Epigenetic regulation of autophagy by histone-modifying enzymes under nutrient stress.

Autophagy is an evolutionarily conserved catabolic process that is induced in response to various stress factors in order to protect cells and maintain cellular homeostasis by degrading redundant components and dysfunctional organelles. Dysregulation of autophagy has been implicated in several conditions such as cancer, neurodegenerative diseases, and metabolic disorders. Although autophagy has been commonly considered as a cytoplasmic process, accumulating evidence has revealed that epigenetic regulation within the nucleus is also important for regulation of autophagy. In particular, when energy homeostasis is disrupted, for instance due to nutrient deprivation, cells increase autophagic activity at the transcriptional level, thereby also increasing the extent of overall autophagic flux. The transcription of genes associated with autophagy is strictly regulated by epigenetic factors through a network of histone-modifying enzymes along with histone modifications. A better understanding of the complex regulatory mechanisms of autophagy could reveal potential new therapeutic targets for autophagy-related diseases. In this review, we discuss the epigenetic regulation of autophagy in response to nutrient stress, focusing on histone-modifying enzymes and histone modifications.

Systemic approaches using single cell transcriptome reveal that C/EBPγ regulates autophagy under amino acid starved condition.

Autophagy, a catabolic process to remove unnecessary or dysfunctional organelles, is triggered by various signals including nutrient starvation. Depending on the types of the nutrient deficiency, diverse sensing mechanisms and signaling pathways orchestrate for transcriptional and epigenetic regulation of autophagy. However, our knowledge about nutrient type-specific transcriptional regulation during autophagy is limited. To understand nutrient type-dependent transcriptional mechanisms during autophagy, we performed single cell RNA sequencing (scRNAseq) in the mouse embryonic fibroblasts (MEFs) with or without glucose starvation (GS) as well as amino acid starvation (AAS). Trajectory analysis using scRNAseq identified sequential induction of potential transcriptional regulators for each condition. Gene regulatory rules inferred using TENET newly identified CCAAT/enhancer binding protein γ (C/EBPγ) as a regulator of autophagy in AAS, but not GS, condition, and knockdown experiment confirmed the TENET result. Cell biological and biochemical studies validated that activating transcription factor 4 (ATF4) is responsible for conferring specificity to C/EBPγ for the activation of autophagy genes under AAS, but not under GS condition. Together, our data identified C/EBPγ as a previously unidentified key regulator under AAS-induced autophagy.

Change in the eye position under general anesthesia in children with intermittent exotropia.

PURPOSE: To evaluate the eye position in subjects with intermittent exotropia and normal subjects under general anesthesia (GA) using the strabismus photo analyzer. METHODS: This retrospective case-control study included 78 subjects with intermittent exotropia and 25 normal control subjects who underwent epiblepharon surgery. Eye position under GA was assessed using the strabismus photo analyzer, based on eye models generated from corneal lights and limbus in pre- and post-anesthesia images. Eye positions under GA in the control and intermittent exotropia groups were compared. Preoperative angle of deviation was also compared with amount of change in eye position under GA in the intermittent exotropia group. RESULTS: Eye position under GA was more divergent in subjects with intermittent exotropia than in controls (P = 0.008). The amount of change in eye position under GA was correlated with the preoperative angle of deviation (r2 = 0.47; P < 0.001). In small preoperative exodeviations, the change in eye position was primarily more divergent, whereas in large exodeviations, a convergent tendency-less exotropic compared with the preoperative angle of exodeviation-was observed. CONCLUSIONS: In subjects with small preoperative exodeviations, there was a tendency for eye position to become more divergent after GA; in those with large exodeviations, there was less exotropia after GA.

Development of New Solitary Retinoblastoma Tumors during and after Chemotherapy.

PURPOSE: To review the occurrence of new solitary tumors during and after intravenous chemotherapy against retinoblastoma. METHODS: From 115 eyes of 78 patients with a diagnosis of intraocular retinoblastoma who underwent intravenous chemotherapy and focal treatment without prior treatment, patient demographics, age at diagnosis, laterality, classification (Reese-Ellsworth and International Classification of Retinoblastoma), and treatment options were recorded. In addition, the occurrence of small tumors during and after chemotherapy was documented with a detailed review of medical records and fundus photographs. RESULTS: Of a total of 115 eyes of 78 consecutive patients, new solitary tumors were observed in 50 eyes (50 / 115, 43%) of 40 patients (40 / 78, 51%). Multinominal logistic regression analyses showed that age at diagnosis (before 1 year) and vitreal seeding at diagnosis were linked to the development of isolated and miliary tumors, respectively. Kaplan-Meier analyses demonstrated that all small tumors developed with 20 months from the start of chemotherapy. Twenty-eight eyes (28 / 34, 82%) were salvaged with additional focal treatment in 34 eyes with isolated tumors. CONCLUSIONS: Small tumors were observed during and after chemotherapy against retinoblastoma in patients who underwent intravenous chemotherapy and focal treatment. It is necessary to promptly identify and address small tumors for the preservation of eyeball and vision.

Postnatal ophthalmological characteristics in patients with congenital cataract diagnosed by fetal ultrasonography.

PURPOSE: To evaluate the findings of postnatal ophthalmological examinations in patients diagnosed with cataracts by prenatal fetal sonography. METHODS: We retrospectively identified patients diagnosed with fetal cataract without other ocular abnormalities using antenatal ultrasound. We analyzed data including the patients' family history, the presence of metabolic or systemic disease-associated cataracts, gestational age at diagnosis, fetal sonography reports, and the results of postnatal ophthalmological examinations. RESULTS: In total, eight patients were diagnosed with cataracts by prenatal fetal ultrasonography, and all these patients showed mild to severe degrees of cataract after birth. Patients showed hyperechoic opacity of the lens in the available images. The mean gestational age at diagnosis was 26 ± 3 weeks (range, 22-28 weeks), and three patients (37.5%) had a family history of congenital cataract. All patients, except one patient who had patent ductus arteriosus, showed no systemic abnormalities associated with cataracts on postnatal evaluation. Six (75.0%) were diagnosed with bilateral cataracts after birth, and of these, two patients had been diagnosed with unilateral left cataract prenatally. Postnatal ophthalmological examination revealed that all patients showed opacities involving the nucleus of the lens and three patients (37.5%) had associated abnormalities of microcornea or microphthalmia, which were not diagnosed antenatally. CONCLUSION: In all our subjects, the lens opacities on prenatal fetal sonography were diagnosed postnatally as cataracts involving the nucleus. A careful postnatal examination is essential to detect the presence of associated ocular abnormalities such as microcornea and/or microphthalmia and cataract in the contralateral eye in patients with congenital cataract.

Pontin arginine methylation by CARM1 is crucial for epigenetic regulation of autophagy.

Autophagy is a catabolic process through which cytoplasmic components are degraded and recycled in response to various stresses including starvation. Recently, transcriptional and epigenetic regulations of autophagy have emerged as essential mechanisms for maintaining homeostasis. Here, we identify that coactivator-associated arginine methyltransferase 1 (CARM1) methylates Pontin chromatin-remodeling factor under glucose starvation, and methylated Pontin binds Forkhead Box O 3a (FOXO3a). Genome-wide analyses and biochemical studies reveal that methylated Pontin functions as a platform for recruiting Tip60 histone acetyltransferase with increased H4 acetylation and subsequent activation of autophagy genes regulated by FOXO3a. Surprisingly, CARM1-Pontin-FOXO3a signaling axis can work in the distal regions and activate autophagy genes through enhancer activation. Together, our findings provide a signaling axis of CARM1-Pontin-FOXO3a and further expand the role of CARM1 in nuclear regulation of autophagy.

Spontaneous regression of congenital corneal opacity.

PURPOSE: To determine the incidence of spontaneous regression of congenital corneal opacity (CCO) and identify clinical factors associated with the regression. METHODS: Medical records and anterior segment photographs were reviewed of 57 eyes in 35 patients with CCO that were not related to congenital glaucoma, tumors, infection, trauma, or metabolic disorders and were followed up without corneal transplantation for longer than one year at Seoul National University Hospital. Spontaneous regression of corneal opacity was defined as a decrease in corneal opacity significant enough for visual axis clearance. Data on demographics, systemic, and ocular characteristics were collected and compared between patients who had spontaneous regression of CCO and those who did not. RESULTS: Spontaneous regression of corneal opacity developed in 32 eyes (22 patients, 56.1%) out of 57 CCO eyes (35 patients) at the mean 8.2 ± 5.4 months of age (the median 6.7 months). Absence of combined ocular anomalies such as iris anomaly, lens opacity, and peripheral corneal vascularization was significantly associated with the regression of opacity. CONCLUSIONS: Corneal opacity can spontaneously regress in 56.1% of eyes with CCO during the first year of life. Careful follow-up with amblyopia management can be one of treatment options for CCO.

Factors Associated with the Prognosis after Operation in Children with Recurrent Intermittent Exotropia.

BACKGROUND: To describe factors affecting the prognosis after operation for recurrent intermittent exotropia (X[T]) in children. METHODS: Clinical records of 50 patients who underwent operation for recurrent X(T) by a single surgeon were reviewed. The age at diagnosis of X(T), and first and second operations, deviation angle at distance and near, surgical method, concurrent vertical strabismus, stereoacuity, and Worth's Four Dot (W4D) examination before reoperation were analyzed, along with the postoperative deviation angle. A successful surgical outcome was defined as orthophoria, esodeviation ≤ 5 prism diopters, or exodeviation ≤ 10 prism diopters at distance. RESULTS: Among the 50 recurrent exotropes who underwent surgery and were followed up for more than 1 year postoperatively, 13 showed recurrent exotropia and 1 showed consecutive esotropia. The mean age at reoperation was 8.49 ± 2.19 years, and the mean duration of postoperative follow-up was 27.78 ± 12.02 months. Good near fusion before reoperation was a significant factor in the success of surgery (P = 0.006). Smaller postoperative deviation angle measured immediately and 2 months after surgery were related to smaller final deviation angle (P = 0.027 and P = 0.022, respectively). CONCLUSION: Peripheral suppression lowers the success rate of operation for recurrent X(T) in children. Overcorrection rather than orthotropia should be the target of immediate postoperative deviation angle. Peripheral suppression status and immediate and 2-month postoperative deviation angle may be important clues for predicting the final result of operation for recurrent X(T).

RORα is crucial for attenuated inflammatory response to maintain intestinal homeostasis.

Retinoic acid-related orphan receptor α (RORα) functions as a transcription factor for various biological processes, including circadian rhythm, cancer, and metabolism. Here, we generate intestinal epithelial cell (IEC)-specific RORα-deficient (RORαΔIEC) mice and find that RORα is crucial for maintaining intestinal homeostasis by attenuating nuclear factor κB (NF-κB) transcriptional activity. RORαΔIEC mice exhibit excessive intestinal inflammation and highly activated inflammatory responses in the dextran sulfate sodium (DSS) mouse colitis model. Transcriptome analysis reveals that deletion of RORα leads to up-regulation of NF-κB target genes in IECs. Chromatin immunoprecipitation analysis reveals corecruitment of RORα and histone deacetylase 3 (HDAC3) on NF-κB target promoters and subsequent dismissal of CREB binding protein (CBP) and bromodomain-containing protein 4 (BRD4) for transcriptional repression. Together, we demonstrate that RORα/HDAC3-mediated attenuation of NF-κB signaling controls the balance of inflammatory responses, and therapeutic strategies targeting this epigenetic regulation could be beneficial to the treatment of chronic inflammatory diseases, including inflammatory bowel disease (IBD).

Incidence and Risk Factors for Glaucoma Development After Bilateral Congenital Cataract Surgery in Microphthalmic Eyes.

PURPOSE: To evaluate the long-term incidence and risk of glaucoma after bilateral congenital cataract surgery in microphthalmic eyes. DESIGN: Retrospective, observational case series. METHODS: Subjects: Children with microphthalmic eyes who had undergone surgery for bilateral congenital cataract within 6 months of birth and been followed up for at least 5 years. PROCEDURES: Review of medical records at our institution. MAIN OUTCOME MEASURES: Probability of an eye's developing glaucoma after bilateral congenital cataract surgery and associated risk factors. RESULTS: Thirty-eight eyes of 19 children with bilateral congenital cataract were included. The mean age at surgery was 3.2 ± 1.7 months, and the mean follow-up duration was 7.79 ± 2.61 years. After cataract surgery, 11 eyes (29.0%) developed glaucoma at the age of 4.0 ± 1.4 years. Three of these eyes underwent Ahmed glaucoma valve implantation surgery. The probability of an eye's developing glaucoma was estimated to be 32.0% by 10 years after surgery. In a multivariate analysis, axial length was significantly associated with glaucoma development (odds ratio = 0.364, P = .025). Age at the time of cataract surgery, corneal diameter, and aphakia did not affect the risk of glaucoma (P > .10). Eyes without glaucoma had a better final visual outcome than those with glaucoma (0.75 ± 0.60 and 1.47 ± 1.10 logMAR, respectively, P = .049). CONCLUSIONS: The long-term cumulative risk of postoperative glaucoma development was 32.0% by 10 years after bilateral congenital cataract surgery. Because the risk of developing glaucoma persists for several years after surgery, careful monitoring and control of intraocular pressure is needed to preserve vision in such patients.

Long-term outcomes of bilateral lateral rectus recession versus unilateral lateral rectus recession-medial rectus plication in children with basic type intermittent exotropia.

PURPOSE: To compare the long-term surgical outcomes between bilateral lateral rectus recession (BLR) and unilateral lateral rectus recession-medial rectus plication (RP) in intermittent exotropia. METHODS: Children who underwent BLR or RP for basic type intermittent exotropia between 2015 and 2016 with a minimum follow-up period of 2 years were retrospectively reviewed. Surgical outcomes were classified based on postoperative angle of deviation as follows: success (esodeviation ≤ 5 prism diopters [PD] to exodeviation ≤ 10 PD), and failure (overcorrection [esodeviation > 5 PD] and undercorrection or recurrence [exodeviation > 10 PD]). RESULTS: Of 144 patients, 90 underwent BLR and 54 underwent RP. The angle of exodeviation of the RP group steadily increased over time after the surgery. The BLR group showed an earlier exodrift and a more stable course compared to the RP group. Kaplan-Meier survival analysis showed a better survival in the BLR group, with final success rates of 48.9% in the BLR group and 25.9% in the RP group after a mean follow-up of 2.2 years. Patients with a successful outcome had greater esodeviation at 1 week postoperatively (at distance 7.6 PD in the BLR group, 11.4 in the RP group). CONCLUSIONS: Surgical outcomes were better in the BLR group than in the RP group. The RP group showed higher rates of recurrence of exodeviation, while the BLR group presented a more stable course. Establishing more esodeviation at postoperative week 1 in the RP group compared to the BLR group would be required to achieve successful results.

Development of a patient-derived xenograft model of glioblastoma via intravitreal injection in mice.

Currently, the two primary patient-derived xenograft (PDX) models of glioblastoma are established through intracranial or subcutaneous injection. In this study, a novel PDX model of glioblastoma was developed via intravitreal injection to facilitate tumor formation in a brain-mimicking microenvironment with improved visibility and fast development. Glioblastoma cells were prepared from the primary and recurrent tumor tissues of a 39-year-old female patient. To demonstrate the feasibility of intracranial tumor formation, U-87 MG and patient-derived glioblastoma cells were injected into the brain parenchyma of Balb/c nude mice. Unlike the U-87 MG cells, the patient-derived glioblastoma cells failed to form intracranial tumors until 6 weeks after tumor cell injection. In contrast, the patient-derived cells effectively formed intraocular tumors, progressing from plaques at 2 weeks to masses at 4 weeks after intravitreal injection. The in vivo tumors exhibited the same immunopositivity for human mitochondria, GFAP, vimentin, and nestin as the original tumors in the patient. Furthermore, cells isolated from the in vivo tumors also demonstrated morphology similar to that of their parental cells and immunopositivity for the same markers. Overall, a novel PDX model of glioblastoma was established via the intravitreal injection of tumor cells. This model will be an essential tool to investigate and develop novel therapeutic alternatives for the treatment of glioblastoma.

Long-term surgical outcomes of patients with consecutive exotropia.

PURPOSE: To evaluate the long-term surgical outcome of patients with consecutive exotropia. METHODS: Patients who underwent surgery for the treatment of consecutive exotropia between January 2008 and July 2016 with a minimum follow-up period of 2 years were retrospectively reviewed. Surgical outcomes were classified based on postoperative angle of deviation at 2 years as follows: success (esodeviation ≤ 5 prism diopters [PD] to exodeviation ≤ 10 PD), and recurrence [exodeviation > 10 PD]). Postoperative angles of deviation at 1 week, 1 month, 6 months, 1 year, and 2 years and at the final follow-up were investigated. RESULTS: A total of 37 patients (28 in the success group and 9 in the recurrence group) were included. Surgical success rate at 2 years was 75.7%, and reoperation rate was 10.8% during a mean follow-up period of 42.4 ± 18.3 months after consecutive exotropia surgery. After surgery, exodrift occurred mostly during 1-month follow-up in both groups, and those with no exodrift within 1 month presented a higher surgical success. Thereafter, patients in the success group showed a more stable course during follow-up than those in the recurrence group. Stereopsis was an important factor associated with surgical outcome. CONCLUSIONS: Exodrift occurs mostly within 1 month after surgery for consecutive exotropia. Targeting initial overcorrection and establishing esodeviation at postoperative month 1 is important to achieve successful results.

Progressive Intrascleral Epithelial Cyst With Intracorneal Extension.

The authors report a case of primary sclerolimbal cyst with corneal extension in a 3-year-old boy. The cyst enlarged and dissected into the cornea over time. Surgical excision with tectonic allograft was performed. Preoperative, intraoperative, and postoperative anterior segment findings were shown, and results from histologic and cytologic assays were presented. [J Pediatr Ophthalmol Strabismus. 2019;56:e20-e23.].

Predictability of formulae for intraocular lens power calculation according to the age of implantation in paediatric cataract.

AIMS: To analyse the predictability of diverse intraocular lens (IOL) power calculation formulae in paediatric patients with congenital cataract. METHODS: The medical records of patients who underwent cataract surgery and posterior chamber IOL implantation (in-the-bag) for congenital cataract before 17 years of age were reviewed retrospectively. Target refractions calculated by Sanders-Retzlaff-Kraff (SRK)/II, SRK/T and Hoffer-Q formulae were compared with the actual refraction. Patients were subgroup according to the age at IOL implantation (age group 0-24 months, 25-60 months, 61-120 months, 121-203 months), and we compared mean prediction error (PE) and mean absolute error (AE) for each formula. Corrected AE was obtained by linear regression analysis. RESULTS: Totally 481 eyes were included in the final analysis. Both SRK/II and SRK/T yielded the lowest mean AE in the age group 0-24 months and SRK/II yielded the lowest mean AE in the age group 25-60 months. For every formula, the mean PE was positive during the first five years of age, which converged to zero according to age as IOL implantation increases. The tendency for immediate postoperative overcorrection in younger patients (<6 years) could be improved by corrected formulae based on the linear regression equation. CONCLUSIONS: Patients with congenital cataract who underwent IOL implantation within 5 years of age showed higher AE than the older ones did. Among the three formulae evaluated, SRK/II consistently provided the best predictive result in these patients. For patients aged >10 years, all three formulae showed favourable predictive abilities.

Long-Term Outcome of Nd:YAG Laser Posterior Capsulotomy in Children: Procedural Strategies and Visual Outcome.

PURPOSE: To evaluate the long-term outcome of neodymium:yttrium-aluminum-garnet (Nd:YAG) laser posterior capsulotomy after cataract surgery in children. DESIGN: Retrospective case series. METHODS: Electronic medical records of pediatric patients who underwent Nd:YAG laser posterior capsulotomy between January 1, 2008, and October 31, 2012, and followed up for more than 5 years were reviewed. RESULTS: Thirty-one eyes of 25 patients were included. Only compliant patients assessed during slit-lamp examination and anterior segment photography underwent laser treatment. The mean age at the time of initial laser treatment was 9.04 ± 3.51 years (minimum 56 months), and the mean interval between cataract surgery and initial laser treatment was 28.1 ± 22.1 months. Posterior capsular openings were successfully made in 26 (83.9%) eyes with a single attempt and in 3 (9.7%) eyes with a second attempt. Overall success rate was 93.5%. The logMAR best-corrected visual acuity was significantly improved from 0.61 ± 0.36 to 0.19 ± 0.25 at 1 month posttreatment (P < .0001) and well maintained, at least for 5 years of follow-up, without serious complications. The recurrence of posterior capsular opacity was observed in 7 (24.1%) eyes, which was successfully managed by repeated laser procedure or surgical capsulectomy. CONCLUSIONS: By selecting compliant patients and repeated attempts, Nd:YAG laser posterior capsulotomy can be successfully performed in a pediatric population without serious complications. Laser treatment is also a good option for managing recurred posterior capsular opacity. Restored visual acuity can be maintained for at least 5 years.

Relation between preoperative hyperopia and surgical outcome in infantile esotropia.

AIM: To evaluate the relation between preoperative hyperopia and surgical outcomes of infantile esotropia in patients younger than 24 months of age. METHODS: Medical records of patients who underwent bilateral medial rectus muscle recession for infantile esotropia between November 1, 2002 and December 1, 2011 were retrospectively reviewed. Patients were divided into two groups according to the degree of preoperative hyperopia. Group I had less than +3.0 diopter (D) of hyperopia and group II had between +3.0 and +5.0 D of hyperopia. Postoperative alignments were evaluated 1wk, 3, 6mo, and 1y after surgery. Following the 1-year postoperative visit, patients were monitored yearly. Relationships between preoperative factors including hyperopia and postoperative outcomes were evaluated. RESULTS: Forty-six patients were included, with 33 patients in group I and 13 patients in group II. The preoperative mean refractive error was +0.88 D in group I and +3.45 D in group II. Surgical outcomes were not significantly different between groups at any postoperative time point examined. Cumulative probability of surgical success, prevalence of inferior oblique overaction, dissociated vertical deviation, and re-operation rate were not significantly different between groups. CONCLUSION: Preoperative moderate hyperopia (less than +5.0 D) did not affect the surgical outcome of infantile esotropia. Therefore, the surgical correction of esotropia should be considered when the angle of esodeviation is unchanged following hyperopia correction, even in children with moderate hyperopia.

A comparison of surgical outcomes between pre-and full-term patients with exotropia.

PURPOSE: To compare the surgical outcomes between pre- and full-term patients with exotropia and to examine the factors associated with surgical outcomes. METHODS: This retrospective study included 48 pre- and 432 full-term patients with basic-type exotropia who underwent unilateral or bilateral lateral rectus muscle (ULR or BLR) recession. Preoperative characteristics and surgical outcomes were compared between the pre- and full-term infants. Additionally, factors affecting the surgical outcomes were evaluated in all patients. RESULTS: The preoperative characteristics were significantly different between the pre- and full-term groups in terms of neurodevelopmental disabilities (p = 0.020). There were no significant differences between the pre- and full-term groups in terms of the success, overcorrection, and recurrence rates after the mean follow-up period of 34.6 ± 13.9 months (p = 0.697). The major cause of surgical failure was recurrence in both groups. Pre-term birth was not a risk factor for overcorrection and recurrence. However, regardless of the pre- or full-term birth status, the presence of neurodevelopmental disabilities significantly affected final overcorrection (p = 0.004). CONCLUSIONS: Pre-term patients with exotropia showed similar surgical outcomes to full-term controls. The presence of neurodevelopmental disabilities was a risk factor for final overcorrection.

Clinical features and outcome of corneal opacity associated with congenital glaucoma.

BACKGROUND: To investigate the clinical features of corneal opacity and the surgical outcome of penetrating keratoplasty (PK) in eyes with congenital glaucoma. METHODS: A retrospective review was made of the records from 320 eyes of 193 patients who were diagnosed with congenital glaucoma between January 1981 and January 2016. Anterior segment photographs at disease presentation were examined for the presence and severity of corneal opacity. Data on patient demographics, intraocular pressure (IOP), ocular and systemic comorbidities, ocular surgery and its outcome were collected. RESULTS: Overall, corneal opacification was observed in 248 of 320 eyes (77.5%). Out of 248 eyes with corneal opacification, 53 eyes had Haab striae alone, and 195 eyes presented with either nebulomacular corneal opacity (128 eyes, iris details visible through opacity) or leukomatous corneal opacity (67 eyes, iris details invisible through opacity). In 12 eyes with severe leukomatous corneal opacity, PK was performed at the mean age of 18.6 months (range 4-57 months). The grafts failed in 6 eyes (50%) due to endothelial rejection (4 eyes) or graft infection (2 eyes) during the mean 80.6 months of follow-up (range 15-228 months). The median survival time was 36 months. The graft failure was significantly associated with smaller corneal diameter at the time of surgery, but not with the age, IOP, combined aniridia, simultaneous glaucoma or lens surgery. CONCLUSION: Congenital glaucoma was combined with corneal opacity in 77.5%. The corneal transplant survival was 50% in eyes with congenital glaucoma and total corneal opacity.