Macular corneal dystrophy with iridofundal coloboma in the same patient: a unique combination.

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.

Sudden unilateral corneal clouding in diabetic patient: A case report and literature review.

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.

Cornea opacity, uveitis with iris atrophy and lens damage following cosmetic high-intensity ultrasound of the eyelid: a case report.

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.

Outcomes of manual small incision cataract surgery (MSICS) in eyes with corneal opacity.

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.

Upadacitinib inhibits corneal inflammation and neovascularization by suppressing M1 macrophage infiltration in the corneal alkali burn model.

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-1ß, 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.

Axenfeld-Rieger syndrome: a novel histopathologic finding associated with corneal abnormalities.

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.

Corneal opacification, an atypical presentation of cystic fibrosis: a case report and review of the literature.

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.

Endoilluminator-aided cataract surgery in eyes with corneal opacity - A modified surgical approach.

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-_wD8.

Peter's anomaly-A homeotic gene disorder.

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.

Case report of the rare Peters' anomaly complicated with Axenfeld-Rieger syndrome: A case report and brief review of the literature.

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.

Phacoemulsification in patients with corneal opacities using slit illumination of the surgical microscope.

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.

Late Onset Interface Calcium Deposition After Laser In Situ Keratomileusis.

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.

Effects of Superficial Keratectomy in Peripheral Hypertrophic Subepithelial Corneal Opacification on Front and Back Corneal Astigmatism.

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.

Bilateral severe microphthalmia in a neonate with trisomy 8 mosaicism: A new finding.

Mosaic Trisomy 8 is a rare chromosomal abnormality estimated to occur one in 30,000 newborns. The phenotype is highly variable and the severity does not appear to be correlated with the proportion of cells that contain the additional chromosome. Ocular involvement in Trisomy 8 mosaicism has previously been described to include corneal opacities, retinal dystrophy, coloboma, and unilateral microphthalmia. We report a case of severe bilateral microphthalmia in a neonate with Trisomy 8 mosaicism, a previously unrecognized ophthalmic manifestation.

A donor twin discordant with Peters anomaly in a twin-twin transfusion syndrome case: a case report.

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.

Slit-beam retro-illumination assisted phacoemulsification for cataract with coexisting 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.

Prevalence and causes of visual impairment among older persons living in low-income old age homes in Durban, South Africa.

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.

Causes of congenital corneal opacities and their management in a tertiary care center / Causas de opacidades corneanas congênitas e seu controle em um centro de cuidados terciários

ABSTRACT 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.

RESUMO Objetivo: Avaliar as causas e o controle das opa cidades corneanas congênitas diagnosticadas em um centro oftal mológico de atendimento terciário e comparar os dados com um estudo anterior realizado na mesma instituição. Métodos: Prontuários médicos informatizados de todos os pacientes com opacidade corneana congênita diagnosticada no Serviço de Córnea no Wills Eye Hospital (Filadélfia, PA) entre 1º de ja neiro de 2007 e 31 de dezembro de 2015 foram revisados retrospectivamente. Crianças com 12 anos ou menos na primeira consulta foram incluídas no estudo. A demografia dos pacientes, o diagnóstico ocular, a lateralidade, as anormalidades oculares associadas, outras cirurgias oculares realizadas antes ou após a primeira consulta e o tratamento foram extraídos dos prontuários médicos. Resultados: Um total de 77 olhos de 56 pacientes foi examinado. A idade média de apresentação foi de 32,8 ± 44,2 meses, com um tempo médio de acompanhamento de 26,7 ± 30,1 meses. O diagnóstico mais frequente foi anomalia de Peters (53,2%), seguido por dermóide límbico (13,0%), aniridia com glaucoma e microftalmia (6,5%), esclerocórnea e glaucoma congênito (5,2%), idiopático (3,9%), síndrome de Axenfeld-Rieger e síndrome de Hurler (2,6%) e microcórnea (1,3%). Ceratoplastia primária foi realizada em 26 olhos, com desfecho de córnea clara de 76,0% durante o acompanhamento. Conclusão: A anomalia de Peters é a causa mais comum de opacidade corneana congênita encontrada em nossa instituição. A ceratoplastia penetrante é a escolha mais frequente de cirurgia corneana para o tratamento de opacidades corneanas congênitas. Intervenções adicionais durante a ceratoplastia penetrante foram moderadamente correlacionadas positivamente com a falha do enxerto. Este estudo também mostra as taxas de algumas etiologias do que mudou ao longo faz últimas décadas em nosso serviço de córnea de atendimento terciário. Embora a anomalia de Peters continue a ser a causa mais comum das opacidades congênitas da córnea, sua taxa parece estar aumentando na última década. Opacidades congênitas da córnea devido a trauma no nascimento, que é uma das causas evitáveis, foram observadas em um estudo anterior em nossa clínica; no entanto, nenhum caso novo foi observado neste estudo.

Causes of congenital corneal opacities and their management in a tertiary care center.

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.