal transplantation. This method has serious preoperative, intraoperative, and postoperative drawbacks, such as a long wait for a donor cornea, choroidal hemorrhage, and delayed visual rehabilitation. In this case series, the technique of intracameral endoilluminator-assisted phacoemulsification surgery in patients with severe corneal opacity was shown to provide better visualization and reduced scattering and reflection in patients with corneal opacity.

Introduction: We describe a novel surgical technique of slit-beam retro-illumination assisted phacoemulsification for cataract with coexisting corneal opacity., Cases: We present two cases with cataract and coexisting opacity, who underwent slitbeam retro-illumination assisted phacoemulsification and further application of same process in 12 patients., Conclusion: This technique is safe and provides excellent visualization during cataract surgery in patients with corneal opacities., (© NEPjOPH.)

Objective: In this retrospective single institution study, we investigated the clinicopathologic features and treatment characteristics of 90 patients with congenital corneal opacities (CCO) (117 eyes) who were 3 years and younger and treated at our hospital., Subject and Methods: We reviewed the clinical data of patients with CCO who presented for the first time for treatment at our hospital between January 1, 2017, and December 31, 2017. CCO were classified using the "STUMPED" (Sclerocornea, Tears in Descement's membrane, Metabolic, Peters, Endothelial dystrophy and Dermoid) method and confirmed by pathological examination. -Results: Seventy percent of the patients had unilateral CCO. Iridocorneal adhesions (61 eyes, 52.1%) and cataracts (22 eyes, 18.8%) were the 2 most common ocular abnormalities. Systemic abnormalities were present in 5 patients (5.6%), including growth retardation (4 patients) and congenital brain defects (1 patient). Eighty-five eyes (72.6%) underwent penetrating keratoplasty (PK), and lamellar keratoplasty (LK) was performed in 30 (25.6%) eyes. Forty-seven (95.9%) eyes with Peters anomaly and all 16 eyes with sclerocornea received PK, and all 24 eyes with dermoids were treated with LK., Conclusion: Our study demonstrates that CCO has varied manifestations in infants and young children in China. A thorough medical history, careful clinical examination, and the use of accessory examinations such as ultrasound biomicroscopy are critical for the accurate diagnosis and classification of CCO and to provide guidance on therapeutic choices., (© 2019 The Author(s) Published by S. Karger AG, Basel.)

Purpose: To conduct a qualitative description of corneal and glaucoma outcomes in Peters anomaly., Design: Retrospective case series., Methods: Children with Peters anomaly who presented between 1982-2017 were included. Visual acuity, intraocular pressure (IOP), survival of corneal grafts, and success of glaucoma surgery (defined as IOP of 5-20 mm Hg and no additional IOP-lowering surgery or visually devastating complications) were analyzed., Results: Fifty-eight eyes of 37 patients (19 males) with Peters anomaly presented at 0.7 ± 1.5 years of age and had a follow-up of 10.2 ± 10.8 years. Twenty-three eyes underwent penetrating keratoplasty (PKP) with an average of 2.0 ± 1.3 grafts per eye. PKP survival at 1 year was 60% (95% confidence interval 41-75%) but decreased at 10 years to 34% (95% confidence interval 18-51%). Thirty-four eyes were diagnosed with glaucoma at 2.8 ± 6.4 years of age. Twenty eyes required glaucoma surgery, and the average number of IOP-lowering surgeries was 2.9 ± 2.0 per eye. Glaucoma drainage devices (15 eyes) had a 53% success rate. Laser cycloablation (9 eyes, transcleral or endoscopic) had a success rate of 67% with 2.0 ± 1.0 treatments per eye. Trabeculectomy with mitomycin C (8 eyes) and trabeculotomy (8 eyes) had 25% and 0% success rates, respectively. Fifteen eyes that underwent glaucoma surgery obtained IOP control, and GDD with or without cycloablation accounted for 80% of the successes. Eyes that underwent PKP did not show increased rates of glaucoma, higher IOP, or a greater necessity for IOP-lowering surgery., Conclusions: In Peters anomaly, PKP shows poor long-term success. In addition, >50% of eyes with Peters anomaly have secondary glaucoma that often requires multiple surgeries., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Purpose: To evaluate corneal edema in different-aged pediatric patients with Peters anomaly and to correlate in vivo with ex vivo histopathologic findings., Methods: A retrospective cross-sectional study was performed. The medical records of patients diagnosed with Peters anomaly who underwent examination under anesthesia (EUA) between 2011 and 2015 were reviewed. Eyes in which central corneal thickness (CCT) measurements were taken were included. The thickest point in the CCT pachymetric map was used to objectively quantify corneal edema. Correlation between CCT and age was calculated. Additionally, a retrospective review of histopathologic studies of excised corneal buttons from pediatric eyes with Peters anomaly between 2011 and 2015 was performed., Results: Eighteen eyes of 12 children were included. Mean age was 14 ± 15 months, and mean CCT was 842 ± 304 µm. A significant inverse correlation was noted between the CCT and the age of the patients, with lower CCT values in older children (r = 0.6; P = 0.01). Seven excised corneal buttons that underwent penetrating keratoplasty were reviewed. All corneal buttons showed absence of Descemet membrane and localized absence of endothelium. However, three specimens showed presence of corneal endothelium in areas of absent or attenuated Descemet membrane., Conclusions: In Peters anomaly, the CCT decreases with age, possibly due to a decrease in corneal edema. Histopathologic studies show cases of endothelial expansion in areas of absent or attenuated Descemet membrane. This may contribute to improved endothelial function and decreased edema with age.

Background: To assess the prevalence and causes of vision impairment in North Africa and the Middle East (NAME) from 1990 to 2015 and to forecast projections for 2020., Methods: Based on a systematic review of medical literature, the prevalence of blindness (presenting visual acuity (PVA) <3/60 in the better eye), moderate and severe vision impairment (MSVI; PVA <6/18 but ≥3/60) and mild vision impairment (PVA <6/12 but ≥6/18) was estimated for 2015 and 2020., Results: The age-standardised prevalence of blindness and MSVI for all ages and genders decreased from 1990 to 2015, from 1.72 (0.53-3.13) to 0.95% (0.32%-1.71%), and from 6.66 (3.09-10.69) to 4.62% (2.21%-7.33%), respectively, with slightly higher figures for women than men. Cataract was the most common cause of blindness in 1990 and 2015, followed by uncorrected refractive error. Uncorrected refractive error was the leading cause of MSVI in the NAME region in 1990 and 2015, followed by cataract. A reduction in the proportions of blindness and MSVI due to cataract, corneal opacity and trachoma is predicted by 2020. Conversely, an increase in the proportion of blindness attributable to uncorrected refractive error, glaucoma, age-related macular degeneration and diabetic retinopathy is expected., Conclusions: In 2015 cataract and uncorrected refractive error were the major causes of vision loss in the NAME region. Proportions of vision impairment from cataract, corneal opacity and trachoma are expected to decrease by 2020, and those from uncorrected refractive error, glaucoma, diabetic retinopathy and age-related macular degeneration are predicted to increase by 2020., Competing Interests: Competing interests: JBJ: consultant for Mundipharma (Cambridge, UK); patent holder with Biocompatibles UK (Farnham, Surrey, UK) (Title: Treatment of eye diseases using encapsulated cells encoding and secreting neuroprotective factor and/or antiangiogenic factor; patent number: 20120263794) and patent application with the University of Heidelberg (Heidelberg, Germany) (Title: Agents for use in the therapeutic or prophylactic treatment of myopia or hyperopia; Europäische Patentanmeldung 15 000 771.4). JHK: consultant for Gilead and Santen. SR: consultant for Brien Holden Vision Institute., (© Author(s) (or their employer(s)) 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

The treatment of choice for cases of corneal opacity with significant cataract is often a triple procedure. However, in certain situations the chances of graft survival are poor, for example in cases with deep vascularization, secondary glaucoma, and healed viral keratitis. Under these circumstances, performing cataract surgery only might improve the visual acuity enough to maintain the patient's day-to-day activities. Performing cataract surgery, especially phacoemulsification, in these cases is challenging. Proper case selection, choosing the right technique, and a thorough idea about the difficulties and methods of tackling such cases is paramount to achieving optimum visual outcomes. In this review, we discuss the case selection and surgical modifications of performing phacoemulsification in cases with coexisting corneal opacities., (Copyright © 2018 ASCRS and ESCRS. Published by Elsevier Inc. All rights reserved.)

Background: We aimed to assess the knowledge and attitude of health professionals towards eye donation at an apex tertiary care centre of northern India., Methods: We interviewed 600 health professionals, comprising doctors, nurses, medical as well as nursing students, social workers and allied paramedical staff. A structured questionnaire (12 questions for assessing knowledge and 5 questions for assessing attitude) was used to estimate the awareness of eye donation and willingness to pledge eyes for donation. The responses pertaining to knowledge were graded as 'excellent', 'good' and 'poor' and those pertaining to attitude were grouped into 'positive' and 'negative'., Results: Of the 600 participants, 138 participants (23%) had 'excellent' knowledge and 234 participants (39%) had 'good' knowledge about eye donation. Awareness of eye donation was positively related to the level of literacy (odds ratio [OR] 8.5 [2.30-31.2]; p<0.001). Medical social workers and health supervisors had better knowledge about eye donation (OR 2.01 [1.08-3.72]; p=0.026) than other professional groups. Knowledge of eye donation had no significant association with age, gender, religion, family type and marital status of the respondent. Willingness to pledge eyes for donation was observed in only 6% of the participating health professionals. Pledging of eyes for donation was higher among older participants (OR 7.8 [2.67-22.77]; p<0.001)., Conclusion: Our study shows that there is sufficient knowledge about eye donation, but an alarmingly low willingness to pledge eyes for donation among health professionals. Concerted efforts are required to alter their attitude to strengthen the Hospital Cornea Retrieval Programme., Competing Interests: None

Purpose: To report 4 cases undergoing 25-gauge endoscopic vitrectomy for the treatment of proliferative vitreoretinopathy with severe corneal opacity in which a transpupillary view of the fundus was not possible., Study Design: A retrospective interventional case series., Methods: The main outcomes measured were postoperative anatomic status of the retina and subjective improvement of vision., Results: Postoperative reattachment of the retina and subjective improvement of vision were achieved in all 4 eyes., Conclusion: Twenty five-gauge endoscopic vitrectomy provides a clear view making it possible conduct pars plana vitrectomy in order to reattach the retina in cases of proliferative vitreoretinopathy with severe corneal opacity.

Background: Investigating the efficacy and safety of phacoemulsification with intraocular lens (IOL) implantation in corneal opacities., Methods: This retrospective study was conducted in a tertiary medical center. Twenty-three eyes of 19 patients with cataracts and corneal opacities obscuring the pupillary center having received phacoemulsification with IOL insertion without any ancillary techniques were enrolled. The primary study outcome measures were uncorrected and best corrected visual acuity (BCVA), and complications. Backscatters of corneal scar lesions were evaluated by slit lamp-based haze grading, Scheimpflug Pentacam and anterior segment optical coherence tomography (ASOCT). Visual outcomes after cataract surgeries and improvement range were used to determine the safety and efficacy of cataract surgery for our patients., Results: All patients underwent uneventful capsulorhexis and phacoemulsification. The mean age was 72.22 ± 10.1 years, and the mean follow-up period was 18.57 ± 15.42 months. The mean BCVA significantly improved from 1.45 ± 0.65 preoperatively to 0.94 ± 0.55 logMAR postoperatively (p < 0.001), and the number of eyes with a BCVA of 20/100 or better increased from 4 to 14. Complications included corneal edema in two eyes and reactivation of the previous corneal pathology in five eyes. Four eyes did not achieve an improvement in visual acuity after surgery, which may have been due to co-existing ocular co-morbidities. Both Pentacam corneal densitometry and ASOCT demonstrated no significant correlations with final visual outcome. However, a statistically significant relationship between the severity of corneal opacity and improvement range in BCVA (r = - 0.782, P = 0.001) was found by our OCT grading method., Conclusions: Phacoemulsification and IOL implantation in selected cases of coexisting cataracts and corneal opacities is safe that can provide suboptimal but long-term vision when penetrating keratoplasty is not possible or at high-risk of graft failure. ASOCT is a simple tool to predict visual outcomes after cataract surgery in opacified corneas.

An estimated 1.4 million of the world's children are blind. A blind child is more likely to live in socioeconomic deprivation, to be more frequently hospitalised during childhood and to die in childhood than a child not living with blindness. This update of a previous review on childhood visual impairment focuses on emerging therapies for children with severe visual disability (severe visual impairment and blindness or SVI/BL).For children in higher income countries, cerebral visual impairment and optic nerve anomalies remain the most common causes of SVI/BL, while retinopathy of prematurity (ROP) and cataract are now the most common avoidable causes. The constellation of causes of childhood blindness in lower income settings is shifting from infective and nutritional corneal opacities and congenital anomalies to more resemble the patterns seen in higher income settings. Improvements in maternal and neonatal health and investment in and maintenance of national ophthalmic care infrastructure are the key to reducing the burden of avoidable blindness. New therapeutic targets are emerging for childhood visual disorders, although the safety and efficacy of novel therapies for diseases such as ROP or retinal dystrophies are not yet clear. Population-based epidemiological research, particularly on cerebral visual impairment and optic nerve hypoplasia, is needed in order to improve understanding of risk factors and to inform and support the development of novel therapies for disorders currently considered 'untreatable'., Competing Interests: Competing interests: None declared., (© Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

PHACE syndrome is a rare neurocutaneous disorder, with a complex pathogenesis. It presents with a large facial hemangioma associated with anomalies of the posterior fossa of the brain, arterial anomalies, cardiac anomalies, coarctation of the aorta, and eye anomalies. Ocular abnormalities are rare. We report the first published case of an infant with PHACE syndrome and Peters anomaly., (Copyright © 2017 American Association for Pediatric Ophthalmology and Strabismus. Published by Elsevier Inc. All rights reserved.)

Purpose: To assess the reproducibility and accuracy of ultrasound (US) measurements in determining the distance between corneoscleral limbus and retinal break and its relation with the distance measured by indirect ophthalmoscopy, in patients undergoing a laser retinopexy procedure., Methods: Forty-four patients with a single retinal break, scheduled for laser a retinopexy procedure (26 phakic patients and 18 pseudophakic patients), underwent 5 repeated measurements by high-resolution US and 3 measurements (at the time of the laser procedure, 1 and 3 months) by indirect ophthalmoscopy with scleral indentation of the corneoscleral limbus-retinal break distance with a caliper., Results: In the phakic patients group, measurements ranged from 8.75 mm to 14.45 mm (12.56 ± 1.24, mean ± SD) and from 9.5 mm to 15 mm (12.35 ± 1.32) with US and indirect ophthalmoscopy, respectively. In the pseudophakic patients group, measurements ranged from 9.04 mm to 13.95 mm (11.88 ± 1.33) and from 8.5 mm to 13.2 mm (11.93 ± 0.99) with US and indirect ophthalmoscopy, respectively. The correlation coefficient was greater than 0.97. Measurement variability was very small. In phakic eyes, it was 0.13 ± 0.08 mm and 0.13 ± 0.07 mm with US and indirect ophthalmoscopy, respectively. In pseudophakic eyes, it was 0.12 ± 0.05 mm and 0.14 ± 0.05 mm with US and indirect ophthalmoscopy, respectively. US and indirect ophthalmoscopy measurements were not statistically different (Student's t-test, P = 1.71). The analysis of the variance among phakic and pseudophakic patients confirmed that measurements of the two groups do not differ significantly (Fisher's exact test, P = 0.16). The univariate analysis showed no significant difference in both US and indirect ophthalmoscopy measurements (ANOVA, P = 0.09) and between the two types of measurements and patient groups (ANOVA, P = 0.38)., Conclusion: This study suggests relevant accuracy and reliability of US readings and provides the possibility of using this technique for localizing tears in eyes with media opacities by identifying the meridian and corneoscleral limbus-retinal break distance.

Background: To evaluate the feasibility and efficacy of small incision lenticule extraction (SMILE) in the treatment of myopia with corneal opacity., Methods: To evaluate the treatment of myopia with corneal opacity, 9 patients (4 males, 5 females) who underwent SMILE were enrolled in this prospective clinical study. One eye of each patient was treated. The results of laser scanning and lenticule extraction were observed during the surgery, and the surgical videos were again reviewed after surgery. Uncorrected distance visual acuity (UDVA), corrected distance visual acuity (CDVA), and spherical equivalence (SE) were noted at 1 month after surgery. The depth and density of the corneal opacities were measured by anterior segment optical coherence tomography (AS-OCT) and the Pentacam anterior eye segment analyzer., Results: All procedures were uneventful and no intraoperative complications were observed. At 1 month after surgery, the UDVA of all patients was 20/25 or better and no patients lost Snellen lines. The mean safety and efficacy indexes were 1.10 ± 0.24 and 1.08 ± 0.16, respectively, at 1 month postoperatively. The mean postoperative spherical equivalent was 0.27 ± 0.23 diopter (D). All eyes were within ±0.75 D and 8 eyes (88.9%) were within ±0.50 D. There was no eccentric corneal topography or abnormal morphology in the corneal caps. The corneal opacities of all patients were within the optical zone. The mean preoperative depths in the deepest areas of corneal opacity were 152 ± 38 μm (range: 86-217 μm); at 1 month after surgery (P < .01), they were 117 ± 28 μm (range: 86-189 μm). The preoperative maximum density of corneal opacity was 48.5 ± 20.7 (range: 20.4 to 85.8); at 1 month after surgery (P > .05), it was 49.8 ± 26.7 (range: 19.8 to 82.5) at 1 month after surgery., Conclusion: Patients with corneal opacity can be successfully treated with the SMILE operation. The short-term outcome was good, however the long-term results need further study., Trial Registration: The trial registration number: ChiCTR-ONRC-13003114 , Date of Last Refreshed on: 2016-01-27, Date of registration on 2013-03-17(retrospectively registered).

Objective: Many blind children in the developing world are unable to obtain timely treatment due to lack of financial and medical resources. Can public health programs that identify and treat such children several years after the onset of blindness enhance their quality of life? The notion that visual development is subject to an early 'critical period' argues against this possibility. However, there are inadequate empirical data from humans on this issue. To address this need, we examined the quality of life of children living in India and who were treated for early-onset blindness (before one year of age), due to cataracts or corneal opacities., Study Design: Survey study., Methods: As part of an ongoing scientific effort named Project Prakash, we screened over 40,000 children in rural northern India to identify those suffering from early-onset blindness. They were provided eye surgeries in a tertiary care ophthalmic center in New Delhi. We subsequently surveyed 64 Prakash children, ranging in age from 5 to 22 years and obtained their responses on a multi-dimensional quality of life questionnaire., Results: Nearly all of the subjects indicated that their quality of life had improved after treatment. Children reported marked enhancement in their mobility, independence, and safety, and also in social integration. Surprisingly, we found no significant correlations between quality of life metrics and factors such as age at treatment, gender, time since treatment, and pre-surgery and post-surgery acuity., Conclusions: A key question for public health policy makers is whether a program of surgical intervention for older blind children is likely to be beneficial, or if the resources are better spent on rehabilitation via vocational training and assistive devices. The marked improvements in quality of life we find in our data strongly argue for the provision of surgical care regardless of a child's age., (Copyright © 2017 The Royal Society for Public Health. Published by Elsevier Ltd. All rights reserved.)

Cutaneous horns uncommonly involve the periocular region. Involvement of the ocular surface is particularly rare. The authors present a patient who underwent a perinatal buccal mucosal graft for corneal perforation due to congenital corneal ectasia, most likely resulting from Peters anomaly. She developed a giant ocular horn 10 years later.

Purpose: Direct visualization of the posterior segment is not possible with conventional imaging techniques in eyes with corneal opacification. This study evaluated the efficacy of videoendoscopy in visualizing and hence prognosticating the visual outcomes in such eyes., Design: Prospective, noncomparative, consecutive interventional case series., Methods: This study included 64 eyes of 64 patients with corneal opacification who underwent a diagnostic ophthalmic endoscopy procedure from March 2014 to May 2016. All eyes also underwent conventional B-scan ultrasonography as part of their comprehensive ocular examination. The findings that were noted during endoscopy included the optic disc characteristics, anatomic integrity of the retina, and condition of the retinal blood vessels. The principal outcome measure was the determination of whether the results of the videoendoscopy helped in establishment of or brought a change in the management plan; accordingly, they were classified as either "contributory" or "noncontributory.", Results: The videoendoscopy findings were classified as contributory in 62 of 64 eyes. The remaining 2 eyes had vitreous hemorrhage; endoscopic vitrectomy was done in them to detect an inoperable retinal detachment in 1 eye. With the help of the stereoscopic picture of the fundus provided by the endoscope, a poor visual prognosis in 17 eyes with retinal detachment and 22 eyes with attached retina was determined. Additionally, in eyes that had an attached retina on ultrasound B-scan, the endoscope helped in detecting a glaucomatous disc in 10 of those eyes; 7 of them had glaucomatous optic atrophy. In 3 patients ultrasound did not detect a retinal detachment (false-negative). One eye with detached retina on ultrasound did not actually have a retinal detachment (false-positive). Thus ultrasound had a sensitivity of 88.4% and a specificity of 97.3% in detecting RD as compared with an endoscopic evaluation. Based on the endoscopic findings, 34 of 64 (53%) eyes were identified as having a better visual prognosis and underwent subsequent vision-restoring procedures. This included 10 (16%) patients who directly underwent a corneal procedure and 24 (37.5%) patients who had endoscopic vitreoretinal procedures initially before undergoing a corneal procedure. Endoscopy helped in identifying poor prognosis in 30 of 64 (46.8%) eyes; thus, further invasive procedures could be avoided. The endoscopy procedure in itself did not lead to any complications., Conclusion: Diagnostic endoscopy provided invaluable supplemental information about the posterior segment in eyes with corneal opacification. This helped in better prognostication and in reserving corneal reconstructive procedures for eyes with a favorable visual prognosis., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Case Report: A case is presented of a 4 week-old female neonate with Peters anomaly (PA) and unilateral persistent foetal vasculature (PFV) referred to our centre due to esotropia. At 12 weeks of age, a penetrating keratoplasty and vitrectomy were performed without major complications in the immediate post-operative period. The patient is currently under an intensive treatment for amblyopia and secondary glaucoma., Discussion: Surgical treatment of PFV is controversial, with prevention of amblyopia, phthisis, and glaucoma being the main reasons for it. Patients with unilateral PFV and type II PA could be good candidates for this combined surgical procedure., (Copyright © 2016 Sociedad Española de Oftalmología. Publicado por Elsevier España, S.L.U. All rights reserved.)

In humans suffering from pulmonary disease and a mouse model, transient receptor potential vanilloid 4 (TRPV4) channel activation contributes to fibrosis. As a corneal alkali burn induces the same response, we determined if such an effect is also attributable to TRPV4 activation in mice. Accordingly, we determined if the alkali burn wound healing responses in wild-type (WT) mice are different than those in their TRPV4-null (KO) counterpart. Stromal opacification due to fibrosis in KO (n = 128) mice was markedly reduced after 20 days relative to that in WT (n = 157) mice. Immunohistochemistry revealed that increases in polymorphonuclear leukocytes and macrophage infiltration declined in KO mice. Semi-quantitative real time RT-PCR of ocular KO fibroblast cultures identified increases in proinflammatory and monocyte chemoattractant protein-1 chemoattractant gene expression after injury. Biomarker gene expression of fibrosis, collagen1a1 and α-smooth muscle actin were attenuated along with macrophage release of interleukin-6 whereas transforming growth factor β, release was unchanged. Tail vein reciprocal bone marrow transplantation between WT and KO chimera mouse models mice showed that reduced scarring and inflammation in KO mice are due to loss of TRPV4 expression on both corneal resident immune cells, fibroblasts and infiltrating polymorphonuclear leukocytes and macrophages. Intraperitoneal TRPV4 receptor antagonist injection of HC-067047 (10 mg/kg, daily) into WT mice reproduced the KO-phenotype. Taken together, alkali-induced TRPV4 activation contributes to inducing fibrosis and inflammation since corneal transparency recovery was markedly improved in KO mice., Competing Interests: The authors have declared that no competing interests exist.

Background: The occurrence of an anorectal malformation with Holt-Oram syndrome, microcephaly, and bilateral corneal opacity is rare and to the best of our knowledge has not previously been reported in the literature. Hence, there is a need to document our experience in this case and learn as much as possible from it., Case Presentation: We present the case of a Nigerian female neonate with a postnatal diagnosis of syndromic anorectal malformation associated with Holt-Oram syndrome, microcephaly, and bilateral corneal opacity. The infant had successful staged correction of her anorectal malformation but developed a metastatic Wilms' tumor and died before other corrective procedures could be instituted., Conclusions: An anorectal malformation is here reported to occur with Holt-Oram syndrome, an association that has not been reported previously. To enhance the prognosis and quality of life of children with syndromic anorectal malformation, prenatal ultrasound monitoring of high-risk pregnancies and expertise in prenatal detection of congenital anomalies are invaluable in antenatal care.

Background: Sight loss from trachoma is the end result of a scarring disease process starting in early childhood and characterised by repeated episodes of conjunctival inflammation (active trachoma). Subsequently, the conjunctiva becomes scarred, causing the eyelashes to turn inwards and scratch the cornea (trichiasis), damaging the corneal surface and leading to corneal opacification and visual impairment. It is thought that this process is initiated and driven by repeated infection with Chlamydia trachomatis. We review published longitudinal studies to re-examine the disease process, its progression rates and risk factors., Methodology/principal Findings: We searched PubMed for studies presenting incidence and progression data for the different stages of trachoma natural history. We only included studies reporting longitudinal data and identified 11 publications meeting this criterion. The studies were very heterogeneous in design, disease stage, duration, size and location, precluding meta-analysis. Severe conjunctival inflammation was consistently associated with incident and progressive scarring in five studies in which this was examined. One study reported an association between C. trachomatis infection and incident scarring. No studies have yet demonstrated an association between C. trachomatis infection and progressive scarring. Several studies conducted in regions with low prevalence active disease and C. trachomatis infection found evidence of on-going scarring progression., Conclusions/significance: Overall, there are few longitudinal studies that provide estimates of progression rates and risk factors, reflecting the challenges of conducting such studies. Our understanding of this disease process and the long-term impact of control measures is partial. Intense conjunctival inflammation was consistently associated with scarring, however, direct evidence demonstrating an association between C. trachomatis and progression is limited. This suggests that on-going chlamydial reinfection may not be mandatory for progression of established scarring, indicating that sight threatening trichiasis may continue to evolve in older people in formerly endemic populations, that will require service provision for years after active disease is controlled.

Purpose: To examine the immunohistochemical alterations in the corneal stroma in Peters anomaly (PA) and congenital glaucoma (CG) compared with age-matched normals and acquired adult corneal scarring (AACS)., Methods: The clinical features of PA and CG patients who underwent penetrating keratoplasty were recorded. Immunohistochemistry of cornea and control tissue (normal and acquired corneal scars) was performed with antibodies against collagen types I, III, keratan sulfate, lumican, decorin, and smooth muscle actin followed by semiquantitative analysis of immunolabeling., Results: Clinical features in 2 groups were consistent with PA and CG. Microscopy showed thickened stromal collagen bundles in PA (n = 15), CG (n = 11), and AACS (n = 20) compared with normals (n = 18). PA and CG had distinct immunophenotypes compared with controls. Type I collagen labeling was more intense in CG compared with PA (intensity grading (IG) 2.73 vs. 2.07; P < 0.001). Decorin, lumican, and keratan sulfate labeling was significantly less intense in PA versus AACS (IG; 1.91, 0.38, 1.75 in PA and 2.7, 1.11, 2.61 in AACS. respectively; P = 0.002, P = 0.001 and P = 0.004) and normals (IG 1.92, 1.06, 2.59 respectively; P < 0.001, P < 0.001 and P = 0.005). Collagen I labeling was less intense in CG versus AACS (IG 2.73 vs. 3.09) (P = 0.007). Collagen III labeling was more intense in PA/CG than in normals (IG 0.9, 0.64, 0.62 retrospectively) (P < 0.001 in both)., Conclusions: The immunophenotype of the corneal scar in PA and CG differs from normal corneas and so does PA from AACS. The similarities between CG and AACS suggest that CG scarring has an acquired component.

Acquired perforating disorders are a group of uncommon skin conditions characterized by transepidermal extrusion of altered dermal material, most often associated with diabetes mellitus and chronic kidney failure. Delusional parasitosis is a primary psychiatric disorder in which affected patients have fixed, false beliefs that their skin is infested by parasites, in the absence of any evidence supporting their statements. A 69 year old malepatient addressed the Dermatology Department for a skin eruption consisting of multiple umbilicated keratotic papules with a generalized distribution. The patient believed that the lesions were produced by small parasites entering and exiting his skin. The histopathological examination confirmed the clinical diagnosis of Kyrle's disease. The psychiatric examination established the diagnosis of delusions of parasitosis. This is the first reported case of Kyrle's disease associated with delusions of parasitosis. There is no evidence supporting the hypothesis that delusions of parasitosis might be a predisposing factor for Kyrle's disease. However, we believe that the pruritic dermatosis might have triggered the delusions of parasitosis due to the associated pruritus. On the other hand the constant excoriations and traumatizing of a skin prone to develop idiopathic Kyrle's disease in the attempt to remove the parasites prevented the complete resolution of the lesions.