[Spontaneous rupture of the anterior lens capsule in Alport syndrome (case study)]. / Samoproizvol'noe vskrytie perednei kapsuly khrustalika pri sindrome Al'porta (klinicheskoe nablyudenie).

Alport syndrome is a hereditary disease characterized by glomerulopathy, manifested by hematuria and/or proteinuria, progressive decline in renal function, often combined with hearing and vision pathology. This article presents a clinical case of spontaneous opening of the anterior lens capsule in a patient with Alport syndrome, accompanied by uveitis and ophthalmic hypertension, and describes the features of the surgical aid and the postoperative period.

Postoperative hyphema due to persistent tunica vasculosa lentis in glaucoma associated with neurofibromatosis-a case report.

An 8-month-old girl referred from her pediatrician with a diagnosis of neurofibromatosis type 1 (NF1) presented with an enlarged cloudy cornea of the left eye and a swollen left side of the face. Her left eye had intraocular pressure (IOP) of 21 mm Hg, corneal diameter of 16 mm, ectropion uvea, cup:disk ratio of 0.9, axial length of 28.06 mm, and S-shaped upper lid deformity. Uneventful combined trabeculotomy-trabeculectomy with mitomycin C was performed. On postoperative day 1, there was a new total hyphema that persisted for 2 weeks. An anterior chamber washout was performed, revealing the source of bleeding to be a persistent tunica vasculosa lentis along the zonules of the lens. Viscotamponade was performed, and the corneal wounds were closed, with the ocular tension slightly elevated. Bleeding did not recur for the following 5 months, and IOP was controlled until final follow-up.

A potential role for inflammatory cytokines in a rare late-onset capsular block syndrome: a case report.

BACKGROUND: Late-onset capsule block syndrome (CBS) is a rare complication of cataract phacoemulsification and the implantation of a posterior chamber intraocular lens (PCIOL), which manifests six months to years after surgery. The hallmark of CBS is the formation of an opaque liquid substance between the implanted intraocular lens (IOL) and the posterior capsule. However, its pathogenesis remains unclear. CASE PRESENTATION: A 64-year-old female patient with chronic angle-closure glaucoma (axis length < 21 mm) underwent trabeculectomy surgery combined with phacoemulsification and PCIOL. After a 4-year follow-up, a decline in visual acuity occurred in her right eye due to the location of opaque fluid in the visual axis and distension of the capsular bag. The initial course of action was to release the trapped fluid. Neodymium: yttrium-aluminum-garnet (Nd: YAG) laser capsulotomy could not be employed due to her non-dilating pupil and high extension of the posterior capsule. Subsequently, anterior capsule peeling and anterior segment vitrectomy surgery were performed. The depth of the anterior chamber (ACD), the distance between the face of the retro-IOL and the posterior capsule, the best-corrected visual acuity (BCVA), and the visual quality (VQ) were measured both before and after surgery. Inflammatory cytokine levels in the opaque substances (OS) trapped between the PCIOL and the posterior capsule were assessed using a flow cytometer and compared to normal statistical data in aqueous humor. After surgery, the patient experienced a significant improvement in BCVA and VQ. The distance between the face of the retro-IOL and the posterior capsule was on the verge of disappearing. However, ACD did not differ between pre- and post-operatively. Interleukin-8 (IL-8) and basic fibroblast growth factor (BFGF) concentrations were higher in the OS than in aqueous humor, especially in the former. However, the concentration of vascular cell adhesion molecule (VCAM) in the OS was lower than in aqueous humor. CONCLUSIONS: Anterior segment vitrectomy surgery proved to be a successful treatment for late-onset CBS, presenting a challenging case. In the human lens, inflammatory cytokines originating from the opaque substances may contribute to abnormal metabolism in the sealed area, a consequence of late-onset CBS.

Using Natural Language Processing to Identify Different Lens Pathology in Electronic Health Records.

PURPOSE: Nearly all published ophthalmology-related Big Data studies rely exclusively on International Classification of Diseases (ICD) billing codes to identify patients with particular ocular conditions. However, inaccurate or nonspecific codes may be used. We assessed whether natural language processing (NLP), as an alternative approach, could more accurately identify lens pathology. DESIGN: Database study comparing the accuracy of NLP versus ICD billing codes to properly identify lens pathology. METHODS: We developed an NLP algorithm capable of searching free-text lens exam data in the electronic health record (EHR) to identify the type(s) of cataract present, cataract density, presence of intraocular lenses, and other lens pathology. We applied our algorithm to 17.5 million lens exam records in the Sight Outcomes Research Collaborative (SOURCE) repository. We selected 4314 unique lens-exam entries and asked 11 clinicians to assess whether all pathology present in the entries had been correctly identified in the NLP algorithm output. The algorithm's sensitivity at accurately identifying lens pathology was compared with that of the ICD codes. RESULTS: The NLP algorithm correctly identified all lens pathology present in 4104 of the 4314 lens-exam entries (95.1%). For less common lens pathology, algorithm findings were corroborated by reviewing clinicians for 100% of mentions of pseudoexfoliation material and 99.7% for phimosis, subluxation, and synechia. Sensitivity at identifying lens pathology was better for NLP (0.98 [0.96-0.99] than for billing codes (0.49 [0.46-0.53]). CONCLUSIONS: Our NLP algorithm identifies and classifies lens abnormalities routinely documented by eye-care professionals with high accuracy. Such algorithms will help researchers to properly identify and classify ocular pathology, broadening the scope of feasible research using real-world data.

A Case of Uveitis-Glaucoma-Hyphema Syndrome Related to a Hydrus Microstent.

Uveitis-Glaucoma-Hyphema (UGH) syndrome is characterized by episodes of anterior chamber inflammation, elevated intraocular pressure and hyphema. It is caused by a mechanical chafing of the iris or ciliary body typically by a malpositioned intraocular lens. We present a rare case of UGH syndrome related to the insertion of a Hydrus Microstent. Because of the increased number of microinvasive glaucoma surgeries being performed and a reduction of UGH syndrome patients related to the insertion of intraocular lenses, it is an important diagnosis to consider in patients with recurrent postoperative inflammation.

Pigment deposits in the retrolental space of Berger: a rare feature of pigment dispersion syndrome? / Depósitos de pigmento no espaço de Berger: uma característica rara de síndrome de dispersão pigmentar?

ABSTRACT Pigment dispersion syndrome is associated with clinical features such as Krukenberg's spindles, trabecular pigmentation, Scheie's stripe and Zentmayer's ring. Another less common feature of this syndrome is retrolental pigment deposits due to anterior hyaloid detachment or a defect in the Wieger's ligament. We present two cases of pigment deposits on the posterior lens capsule. In both cases, there is bilateral dispersion of pigment throughout the anterior segment. The retrolental deposits are unilateral in the first case and bilateral in the second. Both patients report a history of ocular trauma. This is a possible important clinical sign of pigment dispersion syndrome, rarely described.

RESUMO A síndrome de dispersão pigmentar associa-se a sinais clínicos característicos como fuso de Krukenberg, hiperpigmentação da malha trabecular, linha de Scheie e anel de Zentmeyer. Um sinal menos comum dessa síndrome é o depósito de pigmento posterior ao cristalino, que ocorre por um descolamento da hialoide anterior ou um defeito no ligamento de Wieger. Apresentamos dois casos de depósitos de pigmento posterior à cápsula posterior do cristalino. Em ambos os casos, existia dispersão bilateral de pigmento por todo o segmento anterior. No primeiro caso, os depósitos eram unilaterais e, no segundo, estavam presentes em ambos os olhos. Este pode corresponder a um sinal potencialmente importante da síndrome de dispersão pigmentar, raramente descrito.

Risk factors for posterior capsule rupture in mature cataract surgery: A study of 1302 cases.

Purpose: To identify the risk factors predisposing posterior capsule rupture (PCR) during mature cataract surgery. Methods: A total of 1302 consecutive mature cataract cases were included in this retrospective study. A detailed examination was performed for each patient and risk factors including age, gender, systemic diseases, ocular comorbidities, surgeon, and surgery method were recorded. Cases with PCR during surgery were classified as complicated. Multivariate logistic regression analysis with a generalized estimating equations method was applied for statistical analysis. Results: The overall rate of PCR was 7.30% (n=95 eyes). After adjusting for confounders, factors that remained significant on multivariate analysis were strabismus (odds ratio [OR]: 5.70, 95% confidence interval [CI]: 2.17-14.97; P < 0.001), phacodonesis (OR: 4.62, 95% CI: 2.59-8.22; P < 0.001), history of trauma (OR: 4.46, 95% CI: 1.64-12.12; P = 0.003), surgery method (extracapsular cataract extraction/phacoemulsification) (OR: 2.61, 95% CI: 1.60-4.26; P < 0.001), and pseudoexfoliation (OR: 1.94, 95% CI: 1.20-3.16; P = 0. 007). Conclusion: Strabismus, phacodonesis, history of trauma, extracapsular cataract extraction method, and pseudoexfoliation were found to be important risk factors for developing PCR. Appropriate preoperative and perioperative precautions for these higher-risk cases can reduce complications.

When should cataract surgeons seek assistance from experienced colleagues?

PURPOSE: To assess which cases should be assorted exclusively to high-volume surgeons and identify when should a cataract surgeon seek assistance from a senior colleague. METHODS: The medical records of 2853 patients with age-related cataract were reviewed. Preoperative risk factors were documented for each case, and they were divided into surgeons who had more (> 400 surgeries/year) or less experience (< 400 surgeries/year). Ophthalmology residents were excluded from this review. The cases that involved posterior capsule rupture, dropped nucleus, zonular dehiscence and anterior capsular tear with or without vitreous loss were defined as "complicated". RESULTS: From the 3247 cataract extraction surgeries that were reviewed, we were unable to identify any statistically significant difference in the complication rates between the two surgeon groups. In the stepwise regression analysis, both groups supported advanced age (> 85) and mature cataracts with up to fourfold odds ratios (OR). Low-volume surgeons had a fivefold OR in the presence of phacodonesis and a fourfold OR in the case of posterior polar cataract. Finally, the low- and high-volume groups had their highest complication rates in the cumulative four and five risk factors, respectively. CONCLUSION: In the presence of advanced age, mature cataracts, phacodonesis and posterior polar cataract, the complication rates appear to be higher for the less experienced surgeons. Meticulous preoperative assessment with detailed documentation of each patient's risk factors can result in fewer complications. The medical complexity of each case can be used as indicator of whether a more experienced surgeon should perform the surgery or not.

Current Concepts of the Uveitis-Glaucoma-Hyphema (UGH) Syndrome.

PURPOSE: To discuss the pathophysiology, etiology, and current management strategies of uveitis-glaucoma-hyphema (UGH) syndrome. METHODS: Literature review. RESULTS: The classic UGH syndrome associated with anterior chamber intraocular lenses (ACIOL) have decreased in incidence with the modernization of IOL design and surgical techniques. The current UGH syndrome is increasing in prevalence largely related to a parallel increase in late onset dislocations of intraocular lenses (IOLs) and the developing techniques to remedy that condition. The modern features of UGH can present as cystoid macular edema, intraocular pressure elevation typically not attributed to UGH, and recurrent vitreous hemorrhage, unlike the original description as described by Ellingson in 1978. Medical management to control inflammation, reduce intraocular pressure, and reduced the bleeding diathesis are mainstays of therapy. However, surgery with IOL repositioning or exchange should be reserved for cases that are refractory to or progressing despite medical treatment. CONCLUSIONS: UGH syndrome is an increasingly common, poorly understood, and often subtle, manifestation of an anatomic disturbance post intraocular surgery that persists with continued evolution of intraocular surgical techniques and new imaging modalities to aid in its diagnosis.

Endoscopic Visualization for Atypical Uveitis Glaucoma Hyphema Syndrome Management.

We discuss how ophthalmic endoscopy was used in the management of 6 cases with atypical uveitis glaucoma hyphema syndrome. For case 1, the endoscope was used to remove a retained haptic foreign body after an intraocular lens (IOL) exchange with an iris-sutured IOL for a complete capsular bag-IOL complex dislocation. In case 2, the endoscope was key in identifying the presence and location of vascular lesions at the site of previous pars plana sclerotomies. In case 3, the endoscope enabled visualization of a large segmental Soemmering's Ring pushing a 3-piece IOL haptic into the posterior iris. For case 4, the endoscope allowed viewing of the sharp edge of the optic where the haptic of a one-piece lens had been amputated, and the sharp edge of the cut optic was anteriorly oriented and continuing to rub the posterior iris. In case 5, the endoscope confirmed the presence of 1 haptic of a 1-piece lens out of the capsular bag and in the sulcus space. Also, it showed that the capsular bag had inadequate zonular support to attempt repositioning the haptic into the bag. In case 6, the endoscope was helpful in identifying a 1-piece plate haptic IOL in the sulcus, with synechiae and anterior location causing iris bulging inferiorly.

Síndrome de disfunción del cristalino y su intercambio refractivo / Lens Dysfunction Syndrome and its Refractive Exchange

La capacidad de ver bien es una preocupación para todos, especialmente a medida que se envejece. Los cambios en los ojos con el tiempo son inevitables, pero existen opciones de tratamiento para ayudar a tratar los problemas que pueden aparecer en la visión. El síndrome de disfunción del cristalino se caracteriza por englobar algunas de las alteraciones visuales más comunes relacionadas con la edad y que suelen aparecer a partir de los 40 años. La principal causa del síndrome de disfunción del cristalino es el envejecimiento natural de esta estructura ocular. Los síntomas más comunes son visión borrosa, disminución de la sensibilidad a los colores o dificultad y cambios en la visión cercana. El término es poco usado en Cuba por lo que el objetivo fue realizar una revisión sobre el síndrome de disfunción del cristalino y su intercambio refractivo. Existen diferentes opciones de tratamiento, desde gafas hasta el recambio refractivo del cristalino por una lente intraocular, que se usa cada vez a más temprana edad y que gracias al adelanto tecnológico y aumento de la pericia del médico, constituye un tratamiento eficaz en pacientes pregeriátricos(AU)

The ability to see well is a concern for everyone, especially as one ages. Changes in the eyes over time are inevitable, but there are treatment options to help address the problems that can occur in vision. The lens dysfunction syndrome is characterized as encompassing some of the most common age-related visual disorders that usually appear after the age of 40. The main cause of lens dysfunction syndrome is the natural aging of this ocular structure. The most common symptoms are blurred vision, decreased sensitivity to colors or difficulty and changes in near vision. The term is hardly ever used in Cuba, so the objective was to review the crystalline lens dysfunction syndrome and its refractive exchange. There are different treatment options, from glasses to the refractive exchange of the crystalline lens for an intraocular lens, which is used at an increasingly younger age and which, thanks to technological advances and increased medical expertise, is an effective treatment in pregeriatric patients(AU)

Uveitis-Glaucoma-Hyphema Syndrome: Clinical Features and Differential Diagnosis.

PURPOSE: to study the clinical features of uveitis-glaucoma-hyphema (UGH) syndrome, particularly those useful for a differential diagnosis from unilateral hypertensive acute anterior uveitis. METHODS: A retrospective chart review was conducted on the clinical features of 9 patients with UGH syndrome. These features were then compared with those detected in 50 patients with unilateral hypertensive acute anterior uveitis. RESULTS: Fine and pigmented keratic precipitates (p = .0002 and p = .00004, respectively), iris atrophy (p = .0122), hyphema and vitreous opacities > 2+ (p = .0003), and cystoid macular edema (p = .009) were statistically associated with UGH syndrome. These clinical signs show a high specificity, ranging from 58 to 100%; the presence of pigmented keratic precipitates in the setting of a unilateral acute hypertensive anterior uveitis has a sensitivity and specificity of 89% and 84%, respectively. CONCLUSION: In patients operated on for cataract, UGH syndrome can be differentiated from unilateral hypertensive acute anterior uveitis considering specific clinical signs.

OCULAR MANIFESTATIONS OF ASP38ALA AND THR59LYS FAMILIAL TRANSTHYRETIN AMYLOIDOSIS.

PURPOSE: To describe the ophthalmic manifestations of familial transthyretin amyloidosis (FTA) mutations, including Asp38Ala and Thr59Lys, which have not been previously reported to have ocular involvement. METHODS: This is an observational case series of prospectively collected data of 16 patients with FTA who were taking tafamidis for mild peripheral neuropathy and underwent a comprehensive ophthalmic examination at a single tertiary center, between January 2013 and March 2020. The ocular involvement of each FTA mutation type and the specific manifestations were the main outcome measures. RESULTS: Six of 16 patients with FTA manifested ocular involvement. Ocular involvement was noted in two of three patients with Glu89Lys mutations having retinal deposits, retinal hemorrhages, and corneal opacity. Three of nine patients with Asp38Ala mutations and one of two patients with Thr59Lys mutations showed ocular involvement that had not been previously described. The ophthalmic findings included glaucoma, anterior lens capsule opacity, vitreous opacity, and retinal deposits. The decrease in vascular flow due to perivascular cuffing of the amyloid deposits was detected by optical coherence tomography angiography. CONCLUSION: The current study newly described that two transthyretin mutation types of FTA, Asp38Ala and Thr59Lys, may manifest with ocular findings such as anterior lens capsule opacity and retinal deposits.

The role of anterior segment optical coherence tomography in uveitis-glaucoma-hyphema syndrome.

PURPOSE: To describe features of uveitis-glaucoma-hyphema (UGH) syndrome, using Anterior Segment-Optical Coherence Tomography (AS-OCT) and Ultrasound Biomicroscopy (UBM) and to evaluate the diagnostic role of AS-OCT as an imaging technique alternative to UBM. DESIGN: Retrospective case series. METHODS: Four eyes of 4 patients with UGH syndrome were analyzed. All patients reported previous uncomplicated cataract surgery with in-the-bag implantation of single-piece-intraocular lens (IOL). They underwent at presentation complete ophthalmological examination and imaging with slit-lamp anterior segment photographs, UBM and AS-OCT. RESULTS: Although AS-OCT did not allow to visualize the structures behind the iris, it displayed a contact between IOL (plate and/or haptics) and iris and IOL tilting in 3 out of 4 eyes. AS-OCT directly detected the cause of UGH syndrome in one eye, 2 eyes required some expedients to display the iris chafing, like scans in mydriasis and/or patient's gaze direction change. AS-OCT did not allow to appreciate the IOL-iris contact (showed by UBM technique) only in one eye, probably due to the change of patient position from supine to sitting, and consequent anteriorization of iris diaphragm. Furthermore AS-OCT showed fine details, as capsular bag collapse and indirect signs of haptic malposition in 3 out of 4 eyes. CONCLUSION: AS-OCT is a non-invasive technique that allows to determine IOL position and IOL-uveal contact in selected cases of UGH syndrome. Considering AS-OCT and UBM advantages and limitations, AS-OCT should be used as first imaging modality when clinical diagnosis is uncertain. When UGH diagnosis cannot be verified using AS-OCT, UBM should be performed.