Follow-Up of Bilateral Iridoschisis in a Patient With Alport Syndrome.

This case report discusses the long-term follow-up of a patient with bilateral iridoschisis and Alport syndrome.

A novel iridoplasty suture technique to repair iris defects and traumatic mydriasis.

A new iridoplasty method is described, which uses the U-suture technique to repair traumatic mydriasis and large iris defects. Two 0.9 mm opposing corneal incisions were made. The needle was inserted through the first incision, passed through the iris leaflets, and removed through the second incision. The needle was reinserted through the second incision and removed through the first incision by re-passing the needle through the iris leaflets to form a U-shaped suture. The modified Siepser technique was applied to fix the suture. Thus, with a single knot, the iris leaflets were brought closer (shrinking like a pack), fewer sutures were used and fewer gaps were left. Satisfactory aesthetic and functional results were obtained in all cases in which the technique was applied. There was no suture erosion, hypotonia, iris atrophy, or chronic inflammation during the follow-up.

Impacto del síndrome de iris flácido intraoperatorio (IFIS) en cirugía de cataratas mediante facoemulsificación: análisis de 622 casos / Impact of intraoperative floppy IRIS syndrome in cataract surger by phacoemulsification: Analysis of 622 cases

Introducción Los síndromes de pupila estrecha, incluido el síndrome de iris flácido intraoperatorio (IFIS), aumentan el riesgo de complicaciones durante la cirugía de cataratas si no se realiza una correcta planificación quirúrgica. La tamsulosina se asocia a un incremento muy significativo del riesgo de IFIS, debido a la inactivación prolongada de los receptores alfa-1 adrenérgicos en la fibra muscular lisa del iris. Material y métodos Estudio prospectivo observacional unicéntrico, llevado a cabo en el Hospital de l’Esperança - Parc de Salut Mar.ResultadosSe incluyeron 622 ojos de 502 pacientes, de los cuales 337 (62%) eran mujeres. La media de edad de la muestra era de 74,8 años. Se observaron 61 casos de IFIS (11%), de los cuales 13 recibían tratamiento con tamsulosina y uno con doxazosina. Se observaron 23 casos de IFIS en pacientes mujeres. La ratio mujer:hombre fue de aproximadamente 1:3. Se observaron 19 casos (3%) de IFIS severo, de los cuales 6 recibían tratamiento con alfa-antagonistas, sin correlación estadísticamente significativa.La media del tiempo quirúrgico fue de 13,80min (desviación estándar [DE]: 4,01min) en pacientes sin IFIS y de 16,93min (DE: 4,32min) en pacientes con IFIS. La relación entre la duración del procedimiento quirúrgico en minutos y la presencia de IFIS fue estadísticamente significativa, aplicando un test t-Student «a dos colas» o bilateral con un p valor de 0,01. Conclusión Independientemente del grado de severidad, el diagnóstico de IFIS alarga el tiempo quirúrgico en cirugía de cataratas. Esto supone otra evidencia más que apoya la utilización de tratamientos antagonistas adrenérgicos menos alfa-1 selectivos que la tamsulosina o la realización de la cirugía de cataratas antes de iniciar dichos tratamientos. (AU)

Background Small pupil syndromes, including intraoperative-floppy iris syndrome (IFIS), increase the risk of complications during cataract surgery if proper surgical planning is not performed. Tamsulosin is associated with a very significant increase in the risk of IFIS, due to the prolonged inactivation of alpha-1 adrenergic receptors in the smooth muscle fiber of the iris. Material and methods Single-center prospective observational study, carried out at the Hospital de l’Esperança – Parc de Salut Mar.ResultsSix hundred and twenty-two eyes of 502 patients were included, of which 337 (62%) were women. The mean age of the sample is 74.8 years. Sixty-one cases of IFIS (11%) were observed, of which 13 received treatment with Tamsulosin and 1 with Doxazosin. Twenty-three cases of IFIS were observed in female patients. The female:male ratio was approximately 1:3. Nineteen cases (3%) of severe IFIS were observed, of which 6 received treatment with alpha-antagonists, with no statistically significant correlation.The mean surgical time was 13.80min (standard deviation – SD: 4.01min) in patients without IFIS and 16.93min (SD: 4.32min) in patients with IFIS. The relationship between the duration of the surgical procedure in minutes and the presence of IFIS was statistically significant, applying a ‘two-tailed’ or bilateral t-Student test with a p value of 0.01. Conclusion Regardless of the degree of severity, the diagnosis of IFIS lengthens the surgical time in cataract surgery. This represents yet another piece of evidence that supports the use of less selective alpha-1 adrenergic antagonist treatments than tamsulosin or the performance of cataract surgery before starting these treatments. (AU)

Endodiathermal Tautening of Floppy or Irregular Iris in Endothelial Keratoplasty (Iridodiathermy).

ABSTRACT: Floppy or irregular irides may be seen during endothelial keratoplasty in complex cases or in eyes with damaged irides and may cause uneven air fill, retro-pupillary air escape, anterior bowing of iris, forward movement of lens-iris diaphragm, shallowing of anterior chamber (AC), bellowing and floppiness of iris, uneven AC depth, difficulty in inserting and opening graft, iris trauma, intraoperative bleeding, and iridodialysis. We present a technique of iridodiathermy for tautening and flattening such irides. With continuous irrigation using AC maintainer, the bipolar endodiathermy probe tip is applied in localized spots to midperipheral iris in the affected area with power and duration adjusted to induce mild localized shrinkage and tightening of iris stroma. Such iris tautening decreases its floppiness and prevents anterior bowing, excessive mobility, irido-corneal touch, and peripheral anterior synechiae formation. It provides a stable AC with regular depth and improved, uniform, and nonmigratory air fill, thus decreasing intraoperative challenges.

'Progressive peripheral anterior synechiae in iridocorneoendothelial syndrome- a crawling disaster'.

Purpose: Iridocorneal endothelial (ICE) syndrome is well known to cause refractory glaucoma in young adults. Commonly acclaimed mechanism for trabeculectomy failure in these cases include accelerated subconjunctival fibrosis, abnormal endothelial proliferation, and closure of ostium. In the following article, we present a case of Iridocorneal endothelial syndrome that presented with refractory glaucoma after trabeculectomy due to rapidly progressive peripheral anterior synechiae causing angle closure and corneal decompensation that mandated a tailored surgical approach of management. Methods: This is a descriptive case report based on electronic medical records, patient observation, surgical intervention, and follow-ups. Case description: A thirty-eight-year-old-male presented to us with signs suggestive of iridocorneal endothelial syndrome with gonioscopy revealing peripheral anterior synechiae (PAS) over four clock-hours temporally. Uncontrolled intraocular pressure (IOP) despite maximal medical therapy mandated augmented trabeculectomy with anti-fibrotics. The bleb failed within 3 weeks of trabeculectomy, with evidence of progressive crawling PAS causing endothelial decompensation and raised IOP. He underwent Ahmed glaucoma valve (AGV) implant surgery with viscosynechiolysis and sectoral iridectomy under antiviral cover. This helped control IOP and retain corneal clarity, with no recurrence of PAS in the affected area. Conclusion: Progressive peripheral synechiae in ICE syndrome can cause early bleb failure and refractory glaucoma. Careful viscosynechiolysis and sectoral iridectomy alongside a second implant surgery can help salvage visual functions and preserve corneal clarity while preventing further progression of PAS in these eyes.

Bilateral symmetrical maculopathy and heterochromia in Waardenburg syndrome / Maculopatia simétrica bilateral e heterocromia na síndrome de Waardenburg

ABSTRACT Waardenburg syndrome is a rare congenital genetic disorder characterized by sensorineural hearing loss and pigmentary abnormalities of the hair, skin, and eyes. Based on the different clinical presentations, it is divided into four subtypes as in WS1 to WS4. This report describes a 15-year-old boy who presented with low vision and bilateral hearing loss. His visual acuity was 20/200 in both eyes. Slit-lamp examination revealed complete iris heterochromia, with one blue iris and one brown iris. Fundus examination showed symmetrical pigmentation of the retina and choroid, with atrophy of the pigment epithelium in the macular region, notably also in the eye with normal iris pigment illustrating the broad spectrum of the iris and fundus pigmentation as part of this syndrome. A carefully clinical and ophthalmological evaluation should be done to differentiate various types of Waardenburg syndrome and other associated auditory-pigmentary syndrome. Early diagnosis in some cases may be crucial for the adequate development of patients affected with this condition.

RESUMO A síndrome de Waardenburg é uma doença genética congênita rara caracterizada por perda auditiva neurossensorial e anormalidades pigmentares do cabelo, da pele e dos olhos. Com base nas diferentes apresentações clínicas, é dividida em quatro subtipos (WS1 a WS4). Este relato descreve o caso de um menino de 15 anos que apresentava baixa visão e perda auditiva bilateral. Sua acuidade visual era de 20/200 em ambos os olhos. O exame em lâmpada de fenda revelou heterocromia completa da íris, com uma íris azul e uma íris marrom. A fundoscopia mostrou pigmentação simétrica da retina e coroide, com atrofia do epitélio pigmentar na região macular, notadamente também no olho com pigmento de íris normal, ilustrando o amplo espectro de pigmentação de íris e fundo como parte dessa síndrome. Uma avaliação clínica e oftalmológica criteriosa deve ser feita para diferenciar os vários tipos de síndrome de Waardenburg e outras síndromes auditivo-pigmentares associadas. O diagnóstico precoce em alguns casos pode ser crucial para o desenvolvimento adequado dos pacientes acometidos por essa condição.

Reversed scleral tunnel technique for repair of iridodialysis after blunt force trauma: a retrospective clinical study.

BACKGROUND: To investigate the efficacy and safety of reversed scleral tunnel technique for repairing iridodialysis after blunt force trauma. METHODS: A total of 51 eyes of 51 patients with iridodialysis undergoing surgery were included in this study. Patients were divided into 2 groups: group A (the reversed scleral tunnel technique) and group B (the control group). Before the procedure and at 1, 3, and 6 months afterward, data on the patients' age, gender, treatments, diagnosis, mechanism of injury, best-corrected visual acuity (BCVA), intraocular pressure (IOP), degree of iridodialysis, lens status, concomitant ocular damage, number of sutures, complications, and follow-up time were collected and compared between the 2 groups. RESULTS: Iridodialysis was repaired and the pupil shape was restored to nearly round in all eyes. Standard phacoemulsification or lens removal was performed in all eyes. A final BCVA ≥20/60 was achieved in 13 eyes (48.1%) in Group A and 13 eyes (54.2%) in Group B. The IOP remained stable during the follow-up period in all eyes except 2 eyes (7.4%) in Group A and 3 eyes (12.5%) in Group B with angle recession. There were no statistically significant differences in BCVA and IOP between group A and group B. Intraoperatively, A significantly lower percentage of extensive subconjunctival hemorrhage occurred in Group A compared to Group B (1 eye versus 24 eyes, χ2 = 47.1, P = 0.00). Hyphema was observed in 2 eyes (7.4%) in Group A and 1 eye (4.2%) in Group B. Postoperatively, two eyes (7.4%) in Group A and 2 eyes (8.3%) in Group B developed retinal detachment. No other complications were noted during the follow-up period. CONCLUSIONS: The reversed scleral tunnel technique is a safe and effective approach for repairing iridodialysis after blunt force trauma with few complications, favorable cosmetic and visual outcomes.

Iridodialysis repair - A simplified approach.

BACKGROUND: Iris root is the thinnest and weakest portion of the iris stroma. It can detach easily due to blunt trauma or accidental engagement of the iris during intraocular surgery resulting in glare, photophobia and monocular diplopia. Multiple techniques described for iridodialysis repair such as hang back technique, stroke and dock technique and sewing machine technique are technically challenging. PURPOSE: To describe an simplified approach of iridodialysis repair using 9-0 prolene suture. SYNOPSIS: We demonstrate the technique of iridodialysis repair using animation for better understanding. Scleral flap is made adjacent to the iridodialysis area and a paracentesis is made oppsite to the iridodialysis. One arm of the double armed straight needle with 9-0 prolene suture is passed through the paracentesis into the iris root and docked in the 26G needle which is passed underneath the scleral flap 1.5mm posterior to the limbus. Then the needle is pulled out underneath the scleral flap and the manoeuvre is repeated for the second arm as well. The sutures are secured with 5-6 knots under the scleral flap. Intra-operative surgical videos of two patients with traumatic cataract and iridodialysis following blunt trauma are shown. After stabilizing the detached iris using iris hooks, phacoemlsification is done with implantation of foldable acrylic IOL, followed by iridodialysis repair as described above. Both the patients were relieved of their pre-operative symtoms and had good visual recovery. HIGHLIGHTS: We describe a simplified approach of iridodialysis repair that can significantly reduce the patient's troublesome symptoms such as glare and monocular double vision. ONLINE VIDEO LINK: https://youtu.be/-axYnSfWSb0.

Iris Manifestations in Inadequately Treated Chronic Recurrent Vogt-Koyanagi-Harada Disease.

PURPOSE: To describe the iris changes in chronic recurrent Vogt-Koyanagi-Harada Disease (VKH). METHODS: Retrospective case series. Clinical features of 3 VKH patients who developed bilateral depigmentation of the iris are described. RESULTS: Patients had delayed diagnosis and inadequately treated chronic recurrent VKH. Patient 1 presented with bilateral multiple contiguous small granulomatous iris stromal lesions, severe diffuse iris swelling more severe near the iris root, producing peripheral iris undulations. Diffuse iris atrophy and peripheral depigmentation subsequently developed. Patient 2 presented with peripheral iris depigmentation, atrophy of the ciliary zone iris stroma and sparing of the sphincter pupillae, giving a 'sunflower appearance.' Patient 3 had extensive iris depigmentation, atrophy, and transillumination defects involving the entire iris. CONCLUSION: Uncontrolled bilateral diffuse granulomatous stromal iris inflammation leads to subsequent depigmentation, transillumination defects and atrophy, beginning in the peripheral iris. Sphincter pupillae is initially spared but complete iris atrophy may develop.

Complications of a congenital iris cyst in a newborn.

ABSTRACT: Congenital iris cysts are a rare condition in infants that can lead to multiple complications. This report describes an infant born with an iris cyst who developed complications including secondary glaucoma, cataracts, and visual impairment, requiring management with multiple surgical interventions and medications.

COVID-19 Associated Bilateral Acute Iris Transillumination.

Purpose: To report a case of bilateral acute iris transillumination (BAIT) in association with coronavirus disease 2019 (COVID-19).Case report: A 44-year-old woman patient presented with decreased visual acuity, pain, photophobia, and redness in both eyes. The patient reported that she had recent close contact with severe acute respiratory syndrome - coronavirus-2 (SARS-CoV-2) case; also, she mentioned that she was hospitalized for bilateral pneumonia for 14 days. On examination, visual acuity of both eyes was 20/40. Slit-lamp biomicroscopy showed bilateral pigment deposition on the corneal endothelium, 4+ pigment dispersion in the anterior chamber, iris depigmentation with iris transillumination defects. Intraocular pressure was measured as 32 mmHg in right eye and 38 mmHg in left eye. The patient was started on bilaterally topical anti-inflammatory and anti-glaucomatous therapy.Conclusion: It is important to keep in mind that ocular manifestations associated with COVID-19 may include rare entities such as BAIT.

Management of an iris stromal cyst by fine needle aspiration with absolute alcohol irrigation and argon laser photocoagulation to induce sclerosis.

PURPOSE: To describe the management of an iris stromal cyst by fine needle aspiration and irrigation with absolute alcohol to induce cyst sclerosis. METHODS: We present the case of a 45-year-old patient with no previous history referred for a 3-month history of an iris lesion in his right eye. Examination revealed a 2.1×3mm cyst with transparent walls at the right inferonasal iris root, and a primary acquired iris stromal cyst was diagnosed. Initially, observation was recommended, but at the 2-month follow-up, growth of the lesion was observed, so fine needle aspiration and cryotherapy were performed, with recurrence after 2 months. Subsequently, fine needle aspiration and irrigation with absolute alcohol for 2minutes to induce sclerosis of the cyst was carried out. Two weeks after surgery, argon laser was performed on the iris root and remaining walls of the cyst to induce adherence. RESULTS: After 1 year of the procedure, the patient remains asymptomatic, without associated complications. A depression and hyperpigmentation of the iris in the inferonasal sector persists, without recurrence of internal fluid or increase in cyst volume. Endothelial cell count and intraocular pressure monitoring have remained stable. CONCLUSION: Iris stromal cyst sclerosis by aspiration and irrigation with absolute alcohol was effective and avoided complications associated with resection of the lesion.

Ectropion Uveae in neurofibromatosis type 1: a new manifestation.

Abstract: Neurofibromatosis type 1 (NF1) is a rare genetic disorder with an autosomal dominant transmission and an estimated incidence of 1:2500-3500 live birth. Penetrance is virtually 100%, but the expression is highly variable and almost every organ can be affected. Diagnosis of NF1 is made with at least two of the following diagnostic criteria: six or more cafè-au-lait spots, two neurofibromas or one plexiform neurofibroma, axillary or groin freckling, optic glioma, two Lisch nodules, bone dysplasia and first-degree relative with NF1. Other ocular manifestations include orbital neurofibromas, cafè-au-lait spots on the eyelids, congenital dysplasia of the sphenoids wing and con-genital glaucoma and choroidal abnormalities. Congenital Ectropion Uveae (CEU) is a rare, non-progressive anomaly characterized by the presence of iris pigment epithelium on the anterior surface of the iris stroma, resulting from its proliferation. CEU probably depends on embryological disorders in neural cells and/or neuroectoderm of the optic cell. In this paper the authors describe three patients with CEU and NF1 found in 243 consecutive NF1 patients.