[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.

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.

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.

Intra-lenticular caterpillar seta in ophthalmia nodosa.

PURPOSE: To report the clinical course of ophthalmia nodosa with a retained lenticular seta misdiagnosed and treated as non-infectious posterior uveitis for 7 consecutive years. METHODS: Meticulous clinical examination led to discovery of the caterpillar seta embedded in the crystalline lens and the intravitreal setae. RESULTS: Lens-sparing pars plana vitrectomy and removal of free-floating vitreal seta resulted in complete resolution of vitritis and uveitis. CONCLUSION: Embedded seta within a clear lens may remain sequestered, and may be left untouched under close observation, precluding a clear lens extraction in such patients.

Differential optical density of fluid in capsular bag distension syndrome.

Capsular bag distension syndrome (CBDS) is a rare complication of cataract surgery due to collection of fluid behind the intraocular lens (IOL). Nd:Yag laser capsulotomy is commonly performed to release the fluid collection; however, the potential risk of infection and inflammation has led to adaptation of numerous surgical procedures. Clinical examination in an established case of CBDS reveals opalescent fluid behind the IOL with ballooning of the posterior capsule, which has different reflectivity on optical coherence tomography. However, the exact optical properties of this fluid are still unknown. A case of delayed CBDS with induced myopia due to change in IOL position because of fluid collection behind the IOL is presented.

Lenticular fungal infection caused by Aspergillus in a patient with traumatic corneal laceration: a case report.

BACKGROUND: To report a case of lenticular infection caused by Aspergillus, which was diagnosed 13 weeks after traumatic corneal laceration. CASE PRESENTATION: A 60-year-old woman presented with traumatic corneal laceration including anterior lens capsule rupture and traumatic cataract after being hit with a chestnut in the right eye. There were multiple injuries due to tiny thorns of the chestnut, including the conjunctiva, sclera, cornea, and anterior lens capsule. But no visible foreign body was detected by slit-lamp examination. Topical corticosteroid was prescribed to resolve the conjunctival inflammation induced by the thorns of chestnut, which could have caused persistent irritation. As conjunctival injection and edema being decreased during outpatient clinical follow-up, embedded conjunctival foreign body was detected and surgically removed (1st surgery). Approximately 10 weeks after the trauma, severe inflammation of the anterior segment accompanied with hypopyon developed suddenly and at the same time embedded scleral foreign body was revealed. After removal of scleral foreign body (2nd surgery), unspecified mold species was cultured from the scleral foreign body in SDA (Sabouraud dextrose agar) plate. Suspicious corneal foreign body was removed as 3rd surgery and phacoemulsification of traumatic cataract was planned as 4th surgery. Aspergillus was finally detected from removed anterior capsule and fibrotic membrane during the operation. Fungal infection resolved successfully after administration of topical (1% voriconazole and 5% natamycin) and systemic (fluconazole) antifungal agents and phacoemulsification of traumatic cataract. CONCLUSION: Chestnut thorns can damage multiple ocular tissues simultaneously. Lens capsular rupture could result in fungal inoculation and lead to delayed lenticular fungal infection with complicated cataract formation. In cases of ocular trauma due to organic substances such as thorns and branches, the possibility of fungal infection should be considered.

Characterization of Unique Lens Morphology in a Cohort of Children with Familial Exudative Vitreoretinopathy.

Purpose: To characterize the lens morphology and to measure the clinical features of familial exudative vitreoretinopathy (FEVR) in children. Methods: Unique lens changes were observed in a cohort of children with FEVR from March 2015 to November 2017 using slit lamp examination and all the patients underwent cycloplegic refraction, ultrasound A and B, keratometry and fundus fluorescein angiography. Results: Twelve eyes of eight children with FEVR had unique lens changes. The contraction of the posterior capsule caused unique lens changes resulting in myopia in nine eyes of six children and astigmatism in eight eyes of five children. Retinal lesions in the affected eyes were all stage 1 to 2. Six eyes of three patients underwent lensectomy and intraocular lens implantation due to high anisometropia which could not be corrected by conventional optical correction. During lensectomy, the opacification in the posterior capsule was found to be due to the fibrous membrane that protruded into the anterior vitreous and not due to lens opacification. Three patients had bilateral lensectomy, in two of whom significant macular involvement was observed in one eye and in one of whom significant macular involvement was observed in both eyes. After surgery visual acuity (VA) improved obviously in two eyes without significant macular involvement and did not improve in the four eyes which had significant macular involvement. Among the five patients who did not have lensectomy, one patient was lost to follow-up and one patient had VA improved in both eyes without significant macular involvement. The other three patients did not have much change in VA. Conclusions: Clinicians should be aware that when a high myopia or astigmatism does not match the corneal curvature and the length of the eye, one should check carefully the changes of lens and fundus after dilating the pupil, to avoid misdiagnosis and missed diagnosis.

Assessment of Corneal and Lens Density in Children With Celiac Disease.

PURPOSE: To assess the early changes of corneal and lens density in a pediatric population with celiac disease. METHODS: One hundred one patients were included in this observational and prospective study. Patients with celiac disease formed the celiac disease group. Healthy individuals with no medical history formed the control group. Corneal and lens density were assessed with Pentacam HR (Oculus Optikgeräte GmbH, Wetzlar, Germany). RESULTS: The mean lens and corneal density outcomes in all zones did not differ between groups (P > .05 for each). Maximum lens density outcome was significantly higher in the celiac disease group than in the control group (P = .028). The mean corneal density at the peripheral cornea was significantly higher in females than males in the celiac disease group (P < .05 for each). Compliance with a gluten-free diet, body mass index, and histological classification of celiac disease had no significant effect on lens and corneal density in patients with celiac disease (P > .05 for each). CONCLUSIONS: Celiac disease did not affect the mean lens and corneal density in this pediatric population, but higher maximum lens density in patients with celiac disease and higher peripheral corneal density in female patients with celiac disease may indicate early stages of ocular involvement of celiac disease. [J Pediatr Ophthalmol Strabismus. 2019;56(6):402-406.].