[Primary stromal cyst of the iris: case report].

Primary stromal cysts of the iris are rare, often asymptomatic, and incidentally found entities. Treatment is usually indicated in cases of enlargement or complications. However, imaging tests are required to determine their cystic nature and make an accurate differential diagnosis with malignant tumors, as well as for long-term follow-up. Ultrasound biomicroscopy is the technique of choice, although in most centers anterior segment optical coherence tomography is a more accessible and available imaging modality. We present a case of primary stromal cyst of the iris with an atypical presentation to illustrate the diagnosis and initial follow-up using anterior segment optical coherence tomography and photographs, and the management of complications. Anterior segment optical coherence tomography may be useful in the initial study and follow-up of anterior non-pigmented lesions where the cyst can be fully seen.

Anterior segment swept-source optical coherence tomography and ultrasound biomicroscopy in iris and ciliary body lesions.

OBJECTIVES: Differentiation of iris and ciliary body lesions as benign or malignant and cystic or solid is important. The aim of this study was to compare anterior segment swept-source optical coherence tomography (AS SS-OCT) and ultrasound biomicroscopy (UBM) findings in iris and ciliary body tumors. RESEARCH DESIGN AND METHODS: Forty-two eyes of 38 cases with iris and ciliary body tumors imaged with UBM and AS SS-OCT between September 2018 and September 2023 were evaluated retrospectively. RESULTS: Of 42 eyes, 14 had melanoma, 14 iris pigment epithelial (IPE) cysts, 7 nevi, 3 Lisch nodules, 2 iris stromal cysts, 1 pars plana cysts, and 1 iris mammillations. An equivalent (100%) visualization of the anterior tumor margin was obtained with both techniques. Compared to AS SS-OCT, UBM was superior for posterior margin visualization in melanocytic tumors and IPE cysts. Bland-Altman plots demonstrated good agreement between UBM and AS SS-OCT for melanocytic tumors < 2.5 mm in base diameter and < 2 mm in thickness. CONCLUSIONS: Although, UBM is the gold standard for ciliary body and iridociliary tumors. AS SS-OCT should be considered as an excellent alternative to UBM, especially in minimally elevated iris lesions.

Spontaneous collapse of an iris stromal cyst in a young child.

A 4-month-old girl was referred for evaluation of an anterior chamber cyst in the left eye. Examination under anesthesia (EUA) revealed a large iris stromal cyst in the anterior chamber involving the angle and the pupillary border, with touch of the corneal endothelium and anterior lens capsule. Intraocular pressure was normal. There was no anisometropic refractive error by retinoscopy. Close observation with amblyopia management was recommended as the pupil was only partially occluded by the cyst, and there was a clear red reflex around the cyst. Repeat EUA 6 months following diagnosis showed spontaneous collapse of the cyst. There was no recurrence noted at her most recent follow-up, 2 years after presentation, and most of the pupillary axis remains unobstructed by the cyst. The patient continues to be followed to monitor for possible cyst progression, anisometropia, and development of amblyopia. Though rare, spontaneous resolution of an iris stromal cyst may occur and thus, observation may be considered, particularly if the cyst is not causing anterior segment complications or obstruction of the visual axis.

A case report of iridoschisis and cataract: a challenging diagnosis.

PURPOSE: To report the cataract surgery of a 79-year-old female patient with iridoschisis and senile nuclear cataracts. OBSERVATIONS: Ophthalmologic examination of the 79-year-old female patient who came to our clinic with complaints of decreased vision in the right eye was observed to have a grade-3 senile nuclear cataract and iridoschisis in quadrants 2-3, 4-7, and 10-11 of the iris in the right eye and a grade-2 senile nuclear cataract and iridoschisis in quadrants 5-7 in the left eye to a lesser extent compared to the right eye. The iris fibrils were not connected to the corneal endothelium. A safe surgical area was created by administering the viscoelastic material several times during cataract surgery. CONCLUSIONS AND IMPORTANCE: Although iridoschisis has a low incidence rate, it is important to also consider comorbid ocular pathologies when treating iridoschisis patients. Since cataract surgery for these patients is more specialized than for uncomplicated cases, the necessary surgical planning must be paid due diligence.

Phacoemulsification combined with goniosynechialysis versus phacoemulsification alone for patients with primary angle­closure disease: A meta-analysis.

This meta-analysis aims to systematically compare the efficacy between phacoemulsification (PE) combined with goniosynechialysis (GSL) and PE alone for primary angle-closure disease (PACD) patients. All the data were searched from the PubMed, EMBASE and the Cochrane Library. The Cochrane Handbook was used to evaluate the quality of the included studies. Additionally, this meta-analysis was performed by using the Revman 5.4 software. Nine randomized controlled trials (RCTs) were included in this study. Compared with PE alone group, PE+GSL could result significant reduction in the IOP (MD, 1.81; p = 0.002). In the instrumental subgroup, also more reduction of IOP was shown in the PE+GSL group (MD, 2.11; p = 0.02). In the viscogonioplasty (VGP) subgroup, there was not no statistical difference between PE alone group and PE+GSL group (MD, 1.53; p = 0.11). Also, more reduction of peripheral anterior synechiae (PAS) was shown in the PE+GSL group (MD,59.15; p<0.00001). For the change in angle open distance (AOD)500, AOD 750, trabecular-iris space (TISA)500, number of glaucoma medications and best corrected visual acuity (BCVA), there was no difference between two groups (p = 0.25, 0.35, 0.17, 0.56, 0.08). For TISA 750, more improvement was shown in the PE+GSL group (p<0.00001). Instrumental separation had better effect on lowering IOP when it combined with PE. Both instrumental separation and VGP could reduce postoperative PAS. The operation of GSL has no obvious effect on postoperative vision.

Unilateral progressive anterior iris adhesions in Mowat-Wilson syndrome: a new ocular finding.

Ocular associations in Mowat-Wilson syndrome (MWS) are rare. Those involving the anterior segment are scarce in the literature. We describe a child with genetic confirmation of MWS that presented with acquired onset of unilateral anterior iris adhesions with no known trauma.

Bilateral acute iris transillumination (BAIT) syndrome: Differential diagnosis based on clinical, OCT and UBM findings.

INTRODUCTION: Bilateral acute iris transillumination (BAIT) is a relatively new syndrome whose etiopathogenesis is still not fully understood. It is characterized by acute bilateral onset of intense pigment dispersion in the anterior chamber, iris depigmentation with severe transillumination defects, accentuated pigment deposition in the angle, and elevated intraocular pressure (IOP). In literature, the first case was of bilateral acute iris depigmentation (BADI) reported in 2004 in a 77-year-old woman. In 2019, Perone et al. published a review about BAIT syndrome. They reported a total of 79 cases have been published up that date, mainly in Europe and especially in Turkey and Belgium. The majority of reported cases were of bilateral acute iris depigmentation (BADI). BAIT syndrome might be mainly confused with acute iridocyclitis, acute primary angle-closure (APAC) and pigment dispersion syndrome (PDS). In relation to BAIT, controversies still exist regarding the etiology being the differential diagnosis of paramount importance for adequate treatment. PURPOSE: To report a case of BAIT syndrome associated to refractory glaucoma and to discuss the differential diagnosis based on clinical, OCT and UBM findings. METHODS: We present a case of BAIT syndrome in which clinical, OCT and UBM findings have pointed out the similarities and, mainly the diagnosis differences with other ocular diseases. CONCLUSIONS: BAIT syndrome with accentuated IOP rise must be differentiated of other ocular diseases. It requires urgent clinical therapy and/or surgical management as occurred in the present case for avoiding structural damage in OCT and visual field loss. OCT and UBM are critical for early recognition, differential diagnosis and management.

Intraocular medulloepithelioma clinical features and management of 11 cases.

AIMS: To describe the clinical features, imaging characteristics, histopathology, treatment and outcomes of intraocular medulloepithelioma. METHODS: Medical records of 11 patients with clinically or histopathologically confirmed medulloepithelioma were retrieved and reviewed. Clinical features, diagnostic challenges, imaging characteristics, management, histopathology and prognosis were assessed. RESULTS: The median age of the patients at initial diagnosis was 4 years, with the most common manifestations being leukocoria (five eyes), loss of vision (four eyes), ocular pain (one eye) and ophthalmic screening (one eye). The clinical signs include a grey-white ciliary body lesion, cataract or lens subluxation, secondary glaucoma and evident cysts. The ultrasound biomicroscopy (UBM) imaging most commonly displays ciliary body mass with intratumoural cysts (nine eyes). Three patients underwent surgery for cataract or glaucoma while the tumours were incidentally found. Two of the three patients managed by eye preserve treatments eventually required enucleation because of local tumour recurrence or phthisis. One patient treated with intra-arterial chemotherapy and cryotherapy had successful tumour regression and globe salvage. CONCLUSIONS: Initial misdiagnosis, delay in diagnosis and subsequent misdirected management is not uncommon in medulloepithelioma. The presence of multiple cysts in the tumour and retrolental neoplastic cyclitic membrane detected by UBM can offer certain information. Selective intra-arterial melphalan may prevent further tumour growth, but longer follow-up is necessary until treatment efficacy is fully evaluated.

Prospective analysis of anatomic features predisposing patients to intraoperative floppy iris syndrome.

PURPOSE: To aid preoperative risk assessment by identifying anatomic parameters corresponding with a higher risk of intraoperative floppy iris syndrome (IFIS) during cataract surgery. METHODS: Prospective cohort study of 55 patients with α1-adrenergic receptor antagonist (α1-ARA) treatment and 55 controls undergoing cataract surgery. Anterior segment optical coherence tomography (AS-OCT), video pupilometer, and biometry measurements were performed preoperatively and analyzed regarding anatomic parameters that corresponded with a higher rate of IFIS. Those statistically significant parameters were evaluated with logistic regression analysis and receiver operating characteristic (ROC) curve. RESULTS: Pupil diameter was significantly smaller in patients who developed IFIS compared to those who did not develop IFIS (AS-OCT 3.29 ± 0.85 vs. 3.63 ± 0.68, p = 0.03; Pupilometer 3.56 ± 0,87 vs. 3.95 ± 0.67, p = 0.02). Biometric evaluation revealed shallower anterior chambers in the IFIS group (ACD 3.12 ± 0.40 vs. 3.32 ± 0.42, p = 0.02). Cutoff values for 50% IFIS probability (p = 0.5) were PD = 3.18 mm for pupil diameter and ACD = 2.93 mm for anterior chamber depth. ROC curves of combined parameters were calculated for α1-ARA medication with pupil diameter and anterior chamber depth, which yielded an AUC of 0.75 for all IFIS grades. CONCLUSION: The combination of biometric parameters with history of α1-ARA medication can improve assessment of risk stratification for IFIS incidence during cataract surgery.

Posterior synechia formation after phacovitrectomy - Predicting factors and the role of short-acting mydriatics.

PURPOSE: To evaluate the influence of topical short-acting mydriatics on the formation of posterior synechia after phacovitrectomy surgery of pars plana vitrectomy and phacoemulsification with intraocular lens implantation. METHODS: A prospective randomised controlled trial. Fifty-seven adult (>18 years old) patients (57 eyes) who underwent phacovitrectomy surgery at a single tertiary hospital, were randomly divided into two groups. The control group (29 eyes) received standard postoperative treatment (topical antibiotics and steroids). The study group (28 eyes) received short-acting mydriatics together with standard therapy. Patients were followed until 24 months after surgery. The primary outcome measure was the formation of posterior synechia during the follow-up period. RESULTS: A total of 7 patients developed posterior synechia during the follow-up period (12%), 3 in the study group (11%) and 4 in the control group (14%). There was no statistical difference between the groups. Significant associations for the development of posterior synechia were surgery for retinal detachment, longer surgery duration (>93 min) and the use of tamponade, in particular silicone oil. CONCLUSIONS: The use of topical short-acting mydriatic drops after phacovitrectomy surgery, in addition to standard post-operative treatment, did not reduce the formation of posterior synechia. However, we identified several factors that may influence or act as predictors for the development of posterior synechia: surgery for retinal detachment, using silicone oil tamponade and a longer surgery duration. Our findings may aid in the standardisation of post-phacovitrectomy surgery treatment and define potential at-risk patients who should be monitored more closely.

Intracameral phenylephrine for surgical mydriasis and intraoperative floppy-iris syndrome: systemic adverse effects and optimal dose.

Phenylephrine, a potent sympathomimetic, induces mydriasis via iris dilator muscle contraction. Intracameral (IC) phenylephrine has been successfully used in cataract surgery for initial mydriasis, maintaining mydriasis, and management of intraoperative floppy-iris syndrome. Serious systemic adverse events (mainly cardiovascular) have been described with topical phenylephrine drops, but we found very little evidence of such adverse events associated with IC phenylephrine use. However, we suspect under-reporting of such adverse events, as they may instead be ascribed to anxiety, positioning, anesthesia, etc. Optimal dosage/concentrations for IC phenylephrine use in different purposes have not been fully studied. In the absence of robust evidence, we suggest that lower but effective IC phenylephrine concentrations are used: a lower concentration (0.31%), in conjunction with an anticholinergic and lidocaine, may be used for initial mydriasis. For management of intraoperative floppy-iris syndrome, 0.31% may be effective, though a higher concentration (1% to 1.25%) may be required.

Surgical Management of Iris Bombe in Muscle-Eye-Brain Disease.

A 2-year-old girl with severe muscular dystrophy presented with unilateral eye pain and corneal clouding. She was found to have absent red reflex, hypotonia, cerebral hypoplasia, and iris bombe on ultrasound biomicroscopy, a feature not previously reported in this syndrome. She responded favorably to surgical management. Iris bombe can be a cause of glaucoma in muscle-eye-brain disease. This highlights the importance of incorporating ultrasound biomicroscopy into the diagnostic algorithm of muscle-eye-brain disease and other types of congenital syndromic glaucoma. [J Pediatr Ophthalmol Strabismus. 2023;60(4):e35-e37.].

Qualitative ultrasound biomicroscopy in glaucoma.

Background: UBM is a high-resolution ultrasound technique which allows non-invasive, in vivo imaging of the anterior segment and iridocorneal angle. Purpose: This video is compilation of short video clips and images which gives description on identification of angle closure due to pupillary block, peripheral anterior synechiae, iris bombe, plateau iris, supra ciliary effusion, and malignant glaucoma. It also shows video demonstrating partially and fully patent iridotomy and features of trabeculectomy bleb. Synopsis: This video summarizes importance of UBM application in angle closure glaucoma to understand its pathophysiology by showing the relationship between the peripheral iris, trabecular meshwork and ciliary processes. Highlights: UBM provides two-dimensional, grayscale images of the angle structures and allows identification of non-pupillary block mechanism in angle closure glaucoma, which can be recorded for qualitative and quantitative analyses. Video Link: https://youtu.be/prsmGnR8jYc.

Iris color as a predictive factor for intraoperative floppy iris syndrome.

PURPOSE: To assess the influence of iris color on the predisposition for intraoperative floppy iris syndrome (IFIS) during cataract surgery. METHODS: Medical records of patients who underwent cataract surgery in two medical centers between July 2019 and February 2020 were reviewed. Patients younger than 50 years, with preexisting ocular conditions affecting pupillary size or anterior chamber depth (ACD), and combined procedures were excluded. The remaining patients were questioned via telephone regarding their iris color. The association of IFIS occurrence and severity with iris color was tested using univariant and multivariant analyses. RESULTS: Overall, 155 eyes of 155 patients were included, 74 with documented IFIS and 81 without. The mean age was 74.03 ± 7.09 years, and 35.5% were female. The most common iris color among study eyes was brown (110/155, 70.97%), followed by blue (25/155, 16.13%) and green (20/155, 12.90%). Compared to brown-colored eyes, blue irises exhibited a 4.50-fold risk for IFIS (OR = 4.50, 95% CI: 1.73-11.70, p = 0.002), and green irises 7.00-fold risk (OR = 7.00, 95% CI: 2.19-22.39, p = 0.001). After adjusting for possible confounders, the results remained statistically significant (p < 0.01). Light-colored irises tended to exhibit a more severe IFIS compared to the brown iris group (p < 0.001). IFIS bilaterality was also affected by iris color (p < 0.001), with a 10.43-fold risk for fellow eye IFIS in the green iris group compared to eyes with brown irises (OR = 10.43, 95% CI: 3.35-32.54, p < 0.001). CONCLUSIONS: Light iris color was associated with a significantly increased risk of IFIS occurrence, severity, and bilaterality on univariate and multivariate analysis in this study.

A novel transconjunctival, intrascleral, ab-externo, knotless, and double-flanged technique with 6-0 Prolene for iridodialysis repair.

Small traumatic iridodialysis (ID) may be asymptomatic, but large ones usually cause polycoria and corectopia, leading to symptoms like diplopia, glare, and photophobia. The management of ID, including medical and surgical methods, depends upon the patient's symptoms. Mild glare and diplopia can be treated either with atropine, antiglaucoma medications, tinted spectacles, colored contact lens, or corneal tattooing, but extensive IDs require surgical options. The surgical techniques are challenging due to the iris texture and the damage encountered during the primary surgery, the narrow anatomical workspace for repair, and the associated surgical complications. Numerous techniques have been described by several authors in the literature; each has its advantages and disadvantages. All the procedures described previously involve conjunctival peritomy, scleral incisions, and suture knots and are time consuming. Here, we report a novel transconjunctival, intrascleral, knotless, and ab-externo, double-flanged technique for repair of large ID with a 1-year follow-up.

Phaco-goniosynechialysis versus phaco-trabeculectomy in patients with refractory primary angle-closure glaucoma: a comparative study.

PURPOSE: To compare the effects of phacoemulsification with intraocular lens implantation (phaco) combined with goniosynechialysis (phaco + GSL) versus phaco with trabeculectomy (phaco + trab) for the management of primary angle-closure glaucoma (PACG) refractory to peripheral anterior synechiae (PAS) of over 180°. METHODS: This retrospective study followed 77 eyes of 77 patients for at least 6 months. Intraocular pressure (IOP), best-corrected visual acuity (BCVA), number of glaucoma drugs, and PAS were recorded at the preoperative baseline and evaluated at each postoperative follow-up visit. The National Eye Institute Visual Functioning Questionnaire-25 (NEI VFQ-25) was administered to patients enrolled in this study. Pearson's correlation analysis and multivariate linear analysis were performed to identify factors influencing changes in NEI VFQ-25 scores and to identify factors associated with increases in NEI VFQ-25 scores after the operation. RESULTS: In total, seventy-seven eyes were included (43 with phaco + GSL and 34 with phaco + trab). Comparing preoperative baseline and month 6 after surgery measurements revealed that both groups found significant improvements in IOP, PAS, BCVA and the number of glaucoma drugs (P < 0.05). Baseline NEI VFQ-25 scores were similar in the two groups, but there was a significant difference in postoperative NEI VFQ-25 scores (74.47 ± 10.39 in phaco + GSL vs. 69.57 ± 8.54 in phaco + trab, P = 0.048 < 0.05), and the phaco + GSL group had better scores at the time of the last follow-up. The change in preoperative scores and the number of glaucoma drugs was significantly correlated with postoperative scores in the phaco + GSL group. CONCLUSION: Phaco + GSL treatment is as safe and effective as phaco + trab for refractory PACG patients, and patients' subjective experience improved significantly after phaco + GSL surgery.

[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.