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Rationale: Multisystemic smooth muscle dysfunction syndrome (MSMDS) is a genetic disease that affects multiple organs. The report here concerns a patient with MSMDS, who is known so far as the youngest among all the reported patients. In addition to the typical manifestations, we observed previously unreported ocular abnormalities, including persistent anterior tunica vasculosa lentis (TVL) and early-onset retinal arteriolar tortuosity, by the fluorescein angiography (FA)., Patient Concerns: The patient was admitted to the neonatal intensive care unit immediately after birth for a diagnosis of urinary system dysplasia during fetal life. After a thorough examination, the patient was found with patent ductus arteriosus, pulmonary hypertension, cerebrovascular disease, hypotonic bladder, intestinal malrotation, and congenital mydriasis. The FA of the eyes undertaken in her 6-week demonstrated perfused vasculature in the persistent anterior TVL and prominent retinal arteriolar tortuosity. The whole exome sequencing revealed a de novo heterozygous ACTA2 gene missense mutation p.R179H., Diagnoses: The patient was diagnosed with MSMDS., Interventions: Follow-up observation., Outcomes: At the 3-month follow-up, no change of the ocular disease was observed., Lessons: The persistent anterior TVL in this case implies that ACTA2 p.R179H mutation affects not only the smooth muscle cells but also the pericytes, and further affects the TVL regression. The prominent retinal arteriolar tortuosity in this 6-week-old infant indicates that the retinal arteriolar tortuosity can present early in MSMDS., Competing Interests: The authors have no conflicts of interests to disclose., (Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc.)

A postcataract surgery complication in patients with retinitis pigmentosa (RP) is lens capsular contraction. To identify potential proteins contributing to this phenomenon, high-performance liquid chromatography/mass spectrometry-based proteomic analysis was conducted with aqueous humor samples collected from 11 patients who underwent cataract surgeries, with four patients diagnosed as RP and cataract (RP group) and the other seven with only senile cataract group. The upregulated proteins in the RP group were enriched in wound response, while downregulated proteins were enriched in cell adhesion and lens crystallins. Receptors of two dramatically upregulated proteins tenascin-C (TNC) and serotransferrin were found expressed in human lens epithelial cells (HLEs). TNC can promote primary HLEs proliferation and cell line HLE-B3 migration. This study indicates aqueous humor proteomic analysis serves as an effective way to unveil the pathogenesis of RP complications. TNC is a potential target of stimulating HLEs proliferation in RP concomitant cataract patients that worth further research., (© 2020 Wiley Periodicals LLC.)

Background : To first report and study the ultrastructural and immunofluorescence abnormalities of the lens anterior capsules in a patient with autosomal recessive Alport syndrome. Methods : Two anterior lens capsules were collected in femtosecond laser-assisted cataract surgeries from a 29-year-old male patient with bilateral lenticonus caused by autosomal recessive Alport syndrome. The left capsule was examined by transmission electron microscopy and the right capsule was serial sectioned and stained with antibodies against the α2, α3, and α4 chains of type Ⅳ collagen. Anterior lens capsules of another two uncomplicated age-related cataract patients were collected and treated in the same way as the control. Results : The novel findings are that the mitochondria in lens epithelial cells in autosomal recessive Alport syndrome patients increased, twisted, and exhibited high electron density. Characteristic ultrastructure changes of capsule thinning, vertical dehiscence, and irregular-shaped lens epithelial cells were also observed in the left anterior lens capsule. Normal reactivity against the α2 chain and decreased reactivity against the α3 and α4 chains were observed in the right anterior lens capsule. Conclusions : The homozygous c.4599 T > G mutation of COL4A4 not only affects the formation of type Ⅳ collagen networks in the extracellular matrix, but also affects the morphology and survival of the lens epithelial cells in the patient with autosomal recessive Alport syndrome. This study is the first report of the ultrastructural and immunofluorescence changes of anterior lens capsules in autosomal recessive Alport syndrome.

Purpose: N-methyl-N-nitrosourea (MNU) is an alkylating toxicant with potent mutagenic ability. This study was designed to induce apoptosis in lens epithelial cells (LECs) and corneal endothelial cells (CECs) via MNU administration. We sought to build ocular disease models of cataract and corneal endothelial decompensation., Methods: MNU was delivered into the intraperitoneal cavities of neonatal rats and the anterior chambers of adult rabbits. The MNU-treated animals were then subjected to a series of functional and morphological analyses at various time points., Results: MNU treatment induced pervasive apoptosis of LECs and CECs. These effects were dose and time dependent. Mature cataracts were found in neonatal rats 3 weeks after MNU treatment. Histological analysis revealed that MNU toxicity induced swelling, vacuolation, and liquefaction in lens fibers of MNU-treated rats. Pentacam examination showed that the average density of rat lens increased significantly after MNU administration. Terminal deoxynucleotidyl transferase-mediated nick end labeling (TUNEL) analysis showed pervasive apoptotic staining in the lenses of MNU-treated rats. In rabbit eyes, intracameral treatment with MNU induced corneal edema and significantly increased central corneal thickness, which peaked at P14. Morphological and immunohistochemical analysis showed that CECs were effectively ablated in the MNU-treated rabbits. The expression of 8-OHdG increased significantly in the cornea of MNU-treated rabbits, compared with vehicle-treated controls., Conclusions: MNU is sufficient to induce ocular cell apoptosis in animal models. These models of MNU-induced cataract and corneal endothelial decompensation represent valuable tools for efforts to develop relevant therapies.

During an ENU (N-ethyl-N-nitrosourea) mutagenesis screen, we observed a dominant small-eye mutant mouse with viable homozygotes. A corresponding mutant line was established and referred to as Aey69 (abnormality of the eye #69). Comprehensive phenotyping of the homozygous Aey69 mutants in the German Mouse Clinic revealed only a subset of statistically significant alterations between wild types and homozygous mutants. The mutation causes microphthalmia without a lens but with retinal hyperproliferation. Linkage was demonstrated to mouse chromosome 3 between the markers D3Mit188 and D3Mit11. Sequencing revealed a 358 A-> C mutation (Ile120Leu) in the Hist2h3c1 gene and a 71 T-> C (Val24Ala) mutation in the Gja8 gene. Detailed analysis of eye development in the homozygous mutant mice documented a perturbed lens development starting from the lens vesicle stage including decreasing expression of crystallins as well as of lens-specific transcription factors like PITX3 and FOXE3. In contrast, we observed an early expression of retinal progenitor cells characterized by several markers including BRN3 (retinal ganglion cells) and OTX2 (cone photoreceptors). The changes in the retina at the early embryonic stages of E11.5-E15.5 happen in parallel with apoptotic processes in the lens at the respective stages. The excessive retinal hyperproliferation is characterized by an increased level of Ki67. The hyperproliferation, however, does not disrupt the differentiation and appearance of the principal retinal cell types at postnatal stages, even if the overgrowing retina covers finally the entire bulbus of the eye. Morpholino-mediated knock-down of the hist2h3ca1 gene in zebrafish leads to a specific perturbation of lens development. When injected into zebrafish zygotes, only the mutant mouse mRNA leads to severe malformations, ranging from cyclopia to severe microphthalmia. The wild-type Hist2h3c1 mRNA can rescue the morpholino-induced defects corroborating its specific function in lens development. Based upon these data, it is concluded that the ocular function of the Hist2h3c1 gene (encoding a canonical H3.2 variant) is conserved throughout evolution. Moreover, the data highlight also the importance of Hist2h3c1 in the coordinated formation of lens and retina during eye development., (Copyright © 2019 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Intraocular lens (IOL) opacification is a rare phenomenon noted with hydrophilic acrylic IOLs. We report a case of advanced IOL opacification appreciated on anterior segment optical CT (ASOCT)as a shrunken biconcave optic retracted away from the posterior capsule (PC), unlike the other eye which had a clear biconvex IOL of similar material abutting the PC. After IOL exchange, the affected eye was noted to have more folds and Elschnig's pearls on the PC when compared with the other eye. Our case points towards rare IOL changes seen in advanced cases of opacification, their association with posterior capsular changes and the aid of ASOCT as a non-invasive tool in diagnosing them correctly., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

Purpose : The aim of the present study was to demonstrate novel findings recognized within the basement membrane of the anterior lens capsules (ALCs) in exfoliation syndrome, in a transmission electron microscopy (TEM) study. Materials and methods : Twenty-one patients with age-related cataract were included in the study. Eleven out of them suffered from exfoliation syndrome (XFS). Anterior lens capsules were obtained from patients during phacoemulsification, applying continuous curvilinear capsulorhexis and then were examined by transmission electron microscope. Results : Six out of 11 examined basement membranes with XFS had a granular appearance with dotted deposits of electron-dense material. Fibrils were clearly detected, longitudinally, within the basement membrane, usually located in the outer quarter of the basement membrane, towards the anterior chamber. Conclusion : New data about the exfoliation material and its location within the basement membrane of the anterior lens capsules of XFS patients are presented. These findings constitute new evidence for XFS's histopathogenesis and might help clarify the lenticular exfoliation material's (XFM) mechanisms of origin.

Thioredoxin-binding protein-2 (TBP-2) has an important role in the redox system, but it plays a different role in many different diseases (e.g., various cancers, diabetes mellitus (DM), cardiovascular disease, and cataracts) by influencing cell proliferation, differentiation, apoptosis, autophagy, and metabolism. Distinct transcription factors (TFs) stimulated by different factors combine with binding sites or proteins to upregulate or downregulate TBP-2 expression, in order to respond to the change in the internal environment. Most research disclosed that the main function of TBP-2 is associating with thioredoxin (Trx) to inhibit the antioxidant capacity of Trx. Furthermore, the TBP-2 located in tissues, whether normal or abnormal, has the ability to cause the dysfunctioning of cells and even death through different pathways, such as shortening the cell cycle and inducing apoptosis or autophagy. Through these studies, we found that TBP-2 promoted the development of diseases which are involved in inflammatory and oxidative damage. To a certain extent, we believe that there is some hidden connection between the biological functions which TBP-2 participates in and some distinct diseases. This review presents only a summary of the roles that TBP-2 plays in cancer, DM, cataracts, and so on, as well as its universal mechanisms. Further investigations are needed for the cell signaling pathways of the effects caused by TBP-2. A greater understanding of the mechanisms of TBP-2 could produce potential new targets for the treatment of diseases, including cancer and diabetes, cardiovascular disease, and cataracts.

People with the long anterior zonule (LAZ) trait, which may have prevalence near 2%, have zonular fibers that extend more central than usual along the anterior capsule of the crystalline lens. The anomalous fibers can be observed in vivo with clinical slit lamp biomicroscopy after pharmacologic pupil dilation, and although minimally studied, the LAZ trait may have importance to glaucoma, retinal degeneration, and cataract surgery. To further characterize LAZ morphology, a custom computer program was used to trace LAZ fibers seen on retro-illumination photos acquired during previous study at an academic, urban eye care facility in Chicago, IL. There were 59 African-Americans (54 female; median age = 70 years, 53-91 years) included in the analysis. After initial review of the zonule tracings, we identified three basic LAZ patterns. We called one pattern (47% of right eyes) a "non-segmental LAZ pattern," which was predominated by fibers that could be visually traced to the dilated pupil border where they became obscured by the iris. Another pattern (35% of right eyes), the "segmental LAZ pattern," was predominated by fibers that appeared to terminate abruptly without detectable extension to the pupil border. The third pattern (18% of right eyes), the "mixed LAZ pattern," had a more equivalent mixture of the other two fiber morphologies. Compared to the "non-segmental" group, the "segmental" LAZ eyes had smaller central zonule-free zones (P < 0.0001), and they tended to exhibit fewer LAZ fibers (P = 0.07). These data improve understanding of LAZ clinical anatomy and may be helpful to future investigation. Anat Rec, 300:1336-1347, 2017. © 2017 Wiley Periodicals, Inc., (© 2017 Wiley Periodicals, Inc.)

Purpose: The purpose of this study was to determine in which species and under what conditions lens tumors occur., Design: A review of databases of available human and veterinary ocular pathologic material and the previously reported literature., Participants: Approximately 18 000 patients who had ocular surgical specimens submitted and studied at the University of Wisconsin School of Medicine and Public Health between 1920 and 2014 and 45 000 ocular veterinary cases from the Comparative Ocular Pathology Laboratory of Wisconsin between 1983 and 2014., Methods: Material in 2 major archived collections at the University of Wisconsin medical and veterinary schools were studied for occurrence of lens tumors. Tumor was defined as a new growth of tissue characterized by progressive, uncontrolled proliferation of cells. In addition, cases presented at 3 major eye pathologic societies (Verhoeff-Zimmerman Ophthalmic Pathology Society, Eastern Ophthalmic Pathology Society, and The Armed Forces Institute of Pathology Ophthalmic Alumni Society) from 1975 through 2014 were reviewed. Finally, a careful search of the literature was carried out. Approval from the institutional review board to carry out this study was obtained., Main Outcome Measures: The presence of tumors of the lens., Results: The database search and literature review failed to find an example of a lens tumor in humans. In contrast, examples of naturally occurring lens tumors were found in cats, dogs, rabbits, and birds. In the veterinary school database, 4.5% of feline intraocular and adnexal neoplasms (234/5153) were designated as feline ocular posttraumatic sarcoma, a tumor previously demonstrated to be of lens epithelial origin. Similar tumors were seen in rabbit eyes, a bird, and in a dog. All 4 species with lens tumors had a history of either ocular trauma or protracted uveitis. The literature search also revealed cases where lens tumors were induced in zebrafish, rainbow trout, hamsters, and mice by carcinogenic agents (methylcholanthrene, thioacetamide), oncogenic viruses (SV40, HPV-16), and genetic manipulation., Conclusions: Our results suggest that lens tumors do not occur in humans. In contrast, after lens capsule rupture, a lens tumor can occur in other species. We hypothesize that a genetic mechanism exists that prevents lens tumors in humans., (Copyright © 2015 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.)

An adult, female, captive, Matshchie's tree kangaroo was diagnosed with an anterior lens luxation in the right eye and a lens subluxation in the left eye. Both eyes were treated surgically with intracapsular lens extractions. A 360° rhegmatogenous retinal detachment was diagnosed 6 months postoperatively in the left eye. Aphakic vision was maintained in the right eye 9 months postoperatively. Based on family history and the lack of antecedent ocular disease, the lens luxations were presumed to be inherited and veterinarians should be aware of this condition within the captive tree kangaroo population., (© 2014 American College of Veterinary Ophthalmologists.)

A 29-year-old woman presented with symptoms of gradual, progressive, painless diminution of vision in both eyes for past 8-10 years. On examination, uncorrected visual acuity was 20/60 in the right eye and 20/126 in the left eye. Anterior segment examination was essentially normal except for a conical protrusion in the pupillary axis of the anterior lens capsule with a clear underlying lens. During surgery, central protruded fragile lens capsule was meticulously handled to create well-centered continuous curvilinear capsulorhexis by using paediatric rhexis forceps. Histopathology of excised capsule showed markedly thinned lens capsule with normal epithelium. Electron microscopy of the anterior lens capsule showed multiple linear and irregular zones of dehiscence. Few of these had fibrillar, irregular electron-dense material and vacuoles; and adjacent cells were irregular suggestive of Alport syndrome. Postoperative course was uneventful and visual acuity improved to 20/20 unaided. Clear lens extraction is an effective means of rehabilitating visually symptomatic anterior lenticonus of Alport syndrome., (2014 BMJ Publishing Group Ltd.)

This case report describes the clinical manifestations and ocular pathology of an intraocular sarcoma in a spayed female 7-year-old rabbit that developed clinical signs consistent with phacoclastic uveitis at 6 months of age, which was left untreated for most of the next 6 years. Ophthalmologic examination confirmed pupillary occlusion, aqueous flare, fibrin, and lens debris within the anterior chamber of the right eye. The right eye was blind and phthisical. The tentative clinical diagnosis was chronic phacoclastic uveitis, and a routine transconjunctival enucleation of the right eye was completed. The globe was formalin-fixed and submitted for histologic examination. Light microscopic examination revealed a ruptured cataractous lens, uveitis, retinal detachment, and an intraocular sarcoma centered around the lens. Immunohistochemical (IHC) staining was positive for smooth muscle actin and vimentin and negative for Desmin and cytokeratin intermediate filaments., (© 2013 American College of Veterinary Ophthalmologists.)

PURPOSE  To describe histologic anterior segment changes in eyes affected with primary lens displacement (PLD) and secondary glaucoma. METHODS  Histologic sections stained with H&E from canine eyes enucleated because of PLD and secondary glaucoma were examined. RESULTS  Thirteen eyes from 12 patients were evaluated. Four dogs were castrated males and eight spayed females. Median age was 8 years of age (range 3-13). Breeds included seven terriers and five other breeds. All eyes examined demonstrated varying degrees of inflammation involving the iris and cleft. Mononuclear and melanophagic infiltration of the cleft was found in all specimens. Four globes also showed polymorphonuclear infiltrate. Pre-iridal fibrovascular membranes were clearly identified in 10 of 13 eyes. Total inflammatory score was significantly greater in all globes examined compared with an age-matched group of normal dogs. The posterior pigmented iris epithelium demonstrated a consistent pattern of hyperplasia and/or hypertrophy and cystic degeneration, more prominent in the more central regions. In some cases, hyperplasia was of greatest severity in the mid-iris and associated with thinning or flattening of the pupillary region. CONCLUSIONS  These results suggest that lens instability may be associated with chronic inflammation and secondary glaucoma. Mechanical irritation from an unstable lens may result in hypertrophy and/or hyperplasia of the posterior pigmented iris epithelium and subsequent cellular exfoliation and release of melanin. An inflammatory reaction directly or indirectly related to melanin release may obstruct the outflow pathways ultimately leading to glaucoma and loss of vision. Use of topical steroids may be warranted in dogs with PLD., (© 2012 American College of Veterinary Ophthalmologists.)

The author reports the use of pars plana air-fluid exchange in promoting adhesion of the donor endothelial graft to the host cornea during Descemet's membrane stripping with endothelial keratoplasty in the absence of an intact lens-iris diaphragm. A three-port pars plana 25-gauge core vitrectomy is performed, followed by the insertion of the endothelial graft into the anterior chamber with a glide. After the limbal incision and the paracentesis site are sutured, the air-fluid exchange is done from the vitreous cavity toward the anterior chamber until the eye is completely filled with air. The air infusion pressure is maintained at 30 mm Hg for 10 minutes. The endothelial graft is centered with a roller. The pars plana cannulas are removed, and the scleral incisions seal spontaneously. This technique ensures that the anterior chamber is completely filled with air at the end of case and provides fine control of the intraocular pressure throughout the surgery., (Copyright 2012, SLACK Incorporated.)

Background: We have previously shown that the slow Wallerian degeneration mutation, whilst delaying axonal degeneration after optic nerve crush, does not protect retinal ganglion cell (RGC) bodies in adult rats. To test the effects of a combination approach protecting both axons and cell bodies we performed combined optic nerve crush and lens injury, which results in both enhanced RGC survival as well as axon regeneration past the lesion site in wildtype animals., Results: As previously reported we found that the Wld(S) mutation does not protect RGC bodies after optic nerve crush alone. Surprisingly, we found that Wld(S) transgenic rats did not exhibit the enhanced RGC survival response after combined optic nerve crush and lens injury that was observed in wildtype rats. RGC axon regeneration past the optic nerve lesion site was, however, similar in Wld(S) and wildtypes. Furthermore, activation of retinal glia, previously shown to be associated with enhanced RGC survival and axon regeneration after optic nerve crush and lens injury, was unaffected in Wld(S) transgenic rats., Conclusions: RGC axon regeneration is similar between Wld(S) transgenic and wildtype rats, but Wld(S) transgenic rats do not exhibit enhanced RGC survival after combined optic nerve crush and lens injury suggesting that the neuroprotective effects of lens injury on RGC survival may be limited by the Wld(S) protein.

Persistent pupillary membrane is a congenital, incomplete involution of the tunica vasculosa lentis. These membranes are usually tenacious, with fine fibrils attached to the iris collarette on one side, with the other end either free floating or attached to the lens or iris focally on the opposite side. This condition is thought to represent ectopic iris tissue on the lens with abnormal iris stroma caused by aberrant involutional changes in the primitive embryological vascular system of anterior segment. Dense and thick membranes, particularly within the pupil, may result in deprivation amblyopia. We report clinical and histopathological findings in a case of a 12-year-old boy with bilateral extensive hyperplastic persistent pupillary membrane that was surgically removed without injury to the crystalline lens., (Copyright © 2012 American Association for Pediatric Ophthalmology and Strabismus. Published by Mosby, Inc. All rights reserved.)

Millions are rendered blind or exhibit visual impairment due to pathologies of the lens of the eye. Lens research therefore addresses the direct need to gain insights into the cellular and molecular basis of disease, but, moreover, serves as a valuable experimental system to answer fundamental biological questions. This themed issue showcases the scientific knowledge of the processes involved in the development, structure, ultrastructure, physiology and pathology of the lens and how this information has the potential to significantly further knowledge in various fields of research. The issue is divided into three main areas. Firstly, the lens is discussed as a developmental model for embryonic induction, as an elegant system for studying the role of growth factors in development, and for analysis of the molecular control and cellular basis of cellular differentiation. The genetic basis of disorders of lens development, including paediatric cataract (lens opacity), are also discussed in this section. Secondly, adult lens structure and ultrastructure are covered, as well as the lens as a model for homeostasis and solute exchange. Finally, the papers in the latter part of the special issue review lens pathology, including the lens as a model for normal and pathological ageing, vitreoretinal influences on lens function and cataract and the lens as a model for fibrotic disease. Overall, the articles highlight the lens as a continuing, very important and attractive model system for biologists working in many different research areas.

Purpose: To report the pathologic changes in the cornea and the lens capsule resulting from copper deposition in a variant of multiple myeloma., Methods: Case report., Results: Light microscopy of the cornea revealed endothelial cell attenuation with diffuse copper deposition in the central Descemet membrane, which showed thinning, whereas copper banding was seen in the midperipheral and peripheral cornea where the Descemet membrane was normal in thickness. Copper deposition was confirmed by x-ray microanalysis. The anterior lens capsule showed subepithelial copper deposits. Thickening, multilayering, and splitting of the lens capsule were also noted., Conclusions: We report the pattern of deposition of copper in the Descemet membrane of the cornea and the anterior lens capsule in multiple myeloma, associated with hypercupremia. Descemet membrane thinning and regional differences in copper deposition were noted. Also, thickening and splitting of the lens capsule are a novel observation.

This case report retrospectively reviews the outcome of a 43-year-old man with retinitis pigmentosa who suffered rapid anterior capsular phimosis in each eye within 3 weeks following uncomplicated phacoemulsification with continuous curvilinear capsulorrhexis and single-piece acrylic intraocular lens (IOL) implantation. Anterior YAG capsulotomy was successfully performed in both eyes. It has been previously documented in the literature that capsular phimosis is more common in patients with retinitis pigmentosa. However, in the cases that were reviewed, capsular contracture progressed much less rapidly than in this patient. It is proposed that zonular dehiscence or increased lens epithelial cell fibrosis accompanying retinitis pigmentosa may be responsible for phimosis following phacoemulsification; in this case, the single-piece IOL may have offered less resistance to capsular contraction. Using a three-piece IOL, capsular tension ring, or making radial relaxing incisions in the anterior lens capsule may be useful approaches to minimize the aforementioned complications in patients with retinitis pigmentosa and cataracts., (Copyright 2010, SLACK Incorporated.)

This study investigated the encapsulation of newt iris pigment epithelial cells (PECs), which have the ability to regenerate a lens by trans-differentiation in vivo, within a biodegradable hydrogel of oligo(poly(ethylene glycol) fumarate) crosslinked with poly(ethylene glycol)-diacrylate. Hydrogel beads of initial diameter of 1 mm were fabricated by a molding technique. The swelling ratio and degradation rate of the hydrogel beads decreased with increasing crosslinking ratios. Confocal microscopy confirmed the cytocompatibility of crosslinking hydrogel formulations as evidenced by the viability of an encapsulated model cell line within a crosslinked hydrogel bead. Hydrogel beads encapsulating iris PECs were also implanted into lentectomized newts in vivo; histological evaluation of explants after 30 days revealed a regenerated lens, thus demonstrating that the presence of degrading hydrogel did not adversely affect lens regeneration. The results of this study suggest the potential of a method for lens regeneration involving oligo(poly(ethylene glycol) fumarate) hydrogels for iris PEC encapsulation and transplantation.

An 8-month-old boy was found to have unilateral persistent fetal vasculature (PFV) and a small visually insignificant lens opacity. No changes were noted in the appearance of the eye on examinations up to age 3 years. At the age of 4 years, a posterior lentiglobus requiring surgical intervention developed. The thinned and bulging posterior capsule ruptured at surgery. PFV, when treated conservatively, has not been reported to lead to progressive lentiglobus. This case documents the rare concurrence of PFV and lentiglobus., (Copyright (c) 2010 American Association for Pediatric Ophthalmology and Strabismus. Published by Mosby, Inc. All rights reserved.)

Purpose: To evaluate the intraoperative and early postoperative complications of phacoemulsification using a 'phaco chop' technique in previously vitrectomized eyes., Methods: A prospective interventional case series. Seventy-five previously vitrectomized eyes of 73 consecutive patients underwent phacoemulsification using a 'phaco chop' technique via clear corneal incision. Patients were observed for any intraoperative or early postoperative complications., Results: Fifty-four eyes (72%) had predominant nuclear sclerosis or posterior subcapsular cataract. The intraoperative findings included intraoperative miosis [seen in 21 eyes (28%)] and posterior capsule plaques [in 15 eyes (20%)]. No other significant intraoperative complication (posterior capsule rupture, zonular lysis or dropped nuclei) was observed. In the early postoperative period, one patient had massive serous choroidal detachment, which resolved with conservative treatment. Mean preoperative visual acuity (LogMar scale) was 0.74, which improved to 0.36 postoperatively (p < 0.001)., Conclusion: Phacoemulsification using a 'phaco chop' technique is a safe procedure in vitrectomized eyes.

The case of a 29-year-old man is reported who presented with a gradually progressive, painless decrease in vision over 10 years. Anterior segment examination with a slit lamp revealed anterior lenticonus in both eyes. The patient had previously been diagnosed with bilateral sensorineural deafness, however investigations revealed microscopic haematuria and renal insufficiency that subsequently led to a diagnosis of classical Alport syndrome. Since the patient's quality of vision was severely affected by the bulging anterior lens capsule, surgical treatment was required. Clear lens phacoemulsification with continuous curvilinear capsulorhexis and foldable intraocular lens implantation were performed in each eye 2 days apart. One week after surgery, visual acuity was excellent in both eyes. Clear lens phacoemulsification with continuous curvilinear capsulorhexis and foldable intraocular lens implantation was a safe and effective therapeutic choice in this patient for the management of anterior lenticonus due to Alport syndrome.

We describe a rare case of pupillary-iris-lens membrane with goniodysgenesis, a unilateral neurocristopathy. The membrane represents ectopic iris on the lens with abnormal iris stroma and anterior chamber angle from aberrant induction, migration or regression of neural crest cells. The membrane can be progressive. Catastrophic vision loss from angle closure can occur and may be controlled with surgery. This subject needed treatment for amblyopia.

Microbial products are assumed to play a major role in triggering pathogenic autoimmunity. Recently accumulated data have shown that these products stimulate the immune system by interacting with TLRs, expressed on APCs. To examine the capacity of various TLR ligands to trigger pathogenic autoimmunity, we used a system in which naive CD4 cells, specific against hen egg lysozyme (HEL), are injected into recipient mice expressing HEL in their eyes. Only when stimulated, the naive cells acquire pathogenic capacity and induce ocular inflammation. Seven TLR ligands were tested in this system: lipoteichoic acid/peptidoglycan, zymosan, poly (I:C), LPS, pertussis toxin (PTX), flagellin, and CpG oligodeoxynucleotide. Treatment of recipient mice with HEL alone stimulated proliferation of the transferred cells, but no disease, whereas ocular inflammation did develop in recipient mice coinjected with HEL and any one of the seven TLR ligands. Inflammation induced by PTX surpassed by its severity those induced by all other tested TLR ligands and was accompanied by a dramatic increase in number of the transferred cells that acquired features of effector Th1 lymphocytes. Ocular inflammation and number of transferred cells in recipients injected with PTX and HEL were substantially reduced by treatment with Abs against IFN-gamma or IL-12, thus indicating the role of these cytokines in the PTX effect. Overall, our observations demonstrate that various TLR ligands are capable of triggering pathogenic autoimmunity and that PTX surpasses other microbial products in this activity, by stimulating excessive proliferation and polarization toward Th1 of naive T cells.

Presence of exfoliation syndrome (XFS) is associated with an increased risk of complications during cataract surgery. This risk is, in part, dependent on the severity of XFS. We describe a central bulge ("lenticonus") in the anterior lens capsule of some eyes with XFS. This finding is associated with very fragile zonules and a high risk for intraoperative zonular dialysis.

Alport syndrome is a hereditary, progressive disease characterized by progressive nephritis, sensorineural deafness, and ocular abnormalities, including anterior lenticonus. The ultrastructure of the lens capsule abnormalities in Alport syndrome is reported. Four anterior lens capsules from 31-year-old patient and 26-year-old patient with lenticonus who were affected by the Alport syndrome were obtained at capsulectomy. And all four anterior lens capsules were examined by transmission electron microscopy. The histopathologic findings showed that the thickness of the anterior lens capsules was decreased (4-13 microm) and that there were many vascular dehiscences localized at the inner part of the lens capsule. There were large numbers of capsular dehiscences containing fibrillar materials and vacuoles. The anterior capsules were clearly fragile in this disease, forming the basis for the progressive lenticonus and anterior polar cataract.

Background: Cyclitic retrolental membranes (CRM) in children are usually associated with chronic uveitis or genetic syndromes. We report two rare cases of idiopathic CRM., Patients and Methods: Two girls aged 9 and 13 years with visual acuities (VA) of 0.05 underwent lensectomy and anterior vitrectomy with dissection of the central part of the retrolental membrane and intraocular lens (IOL) implantation., Results: The clinical evolution was excellent for the 9 year old girl who recovered 1.0 VA after 2 months. Histological examination revealed a fibroelastic tissue of unknown origin without inflammatory components. The 13 year old girl showed VA of 0.6 within 1 month. However, a recurrent CRM developed with retinal detachment and proliferative vitreoretinopathy (PVR). Vitrectomy, complete excision of the CRM and 360 degrees retinotomy with silicon oil tamponade attached the retina with limited visual recovery. Histology showed fibrovascular tissue with inflammatory components infiltrating the CRM., Conclusions: Idiopathic CRM in children are rare and can be composed of different histological tissues with very different clinical outcomes.

Purpose: To describe the familial occurrence of a peripheral ring of anterior lens capsule discoloration and iridescence in three families., Methods: Clinical ophthalmologic examination with visual acuity, slit-lamp biomicroscopy, and dilated ophthalmoscopy. Pedigree construction and evaluation for possible mode(s) of inheritance., Results: In family 1, 25 members from four generations were available for examination. Twelve had identical findings consisting of a peripheral circumferential polychromatic band of anterior lens capsule. The band was predominantly iridescent green but exhibited a rainbow of colors on direct illumination with the slit-lamp beam. There were affected individuals in all four generations. The proband, one of her sons, and her granddaughter had no clinical, serologic, or other laboratory evidence of Wilson's disease, hypercupremia, or myotonic dystrophy. In family 2, three individuals in three generations were similarly affected. In family 3, a man and his son and daughter had identical peripheral lens capsule discoloration., Conclusions: Polychromasia capsulare is a rare benign autosomal dominant ocular trait that does not appear to be associated with ophthalmologic or systemic disease. The occurrence in consecutive generations and the presence of male-to-male transmission are consistent with autosomal dominant inheritance.

It is a long-accepted dogma in ophthalmology that the lens is a tumour-free tissue. Yet, in the lens, there is lifelong mitotic activity in the subcapsular epithelium. Therefore, these subcapsular epithelial cells must have the potential for cellular transformation. How then can we explain the fact that no scientist has ever seen a naturally occurring primary tumour of the lens in vivo? This review discusses the early work of Mann, von Hallermann, Courtois and others who addressed the issue of tumour resistance of the lens.

An 86-year-old woman came to our emergency ward, and the impression was acute-angle-closure glaucoma attack with corneal edema of the left eye. Incidentally, we found a diaphanous membrane in the anterior chamber, and it was confirmed to be capsular delamination (true exfoliation) of the crystalline lens using ultrasound biomicroscopy. Capsular delamination of the lens can easily be missed because it is transparent and very thin. It is more difficult to find this membrane if the cornea is not sufficiently clear. Ultrasound biomicroscopy at 50 MHz produces good resolution of the fine diaphanous capsular split membrane. Ultrasound biomicroscopy is an effective method for investigating these difficult cases.

Purpose: To describe the occurrence of phacoanaphylaxis in enucleated eyes of patients with Behçet's disease., Design: Retrospective, observational, case series and human tissue study., Participants: Twenty-six patients with Behçet's disease who underwent enucleation., Methods: Histopathologic analysis was performed on 28 enucleated eyes of 26 patients with Behçet's disease. The eyes were divided into two groups, based on the absence or presence of tractional retinal detachment associated with cyclitic membrane formation. Selected eyes with tractional retinal detachment were stained for immunohistochemical examination., Main Outcome Measures: Histopathologic examination of enucleated eyes, including routine histopathologic and immunohistochemical studies., Results: None of the five eyes without retinal detachment showed phacoanaphylaxis. Nine of the 23 eyes with detachment exhibited phacoanaphylaxis, 10 showed no inflammation of the lens, and four were aphakic. There was marked inflammatory cell infiltration in the cyclitic membrane of all nine eyes with phacoanaphylaxis. Immunohistochemical examination demonstrated positive staining for the macrophage markers in the epithelioid and giant cells. The average interval between onset of the ocular manifestations of Behçet's disease and enucleation was 63 months for eyes with phacoanaphylaxis and 35 months for eyes without phacoanaphylaxis (P<0.005)., Conclusions: In Behçet's disease, eyes with long-standing intraocular inflammation complicated by cyclitic membrane formation may develop phacoanaphylaxis. Such patients may benefit from surgical removal of the cyclitic membrane along with the lens in eyes with significant visual function.