Cataract surgery and artificial iris implantation in patient with oculocutaneous albinism: a case report.

We present a case report detailing the successful phacoemulsification surgery with artificial iris implantation for two individuals with oculocutaneous albinism. These women suffered from cataracts, resulting in reduced visual acuity and heightened photophobia due to iris pigmentary epithelium deficiency. The patients underwent phacoemulsification along with prosthetic artificial iris implantation into the posterior chamber. This intervention resulted in improved visual acuity, reduced photophobia and glare, and an overall enhanced quality of life. Our report highlights two cases of successful phacoemulsification and artificial iris implantation in patients with oculocutaneous albinism and cataracts, leading to improved visual acuity, reduced photophobia, and enhanced quality of life. Notably, there are no prior records in South American literature of cataract surgery combined with artificial iris implantation for oculocutaneous albinism patients up to the time of this publication.

Custom Iris Prosthesis for Subtotal Traumatic Aniridia From Combat Ocular Trauma.

Traumatic aniridia from combat ocular trauma can cause visual disability. A 41-year-old male Army Veteran was referred for evaluation of light sensitivity and glare secondary to subtotal traumatic aniridia of his left eye from an improvised explosive device blast. A custom-made artificial iris prosthesis was implanted in the ciliary sulcus and secured using Gore-Tex sutures. After surgery, the patient reported improvement of his light sensitivity and quality of life. The custom iris prosthesis is a surgical option for visual disability resulting from traumatic aniridia from combat ocular trauma.

Aniridic scleral fixation of intraocular lens: The answer to vision loss with photophobia!

Background: Aniridia is defined as missing iris tissue which can be partial, subtotal, or total. Characteristic clinical symptoms include photophobia and decreased visual acuity due to an increased light perception. In addition to this, disturbing cosmetic problems are prevalent. Even after implantation of an intraocular lens, patients often tend to be unsatisfied. Purpose: The answer to this problem lies in the implantation of an aniridic scleral fixation of intraocular lens (SFIOL), which has a central optical axis that acts as the refractive lens and a peripheral rim of a hyperpigmented area that mimics the iris and hence reduces photophobia. The purpose of the video was to demonstrate the use of black diaphragm intraocular lens (BDIOL) implantation, its surgical steps, and its outcomes. Synopsis: We report one such case where a young patient presented with post-traumatic aniridia with subluxated total cataract and spillover vitreous hemorrhage. He was subjected to a vitrectomy, cataract removal, and placement of an aniridic SFIOL with prolene 9-0 using the four-point fixation method. This gave an extremely gratifying outcome and solved both problems, that is, vision and photophobia. Highlight: Before implantation of the SFIOL, the patients had reduced visual acuity from aphakia and intolerable glare from aniridia. In this case-based approach and with relevant example, we tried to provide a solution for tricky scenarios like co-existing traumatic cataract and traumatic aniridia. The patient showed improved visual acuity and marked glare reduction after black diaphragm SFIOL implantation. Video link: https://youtu.be/atl60WetFsM.

Misdiagnosis and Treatment of Corneal Complications Caused by Suture Exposure After Buried-Suture Double-Eyelid Blepharoplasty.

PURPOSE: The purpose of this study was to summarize the misdiagnosis and treatment of corneal complications associated with suture exposure in cases of buried-suture double-eyelid blepharoplasty. METHODS: This study retrospectively analyzed 14 patients with palpebral conjunctival and corneal complications due to suture exposure after buried-suture double-eyelid blepharoplasty at the First Affiliated Hospital of Harbin Medical University from January 2020 to July 2022. The patients' clinical symptoms included photophobia, lacrimation, pain, foreign body sensation, swelling of the eyelids, conjunctival hyperemia, secretion, etc. We recorded the patient's sex, age, surgical method, length of exposed suture, suture type, number of double-eyelid surgeries, surgical site, timepoint when eye discomfort occurred, misdiagnosed disease and treatment. RESULTS: Three patients were misdiagnosed with dry eye, nine patients were misdiagnosed with viral keratitis, and two patients were misdiagnosed with allergic conjunctivitis. All 14 patients had manifestations of photophobia, lacrimation, pain, foreign body sensation and conjunctival hyperemia. Eight patients had manifestations of swelling of the eyelids. Five patients had manifestations of eye secretions. There were 8 patients with corneal epithelial injuries and 6 patients with corneal ulcers. All patients underwent suture removal without further progression. Ten patients were treated with artificial tears, and 4 patients were treated with calf serum deproteinized gel after suture removal. CONCLUSION: If there is postoperative eye discomfort caused by eyelid and corneal complications in patients after buried-suture double-eyelid blepharoplasty, clinicians should carefully check whether there is suture exposure and determine the cause in a timely manner. Suture removal is the best way to treat this complication. LEVEL OF EVIDENCE IV: This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 .

Results of the United States Food and Drug Administration Clinical Trial of the CustomFlex Artificial Iris.

PURPOSE: To evaluate safety and efficacy of a custom-manufactured artificial iris device (CustomFlex Artificial Iris; HumanOptics AG) for the treatment of congenital and acquired iris defects. DESIGN: Multicenter, prospective, unmasked, nonrandomized, interventional clinical trial. PARTICIPANTS: Patients with photophobia, sensitivity secondary to partial or complete congenital or acquired iris defects, or both. METHODS: Eyes were implanted from November 26, 2013, to December 1, 2017, with a custom, foldable artificial iris by 1 of 4 different surgical techniques. Patients were evaluated 1 day, 1 week, and 1, 3, 6, and 12 months after surgery. At each examination, slit-lamp findings, intraocular pressure, implant position, subjective visual symptoms, and complications were recorded. Corrected distance visual acuity (CDVA) and endothelial cell density (ECD) were measured at 3, 6, or 12 months as additional safety evaluations. The 25-item National Eye Institute Visual Function Questionnaire (NEI VFQ-25) was used to assess health-related quality of life affected by vision. The Global Aesthetic Improvement Scale was used to assess cosmetic results. MAIN OUTCOME MEASURES: Photosensitivity, glare, visual symptoms, NEI VFQ-25 score, Global Aesthetic Improvement Scale rating, prosthesis-related adverse events, intraocular lens (IOL)-related adverse events, and surgery-related adverse events 12 months after surgery. RESULTS: At the 12-month postoperative examination, a 59.7% reduction in marked to severe daytime light sensitivity (P < 0.0001), a 41.5% reduction in marked to severe nighttime light sensitivity (P < 0.0001), a 53.1% reduction in marked to severe daytime glare (P < 0.0001), and a 48.5% reduction in severe nighttime glare (P < 0.0001) were found. A 15.4-point improvement (P < 0.0001) in the NEI VFQ-25 total score was found, and 93.8% of patients reported an improvement in cosmesis as measured by the Global Aesthetic Improvement Scale 12 months after surgery. No loss of CDVA of > 2 lines related to the device was found. Median ECD loss was 5.3% at 6 months after surgery and 7.2% at 12 months after surgery. CONCLUSIONS: The artificial iris surpassed all key safety end points for adverse events related to the device, IOL, or implant surgery and met all key efficacy end points, including decreased light and glare sensitivity, improved health-related quality of life, and satisfaction with cosmesis. The device is safe and effective for the treatment of symptoms and an unacceptable cosmetic appearance created by congenital or acquired iris defects.

Ocular Features in a Large Cohort of Indians With Xeroderma Pigmentosum.

PURPOSE: Xeroderma pigmentosum (XP) is an extreme hypersensitivity to sunlight causing skin freckling and pigmentary changes because of defective DNA repair mechanisms. The purpose of this article is to evaluate the spectrum of ocular and systemic features in XP at a tertiary eye care center in India over 32 years. METHODS: Data from 418 eyes of 209 patients diagnosed with XP from 1987 to 2018 were reviewed retrospectively for demographics, complaints, ocular features, systemic associations, and their management. RESULTS: Median age at diagnosis was 2 years (interquartile range, 0.5-5 years). A total of 124 patients (59.3%) were men. There was parental consanguinity in 74.4% cases. Common ocular complaints were photophobia (47.1%), ocular discomfort (45%), defective vision (36.6%), redness (13.4%), tissue growth (12%), white spot (11.2%), and pain (10.5%). At presentation, 43.5% had corneal scars (45.5% were located inferiorly and 70.9% covered visual axis). Corneal vascularization and limbal stem cell deficiency were noted in 37.4%. A total of 56% of patients had at least 1 tumor, and 6.7% had neurological abnormalities. At least 1 ocular surgery was performed in 37.8% of patients. Ophthalmic surgical interventions included tumor excision (23%), keratoplasty (13.4%), and nontumor ocular surface surgery (3.4%). CONCLUSIONS: XP is a disorder that has high ocular morbidity in Indian patients. The recognition of common signs and symptoms and relative frequency of various ocular complications with time trends will help in managing and reducing the sequelae of this otherwise untreatable and progressive disease.

Salzmann nodular degeneration features in a case of Kabuki make-up syndrome.

Kabuki syndrome (KS) is a multiple congenital anomaly syndrome with diversified ophthalmological manifestations. We report a case of a boy with bilateral features of Salzmann nodular degeneration (SND) associated with KS. An 18-year-old Caucasian man with KS presented for a second opinion regarding incapacitating photophobia in his right eye, refractory to medical therapy. Biomicroscopy revealed bilateral subepithelial nodules in the midperiphery of the cornea, less extensive in the left eye, consistent with SND. Symptomatic improvement was achieved after superficial keratectomy, manually performed with a blade and adjuvant application of mitomycin C. We report a rare case of a KS patient with SND. Since KS manifestations may vary widely, it is important to perform an early ophthalmological examination for prompt detection and treatment of ocular abnormalities and thus improve life quality in these patients.

Endothelial dysfunction in a child with Pearson marrow-pancreas syndrome managed with Descemet stripping automated endothelial keratoplasty using a suture pull-through technique.

A 4-year-old girl with a history of Pearson marrow-pancreas syndrome presenting with severe, progressive photophobia was found to have bilateral, diffuse corneal thickening and peripheral pigmentary retinopathy. She underwent Descemet stripping automated endothelial keratoplasty (DSAEK) surgery in both eyes using a modified suture pull-through technique. Postoperatively there was no evidence of cataract formation or graft detachment; her corneas thinned, and her photophobia improved dramatically.

Case Report: Ocular Gnathostomiasis in Venezuela Most Likely Acquired in Texas.

A 69-year-old male dentist in Caracas, Venezuela, was referred to our Cornea Clinic with a history of pain, photophobia, and blurred vision on his left eye. Routine biomicroscopic examination with a slit lamp showed a worm in the corneal stroma of his left eye. The worm was surgically removed and was identified morphologically as Gnathostoma binucleatum.

Stellate Ganglion Block for Debilitating Photophobia Secondary to Trigeminal, Postherpetic Neuralgia.

OBJECTIVES: The primary objective of this case report was to demonstrate the therapeutic benefit of stellate ganglion block in trigeminal postherpetic neuralgia. METHODS: This was a case report on a single patient who presented with debilitating photophobia secondary to left-sided trigeminal postherpetic neuralgia. A left-sided stellate ganglion block was performed on the patient under fluoroscopic guidance. The primary endpoints were VAS pain scores and changes in functional capacity. RESULTS: The patient demonstrated significant reduction in her VAS pain score and improved functional capacity for approximately 6 months after the intervention. This case report provides evidence that sympathectomy via a stellate ganglion block can treat photophobia secondary to postherpetic neuralgia in the V1 distribution.

Pinhole iris-fixated intraocular lens for dysphotopsia and photophobia.

UNLABELLED: We present the pinhole iris-fixated diaphragm intraocular lens (IOL), which is a 1-piece black polycarbonate IOL with rigid iris-fixated haptics, an 8.5 mm overall length, a 6.0 mm diameter optic, and a central opening, the pinhole. The IOL was implanted in a 36-year-old man who had a history of debilitating dysphotopsia (star burst, halos, glare, and ghosting) and severe light sensitivity in his right eye following several interventions for keratoconus. The diameter of the central opening of the pinhole IOL, 2.0 mm, was based on the patient's corneal aberration profile. Postoperatively, the dysphotopic symptoms and photophobia markedly improved. We recommend the use of the pinhole iris-fixated IOL in patients with severe photophobia or dysphotopsia refractory to other treatment modalities. FINANCIAL DISCLOSURE: No author has a financial or proprietary interest in any material or method mentioned.

Femtosecond-assisted keratopigmentation for functional and cosmetic restoration in essential iris atrophy.

UNLABELLED: We describe a new surgical technique of femtosecond-assisted keratopigmentation (KTP) in a 23-year-old woman with severe visual dysfunction secondary to unilateral essential iris atrophy due to the presence of iridocorneal endothelial syndrome in her left eye. The patient presented with complaints of photophobia and monocular diplopia in the affected eye. Femtosecond-assisted KTP surgery was performed in this eye. Following the procedure, the patient reported complete elimination of photophobia and diplopia, with an excellent cosmetic result. Follow-up of 12 months was unremarkable. To our knowledge, this is the first report of a severe visual function disability caused by essential iris atrophy that was corrected by a new KTP technique. FINANCIAL DISCLOSURE: No author has a financial or proprietary interest in any material or method mentioned.

Ichthyosis follicularis, alopecia and photophobia syndrome (IFAP): report of the first case with ocular and cutaneous manifestations in Brazil with a favorable response to treatment.

Ichthyosis follicular, alopecia, and photophobia (IFAP) syndrome is a rare disease, with possible X-linked mode of inheritance. The patient presented with ocular findings of photophobia, corneal scarring and erosions, superficial and deep corneal vascularization and myopia. He was treated with artificial tears and punctal occlusion with small improvement of photophobia. After three months using systemic retinoid (Acitretina) and posterior amniotic membrane transplantation in the left eye, there was a significant improvement of photophobia, corneal erosions and neuropsychomotor development.

Ichthyosis follicularis, alopecia and photophobia syndrome (IFAP): report of the first case with ocular and cutaneous manifestations in Brazil with a favorable response to treatment / Síndrome de ictiose folicular, alopécia e fotofobia (IFAP): relato do primeiro caso com acometimento ocular e cutâneo do Brasil e da resposta favorável ao tratamento

Ichthyosis follicular, alopecia, and photophobia (IFAP) syndrome is a rare disease, with possible X-linked mode of inheritance. The patient presented with ocular findings of photophobia, corneal scarring and erosions, superficial and deep corneal vascularization and myopia. He was treated with artificial tears and punctal occlusion with small improvement of photophobia. After three months using systemic retinoid (Acitretina) and posterior amniotic membrane transplantation in the left eye, there was a significant improvement of photophobia, corneal erosions and neuropsychomotor development.

A síndrome de ictiose folicular, alopecia e fotofobia (IFAP) é uma doença rara, com possível modo de herança ligado ao cromossomo X. O paciente apresentou achados oftalmológicos de fotofobia, cicatrizes e erosão corneanas, neovascularização superficial e profunda da córnea e miopia. Foi iniciado uso de lubrificantes e oclusão do ponto lacrimal com discreta melhora da fotofobia. Após uso de retinóide sistêmico (Acitretina) por três meses e posterior transplante de membrana amniótica no olho esquerdo apresentou melhora importante da fotofobia, das erosões corneanas e do desenvolvimento neuropsicomotor.

Alleviating debilitating photophobia and secondary exotropia caused by increased straylight by widening a small posterior capsulotomy.

In a patient with complaints of photophobia and an ocular deviation, straylight was found to be increased to 1.61 (log[s]), which is 5 times the normal value. The only relative clinical finding was the edge of a too small posterior capsulotomy. Visual acuity was normal. Six weeks after the posterior capsulotomy was widened to a diameter of 6.0 mm with a neodymium:YAG laser, the symptoms were resolved and the patient was satisfied. Straylight may manifest clinically as complaints of photophobia. Straylight increase, which can be related to slitlamp findings, may lead to an interventional decision. Our clinical decision-making was also guided by straylight measurements and proved to be crucial in resolving the patient's complaints. More study of clinical situations in which straylight measurement can be used is needed.

Open-sky pupilloplasty during phakic penetrating keratoplasty to treat a fixed, dilated pupil.

We report a technique in which penetrating keratoplasty is performed in conjunction with open-sky pupilloplasty in a phakic patient. The technique was used in a 27-year-old man with poor vision and severe light sensitivity in the left eye dating back to an episode of presumed herpes simplex keratouveitis 13 years previously. Examination showed a best corrected visual acuity of 20/40, a paracentral midstromal corneal scar, a fixed dilated pupil, and a clear lens. Postoperatively, the pupil was relatively round with a diameter of approximately 4.0 mm, the cosmetic result was favorable, the photophobia had resolved, and the lens and corneal transplant were clear.

Amniotic membrane transplantation for partial limbal stem cell deficiency.

AIM: To examine the efficacy, safety, and long term outcomes of amniotic membrane transplantation for corneal surface reconstruction in cases of partial limbal stem cell deficiency. METHODS: 17 eyes of 15 patients with partial limbal stem cell deficiency underwent superficial keratectomy of the conjunctivalised corneal surface followed by amniotic membrane transplantation. Cases were followed up for at least a year. RESULTS: All eyes exhibited a stable, intact corneal epithelial surface after a mean follow up period of 25.8 months with no eyes developing recurrent erosion or persistent epithelial defect. The mean time to re-epithelialisation was 22.8 days. Overall improvement in visual acuity was observed in 92.9% of 14 eyes with visual potential. Of those, five eyes gained six or more lines, two eyes gained between four and five lines, six eyes gained between one and three lines, and one eye lost three lines of Snellen acuity. Pain and photophobia were abolished in 86% of cases and substantially reduced in 14%, with all eyes exhibiting decreased vascularisation and inflammation at final follow up. CONCLUSIONS: Amniotic membrane transplantation appears to be a safe and effective method of restoring a stable corneal epithelium for cases of partial limbal stem cell deficiency and can be considered as an alternative to limbal autograft or allograft.