[Modern techniques and features of selective keratoplasty]. / Sovremennye tekhnologii i vozmozhnosti selektivnoi keratoplastiki.

Selective keratoplasty involves replacing the affected layers of the cornea with similar donor tissue. In case of pathological changes in the middle and posterior stroma, deep anterior lamellar keratoplasty (DALK) is performed. Chronic corneal edema caused by endothelial dysfunction is an indication for endothelial keratoplasty - Descemet membrane endothelial keratoplasty (DMEK) or Descemet Stripping Endothelial Keratoplasty (DSAEK). Compared to penetrating keratoplasty (PK), these operations are characterized by a low risk of damage to intraocular structures and a relatively short rehabilitation period. Complications of selective keratoplasty include the formation of a false chamber between the lamellar graft and the recipient's cornea, ocular hypertension during anterior chamber air tamponade. Persistent epithelial defect can be a sign of primary graft failure in DALK, DSAEK and DMEK. Selective keratoplasty is characterized by a lower incidence of immune rejection than PK. In some cases, DALK can be complicated by corneal changes related to suture fixation of the graft. Long-term postoperative use of topical glucocorticoids can cause ocular hypertension and cataracts.

[Severe Conjunctival and Corneal Epithelial Dysplasia: A Case Series]. / Schwere konjunktivale und korneale epitheliale Dysplasie: eine Fallserie.

BACKGROUND/OBJECTIVES: Ocular surface squamous neoplasia (OSSN) are among the most frequent non-pigmented malignancies of the ocular surface. They have a wide range of histological characteristics - ranging from mild epithelial dysplasia to invasive carcinoma of the squamous cells of the cornea. They may be restricted to the conjunctiva or also involve the cornea. As there are no leading symptoms in the early stages, diagnosis may be very delayed in patients who do not receive regular ophthalmological treatment. The present case series describes clinical and histological data on OSSN and includes clinical and histological data on OSSN, including possible clinical presentations, important risk factors, special histological and cytological features and therapeutic options. METHODS: Retrospective case series of patients with histologically confirmed severe epithelial dysplasia of the conjunctiva and cornea consistent with OSSN who presented to the Department of Ophthalmology in Basel University Hospital. The analysis covered demographic data, symptoms, diagnostic testing (photo documentation, brush biopsy), treatment and cytological and/or histological material and findings. RESULTS: We report on five patients aged between 41 and 92 years at the time of diagnosis. The histological findings in all patients included severe epithelial dysplasia, but with a heterogenous clinical presentation. In all cases, the lesion started in the conjunctiva, but traversed the limbus and extended to the cornea. The primary treatment was always surgical removal. In one patient, this had to be repeated several times due to recurrent metaplasia and was complemented by subsequent mitomycin C therapy. The clinical outcome ranged between total restitution of the original state to inevitable enucleation. CONCLUSION: The clinical presentation of OSSN is highly heterogenous, so that the initial diagnosis is difficult. There are no official guidelines for treatment, so that the treatment of choice varied between clinics. Regular ophthalmological follow-ups are recommended, even after complete surgical excision. Possible relevant concomitant diseases and risk factors must be identified before therapy.

Clinical features and comprehensive treatment of persistent corneal epithelial dysfunction after cataract surgery.

OBJECTIVE: Evaluation of clinical efficacy and safety of tobramycin/dexamethasone eye ointment in treating persistent corneal epithelial dysfunction (PED) after cataract surgery. METHODS: 26 cases diagnosed as PED after cataract surgery accept the tobramycin/dexamethasone ophthalmic ointment and intense pulse light treatment in the Xiamen University of Xiamen eye center between September 2016 and April 2022 were retrospectively analyzed, mainly including clinical manifestations, characteristics of morphological changes imaged by in vivo confocal microscopy, meibomian glands infrared photography, lipid layer thickness (LLT), management and therapeutic effects. RESULTS: There were 26 eyes, include 8(35%) males and 15(65%) females with an average age of 69.6 ± 5.2 years(50 to 78 years). The mean hospitalization time was (18.4 ± 7.5) days after cataract surgery. Twenty patients had meibomian gland dysfunction. Infrared photography revealed varying loss in the meibomian glands, with a mean score of 3.8 ± 1.2 for gland loss. The mean LLT was 61.6 ± 8.4 nm. After treatment, 20 patients were cured, and 3 received amniotic membrane transplantation. After treatment, the uncorrected visual acuity (UCVA) and best-corrected vision activity (BCVA) improved (P < 0.001), and there was no significant difference in intraocular pressure (IOP) before and after treatment (P > 0.05). CONCLUSIONS: The early manifestation of PED after surgery is punctate staining of the corneal epithelium. Tobramycin and dexamethasone eye ointment bandages have a good repair effect. The meibomian gland massage combined with intense pulse light treatment can effectively shorten the course of the disease.

Exosomal microRNAs as potential biomarkers and therapeutic targets in corneal diseases.

Exosomes are a subtype of extracellular vesicle (EV) that are released and found in almost all body fluids. Exosomes consist of and carry a variety of bioactive molecules, including genetic information in the form of microRNAs (miRNAs). miRNA, a type of small non-coding RNA, plays a key role in regulating genes by suppressing their translation. miRNAs are often disrupted in the pathophysiology of different conditions, including eye disease. The stability and easy detectability of exosomal miRNAs in body fluids make them promising biomarkers for the diagnosis of different diseases. Additionally, due to the natural delivery capabilities of exosomes, they can be modified to transport therapeutic miRNAs to specific recipient cells. Most exosome research has primarily focused on cancer, so there is limited research highlighting the importance of exosomes in ocular biology, particularly in cornea-associated pathologies. This review provides an overview of the existing evidence regarding the primary functions of exosomal miRNAs and their potential role in diagnostic and therapeutic applications in the human cornea.

Diabetic Ocular Surface Has Defects in Oxygen Uptake Revealed by Optic Fiber Microsensor.

Purpose: Diabetes mellitus causes diabetic keratopathy (DK). This and other ocular surface disorders are underdiagnosed and problematic for affected patients as well as recipients of diabetic donor corneas. Thus, it is important to find noninvasive means to facilitate determination of the potentially vision-threatening DK. It has been reported that diabetic corneas uptake significantly less oxygen (O2) than healthy controls. However, an integral assessment of the ocular surface is missing. Methods: Using an optic-fiber O2 micro-sensor (optrode) we demonstrated recently that the healthy ocular surface displays a unique spatiotemporal map of O2 consumption. We hypothesize that diabetes impairs the spatiotemporal profile of O2 uptake at the ocular surface. Results: Using streptozotocin (STZ)-induced diabetic mice, we found diminished O2 uptake and loss of the unique pattern across the ocular surface. A diabetic cornea consumes significantly less O2 at the bulbar conjunctiva and limbus, but not the central and peripheral cornea, compared to controls. Further, we show that, contrary to the healthy cornea, the diabetic cornea does not increase the O2 consumption at the limbus in the evening as the normal control. Conclusions: Altogether, our measurements reveal a previously unknown impairment in O2 uptake at the diabetic cornea, making it a potential tool to diagnose ocular surface abnormalities and suggesting a new etiology mechanism.

A method for teaching depth of corneal lesions using a portable slit lamp (biomicroscope).

A novel, simple, and inexpensive model for teaching depth of lesions within the cornea using slit lamp biomicroscopy to veterinary students, ophthalmology residents, and general clinicians with access to a slit lamp biomicroscope is described. Using common laboratory items, this method can be created in any clinic and can be used to teach and quiz students through independent study with objective self-testing achieved.

Performance of ChatGPT in Diagnosis of Corneal Eye Diseases.

PURPOSE: The aim of this study was to assess the capabilities of ChatGPT-4.0 and ChatGPT-3.5 for diagnosing corneal eye diseases based on case reports and compare with human experts. METHODS: We randomly selected 20 cases of corneal diseases including corneal infections, dystrophies, and degenerations from a publicly accessible online database from the University of Iowa. We then input the text of each case description into ChatGPT-4.0 and ChatGPT-3.5 and asked for a provisional diagnosis. We finally evaluated the responses based on the correct diagnoses, compared them with the diagnoses made by 3 corneal specialists (human experts), and evaluated interobserver agreements. RESULTS: The provisional diagnosis accuracy based on ChatGPT-4.0 was 85% (17 correct of 20 cases), whereas the accuracy of ChatGPT-3.5 was 60% (12 correct cases of 20). The accuracy of 3 corneal specialists compared with ChatGPT-4.0 and ChatGPT-3.5 was 100% (20 cases, P = 0.23, P = 0.0033), 90% (18 cases, P = 0.99, P = 0.6), and 90% (18 cases, P = 0.99, P = 0.6), respectively. The interobserver agreement between ChatGPT-4.0 and ChatGPT-3.5 was 65% (13 cases), whereas the interobserver agreement between ChatGPT-4.0 and 3 corneal specialists was 85% (17 cases), 80% (16 cases), and 75% (15 cases), respectively. However, the interobserver agreement between ChatGPT-3.5 and each of 3 corneal specialists was 60% (12 cases). CONCLUSIONS: The accuracy of ChatGPT-4.0 in diagnosing patients with various corneal conditions was markedly improved than ChatGPT-3.5 and promising for potential clinical integration. A balanced approach that combines artificial intelligence-generated insights with clinical expertise holds a key role for unveiling its full potential in eye care.

Process and Outcomes of Fitting Corneoscleral Profilometry-Driven Scleral Lenses for Patients With Ocular Surface Disease.

OBJECTIVES: To assess the feasibility of obtaining cornea scleral profile (CSP) measurements using Scheimpflug imaging and report on the fitting process of free-form custom scleral lenses (SLs) for patients with ocular surface disease (OSD). METHODS: This prospective study of patients fit with free-form SLs collected data on the following: demographics, indications for wear, corneal and scleral tomography, scan acquisition process, and SL fitting process. RESULTS: Cornea scleral profile scans were acquired on 15 eyes of nine patients. Mean scan time for right eyes was 10.7, and 9.7 min for left eyes. A mean of 2.9 follow-up visits were required to complete SL fitting, with a mean of 2.1 lenses ordered. One eye did not tolerate lens wear, and one eye could not be fit using the CSP scan because of insufficient data. The initial lens ordered was dispensed at the first follow-up visit for seven of the remaining 13 eyes, all of which were ultimately fit successfully in free-form lenses. CONCLUSIONS: In this study of profilometry-guided SL fitting for eyes with OSD and low magnitude corneal astigmatism, the number of lenses and follow-up visits required were similar to outcomes of previous studies that described the diagnostic approach to SL fitting. In addition, imaging technology does not negate the need for skilled clinical observation while fitting SLs.

Severity Classification of Limbal Stem Cell Failure Due to Steven Johnson Syndrome in the Light of the Classification Consensus of Limbal Stem Cell Deficiency.

OBJECTIVES: To examine and to understand the limbal stem-cell deficiency (LSCD) because of Steven-Johnson syndrome (SJS) in line with the new classification system for the first time in the literature. METHODS: Medical records of patients with LSCD because of SJS were reviewed retrospectively. In addition to demographic data and ophthalmologic or systemic findings, anterior segment photographs of the patients were reviewed retrospectively. Limbal stem-cell deficiency severity was graded according to the classification published by the Limbal Stem Cell Working Group. RESULTS: Twenty-four eyes of 14 patients with eye involvement secondary to SJS were included in the study. The mean age of the patients was 36.09±16.70 (9-58) years and the female-to-male ratio was 11:3. The anterior segment photographs of the patients were evaluated by two independent masked observers. Limbal stem-cell deficiency severity was graded according to the classification published by Deng et al. Corneal opacity was divided into three stages according to the area of involvement. Corneal opacity was classified as Stage I if the central 5 mm region of the cornea was not affected, as Stage II if the central 5 mm region of the cornea was affected, and as Stage III if the entire corneal surface was affected. Limbal involvement was classified as Stage A if it was below 50%, as Stage B if it was between 50% and 100%, and as Stage C if it was 100%. CONCLUSION: This is the first study in the literature to describe and classify LSCD because of SJS, according to the new LSCD classification. Consistent with the results, LSCD follows a bimodal distribution. Most patients demonstrated severe (Stage III-32.14%) or mild (Stage IA-21.42%) LSCD.

Neurotrophic Keratopathy after wide retinal endolaser and postoperative Ketorolac eye drops: A case series.

PURPOSE: We report a series of 5 cases, happened in a period of 5 months, who developed neurotrophic keratopathy (NK) following pars plana vitrectomy (PPV) and retinal endolaser for rhegmatogenous retinal detachment (RRD). In our several decennary experience of surgical center predominantly based on vitreoretinal surgery, we had rare cases of postoperative NK. These recent cases of post-surgical NK happened contextually to our change of postoperative non-steroidal anti-inflammatory drugs (NSAIDs) drops, based on Ketorolac Tromethamine 0.5% eye drops. CASES PRESENTATION: Five patients with a mean age of 61 ± 7.3 years were treated with one or more PPV with intraoperative peripheral endolaser for RRD. Nobody had previous herpetic keratitis, systemic disease like diabetes mellitus or other predisposing factors for NK. In the postoperative period, all patients received Ketorolac Tromethamine 0.5% eye drops for a mean period of 54 ± 25 days. During follow-up visits they developed NK and they were successfully treated with suspension of Ketorolac eye drops, application of therapeutic contact lens or amniotic membrane patch and topical lubricant therapy. CONCLUSIONS: Postoperative Ketorolac eye drops, in patients who underwent PPV with endolaser, may reduce the corneal sensitivity, predispose to epithelial disruption and NK development. Studies are needed to explore the effect of NSAIDs on corneal sensitivity reduction in patient who will undergo PPV and extensive endolaser.

Artificial Endothelial Layer Implantation After Multiple Failed Keratoplasties.

PURPOSE: Presenting the first case of noncellular corneal endothelial substitute after multiple failed penetrating keratoplasty (PK) and lamellar endothelial keratoplasty. METHODS: Our case presented with pseudophakic bullous keratopathy after a history of 2 rejected PKs and 1 rejected Descemet stripping automated endothelial keratoplasty. We implanted an artificial endothelial layer. RESULTS: The implant remained fully attached for a follow-up period of 12 months, and central corneal thickness decreased significantly. The patient reported improvement in her subjective vision, although ocular comorbidities limited the visual potential. CONCLUSIONS: This new device could serve as an alternative to lamellar endothelial corneal transplantation in cases where tissue rejection has occurred and is highly likely to recur. The technique is simple, and the deswelling effect on the cornea persisted, although the visual results require further validation in patients with a higher visual potential.

Descemet stripping automated endothelial keratoplasty via a frown incision.

PURPOSE: To assess the outcomes of Descemet stripping automated endothelial keratoplasty (DSAEK) via a sclerocorneal frown incision. STUDY DESIGN: Retrospective comparative study. METHODS: The outcomes of Descement stripping endothelial keratoplasty (DSAEK) were retrospectively compared between 36 patients (36 eyes) who underwent surgery via a 3.8-mm frown incision (frown incision group) and 20 patients (20 eyes) who underwent surgery via a 4.6-mm straight incision (straight incision group). In all patients, an NS Endo-Inserter was used as the graft inserter and the incision for a frown incision was via the superior sclerocorneal site and for the straight incision via the temporal cornea. DSAEK was performed by the standard technique, except for the incision. At 1 year after surgery, the two groups were compared with respect to the visual acuity, decrease of corneal endothelial cell density, the severity of corneal astigmatism (diopters), the number of sutures for wound closure, and intraoperative/postoperative complications. RESULTS: There was no significant difference between the two groups in terms of postoperative visual acuity, corneal astigmatism, and intraoperative/postoperative complications one year after surgery. On the other hand, the number of sutures required for wound closure was 1.13 ± 0.42 in the frown incision group, whereas in the straight incision group, it was 3.20 ± 0.40, showing a significant difference (P<0.001). In addition, there was no decreased corneal endothelial cell density associated with the reduction in incision width. CONCLUSIONS: A sclerocorneal frown incision is useful for performing DSAEK with an NS Endo-Inserter as it does not affect endothelial cell loss despite its short incision width.

[Comparison of the Effect of Autologous Serum on Therapy Resistant Corneal Erosions and Ulcers on the Corneal Graft vs. the Patient's Own Cornea]. / Die Wirkung von autologen Serumaugentropfen bei therapieresistenten Erosiones und Ulcera der Kornea bei eigenem im Vergleich zu transplantiertem Gewebe.

PURPOSE: The aim of this study is to compare the healing of corneal epithelial defects or ulcers on the corneal graft in comparison with the patient's own cornea after treatment with 100%, undiluted autologous serum eye drops. METHODS: In a retrospective study over 7 years, we analysed 263 treatments with autologous serum eye drops of persistent corneal epithelial defects (erosions [88%] vs. ulcers [12%]). We compared the epithelial healing tendency of patients with defects on their own cornea (51.9%) vs. patients who had previously undergone penetrating keratoplasty (48.1%). Complete epithelial healing during the 28 days of treatment was considered as therapeutic success. In addition, the recurrence rate of the epithelial defects after finishing the therapy was analysed. RESULTS: 88.2% of the epithelial defects healed during 28 days of therapy. The recurrence rate during follow-up was 5.1%. There was no significant difference with respect to success rate between corneal defects on the patient's own cornea (87.8%) and on the graft (88.6%; p = 0.137). There was a significantly lower success rate for corneal ulcers (74.2%) than for erosions (90.3%; p < 0.001). The recurrence rate of erosions was 4.4%, vs. 4.3% in ulcers during follow-up. CONCLUSION: The results of our study suggest that autologous serum eye drops are a non-invasive and safe alternative treatment for persistent corneal epithelial defects - with no significant difference in patients with a defect on their own cornea vs. defects on the corneal graft. The success rate, but not the recurrence rate, is significantly worse in ulcers than in erosions.

Glaucoma after penetrating keratoplasty and keratoprosthesis.

PURPOSE: To compare visual and glaucoma outcomes in patients with known glaucoma after a penetrating keratoplasty (PKP) or a Boston Keratoprosthesis Type 1 (KPro) as a second corneal replacement procedure. DESIGN: Retrospective interventional case series. PARTICIPANTS: Charts of 141 eyes that underwent either a PKP or KPro at the Centre hospitalier de l'Université de Montréal after one failed PKP from 2008 to 2020 were reviewed. Forty-six eyes with preoperative glaucoma were included. METHODS: Data collected included demographics, indication for the initial surgery, best corrected visual acuity (BCVA), concurrent ocular disorders, number of glaucoma medications, need for glaucoma surgery, cup-to-disc ratios (CDRs), mean RNFL thickness, and visual field (VF) characteristics. Primary outcomes were glaucoma progression trends. Secondary outcomes were visual outcomes and need for additional procedures. RESULTS: Mean follow-up was 4.7 years for the PKP and 7.3 for the KPro group (P<0.007). 30.6% of PKP compared to 70.5% of KPro patients were diagnosed with glaucoma preoperatively. Glaucoma worsened similarly in both groups; this is based on an analysis of the number of glaucoma medications, CDR, need for glaucoma surgery, and characteristic VF changes. Patients in the PKP group required significantly more regrafts than patients in the KPro group (31.8 vs. 8.3%; P=0.045). CONCLUSIONS: A preoperative diagnosis of glaucoma does not preclude KPro implantation. In glaucomatous eyes, the disease progressed similarly in both groups. Since both procedures increase the risk of worsening glaucoma, close follow-up is recommended. KPro may decrease the need for further corneal transplantation surgery.

Artifacts in OCT Retinal Nerve Fiber Layer Imaging in Patients with Boston Keratoprosthesis Type 1.

PURPOSE: To determine the clinical utility of OCT retinal nerve fiber layer (OCT RNFL) imaging for glaucoma evaluation in patients with Boston keratoprosthesis type 1 (KPro) by investigating imaging artifacts. DESIGN: Case-control study. SUBJECTS: Patients with KPro and without KPro (controls) matched for age, gender, and glaucoma diagnosis. METHODS: The most recent Cirrus OCT RNFL scan from 1 eye was categorized as having good signal strength (SS; ≥ 6 out of 10) or poor SS (< 6). Those with good SS were analyzed by 2 independent reviewers for artifacts. Images with good SS and no artifacts affecting the scanning circle were considered useful for glaucoma evaluation. MAIN OUTCOME MEASURES: The incidence of poor SS and artifacts in OCT RNFL images; patient characteristics associated with useful scans. RESULTS: Sixty-five patients with KPro and 75 controls were included; 89.2% of KPro patients and 89.3% of control subjects had glaucoma (P = 0.98). Forty percent of KPro patients and 5.3% of controls had poor SS (P < 0.001). The proportion of images with either poor SS or artifacts was similar in KPro (76.9%) vs. controls (72.0%, P = 0.51). The most common artifacts in both groups were missing data (43.6%, 53.2%, respectively, P = 0.32) and motion artifact (25.6%, 19.7%, respectively, P = 0.47). Images were useful for glaucoma evaluation in 43.1% of KPro patients and in 69.3% of controls (P = 0.002). In the KPro group, patients with useful OCT scans, compared with those without, had better visual acuity (0.4 ± 0.3 vs. 0.9 ± 0.7 logarithm of the minimum angle of resolution, P = 0.004), and did not have congenital corneal pathologies (0.0% vs. 24.3%, P = 0.008). A multivariate analysis showed that KPro patients with older age had higher odds of useful OCT images (odds ratio, 1.05; P = 0.03). Among KPro patients with useful OCT scans, retinal nerve fiber layer thickness correlated with observed cup-to-disc ratio (Pearson correlation: r = -0.42, P = 0.03). CONCLUSIONS: The rate of OCT RNFL images with either poor signal strength or artifacts in the KPro and control population was comparable. In patients with KPro, where intraocular pressure measurements are difficult and glaucoma is highly prevalent and often severe, OCT RNFL imaging can be useful for glaucoma evaluation. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

Coexistence of neuropathic corneal pain, corneal nerve abnormalities, depression, and low quality of life.

PURPOSE: To evaluate the quality of life (QoL), mental health conditions and corneal morphology in neuropathic corneal pain (NCP) subjects without a significant ocular surface disease. METHODS: A composite questionnaire was administered to 228 consecutive subjects, assessing the pain intensity, duration, and quality using a modified version of the Self-Administered Leeds Assessment of Neuropathic Symptoms and Signs (S-LANSS) and Pain Detect (PD) questionnaires. Subjects diagnosed with possible central NCP and two sub-groups of patients diagnosed with peripheral ocular pain completed an additional battery of mental health questionnaires and were examined by In Vivo Confocal Microscopy (IVCM). RESULTS: Of the 76 subjects that reported chronic ocular pain (duration >1 month), 53 were classified with probable NCP. Nine subjects without signs that justify the pain and non-responding to topical anaesthesia, were considered affected by central NCP. In these patients, a significant negative correlation was found between the presence pain and the mental component of the QoL (R2 = 0.733), and a positive correlation between the severity of pain the presence post-traumatic stress disorder (R2 = 0.83) and depression (R2 = 0.93). Although neuromas and sprouting had higher frequency in the central NCP group compared the control groups, these differences was not statistically different. CONCLUSIONS: The assessment of ocular pain characteristics using multiple questionnaires and IVCM may help to recognize differences between nociceptive and neuropathic pain. An association between pain intensity and mental health condition may guide the therapeutical choices.

Variable Phenotype of Congenital Corneal Opacities in Biallelic CYP1B1 Pathogenic Variants.

PURPOSE: The aim of this study is to describe the variable phenotype of congenital corneal opacities occurring in patients with biallelic CYP1B1 pathogenic variants. METHODS: A retrospective chart review was conducted to identify patients with congenital corneal opacities and CYP1B1 pathogenic variants seen at UPMC Children's Hospital of Pittsburgh. Ophthalmic examination, high-frequency ultrasound, anterior segment optical coherence tomography, histopathologic images, and details of genetic testing were reviewed. RESULTS: Three children were identified. All presented with raised intraocular pressure. Two patients showed bilateral limbus-to-limbus avascular corneal opacification that did not resolve with intraocular pressure control; 1 showed unilateral avascular corneal opacity with a crescent of clear cornea, iridocorneal adhesions, iridolenticular adhesions, and classical features of congenital glaucoma in the fellow eye (enlarged corneal diameter, Haab striae, and clearing of the corneal clouding with appropriate intraocular pressure control). The first 2 patients were visually rehabilitated with penetrating keratoplasty. Histopathology revealed distinct features: a variably keratinized epithelium; a thick but discontinuous Bowman-like layer with areas of disruption and abnormal cellularity; Descemet membrane, when observed, showed reduced endothelial cells; and no pathological changes of Haab striae were identified. Two patients had compound heterozygous pathogenic variants in CYP1B1 causing premature stop codons, whereas 1 was homozygous for a pathogenic missense variant. CONCLUSIONS: Congenital corneal opacities seen in biallelic CYP1B1 pathogenic variants have a variable phenotype. One is that commonly termed as Peters anomaly type 1 (with iridocorneal adhesions, with or without iridolenticular adhesions) and the other is a limbus-to-limbus opacity, termed CYP1B1 cytopathy. Clinicians should be aware of this phenotypic variability.