Iris Transillumination Research Articles

The uveitis–glaucoma–hyphema syndrome. Part 1. Pathogenesis, clinical features, diagnosis

BACKGROUND: The uveitis–glaucoma–hyphema (UGH) syndrome was first described in 1978 as a complication of anterior chamber polymethyl methacrylate intraocular lenses implantation. Introduction into practice of foldable intraocular lenses with intracapsular fixation has reduced the incidence of UGH. However, this complication still occurs today, especially with extracapsular intraocular lens fixation. AIM: The aim of this study is to describe “uveitis–glaucoma–hyphema” syndrome pathogenesis, clinical features, and diagnosis. MATERIAL AND METHODS: A retrospective analysis of medical data for 2017–2021 identified 100 patients (101 eyes) diagnosed with UGH syndrome, they made up the study group. Complaints, medical history, biomicroscopy, visual acuity and intraocular pressure were assessed. 37 patients underwent anterior segment ultrasound biomicroscopy using the Accutome UBM Plus (USA). RESULTS: The occurrence of UGH syndrome is at least 0.19%. The leading UGH syndrome risk factor was intraocular lens material. In 49% of cases these were AcrySof hydrophobic intraocular lenses. The second UGH risk factor was extracapsular (mixed and sulcus) intraocular lens fixation, occurring, according to ultrasound biomicroscopy, in 54 and 19% of cases, respectively. The most significant diagnostic UGH sign was hyphema (93%). Ocular hypertension was detected in 66%, and uveitis in 57% of cases. CONCLUSIONS: The main UGH syndrome manifestations are hyphema and iris transillumination after phacoemulsification complicated by posterior capsule rupture with extracapsular hydrophobic intraocular lens fixation. The most informative instrumental method to assess intraocular lens position is ultrasound biomicroscopy.

Moxifloxacin-associated acute pigment dispersion and iris transillumination after phakic lens surgery

Introduction: Acute pigment dispersion and elevated intraocular pressure (IOP) were found after phakic intraocular lens surgery where 0.5% moxifloxacin was used. While scattered reports can be found in the literature, to the authors' knowledge, this is the first time it has been linked to intracameral 0.5% moxifloxacin. Patient and Clinical Findings: A 23-year-old man experienced symptoms of unilateral eye pain, increased IOP, diffuse pigment dispersion, transillumination defects, and mydriasis after successful bilateral Starr Visian EVO implantable collamer lens implantation. Diagnosis, Intervention and Outcomes: Intracameral moxifloxacin (0.5%) was given at the time of the procedure and was suspected to be the cause of this patient's postoperative findings. These findings are consistent with iris toxicity associated with systemic moxifloxacin. Supportive care was given, however, the resulting pigment dispersion and mydriasis are permanent. Conclusions: Although generally safe for intracameral use, moxifloxacin has the potential to be toxic to the iris. Patients with phakia may be at higher risk of this toxicity, and lower concentrations of intracameral moxifloxacin should be considered in these patients.

Symptomatic pigment dispersion with intraocular pressure elevation attributed to intracameral moxifloxacin after open-globe injury

Background: Intracameral moxifloxacin is commonly used following intraocular surgery to prevent endophthalmitis. Pigment dispersion is a rare complication of moxifloxacin use that has mainly been reported following systemic administration. Case presentation: We report a case of unilateral pigment dispersion three weeks after open-globe repair with intracameral moxifloxacin presenting with pain, redness, photophobia, elevated intraocular pressure, marked pigment liberation, new iris transillumination defects and pigment deposits on the anterior iris surface. Conclusion: Symptomatic unilateral pigment dispersion with IOP elevation following intracameral moxifloxacin is a rare entity, but it is a crucial complication for clinicians to be aware of as intracameral antibiotic use following intraocular surgery becomes more frequent.

Anterior Segment Optical Coherence Tomography in Uveitis-Glaucoma-Hyphema Syndrome

ABSTRACT Purpose Uveitis-Glaucoma-Hyphema (UGH) syndrome results from contact between the intraocular lens (IOL) and the iris or ciliary body, leading to uveal structure erosion and blood-aqueous barrier breakdown. Treatment involves various drugs, with IOL removal often being necessary. Diagnosis relies on clinical signs, but imaging techniques like ultrasound biomicroscopy (UBM) or anterior segment optical coherence tomography (AS-OCT) are crucial. AS-OCT accurately depicts IOL position and potential contact, emerging as a primary alternative to UBM in the diagnosis. Our study aimed to correlate AS-OCT findings with clinically detectable iris atrophy in pseudophakic patients with IOL-iris chafing and UGH syndrome. Methods The study retrospectively analyzed patients diagnosed with UGH syndrome presenting at the Ocular Immunology Unit of Reggio Emilia, Italy, from January 2019 to August 2023. Patients’ data were collected. Ophthalmological exams and imaging were performed. The peephole sign in AS-OCT images was evaluated. Statistical analyses were conducted, with a significance level of p ≤ 0.05. Results The study reviewed 22 eyes of 22 patients with UGH syndrome. Four eyes were excluded, leaving 18 patients (8 females, 10 males). Common misdiagnoses included idiopathic anterior uveitis (55.5%) and herpetic anterior uveitis (16.7%). All patients had iris transillumination defects, mostly focal (77.8%). AS-OCT revealed IOL chafing in all the eyes, with peephole sign correlation. More peephole signs occurred with IOL in the sulcus (p-value = 0.08). Conclusion The study recommends AS-OCT for UGH syndrome confirmation and UBM when IOL-iris chafing is not observed on AS-OCT scans.

Case series: Fundus autofluorescence abnormalities in a family of ocular albinism carriers.

Carriers of ocular albinism demonstrate signs of retinal mosaicism with unique features on fundus autofluorescence testing, which differentiate this condition from other x-linked retinal disorders in carrier patients. Distinctive findings include a mud-splattered fundus with peripheral hyperpigmented streaks, which correlate with areas of hyperautofluorescence and hypoautofluorescence. This is the first reported case series of a family that demonstrates diagnostic retinal and fundus autofluorescence abnormalities related to retinal mosaicism in three sisters who were unaware they were carriers of ocular albinism type 1. Multimodal imaging, electrodiagnostic testing, and genetic testing can be used to confirm the diagnosis and differentiate this clinical presentation from other sight-threatening hereditary retinal diseases. Three sisters, aged 21, 17, and 13 years, were referred to determine the cause of abnormal retinal pigmentation. All presented with normal vision, and anterior segment examination was unremarkable without iris transillumination. They denied family history of ocular disease. Fundus examination of all three sisters revealed a mud-splattered pattern of pigmentation in the posterior pole and radial pigmentary streaks. Fundus autofluorescence showed a pattern of hyperautofluorescence and hypoautofluorescence corresponding to this pigmentary pattern. Spectral domain optical coherence tomography, electro-oculogram, and electroretinogram were normal in all three sisters. Genetic testing of their father, who was unaware of any disorder, tested positive for ocular albinism. Ocular albinism carriers have abnormal retinal pigmentation in a characteristic pattern. Fundus autofluorescence shows a correlative pattern that can confirm carrier status of ocular albinism in individuals unaware of their status and rule out other retinal degenerations.

Evaluation of pseudoexfoliation syndrome in eyes undergoing cataract surgery in tertiary care centre of Uttarakhand

Background: Pseudoexfoliation (PEX) syndrome, an age related microfibrillopathy having varied prevalence and associated with dense cataract, poorly dilating pupil resulting in challenging cataract surgeries. The present study aimed to evaluate pseudoexfoliation syndrome in eyes undergoing cataract surgery in tertiary care centre of Uttarakhand. Materials and methods: A hospital based prospective study carried out from January 2021 to June 2022 and approved by the Institutional Ethical Committee. A total of 1000 eyes of 1000 patients to be operated underwent complete ophthalmic evaluation including refraction, tonometry, gonioscopy, slit lamp biomicroscopy for presence of pseudoexfoliation, cataract grading, and fundus examination. Patients with PEX were diagnosed and data analysed. Results: A total of 54 PEX cases were diagnosed with 29 (53.7%) males and 25(46.3%) were females having mean age of 67.48 + 9.79 years, mean CCT was 528.46 + 18.49 micron and mean IOP was 19.81 + 5.88 mmHg. Prior to cataract surgery, an IOP of more than 21 mm Hg was noted in 40.7% (n=54) of pseudoexfoliative eyes. PXM on pupillary margin present in 51 (94.4%), on anterior lens capsule in 37 (68.5%), on iris in 31 (57.4%), on cornea in 5 (9.3%), phacodonesis in 6 (11.1%), Iris transillumination defect in 3 (5.6%), PXM on trabecular meshwork in 24 (44.4%) and sampaolesi line in 13 (24.1%). Conclusion: PEX presents challenges that necessitate thorough preoperative planning for a safe surgery and a satisfactory postoperative result. Therefore, dilated anterior segment evaluation should be emphasised in daily practise to avoid early PEX patients from being undiagnosed.

Reduction of lens size in PAX6-related aniridia

Heterozygous mutation of PAX6 in humans leads to congenital aniridia (OMIM 106210) which is typified by congenital iris and foveal defects, and later onset glaucoma, aniridic keratopathy, and cataract. Mice heterozygous for Pax6 mutations phenocopy many aspects of aniridia including the iris defects, keratopathy and cataract, although Pax6 mutant mice have small lenses, a phenotype which is not typically reported in human aniridia, perhaps due to difficulties in measuring lens diameter during typical ophthalmic examinations as the lens periphery is shielded by the iris. In order to overcome this, records of patients diagnosed with congenital aniridia between April 2015 and May 2021 at the Necker-Enfants Malades Hospital, and genetically confirmed with a disease-causing PAX6 variant, were retrospectively reviewed for those with normal axial length whose iris defects allowed visualization of the lens margins and corneal diameter to allow calculation of a lens/corneal diameter ratio. This value was compared with values obtained from a cohort of patients with Sjödell grade IV oculocutaneous albinism type 1 (OCA1; OMIM 203100) which allowed visualization of the lens periphery via iris transillumination. This analysis revealed that patients with congenital aniridia had a significantly lower lens/corneal ratio when compared to those with albinism, suggesting that humans haploinsufficient for PAX6, like mice, rats, frogs, and zebrafish, exhibit reductions in lens size.

Bilateral Acute Iris Transillumination Associated with Moxifloxacin Antibiotic Use

ABSTRACT Purpose To describe the clinical and demographic characteristics and associated factors leading to bilateral acute iris transillumination (BAIT) syndrome. Methods A retrospective review of patients with BAIT syndrome was performed. Results Thirty-five patients with a diagnosis of BAIT were identified. The median age at presentation was 53 years; 80% of the patients were female. Twenty-six patients (74%) had recent histories of systemic antibiotic treatment. Of those with such a history, 24 patients (92%) had been receiving moxifloxacin. Two patients within our cohort were prescribed moxifloxacin prophylactically prior to a systemic surgical procedure and had no evidence of systemic illness or recent viral illness. Conclusions Our data support the notion that moxifloxacin might be associated with the onset of BAIT syndrome. Notably, within our cohort, two patients received moxifloxacin as surgical prophylaxis and subsequently developed BAIT syndrome. This could suggest a potential association between moxifloxacin and the onset of BAIT, though further studies are needed to confirm this finding.

Selective Laser Trabeculoplasty (SLT) for IOP Control in a Patient with Bilateral Acute Iris Transillumination (BAIT)

ABSTRACT Introduction Bilateral acute iris transillumination (BAIT) is a clinical condition characterized by acute bilateral loss of iris pigment epithelium and iris transillumination. Because of the similar presentation, it is commonly confused with anterior uveitis, making it an important differential diagnosis for this condition. Case description We report the case of a 60-year-old male patient who presented all the characteristics of BAIT after undergoing systemic treatment with moxifloxacin and that had the intraocular pressure (IOP) successfully controlled with selective laser trabeculoplasty (SLT). Conclusion SLT might be an effective therapeutic option and should be considered for the control of IOP in BAIT.

Clinical profile and demographic distribution of pigment dispersion syndrome: An electronic medical record-driven big data analytics from an eye care network in India

Objectives: The purpose of this study was to describe the demographics and clinical profile of pigment dispersion syndrome (PDS) in patients presenting to a multi-tier ophthalmology hospital network in India. Material and Methods: This cross-sectional hospital-based study included 2,961,706 new patients presenting between August 2010 and September 2021. Patients with a clinical diagnosis of PDS in at least one eye were included as cases. The data were collected using an electronic medical record system. Results: Overall, 403 (0.014%) patients were diagnosed with PDS. Three fourth of the patients were male (75.43%) and 91.81% had bilateral affliction. The most common age group at presentation was during the fourth decade of life with 100 (24.81%) patients. In the 773 eyes, 443 (57.31%) eyes had mild or no visual impairment (<20/70) and blindness (>20/400) in 81 (10.48%) eyes. Krukenberg spindle was seen in 445 (57.57%) eyes and iris transillumination defects in 33 (4.27%) eyes. About a third of the eyes, 241 (31.18%) eyes had an intraocular pressure (IOP) >21 mm of Hg at presentation. Three hundred and twenty-eight (42.43%) eyes were on more than one anti-glaucoma medication. YAG peripheral iridotomy was documented in 100 (12.94%) eyes. In the 46 (5.95%) eyes that required a glaucoma related surgical intervention, combined surgery was performed in 30 (3.88%) eyes and trabeculectomy in 16 (2.07%) eyes. Conclusion: PDS is more common in males presenting during the fourth decade of life and is predominantly bilateral. A third of the eyes have raised IOP and a tenth of them are affected with blindness at presentation.

Anterior segment phenotypic changes in late-onset retinal degeneration with Ser163Arg mutation in CTRP5/C1QTNF5.

Late-onset retinal degeneration (L-ORD) is a rare retinal dystrophy with anterior segment (AS) abnormalities, including long anterior zonules (LAZ) and iris atrophy. This investigation evaluates AS changes in a L-ORD cohort. Prospective, longitudinal study including L-ORD individuals (Ser163Arg) with ocular exam and standard slit-lamp photographs between 2011 and 2022. AS images were merged and assessed for LAZ number and zonule-free zone (ZFZ) radius. Further clinical findings such as iris atrophy patterns were reported descriptively. Twelve eyes of 6 patients (4 males, median age = 60.5years) were included, showing a median of 160 (11-372) LAZs, mainly localized superiorly (39%) and inferiorly (24%). There was a high inter-ocular correlation (rs = 0.94, p < 0.01), no difference in LAZ count between eyes (p = 0.82), and an inverse relationship between LAZ and age (r = - 0.82; p < 0.05). The ZFZ had median 2.1mm (1.3-5.4), with no inter-ocular difference (p = 0.31). Iris transillumination defects occurred in 11/12 eyes, with 4 major patterns identified: pupillary ruff rarefaction (10/12), patchy atrophy (6/12), notched defects (6/12), and radial streaks (2/12). In a short-term follow-up of 5.9years, 4 eyes showed a reduction in LAZ count to median 139.5 (67-169) (p = 0.50) and a concomitant increase in ZFZ measurement to median 2.2 (1.7-2.6) (p = 0.17). This study confirms symmetric LAZs count and ZFZ in L-ORD, with ZFZ measurements smaller than in previous cohorts. A reduction in LAZs count and an increase in ZFZ with age were suggested longitudinally, yet findings need further evaluation as follow-up was limited to two cases.

Bilateral Acute Iris Transillumination and Elevated Intraocular Pressure After COVID-19 Infection.

Bilateral acute iris transillumination (BAIT) is characterized by abundant pigment discharge into the anterior chamber. Atonic pupil and severe intraocular pressure (IOP) elevations may be observed. Generally, there is a viral upper respiratory tract infection or systemic fluoroquinolone usage before BAIT. Two cases with a recent history of coronavirus disease 2019 (COVID-19) presented with a complaint of decreased vision. Elevated IOP and iris transillumination defects were observed in both patients. Both patients were diagnosed with BAIT. Although elevated IOP was controlled with medical treatment in 1 patient, glaucoma surgery was performed in the other patient because it could not be controlled with medical treatment. As both patients received no systemic treatment for COVID-19, the pressure rise seems to be directly related to the viral infection alone through the inflammatory process. Atypical ocular presentations of COVID-19 should be kept in mind as it can have serious consequences.

SUSPECTED FUCHS’ ENDOTHELIAL CORNEAL DYSTROPHY IN A NEONATE WITH MYOTONIC DYSTROPHY TYPE 1

Purpose: To report a novel case of FECD in a neonate with myotonic dystrophy type 1. Observations: A 32-week neonate admitted to the NICU (Neonatal Intensive Care Unit). Patient was suspected to have myotonic dystrophy, which was confirmed with DMPK PCR (Polymerase Chain Reaction) and Southern Blot Analysis. Ocular evaluation revealed iris transillumination, bilateral hazy cornea, intraocular pressure (IOP) of 18mmHg for right eye and 22mmHg for left eye, and the centre cornea thickness (CCT) was 862 microns and 887 microns for right and left eye, respectively. The thick and hazy corneas were suggestive of Fuchs’ endothelial corneal dystrophy (FECD). Conclusions: This may be the first reported case of FECD in neonate with DM1. Knowledge that FECD may present in neonates with DM1 allows for earlier identification and treatment, resulting in decreased vision loss from FECD.

Bilateral acute iris transillumination after photorefractive keratectomy

A 25-year-old male presented with ciliary congestion, pigmented cells in the anterior chamber, and raised intraocular pressure (IOP) in both eyes (left &gt; right) two weeks after photorefractive keratectomy (PRK). The patient was treated for acute anterior uveitis with topical steroids, moxifloxacin, cycloplegic, and antiglaucoma eye drops. Systemic workup was unremarkable except elevated IgG CMV levels. Despite treatment, inflammation increased and IOP became treatment-refractory (peaked to 50 mmHg), with florid pigment deposition on lens capsule, diffuse iris transillumination defects, and dilated distorted pupils. Suspecting bilateral acute iris transillumination (BAIT), topical moxifloxacin was withdrawn with gradual resolution of disease over four months.

Bilateral Acute Depigmentation of Iris (BADI) and Bilateral Acute Iris Transillumination (BAIT)-An Update.

Bilateral acute depigmentation of the iris (BADI) and bilateral acute iris transillumination (BAIT) are relatively new clinical entities characterized by acute pigment dispersion from the iris stroma or iris pigment epithelium, respectively. While BADI presents with diffuse or geographic areas of iris stromal depigmentation without transillumination, BAIT cases typically develop diffuse iris transillumination and mydriatic atonic pupils. Prolonged pigment dispersion and ocular hypertension are more common in BAIT. Although the exact etiopathogenesis is still unknown, moxifloxacin toxicity appears to be a probable/likely cause. The underlying cause of BADI or BAIT in patients who were not exposed to fluoroquinolone antibiotics remains unexplained. Systemic viral infections, including coronavirus disease 2019, may be the triggering event in several cases.

Bilateral Acute Depigmentation of Iris (BADI) and Bilateral Acute Iris Transillumination (BAIT)Following Acute COVID-19 Infection

ABSTRACT Purpose To report the presenting features and outcomes in patients who developed bilateral acute iris transillumination (BAIT) or bilateral acute depigmentation of iris (BADI) following acute COVID-19 infection. Methods Thirty two eyes of 16 patients were reviewed retrospectively. The severity of COVID-19 infection, use of antibiotics, time of onset of ocular symptoms; ocular signs, the course and surgical procedures were recorded. Results 24 eyes of 12 BAIT and eight eyes of four consecutive BADI patients were included. The mean time between infection and onset of ocular symptoms was 2.5±1.1 weeks. Nine patients were treated with oral moxifloxacin for COVID-19 prior to presentation. Trabeculectomy was performed in 7 eyes (21.8%) of 5 BAIT patients; in the postoperative follow-up, IOP was controlled without medication in 6 eyes, with medication in 1 eye. Conclusion BADI and BAIT can also develop after COVID-19 infection. A significant proportion of BAIT patients may require glaucoma surgery.

Will my child with albinism lose vision over time? Age-related vision loss in adults with albinism

Albinism is associated with congenital nystagmus, foveal hypoplasia, iris transillumination and optic nerve anomalies. Because it is a panocular developmental disorder, we sought to study other common ocular disorders in adults with albinism.

Bilateral acute Iris transillumination after COVID-19 pneumonia.

PurposeTo report the diagnosis, treatment and follow-up of a case of bilateral acute iris transillumination (BAIT) developed after COVID-19 pneumonia.MethodCase report.CaseA 66-year-old male patient with COVID-19 pneumonia received systemic treatments of favipiravir, prednisolone, moxifloxacin and piperacillin-tazobactam during hospitalization. The patient applied to our clinic with the complaint of blurred vision 20 days after the diagnosis of COVID-19. The best corrected visual acuity (BCVA) was 0.3 in the right eye and 0.5 in the left eye. In the anterior segment examination; ciliary injection, intense pigment dispersion in the anterior chamber, pigment deposits on the lens and iris, 2–3 + cells in the anterior chamber, posterior synechia, and 360 degrees diffuse iris transillumination were observed in both eyes. The pupillary response to light was weak. Bilateral fundus examination were normal. In the anterior chamber sample; HSV, VZV, CMV and Toxoplasma PCR were negative. Bilateral acute iris transillumination (BAIT) diagnosed in the patient and topical 0.1% dexamethasone and topical 1% cyclopentolate were started. In the follow-up, visual acuity increased 1.0 in both eyes, there were no cells in the anterior chamber, and the pigment dispersion was still continuing despite a decrease.ConclusionBAIT, which can usually be seen after upper respiratory tract infections, can also be seen after covid 19 pneumonia and be kept in mind as a possible eye involvement in patients with COVID-19 infection.

Bilateral Iris depigmentation, transillumination defects and hypotony in Vogt-Koyanagi-Harada disease.

Bilateral Iris depigmentation, transillumination defects and hypotony in Vogt-Koyanagi-Harada disease.

Characteristics of pigmentary glaucoma in Japanese individuals

PurposeMyopia is a known risk factor of pigmentary glaucoma (PG), and the increased prevalence of myopia in Asian countries indicates that more cases of PG will likely develop soon. However, there are no diagnostic criteria for PG for Asians. Therefore, the purpose of this study is to determine the characteristics of PG in Japanese individuals and establish three diagnostic signs for PG.MethodsThis was a single-center, retrospective, case series study of glaucoma patients who visited the Kagoshima University Hospital between January 2015 and January 2020. The inclusion criteria were age <50 years at time of diagnosis and presence of pigmentation in the anterior chamber (AC) angle including a Sampaolesi line. Eyes with pigmentation of the AC angle caused by other types of glaucoma such as uveitis, trauma, exfoliation, or childhood glaucoma were excluded. We investigated the classic diagnostic triad of signs of PG; posterior corneal pigmentation, mid peripheral iris transillumination defect, and pigmentation of the trabecular meshwork. We also examined the Sampaolesi line, iris concavity, and midperipheral iris depigmentation in eyes with PG.ResultsTen eyes of 5 Japanese men and 10 eyes of 5 Japanese women were studied. Their age ranged from 13 to 46 years at the time of diagnosis. One eye had posterior corneal pigmentation and 6 eyes had pigmentation of the trabecular meshwork. None had mid peripheral iris transillumination defect. The Sampaolesi line, iris concavity, and midperipheral iris depigmentation were found in all patients except one patient who lacked the mid peripheral depigmentation. Two eyes had the pigment reversal sign, none had lens pigmentation, and 2 eyes had peripheral retinal degeneration.ConclusionThe presence of the Sampaolesi line, iris concavity, and midperipheral iris depigmentation may be appropriate signs for the diagnosis of PG in Asians.