Atypical presentation of bilateral iridoschisis with abnormal visibility of iris vessels: Role of anterior segment optical coherence tomography.

PURPOSE: To describe clinical and anterior segment optical coherence tomography (AS-OCT) findings in a patient with bilateral iridoschisis and unilateral angle closure glaucoma (ACG) associated with abnormal visibility of iris vessels. CASE PRESENTATION: A 67-year-old male patient with a history of red and painful left eye (LE) one year earlier, presented to our ophthalmology department for a routine examination.Ophthalmic examination of the right eye revealed narrow anterior chamber with sectorial iris atrophy associated to abnormal visibility of an iris vessel. Intraocular pressure (IOP) was 12 mmHg with normal optic disc appearance. LE anterior chamber was narrow with diffuse iris atrophy and abnormal vessels visibility. IOP was 28 mmHg with an important optic disc excavation. On gonioscopy, angle was narrow without neovessels nor synechiae. AS-OCT of both eyes revealed shallow angles, iris splitting with material release in the anterior chamber, while pigmented epithelium was preservedAnti-glaucoma eye drops were prescribed and peripheral laser iridotomy was performed in both eyes with decreased IOP at 14 mmHg in the LE. CONCLUSION: Iridoschisis is a rare ocular condition characterized by a separation between the anterior and posterior layers of iris stroma with several clinical presentations, and may be associated with abnormal visibility of iris vessels in some cases. The diagnosis of iridoschisis may be challenging and AS-OCT can be a very useful tool to confirm the diagnosis in atypical presentations and to detect associated angle closure.

Glauco-Claw: A novice implant for chronic angle-closure glaucoma.

This article describes 1-year outcomes of a new intra-ocular implant, "Glauco-Claw," in refractory chronic angle-closure glaucoma (ACG). Glauco-Claw is a novice polymethylmethacrylate implant with a central ring and five claws placed circumferentially. It was placed in the anterior chamber and the peripheral iris was tucked into the claws, thus causing goniosynechialysis and preventing the reformation of goniosynechiae. It was implanted in five eyes of five patients, and they were followed up for 1 year. Target intra-ocular pressure was achieved and maintained in all the patients till the last follow-up. Two patients did not require any anti-glaucoma medication. No significant complications were observed in any patient. Glauco-Claw could be another armamentarium in the management of refractory chronic ACG.

[Painful anisocoria in a five-year-old child: A rare diagnosis which must not be missed]. / Anisocorie douloureuse chez un enfant de cinq ans : un diagnostic rare à ne pas méconnaître.

Congenital ectropion uveae (CEU) is a rare anomaly of the embryonic development of the anterior segment of the eye. We report the case of a 5-year-old child with an undiagnosed CEU who was treated urgently for an acute angle closure attack. CASE DESCRIPTION: A 5-year-old child was referred urgently for evaluation of anisocoria with mydriasis of the right eye and severe headache. Brain imaging with contrast injection was initially performed in the pediatric emergency department and ruled out central nervous system pathology. The initial examination of the right eye revealed an intraocular pressure (IOP) of 37mmHg, corneal edema, congenital ectropion uveae, mydriasis with pupillary block, a closed angle on gonioscopy, and a clear lens. The examination of the left eye was unremarkable, with no visible CEU. The initial management consisted of medical treatment with topical glaucoma drops and miotics and acetazolamide at 10mg/kg/d. Re-evaluation under general anesthesia showed persistent mydriasis and no resolution of the pupillary block. Filtering surgery was performed in the absence of a complete response to medical treatment, allowing control of IOP without drops and complete regression of the corneal edema. DISCUSSION: CEU is a rare malformation, and pressure complications represent an insignificant proportion of pediatric glaucoma cases. The acute presentation of acute angle closure in this potentially blinding short-term setting, however, makes detection and management difficult in very young children in a great deal of pain. Only one similar case has been reported in the pediatric literature. CONCLUSION: Acute angle closure complicating CEU is exceptional and difficult to diagnose in a pediatric context. Parents of children with this predisposing condition should be informed of the need to consult urgently when clinical signs of elevated intraocular pressure appear.

'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.

Innovation Urrets-Zavalia syndrome.

Background: Surgeons often notice unexplained dilation of the pupil following an uncomplicated intra-ocular surgery. No definite line of treatment has been proposed for managing Urrets-Zavalia syndrome (UZS). The authors have previously documented the results of surgical pupilloplasty and have outlined this modality of treatment for cases with UZS. Purpose: To highlight the aspect of development of UZS post-operatively in the eyes following an intra-ocular surgery. Synopsis: The video highlights the aspect of prevalence of the persistently dilated pupil that is non-responsive to topical miotics. Apart from associated glare, these cases often have raised intra-ocular pressure because of appositional closure of the anterior chamber angle. Surgical pupilloplasty pulls the iris tissue centrally, eventually releasing the mechanical blockage and often breakage of the peripheral anterior synechias as demonstrated by intra-operative gonioscopy and anterior segment optical coherence tomography. Highlights: Performing a surgical pupilloplasty can resolve the UZS, and timely intervention can also prevent the development of secondary glaucoma because of fallback of the iris tissue on the structures of the anterior chamber angle. Online Video Link: https://youtu.be/IF_w8dVk5_w.

Spontaneous rupture of secondary iris inclusion cyst causing acute glaucoma and other ocular sequelae.

A 63-year-old woman with a known secondary iris inclusion cyst in her right eye presented with headache, blurry vision, and eye pain of 3 days' duration. Initial findings were notable for significant decrease in vision and elevated intraocular pressure in the right eye, with diffuse microcystic corneal edema, diffuse anterior chamber flare with minimal cellular reaction, and a significantly decompressed iris inclusion cyst. On gonioscopy, the right eye was open to scleral spur, and no pigment was visualized. Patient history and presentation were consistent with a diagnosis of spontaneous rupture of iris inclusion cyst causing secondary glaucoma. Iris inclusion cysts are not uncommon; however, ocular outcomes are generally benign and limited to obstruction of the pupillary axis.

A rare case of congenital pupillary abnormality: a case report.

BACKGROUND: Congenital anomalies of the pupil are quite varied, including abnormal size, shape, color, response to stimulus, and function. We are here reporting an unusual case presented with the absence of pupillary opening with folds of iris tissue at the center. Only an extremely small pupil (diameter < 0.5 mm) could be observed during the operation. CASE PRESENTATION: A 15-year-old male patient visited our outpatient clinic due to vision difficulty in his right eye for more than ten years. The best-corrected visual acuity was 2.0 logMAR and 0 logMAR for the right and left eye, respectively. There were amblyopia, astigmatism and constant exotropia in his right eye. Ophthalmic examination of the right eye showed flat iris root, minimal iris pigmentation, and the pupil area was entirely covered by iris tissue. Lens status and fundus evaluation could not be commented. The left eye was found to be within normal limit. Based on ophthalmic examination, the admission diagnosis was given as acorea. Pupilloplasty was performed on the right eye due to the situation that the iris tissue blocked the visual axis, which led to visual impairment and stimulus deprivation amblyopia. However, an extremely small pupil at the center of his pupillary area was observed during the operation. The postoperative course was favorable, and a normal pupil was secured. Hospital discharge diagnosis was given as microcoria, and amblyopia treatment was followed. CONCLUSIONS: We report a rare case of congenital pupillary abnormality. The further diagnosis was given as microcoria, which should be differentiated from acorea. For this kind of pupil disorder which blocks the visual axis, early diagnosis and treatment can help prevent the development of stimulus deprivation amblyopia.

SPONTANEOUS REGRESSION OF A PRIMARY IRIS STROMAL CYST IN A PATIENT WITH KERATOCONUS. A CASE REPORT.

PURPOSE: To report the rare case of a 29-year-old male with a history of keratoconus, who presented with a primary iris stromal cyst which eventually showed spontaneous regression. METHODS: Description of the clinical findings in the case of a 29-year-old male with a prior history of keratoconus, but no eye surgery or trauma, who consulted for an iris cyst in the left eye, diagnosed 9 months earlier. CASE REPORT: Slit-lamp examination revealed mild dyscoria, and a large cyst in the inferior quadrant of the iris. Ultrasound biomicroscopy and anterior segment optical coherence tomography of the left eye confirmed the presence of a giant iris cyst with thin walls, in contact with the corneal endothelium. Corneal endothelial cell density in the inferior cornea (close to the cyst) was 1805 cells/mm2 and 2066 cells/mm2 in the central area. After considering the risk of anterior chamber epithelial downgrowth following any surgical procedure of the cyst, the patient received conservative management. In the following months, the patient presented with 3 episodes of anterior uveitis, managed with topical corticosteroids. Finally, at approx. 21 months after the initial diagnosis, the cyst presented spontaneous regression. Anterior segment optical coherence tomography confirmed the absence of fluid inside the cyst remnants and the final endothelial cell densities evidenced endothelial cell loss (inferior cornea 738 cells/mm2 and central cornea 1605 cells/mm2). CONCLUSION: Conservative management should be considered in patients with cysts that show slow progression and are distant from the visual axis, in order to minimise the risk of complications following any surgical procedure of the cyst. In addition, the present case is one of the few of primary stromal iris cysts with spontaneous regression reported in the literature.

Long-Term Outcome of Femtosecond Laser-Assisted Keratopigmentation: Using Intacs Channels for Precise Pigment Deposition Within the Cornea.

PURPOSE: To describe a long-term outcome of a new technique using the femtosecond laser to assist with keratopigmentation (corneal tattooing). METHOD: Nineteen eyes of 18 patients with a history of significant visual disability related to iris defects underwent femtosecond laser-assisted keratopigmentation (FAK) for functional restoration of their visual disabilities. Postoperative visual acuity and symptomatic resolution were collected during their postoperative visits. RESULTS: After FAK surgery, all patients had improvement in their previous visual-related symptoms. Eighteen of 19 eyes (94%) had improvement in their best-corrected Snellen visual acuity. Eight of 19 eyes (42%) had at least 1 line improvement, 8 of 19 eyes (42%) had at least 2 lines of improvement, and 2 of 19 eyes (10%) had at least 3 lines improvement. The average follow-up period was approximately 60 months. CONCLUSIONS: The FAK technique offers a long-term effective and safe technique for visual disabilities and improves visual acuity in patients with various traumatic iris defects.

Novel mechanism of decreased iris vasculature density after cosmetic iris implants.

A 25-year-old man presented with decreased vision in both eyes, approximately 4 years following bilateral bright ocular cosmetic iris implantation. On examination, he was found to have bilateral elevated intraocular pressures, anterior chamber cells and flare, chronic peripheral anterior synechiae and significantly reduced endothelial cell counts. Ultrasound biomicroscopy demonstrated compression of the peripheral iris, resulting in synechial angle closure in both eyes. Surgical removal of the implants was performed without additional complication. On removal, bilateral iris atrophy was evident with non-reacting pupils and permanent mydriasis. Optical coherence tomography angiography showed a reduction in iris vasculature density that is more pronounced in the area of the iris atrophic defects. This case suggests that cosmetic iris implants may compress iris vasculature, resulting in decreased iris perfusion resulting in atrophic mydriasis and iris defects. This is a potential novel mechanism for complications in eyes with cosmetic iris implants.

Mosaic cat eye syndrome in a child with unilateral iris coloboma.

BACKGROUND: Cat eye syndrome (CES) is a rare chromosomal disorder with a known incidence of 1 per 50,000-150,000 live newborns. The classic triad of iris coloboma, anorectal malformations, and auricular abnormalities is present in 40% of patients. In addition, other ocular malformations and systemic defects can be present. The aim of this report is to present a patient with unilateral iris coloboma related to a mosaicism of cat eye syndrome. METHODS: A complete ophthalmological and systemic evaluation was performed in a three-year-old male. He also underwent a standard karyotype and FISH analysis with a probe against the 22q11.2 locus. RESULTS: The ophthalmological and systemic evaluation revealed a unilateral iris coloboma and ipsilateral auricular malformations. Karyotype analysis of blood leukocytes indicated the presence of a marker chromosome in 6% of the analyzed cells. FISH analysis showed three positive signals in 5.5% of the analyzed nucleus. CONCLUSION: This patient presented two of the three classic manifestations of CES; interestingly, they were unilateral. The 22q11 duplication was identified by standard karyotype and confirmed with FISH. The present case demonstrates the importance of conducting a multidisciplinary approach in patients with congenital malformations associated with known syndromes.

Iris cyst and atypical complications / Cisto de íris e complicações atípicas

ABSTRACT The authors report the case of a male adult presenting significant ocular complications and irreversible visual impairment, resulting from the long-term progression and late diagnosis of an iris cyst in the right eye, probably secondary to trauma. The patient was admitted to Hospital Universitário Antonio Pedro with a total corneal opacity that blocked direct visualization of the anterior chamber. Ultrasound biomicroscopy was crucial for the anatomic study, and the patient was submitted to enucleation for aesthetic improvement and clarifying diagnosis. We concluded athalamia and deformation of the anterior segment, due to expansion of the cyst, led to gradual elevation of the intraocular pressure and damage of the optic nerve, resulting in visual loss.

RESUMO Relatamos o caso de um paciente com evolução e diagnóstico tardios de cisto de íris no olho direito, provavelmente secundário a trauma, com complicações importantes e baixa irreversível da visão, tendo sido admitido no Hospital Universitário Antônio Pedro já com leucoma total da córnea e câmara anterior indevassável. A biomicroscopia ultrassônica se mostrou imprescindível para o estudo anatômico, sendo o paciente finalmente submetido à enucleação, para melhora estética e elucidação diagnóstica. Concluímos que a atalamia e a desestruturação do segmento anterior, consequentes ao crescimento cístico, levaram a um gradativo aumento da pressão intraocular e lesão do nervo óptico, com consequente perda da visão.

Iris Stromal Cyst in a 6-Month-Old With Rapid Progression to Angle Closure.

CASE REPORT: A 6-month-old female presented with an iris cyst in the left eye, first identified at age 4 months. The patient was seen by an ophthalmologist at age 6 months and referred for further management. Our examination 2 weeks later demonstrated a 6 mm diameter iris cyst originating from the anterior surface of the inferior iris, occupying the inferior two thirds of the anterior chamber and obscuring the pupil. Intraocular pressure was normal and the remainder of the anterior chamber was formed. Because of concern for the development of amblyopia, the patient was scheduled for an examination under anesthesia and iris cyst removal 2 days later. In the intervening 2 days, the patient's mother noted worsening photophobia and tearing. At the time of surgery, the intraocular pressure was 51 mm Hg in the left eye. Anterior examination demonstrated interval development of shallowing of the anterior chamber with irido-corneal and cyst-corneal touch. The iris cyst had increased to 8 mm in size and filled the entire pupillary aperture. The iris cyst was excised, and histopathology confirmed the diagnosis of an iris stromal cyst. CONCLUSIONS: Congenital stromal cysts of the iris can enlarge, threatening amblyopia and secondary glaucoma in children. Although angle closure is a known theoretical complication of iris stromal cysts, actual cases are rare in the literature. This case demonstrates the importance of serial examinations to monitor progression of iris stromal cysts, particularly in young children.