UNASSIGNED: Sturge-Weber syndrome (SWS) is a complex rare genetic neuro-cutaneous disorder characterized by the presence of a port-wine stain, ophthalmic and intracranial angiomatosis leading to seizures, ocular, and oral abnormalities.
UNASSIGNED: We report a 39-year-old, non-diabetic, non-hypertensive female refugee who presented initially with heart failure due to anemia for which she received blood transfusions. Later on admission, she developed multiple focal to bilateral seizures, severe irritability, aphasia, and right-sided hemiplegia, leading to admission to the ICU. A repeat medical history and examination revealed a faint left-sided ophthalmic port-wine stain that was initially unnoticed and a remote history of unprovoked seizures 20 years ago. Imaging revealed parietal calcifications and confirmed the diagnosis of SWS. Thus, a multidisciplinary approach was taken to fully understand the patient\'s diagnosis and determine a treatment strategy, involving consultations with the neurology, ophthalmology, otolaryngology, and physiotherapy departments. Successful seizure control was achieved by administering IV phenytoin for 3 days and the up-titrating of oral carbamazepine to 1g daily through a nasogastric tube. Unfortunately, due to the unavailability of personnel or resources, other important assessments for patients with SWS, such as advanced neuroimaging, psychiatric, plastic and neuro-surgery evaluations, as well as dentistry reviews, could not be conducted.
UNASSIGNED: This case highlights the rare occurrence of adult-onset seizures in an undiagnosed SWS and their re-emergence after almost two decades without anti-seizure medications. It also highlights the importance of a comprehensive history and clinical examination, as this patient\'s diagnosis of SWS could have been missed if she had not experienced seizures on admission. Our study also demonstrates the challenges associated with managing such a complex condition in settings with limited resources.

Background: Platinum-based combination chemotherapy, including cisplatin and carboplatin, are important cytotoxic anti-cancer agents that are widely used to treat various solid tumors. Carboplatin has a similar effect on survival in small cell lung cancer, but generally has a milder toxicity profile when compared with cisplatin. Both may cause moderate or severe neurotoxicity, but ocular neurotoxicity from carboplatin is rarely reported. Case presentation: A 79-year-old man underwent intravenous polychemotherapy (atezolizumab, etoposide, and carboplatin) for small cell lung cancer. One week after the second cycle of chemotherapy, he reported bilateral visual loss as hand motion in both eyes. Dilated fundus examination showed retinal arterial narrowing without hemorrhage, and diffuse choroidal and retinal thinning was observed in an optical coherence tomography scan. Fluorescein angiography revealed significantly delayed circulation without evidence of obstructive lesions. 30-Flicker electroretinogram testing showed a complete absence of cone response in both eyes. The patient\'s visual acuity aggravated to no light perception in both eyes, even after the cessation of chemotherapy. Conclusions: Carboplatin combination chemotherapy administered at therapeutic doses can result in irreversible visual loss, a side effect that is not widely acknowledged. When using carboplatin, physicians should be aware of its potential ocular toxicity.

Different techniques for artificial iris implantation with or without an intraocular lens, depending on lens status, are described in the literature. We describe a surgical technique for a custom-made artificial iris and toric-intraocular lens intrascleral flange fixation. We modified the \"Backpack\" artificial iris implantation surgical technique to facilitate an accurate alignment of the toric-intraocular lens in a patient with aphakia, aniridia, and high asymmetric astigmatism secondary to blunt trauma. Two months after the surgery, uncorrected visual acuity was 20/30, corrected to 20/25 with a refraction of -2.00 in the diopter sphere with no residual astigmatism. The artificial iris implant and toric-intraocular lens were well-centered. The patient was satisfied with the visual and cosmetic outcomes. This procedure, however, is not complication-free as our patient developed uveitis and increased intraocular pressure during the postoperative period, which was treated successfully.

The MAF gene encodes a transcription factor in which pathogenic variants have been associated with both isolated and syndromic congenital cataracts. We aim to review the MAF variants in the C-terminal DNA-binding domain associated with non-syndromic congenital cataracts and describe a patient with a novel, disease-causing de novo missense variant. Published reports of C-terminal MAF variants and their associated congenital cataracts and ophthalmic findings were reviewed. The patient we present and his biological parents had genetic testing via a targeted gene panel followed by trio-based whole exome sequencing. A 4-year-old patient with a history of bilateral nuclear and cortical cataracts was found to have a novel, likely pathogenic de novo variant in MAF, NM_005360.5:c.922A>G (p.Lys308Glu). No syndromic findings or anterior segment abnormalities were identified. We report the novel missense variant, c.922A>G (p.Lys308Glu), in the C-terminal DNA-binding domain of MAF classified as likely pathogenic and associated with non-syndromic bilateral congenital cataracts.

The authors present a case of a thirty-eight-year-old patient with Alport syndrome. The patient had several ocular symptoms of the disease and has been treated for systemic problems in connection with Alport syndrome since he was fifteen years old. At that age the patient also underwent a kidney transplant in order to deal with renal insufficiency. To date, he still uses immunosuppressants and antihypertensives. Furthermore, the patient suffers from perceptive deafness. The patient visited our clinic in 2021 with a request to solve his high refractive error, in which the diopters were so high that it was not possible to place them in spectacles. The patient\'s best corrected visual acuity was 0.6 with -8.0sph/-4.0cyl/ax15 in the right eye and 0.7partim with -8.0sph/-4.0cyl/ax155 in the left eye. The autorefractometer values were -6.25sph/-6.75cyl/ax17 in the right eye and -6.75sph/-6.5cyl/ax155 in the left eye. During the eye examination we found a number of ocular manifestations that are typical of Alport syndrome. On the cornea there were opacities as a residue of corneal erosions, and at one of the following check-ups we also found a newly developed corneal erosion. Subsequently, we found an anterior lenticonus and incipient cataract. Upon performing OCT, a typical temporal macular atrophy was evident. Fundus examination in artificial mydriasis showed just a minimal manifestation of fleck retinopathy. Due to the clinical manifestation we decided to perform cataract surgery and implant a monofocal toric intraocular lens in both eyes. There were no complications during the operations, however the surgeon registered a non-standard structure of the lens capsule. The capsule was more fragile, and performing capsulorhexis was much more complicated. A week after the surgery, higher cylinder diopters were still present. A decrease of the higher diopters was noticeable one month after surgery. The time interval between the first operation and the second operation was one month. The patient was highly satisfied with result, and uncorrected visual acuity improved by over four lines. After surgery the patient needed low diopters for near as well as far distance. In the case of this patient, the ocular manifestations were detected and treated in adulthood. Nevertheless, early detection of ocular symptoms of Alport syndrome in young patients before renal failure could lead to timely start of the treatment and delay a possible renal transplant. In case of any suspicion of Alport syndrome it is advised to send the patient to a pediatrician, and at an older age to an internal medicine specialist, for further examination.

OBJECTIVE: Iridocorneal endothelial (ICE) syndrome is a rare disease characterized by abnormal proliferation and structural changes of the endothelium, obliteration of the iridocorneal angle, and anomalies of the iris. The consequence of these changes is secondary glaucoma and corneal decompensation. The etiology is unclear, and the syndrome more commonly affects middle-aged women.
METHODS: In this article we present two different case studies of young patients diagnosed with ICE syndrome with complications. The first case report is about a young woman in whom surgical treatment of glaucoma and corneal edema was successful. On the other hand, the second report presents a complicated case of a 29-year-old patient whose treatment was not successful despite repeated interventions.
CONCLUSIONS: This text highlights the complexity of ICE syndrome, the difficulty of its therapy and the importance of early diagnosis.

UNASSIGNED: To report an atypical presentation of severe toxicity, anterior chamber (AC) inflammation, and transient parafoveal formation of subretinal fluid induced by the subconjunctival injection of 5-fluorouracil (5-FU).
UNASSIGNED: Case presentation.
UNASSIGNED: Seven weeks after trabeculectomy, a 40-year-old white male had a subconjunctival injection of 5-FU. Within minutes after the injection, the lens turned grey and then total white. Initially, AC was clear, and 20 min later, a severe AC reaction was detected. The patient was prescribed hourly dexamethasone eye drops and tropicamide eye drops twice daily. Two days post-injection, vision improved, AC reaction was minimal, and there was whitish fibrinous material on the anterior surface of the lens, extending up to the pupillary margin. Minimal posterior synechiae were observed, and upon dilation, the remainder of the anterior surface of the lens appeared completely clear, indicating that only the portion of the lens not covered by the iris exhibited fibrinous material and deposits. One week post-injection, vision worsened due to severe corneal toxicity. The dimensions of the whitish fibrinous material on the anterior lens capsule decreased, and macular scans revealed parafoveal subretinal fluid. Two weeks later, vision significantly improved, and the dimensions of the whitish fibrinous material on the anterior lens capsule were further decreased. The subretinal fluid had completely resolved.
UNASSIGNED: We describe a rare case of severe toxicity, AC inflammation, and transient parafoveal subretinal fluid formation caused by the 5-FU. This was treated with topical steroid treatment, and eventually some level of lens opacification persisted despite significant clearance of the AC inflammation.

A 61-year-old Malaysian Chinese man who has high myopia complained of both eye floaters. Spectral-domain optical coherence tomography (SD-OCT) of the macula showed bilateral posterior staphyloma with right eye (RE) foveoschisis without macula detachment, which had been stable for a seven-year follow-up. When bilateral YAG laser vitreolysis could not alleviate his symptoms, he underwent pars plana vitrectomy with the inducement of posterior vitreous detachment, first in the left eye, followed by the RE one month later. The best-corrected visual acuity for both eyes was 6/6, N5 two months postoperatively, and he was asymptomatic for floaters. However, six months postoperatively, he complained of metamorphopsia and worsening RE vision. Repeat OCT showed worsening of the foveoschisis bilaterally with left foveal detachment. The patient had to undergo a repeat vitrectomy with peeling of the internal limiting membrane (ILM) in bilateral eyes, which successfully restored his foveal architecture and alleviated his symptoms. This article highlights theimportance of preoperative OCT assessment of the fovea in patients undergoing vitrectomy for floaters, as staining and complete removal of posterior hyaloid with ILM peeling during vitrectomy may mitigate the progression of foveoschisis after core vitrectomy for floaters in myopic patients.

Epithelial inclusion cysts (EIC) are a rare ocular disease and its physiopathology is not well-known. They consist on growths of ocular surface epithelial cells inside the anterior segment of the eye in the form of a cyst. To date several cases have been published in the literature, none of them related to glaucoma surgery. We describe two cases of EIC after glaucoma devices implantation. An 86 year-old male patient with primary open angle glaucoma develop an EIC in right eye three years after removal of PRESERFLO™MicroShunt (Santen, Osaka, Japan) and a 9 year-old female patient with glaucoma secondary to uveitis for juvenile idiopathic arthritis develops an EIC under the tube of an Ahmed valve implant during postoperative period. EIC develop after ocular penetrating wounds and an inflammatory stimulus. They are benign proliferations, follow-up is necessary to detect space complications early, so less mutilating surgery is needed for removal.

BACKGROUND: Cataract surgery in patients after penetrating keratoplasty (PKP) is often challenging because of changes in corneal structure induced by PKP and primary corneal disease. Femtosecond laser-assisted cataract surgery offers several advantages over conventional phacoemulsification, and has been widely used in complicated cataract surgery.
METHODS: We report the use of femtosecond laser-assisted cataract surgery in 3 challenging cases after penetrating keratoplasty. Case 1 involved a patient with hard nuclear grade IV° cataract. After surgery, his corrected distance visual acuity (CDVA) improved from 20/400 to 20/25, and the endothelial cell loss (ECL) % was 12.05 % at 3 months postoperatively. The rotation of the toric IOL in Case 1 was 2°. Case 2 involved a patient with severe nuclear cataract and an endothelial cell density of 837 cells/mm2. After surgery, the CDVA improved from 20/100 to 20/40. The ECL% was 4.06% at 1 week postoperatively. Case 3 was a 91-year-old woman with a short axis length of 21.35 mm and an endothelial cell density number of 1238 cells/mm2. After surgery, the CDVA improved from light perception to 20/133, and the ECL% was 26.09% at 1 week postoperatively; ECL% was 2.67% at 1 month post-operation. The corneal grafts were transparent.
CONCLUSIONS: Femtosecond laser-assisted cataract surgery seems to be an effective, predictable, and safe approach for challenging patients after PKP, and improves visual recovery and optimal refractive outcomes.