Clinical and Optical Coherence Tomography Correlation of Recurrence Patterns After Femtosecond Laser-Assisted Anterior Lamellar Keratoplasty in Reis-Bucklers Corneal Dystrophy.

PURPOSE: The aim of this study was to report the clinical profile and patterns of recurrence after femtosecond laser-assisted anterior lamellar keratoplasty (FALK) in Reis-Bucklers corneal dystrophy. METHODS: This is a case series of 5 eyes of 4 patients with Reis-Bucklers corneal dystrophy. Clinical images of recurrence were correlated with the high-resolution optical coherence tomography. Histopathologic examination of excised corneal samples was performed when possible. RESULTS: The median time to recurrence was 2 (1-5) years after FALK. Of the 5 eyes, 1 eye had primary FALK, whereas 4 eyes had secondary interventions, which included previous phototherapeutic keratectomy (once in 1 eye and twice in 2 eyes), and previous penetrating keratoplasty, followed by phototherapeutic keratectomy (1 eye). Recurrence was noted at the level of the subepithelium. In addition, 1 eye showed interface deposits along with epithelial downgrowth at the graft-host bed. CONCLUSIONS: The 2 distinct patterns of recurrence noted were at the subepithelial region and the interface. The clinical patterns of recurrence favor an epithelial origin of recurrent deposits.

Pregnancy outcomes in the medical management of glaucoma: An international multicenter descriptive survey.

PURPOSE: To determine if glaucoma medications are associated with pregnancy and/or postnatal complications. METHODS: Multicenter descriptive survey. Subjects were female patients 18-45 years who were previously pregnant with a diagnosis of glaucoma or ocular hypertension prior to pregnancy. Chart review queried diagnosis, glaucoma severity, and race. Survey questions were asked for each pregnancy and queried pregnancy age, medications used, and pregnancy outcomes/complications. RESULTS: 114 pregnancies of 56 patients (mean 2.0 pregnancies per patient) were included. Three pregnancies with therapeutic abortion were excluded from further analysis. Mean age during pregnancy was 29.1 ± 5.7 years. Of the 111 pregnancies, 20 (18.0%) used no medications and 91 (82.0%) used at least one medication. Medications were topical carbonic anhydrase inhibitors (n = 45), beta-blockers (n = 55), alpha-agonists (n = 56), and prostaglandin analogues (n = 28). Outcomes were: preterm contractions/labour (6.3%), miscarriage (4.5%), stillbirth (4.5%), induction of labour (11.9%), emergency/unplanned caesarean delivery (13.9%), neonatal intensive care unit (NICU) stay (15.8%), congenital anomalies (8.1%), and low birth weight (10.9%). Fisher exact test assessed outcome associations with individual agents, use of any agent, and different number of agents. Alpha-agonist use was associated with NICU stay: 25.5% rate (p = 0.012) in alpha-agonist use. Most of the alpha-agonist use NICU stays occurred in pregnancies with third trimester use. All other associations were not statistically significant. CONCLUSIONS: The data from this survey suggest an overall favourable safety profile for topical glaucoma medications in pregnancy, but further investigation is needed. Caution should be employed regarding third trimester alpha-agonist use owing to association with NICU stay.

Band-shaped keratopathy in HNF4A-related Fanconi syndrome: a case report and review of the literature.

BACKGROUND: Fanconi's syndrome (FS) is characterized by type-2 renal tubular acidosis, short stature, and renal rickets, along with glycosuria, aminoaciduria, hypophosphaturia, and urinary bicarbonate wasting. The genetic form of FS has been linked to HNF4A variants. Although additional clinical features such as hearing impairment have recently been associated with HNF4A-linked FS, its ocular manifestation has not been described. MATERIAL AND METHODS: Presenting a case of a 5-year-old male child with bilateral progressive corneal opacification and the presence of bilateral greyish-white deposits in the interpalpebral region since infancy. A next-generation sequencing (NGS)-based genetic testing was performed for the child followed by parental genetic testing for the identified variant. Furthermore, relevant works of literature were reviewed related to this condition. RESULTS: Detailed corneal findings showed a bilateral band-shaped keratopathy (BSK) in the patient. Physical and systemic findings showed signs consistent with FS. Sequencing analysis revealed a novel heterozygous c.635C>T, (p.Pro212Leu) variant in the HNF4A gene in the proband and mother, while the father had a normal genotype. CONCLUSIONS: Our case highlights the occurrence of BSK in an exceptionally rare manifestation of hereditary FS linked to HNF4A gene variant. The variant exists both in proband and asymptomatic mother. Therefore, the variable penetrance which is known to exist in HNF4A is acknowledged in this context. This report suggests the first documented instance establishing a plausible connection between BSK and HNF4A-associated FS, characterized by the variable penetrance attributed to the HNF4A gene.

Corneal Vascularization Associated With a Novel PDGFRB Variant.

Purpose: The purpose of this study was to identify the genetic cause of aggressive corneal vascularization in otherwise healthy children in one family. Further, to study molecular consequences associated with the identified variant and implications for possible treatment. Methods: Exome sequencing was performed in affected individuals. HeLa cells were transduced with the identified c.1643C>A, p.(Ser548Tyr) variant in the platelet-derived growth factor receptor beta gene (PDGFRB) or wild-type PDGFRB. ELISA and immunoblot analysis were used to detect the phosphorylation levels of PDGFRß and downstream signaling proteins in untreated and ligand-stimulated cells. Sensitivity to various receptor tyrosine kinase inhibitors (TKIs) was determined. Results: A novel c.1643C>A, p.(Ser548Tyr) PDGFRB variant was found in affected family members. HeLa cells transduced with this variant did not have increased baseline levels of phosphorylated PDGFRß. However, upon stimulation with ligand, excessive activation of PDGFRß was observed compared to cells transduced with the wild-type variant. PDGFRß with the p.(Ser548Tyr) amino acid substitution was successfully inhibited with tyrosine kinase inhibitors (axitinib, dasatinib, imatinib, and sunitinib) in vitro. Conclusions: A novel c.1643C>A, p.(Ser548Tyr) PDGFRB variant was found in family members with isolated corneal vascularization. Cells transduced with the newly identified variant showed increased phosphorylation of PDGFRß upon ligand stimulation. This suggests that PDGF-PDGFRß signaling in these patients leads to overactivation of PDGFRß, which could lead to abnormal wound healing of the cornea. The examined TKIs prevented such overactivation, introducing the possibility for targeted treatment in these patients.

An ophthalmology professionalism survey tool: outcomes from a multi-center study in Central India.

Purpose: To describe a professionalism survey tool and its use to assess knowledge of medical professionalism in ophthalmology training programs in Central India. Settings and Design: Multi-center survey study. Methods: A validated 33-question, scenario-based survey addressing professionalism attributes was administered at five centers in central India. The attributes tested included "personal characteristics," "physician-patient relationships," "workplace practice and relationships," and "socially responsible behaviors." A mean attribute score (%) was calculated and compared to "gold standard" responses by a group of expert senior ophthalmologists (100% agreement for responses). Results: A total of 225 participants completed the survey; 124 residents, 47 fellows, and 54 consultants (98.4% response rate). The total mean attribute score was 80.7 ± 9.1 (min 16.67, max 100). There was variation in the mean attribute score by professionalism attribute (P < 0.001), and a trend toward higher mean attribute scores for consultants compared to trainees across all attribute groups. The scores for "personal characteristics" (93 ± 9.7) and "physician-patient relationship" (82 ± 15.8) were the highest, whereas scores for "socially responsible behaviors" (73.9 ± 18.6) and "workplace practices" were low (72 ± 13). Conclusions: There is a generally high level of professionalism knowledge among ophthalmologists in central India. The results suggest that experience does impact knowledge of professionalism. Potential for improvement in professionalism exists in around "workplace practices", and around "socially responsible behaviors". These findings may serve as a valuable discussion starter and teaching tool to enhance professionalism in ophthalmology training programs.

Inflammation and Fibrosis in Orbital Inflammatory Disease: A Histopathologic Analysis.

PURPOSE: The purpose of this study was to compare the histopathologic inflammation and fibrosis of orbital adipose tissue in orbital inflammatory disease (OID) specimens. METHODS: In this retrospective cohort study, inflammation, and fibrosis in orbital adipose tissue from patients with thyroid-associated orbitopathy (TAO), granulomatosis with polyangiitis (GPA), sarcoidosis, nonspecific orbital inflammation (NSOI), and healthy controls were scored by 2 masked ocular pathologists. Both categories were scored on a scale of 0 to 3 with scoring criteria based on the percentage of specimens containing inflammation or fibrosis, respectively. Tissue specimens were collected from oculoplastic surgeons at 8 international centers representing 4 countries. Seventy-four specimens were included: 25 with TAO, 6 with orbital GPA, 7 with orbital sarcoidosis, 24 with NSOI, and 12 healthy controls. RESULTS: The mean inflammation and fibrosis scores for healthy controls were 0.0 and 1.1, respectively. Orbital inflammatory disease groups' inflammation (I) and fibrosis (F) scores, formatted [I, F] with respective p -values when compared to controls, were: TAO [0.2, 1.4] ( p = 1, 1), GPA [1.9, 2.6] ( p = 0.003, 0.009), sarcoidosis [2.4, 1.9] ( p = 0.001, 0.023), and NSOI [1.3, 1.8] ( p ≤ 0.001, 0.018). Sarcoidosis had the highest mean inflammation score. The pairwise analysis demonstrated that sarcoidosis had a significantly higher mean inflammation score than NSOI ( p = 0.036) and TAO ( p < 0.0001), but no difference when compared to GPA. GPA had the highest mean fibrosis score, with pairwise analysis demonstrating a significantly higher mean fibrosis score than TAO ( p = 0.048). CONCLUSIONS: Mean inflammation and fibrosis scores in TAO orbital adipose tissue samples did not differ from healthy controls. In contrast, the more "intense" inflammatory diseases such as GPA, sarcoidosis, and NSOI did demonstrate higher histopathologic inflammation and fibrosis. This has implications in prognosis, therapeutic selection, and response monitoring in orbital inflammatory disease.

Computer Vision Syndrome in Undergraduate and Medical Students During the COVID-19 Pandemic.

Purpose: The purpose of this study was to evaluate Computer Vision Syndrome (CVS) in undergraduate and medical students since transitioning to online learning during the COVID-19 pandemic. Patients and Methods: This was a cross-sectional single center survey-based study using a validated CVS questionnaire (CVS-Q). The survey was distributed to 20,080 undergraduate students and 680 medical students at the University of Illinois at Chicago. The primary outcome measures were prevalence of CVS (based on CVS severity score of 6 or more), frequency of CVS and intensity of CVS symptoms. Results: The survey was completed by 2300 undergraduate students (11.4% response rate) and 154 medical students (22.6% response rate). The prevalence of CVS was 77.1% in undergraduate students and 69.1% in medical students. CVS-Q severity scores were highest for headaches and eye dryness, with over half of students reporting worsening of symptoms since March 2020. Increased time spent on online learning (undergraduate: P <0.001, medical: P = 0.018), blue light glasses usage (undergraduate: P <0.001, medical: P = 0.0015), and increased number of device usage were associated with higher CVS severity scores (undergraduate: P <0.001, medical: P = 0.0032). Conclusion: CVS among undergraduate and medical students has increased since the start of the COVID-19 pandemic. More focus should be placed on the management of CVS for students in higher education. Physicians should be cognizant of the consequences of online learning and be proactive about providing advice regarding preventative measures.

Topical netarsudil for treatment of glaucoma with elevated episcleral venous pressure: A pilot investigation in sturge-weber syndrome.

PURPOSE: Topical netarsudil 0.02% may reduce intraocular pressure (IOP) by decreasing episcleral venous pressure (EVP), which carries theoretical utility for glaucoma associated with elevated EVP. A role for netarsudil in patients with elevated EVP is evaluated in a pilot investigation using a cohort of individuals with Sturge-Weber syndrome (SWS). METHODS: Retrospective study of patients with SWS and glaucoma who were treated with netarsudil. Five patients (six eyes) were identified. Data collected included demographics, visual acuity, IOP, glaucoma medical and surgical treatments, and adverse effects of netarsudil. RESULTS: Mean age was 13.6 ± 8.5 years. EVP elevation was presumed based on clinical stigmata and/or historical features. Mean number of baseline glaucoma medications was 3.3 ± 1.2. There was a significant reduction in the IOP at netarsudil initiation (mean 26.2 ± 4.5 mmHg) to 1 month of netarsudil therapy (mean 20.2 ± 3.8 mmHg, p = 0.0283) and latest IOP on netarsudil (mean 17.6 ± 1.4 mmHg, p = 0.0034). Mean duration of netarsudil therapy was 18.7 ± 11.8 months. Three patients required additional glaucoma procedures; one patient required an additional glaucoma medication. Three eyes (50%) developed conjunctival hyperemia. One patient discontinued netarsudil at 29 months, to reduce drop burden. CONCLUSIONS: Netarsudil can effectively reduce IOP in patients with SWS, even when used as a fourth or fifth glaucoma medication. A possible role for netarsudil in the management of patients with elevated EVP is suggested pending further future investigations.

Characteristics of Corneal Endothelium in Axenfeld Rieger Spectrum.

PURPOSE: The purpose of this study was to compare the corneal endothelial characteristics in Axenfeld anomaly (AXA), Rieger anomaly (RGA), and Axenfeld-Rieger anomaly/syndrome with age-matched healthy controls. METHODS: This is a retrospective, comparative case-control study of 52 eyes of 30 patients with AXA/RGA and AXA/S and 36 controls. RESULTS: Median age at endothelial imaging was 21.5 years (interquartile range, 13.8-33.3 years). In the study group, the mean endothelial cell density (ECD) was 2112.4 ± 78.5 cells/mm 2 , the mean cell area (MCA) was 526.9 ± 28.5 µm 2 , and the coefficient of variation of cell size was 41.2 ± 1.8%. The ECD was significantly (all, P < 0.0001) lower than controls, while MCA ( P < 0.0001), SD of cell size ( P < 0.0001), and maximum cell area ( P = 0.0007) were significantly higher than controls. Four eyes of 3 patients had guttae on slitlamp evaluation and endothelial imaging. There were no differences in the corneal endothelial characteristics among the clinical subtypes. CONCLUSIONS: Patients with AXA, RGA, and Axenfeld-Rieger anomaly/syndrome have lower ECD and increased MCA compared with normal eyes. The reduced ECD associated with inherent anterior segment alterations can predispose to the risk of postcataract surgery endothelial decompensation in these eyes. The association of guttae in some eyes needs further investigational studies.

Axenfeld-Rieger syndrome in the pediatric population: A review.

Axenfeld-Rieger syndrome (ARS) is a rare autosomal-dominant neurocristopathy that presents with a variety of classical ocular and systemic findings. The pathophysiology of the disease involves anterior segment dysgenesis, and patients may present with ophthalmic complications early in life, including secondary glaucoma, high refractive errors, amblyopia, and permanent visual damage. There are a limited number of studies in the literature that focus primarily on pediatric patients with ARS. The purpose of this article was to review the current literature on clinical presentation, genetic associations, diagnosis, secondary complications, and treatment of ARS in pediatric patients. Evaluating the essential clinical aspects of the disease in children may allow for earlier diagnosis and treatment and prevent visual morbidity from amblyopia and secondary glaucoma that may result in permanent visual damage.

Newer advances in medical management of glaucoma.

The burden of irreversible vision loss from Glaucoma continues to rise. While the disease pathogenesis is not well understood, intraocular pressure (IOP) is the only modifiable risk factor identified to prevent glaucomatous vision loss. Medical management remains the first-line of treatment in most adult glaucomas and the evolution of medical therapy for glaucoma has followed an exponential curve. This review tracks the rapid development of new medications and drug delivery systems in the recent years. Introduction of Rho kinase inhibitors with an entirely new mechanism of action from that of the currently used anti glaucoma medications has been a significant milestone. Latanoprostene Bunod is a novel, single molecule which provides two active metabolites that work through two different pathways for reducing intra ocular pressure. Bimatoprost implants and travoprost punctum plugs attempt to ease chronic medication use in glaucoma patients. Nanotechnology is an evolving route of drug delivery. Role of cannabinoids in medical management of glaucoma remain equivocal. The relatively short term effect on IOP, the risks of developing tolerance and side effects impacting patients' neurocognitive health greatly outweigh the potential benefit. Research on Latrunculin B, Adenosine receptor agonists, Specific gene silencing and Stem cell therapy are poised to make an impact on glaucoma treatment. While there is some evidence to support the role of Brimonidine in neuroprotection, further research is needed to clarify the role of Memantine and Neurotrophins. Evidence for benefit from dietary supplementation with Alpha lipoic acid, Forskolin , and Ginko Biloba is limited.

Periocular Pigmented Basal Cell Carcinomas: Clinicopathologic Features and Mutational Profile.

PURPOSE: Pigmented basal cell carcinomas (PBCC) is an uncommon variant of basal cell carcinoma of the periocular region with limited information in the literature. We highlight the clinicopathological profile and somatic mutations in periocular PBCC. METHODS: The clinicopathological features and somatic mutations in patients with periocular PBCC were examined and compared with periocular non-PBCC reported in the literature. Next-generation sequencing panel analysis for the excised tumors identified somatic mutations. RESULTS: In a total of 31 patients, PBCC was common in females (54%; p = 0.03); as a unilateral lower eyelid (n = 22; 71%), solitary mass (n = 30; 98%). Pathologic subtypes were variable. Most were nodular or mixed variants (n = 23; 74%). During the follow up (2.5-4.5 years), 1 patient (3.5%) had a recurrence. The clinical and pathologic features of PBCC were similar to those reported in nonperiocular locations. Somatic mutations detected in 25/31 tumors. Variants in 50/161 genes in the panel were noted. PTCH1 (14/31), TERT (12/31), and SMO (7/31) variants were common. Fifteen patients had novel drivers, including POLE, FANCD2, and CREBBP. SMO mutations were significantly more common in females (7/7), lower eyelid (5/7), and TERT mutations were more common in nodular subtype (10/12). CONCLUSIONS: In this large cohort of a relatively uncommon variant of BCC, the clinicopathological features and tumor behavior of PBCC was similar to periocular non-PBCC. The somatic mutation spectrum of PBCC resembles that reported in nonperiocular cutaneous BCC with novel drivers identified. We identified several potential actionable mutations that could be targeted with molecular therapy.

Preliminary Surgical Outcomes of a Trimmed-Plate Aurolab Aqueous Drainage Implant (AADI) in Eyes at High Risk of Hypotony.

Purpose: We describe the technique of trimming the 350 mm2 AADI glaucoma shunt plate and report preliminary results that test the hypothesis that the IOP-lowering efficacy of the trimmed AADI glaucoma shunt is comparable to the Baerveldt 250 mm2 glaucoma drainage implant with a comparable safety profile to the standard AADI implant. Methods: Consecutive patients who had received the modified trimmed-plate AADI, standard AADI and Baerveldt 250 mm2 were included in the study. This included patients with refractory or primary or secondary glaucoma of all ages and eyes with and without previous glaucoma surgery. The decision for trimming the AADI plate was made according to the surgeon's perceived risk of hypotony. Pre-operative, intraoperative and post-operative data were collected from the hospital electronic medical record system. Surgical success was defined as IOP ≥5 mmHg and ≤21 mmHg on two consecutive visits after 3 months, whilst maintaining at least LP vision and avoiding re-operation for glaucoma. Results: The sample consisted of 69 eyes (19 with trimmed-plate AADI implant; 36 eyes with the standard AADI implant and 14 eyes who received a BGI-250). The mean IOP reduction at 1 year was 15 mmHg for the Baerveldt-250, 10 mmHg for the AADI and 13 mmHg for the trimmed-plate AADI. The surgical success rate of the implants over 1 year was 85.7% (95% CI, 53.9-96.2%) for BGI-250, 81.5% (62.6-91.5%) for standard AADI and 78.2% (51.7-91.3%) for the trimmed AADI. Conclusion: Trimming the plate of the AADI manually may provide a safe and low-cost method of obtaining a successful surgical outcome in eyes at high risk of hypotony.

Barriers and adherence to glaucoma medication in a paediatric glaucoma population: A cross-sectional survey in central Saudi Arabia.

PURPOSE: To examine barriers and adherence to topical glaucoma medication in a pediatric glaucoma population in Saudi Arabia. METHODS: This cross-sectional study was undertaken at the King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia during 2016-2017. Parents (father or mother) of children aged 6 months to 15 years with a history of glaucoma were interviewed. Rasch analysis was performed to assess the psychometric properties of the developed 12-item barriers-to-adherence tool (BAT-12) and to identify barriers deemed highly important. The 12-items were adapted from previous studies examining different barriers to adherence in different populations. Medication adherence was assessed by asking parents a series of closed-ended questions about the frequency of missed medications. Determinants of missed medications on at least 1 out of the last 3 days were examined using Poisson regression. RESULTS: One hundred and six parents were interviewed. The 12-item scale had acceptable psychometric properties. Barriers deemed important were forgetfulness (logit -0.59), complex dosing regimen (logit 0.09), and being too busy with other work/activities (logit 0.14). When asked how often, on average, their child missed the prescribed medication, 26 (24.5%) reported daily, 17(16.0%) reported few times per week, and 32 (30.2%) reported once per week. A third of parents (n = 37, 34.9%) reported having missed giving all drops on at least one day in the last 3 days. Poisson regression with robust variance revealed that increase in age of the child (prevalence ratio, 1.08 [95% CI, 1.03-1.14]; p = 0.003) and increase in parents' barriers score (prevalence ratio, 2.13 [95% CI, 1.49, 3.03]; p < 0.001) were significantly associated with having missed medications on at least one out of the last three days while adjusting for the effect of father's current employment status and area of residence. CONCLUSIONS: The tool we used to examine barriers to glaucoma medication had acceptable psychometric properties and could be used and strengthened in future studies. Our study highlights poor adherence to glaucoma medications among children with glaucoma.

Open globe injury and intraocular foreign body following crossbow-related penetrating ocular trauma.

PURPOSE: To describe a case of a penetrating ocular trauma and plastic intraocular foreign body (IOFB), undetected on preoperative imaging. OBSERVATIONS: We present the findings of a 40-year-old male who sustained an open globe injury and IOFB composed of plastic following crossbow-related trauma. Preoperative detection of the IOFB was unsuccessful on clinical exam, computed tomography (CT) and ultrasonography. During extraction of the traumatic cataract, an intralenticular IOFB was discovered and removed through an enlarged limbal incision. Postoperative review revealed that a fragmented plastic "nock", from the crossbow arrow bolt, was the likely IOFB source. The bolt was produced by injection molding which may lead to trapped gas within the plastic, causing radiolucency on CT. CONCLUSIONS AND IMPORTANCE: Radiolucent plastic warrants consideration on the differential diagnosis when intraocular gas is noted on computed tomography following penetrating ocular trauma. Multimodal imaging should be considered if IOFB is suspected and not detected by CT.

Spontaneous lens resorption in a patient with Marshall-Stickler Syndrome and glaucoma.

PURPOSE: The purpose of this case report is to describe the increased fragility of anterior capsule in patients with the rare genetic disease Marshall-Stickler syndrome. OBSERVATIONS: We describe a 3-year old patient with Marshall-Stickler Syndrome and congenital glaucoma treated with glaucoma drainage implant placement who subsequently developed spontaneous lens resorption in one eye, which was noted incidentally during one follow-up appointment. CONCLUSIONS AND IMPORTANCE: It is hypothesized that the lens material left the eye through the glaucoma drainage implant over time. This process was not associated with elevation of intraocular pressures or inflammation and did not require subsequent surgery to remove any residual lens fragments, which has not been reported before to the best of the authors' knowledge.

Posterior Embryotoxon Revisited: An Immunohistologic Study.

PURPOSE: This series describes the immunopathologic features of posterior embryotoxon (PE) and demonstrates that it is not an anterior displaced Schwalbe's line as commonly described, but a peripheral corneal stromal nub variable in location with abnormal extracellular matrix. DESIGN: Case series. PARTICIPANTS: Archived specimens from patients with PE. METHODS: Sections from archived formalin-fixed, paraffin-embedded specimens (n = 9; 7 autopsy and 2 trabeculectomy specimens) were examined by light microscopy. Immunohistochemistry was performed on 5 specimens to characterize the extracellular matrix composition of PE. RESULTS: Posterior embryotoxon appeared as nubs of whorled collagen extending from the corneal stroma, lined in some instances, by Descemet membrane. These nubs were located anterior to Schwalbe's line (n = 4), posteriorly (n = 1), partially embedded in the trabecular meshwork (n = 1), or at Schwalbe's line (n = 2). Qualitatively, collagen I labeling of the PE stroma was similar or weaker than the corneal stroma, whereas collagen III staining was focal and slightly more intense compared with the corneal stroma. Lumican and keratan sulfate staining was similar or less intense in PE compared with the corneal stroma. MAIN OUTCOME MEASURES: Identify location of PE and its immunohistochemical features. CONCLUSIONS: In contrast to the widely accepted definition of PE as a prominent, anteriorly displaced Schwalbe line, histologic evidence suggests that it is a direct extension of the corneal stroma with variable locations that may displace the attenuated Descemet membrane when located anterior to or at Schwalbe's line. Immunohistochemical examination revealed that the composition of PE's extracellular matrix was similar to corneal stroma but with some variability in staining intensity.

Secondary developmental glaucoma in eyes with congenital aphakia.

PURPOSE: To describe the clinical spectrum and management of glaucoma in congenital aphakia. METHODS: The demographics and clinical spectrum of eyes with congenital aphakia with and without glaucoma were compared, and management outcomes of congenital aphakia cases with glaucoma were studied retrospectively between April 2000 and June 2020. RESULTS: There were a total of 168 eyes (84 subjects) with a diagnosis of congenital aphakia, of which 29 eyes of 18 subjects were diagnosed with glaucoma. Corneal opacity was the presenting complaint in 26/29 eyes with glaucoma and 139/139 eyes without glaucoma. The (interquartile range (IQR)) horizontal corneal diameterwas 10.5mm (IQR, 9.0-12.5) and 8mm (IQR, 5-10) in eyes with and without glaucoma (P = 0.01), respectively. The median (IQR) axial length was 17.5mm (IQR, 13.5-19.5) and 15mm (IQR, 14-16) mm in eyes with and without glaucoma (P = 0.03), respectively. Nineteen eyes with glaucoma had adequate intraocular pressure (IOP) control with one medication. Three eyes underwent transscleral diode cyclophotocoagulation and maintained IOP without medications. Three eyes underwent trabeculectomy and trabeculotomy, trabeculectomy followed by penetrating keratoplasty, and trabeculectomy, respectively, of which two eyes became phthisical. At the last follow-up, the median (IQR) IOP was 14 mm Hg (IQR, 14-17) Hg. The median(IQR) follow-up duration was 4.53 months (IQR, 2.03- 48.06). CONCLUSION: One-fifth of the eyes with congenital aphakia had secondary developmental glaucoma. The corneal diameter and axial lengths were higher in the eyes with glaucoma compared to eyes without glaucoma. Medical management is the preferred short-term mode of IOP control. Transscleral cyclophotocoagulation may be preferred over surgical intervention.