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BACKGROUND: Clinical pathways are care plans established to describe essential steps in the care of patients with a specific clinical problem. They translate (inter)national guidelines into local applicable protocols and clinical practice. The purpose of this article is to establish a multidisciplinary integrated care pathway for specialists and allied health care professionals in caring for individuals with von Hippel–Lindau (VHL) disease. METHODS: Using a modified Delphi consensus‐making process, a multidisciplinary panel from 5 Dutch University Medical Centers produced an integrated care pathway relating to the provision of care for patients with VHL by medical specialists, specialized nurses, and associated health care professionals. Patient representatives cocreated the pathway and contributed quality criteria from the patients' perspective. RESULTS: The panel agreed on recommendations for the optimal quality of care for individuals with a VHL gene mutation. These items were the starting point for the development of a patient care pathway. With international medical guidelines addressing the different VHL‐related disorders, this article presents a patient care pathway as a flowchart that can be incorporated into VHL expertise clinics or nonacademic treatment clinics. CONCLUSIONS: Medical specialists (internists, urologists, neurosurgeons, ophthalmologists, geneticists, medical oncologists, neurologists, gastroenterologists, pediatricians, and ear‐nose‐throat specialists) together with specialized nurses play a vital role alongside health care professionals in providing care to people affected by VHL and their families. This article presents a set of consensus recommendations, supported by organ‐specific guidelines, for the roles of these practitioners in order to provide optimal VHL care. This care pathway can form the basis for the development of comprehensive, integrated pathways for multiple neoplasia syndromes. [ABSTRACT FROM AUTHOR]

Purpose: To determine if non-mydriatic ultra wide-angle digital retinal imaging alone is effective for screening subjects considered to be at 50% or lower risk for developing retinal angiomas. Methods: Digital records and retinal images of subjects attending a regional Von-Hippel-Lindau disease multi-disciplinary clinic over a 12 month period were reviewed. Individuals were stratified for risk of developing retinal angiomas on the basis of age, clinical features and genetic risk. The image quality and necessity for subsequent mydriatic fundoscopy were assessed. Results: Eighty subjects from 55 pedigrees attended the VHL clinic over 12 months. Of these, 44 (55%) were considered to be at lower risk for retinal angiomatosis: 34 (77%) because they had reached at least 30 years of age without developing an angioma, 16 with a presenting solitary tumour of the type associated with VHL but no identifiable gene mutation, and 12 with a family history giving them a 50% risk of carrying a gene mutation. Eighteen patients fulfilled two low risk criteria. All were able to comply with imaging but poor image quality (limited view of the inferior retinal far-periphery) required subsequent dilated fundoscopy in five (6%) eyes of three patients. Conclusions: Non-mydriatic ultra wide-field retinal imaging enabled satisfactory assessment in over 95% of lower-risk VHL subjects. Virtual clinics or remote imaging of lower-risk subjects may improve both the efficiency and flexibility in the provision of multi-disciplinary VHL services and the patient experience. [ABSTRACT FROM AUTHOR]

Retinal angiomas are rare tumors that develop within the vascular bed of the retina. Their occurrence may be sporadic or as part of the multisystem disorder called von Hippel-Lindau disease. It is well established in literature that retinal angiomas have a natural history of evolving as small aneurysmal dilatations and progressing to vision-threatening retinal detachments. Several approaches to the management of these angiomas have been reported in the literature, but it is difficult to decipher which modality is best suited for which type of angioma. A classification of retinal angiomas is proposed herein. A classification system may help in developing future guidelines for the study, screening, treatment, and prognostication of patients with retinal angiomas. [ABSTRACT FROM AUTHOR]

Von Hippel-Lindau syndrome, an autosomal dominant genetic disorder with high penetrance, is a rare genetic cause of pheochromocytoma and various other tumors, such as hemangioblastomas of retina and cerebellum, renal cell carcinoma, islet cell tumors, endolymphatic sac tumors, etc. Conventionally, Von Hippel-Lindau is diagnosed with the help of biochemical and structural imagining techniques, such as magnetic resonance imaging and computed tomography scans. However, the recent advent of the functional imaging techniques, such as meta-iodo-benzylguanidine, has significantly added to the armamentarium available for the diagnosis as well as localization of these tumors. In this report, we describe a case of a 22-year-old male with VHL syndrome, where functional 68Ga-DOTANOC PET/CT, 99mTc-HYNIC-TOC SPECT/CT, and 131I-MIBG SPECT/CT aided in the diagnostic evaluation of this disease. This patient presented with bilateral pheochromocytoma, retinal angioma, cerebellar hemangioblastoma, pancreatic neuroendocrine tumor, and pancreatic cysts. This case highlights the emerging role of functional imaging as an adjuvant to conventional tools in the diagnostic evaluations of diseases, an area that has not been addressed to date. [ABSTRACT FROM AUTHOR]

Purpose: To report the surgical management of a combined rhegmatogenous and traction retinal detachment associated with a vasoproliferative tumor secondary to sickle cell retinopathy. Observations: A 29 year old man from Ghana presented with unilateral vision loss, ischemic retina and sea fan neovascularization in both eyes and a retinal detachment nearby a vasoproliferative tumor (VPT) in the left eye. Hemoglobin electrophoresis led to the diagnosis of sickle cell disease. The patient underwent vitrectomy with scleral buckle surgery, resection of the tumor, and removal of subretinal membranes in the left eye. Laser photocoagulation was targeted to areas of ischemic retina in both eyes. Conclusions: and Importance: To our knowledge, this is the first report of a combined rhegmatogenous and traction retinal detachment associated with a VPT in sickle cell retinopathy managed by modern vitrectomy techniques. Prompt recognition of the condition and surgical management addressing both rhegmatogenous and tractional components can lead to improved outcome.

Aim : To evaluate the efficacy of modified temporalis muscle transfer (TMT) by silicone sling for the management of paralytic lagophthalmos. Settings and Design: Prospective interventional study. Materials and Methods : Ten patients of lagophthalmos due to facial palsy underwent modified TMT using silicone sling. The patients were followed-up for a period of 3 months. Palpebral aperture in primary gaze and during eye closure were assessed both pre- and postoperatively along with problems associated with lagophthalmos like exposure keratopathy and lacrimation. Statistical Analysis : Paired t-test was applied to measure the statistical outcome. Results : Eight patients achieved full correction of lagophthalmos with no lid gap on closing the eye. The mean (standard deviation (SD)) lid gap on eye closure was 7.7 (0.86) mm preoperatively, 0.5 (0.47) mm at 1 st postoperative day, and 0.7 (0.75) mm at 3 rd month. There was a reduction in mean lid gap on eye closure of 7 mm at 3 months (P < 0.0001) which is highly significant. The mean (SD) vertical interpalpebral distance during primary gaze was 12.05 (1.12) mm preoperatively, 10 (0.94) mm at 1 st postoperative day, and 10.35 (1.08) mm at 3 rd month. There was a reduction in mean vertical inter palpebral distance of 1.7 mm at 3 months (P = 0.001) which is significant. Exposure keratitis decreased in five out of six patients at 3 months. Conclusion : Modified TMT by silicone sling is a useful procedure with lesser morbidity and good outcomes for the treatment of paralytic lagophthalmos due to long standing facial palsy.

Purpose: To evaluate the role of echography in diagnosis and management of a diverse array of choroidal masses. Materials and Methods: Sixty-two cases of clinically suspected choroidal masses were prospectively analyzed with B-scan (10 Hz), A-scan, and ultrasound biomicroscopy (UBM) (50 Hz) after a meticulous history and ocular examination. Ancillary investigations and systemic evaluation were also done. Results: Based on clinical suspicion, acoustic features, response to treatment, and other ancillary tests combined together, the various masses were differentiated. The cases included in the study were as follows: n = 10 malignant melanomas, n = 16 metastasis and infiltrations, n = 9 hemangioma, n = 7 tuberculoma, n = 8 nonspecific inflammatory masses, n = 2 disciform plaques, n = 4 macular cysts or retinoschisis, n = 2 Coat′s disease, n = 1 melanocytoma, and n = 2 osteomas. Ultrasonography (USG) alone could identify n = 51 lesions, while UBM in combination with USG was needed in remaining 11 masses. Conclusion: Standardized echography is an important adjunct in the diagnosis and management of eyes with intraocular masses. A better understanding of the clinicopathological and echographic picture of the diverse lesions can help in detection, differentiation, diagnosis, proposing a therapeutic approach, and also monitoring response to treatment. Echography is essential to evaluate tumors for extrascleral and anterior segment extension.

Aim: To determine profiles of non-chlamydia conjunctival bacteria and their antimicrobial susceptibility from adults who underwent trachomatous trichiasis surgery in rural areas of Ethiopia. Materials and Methods: A cross-sectional study was conducted in rural districts in West Gojjam administrative zone. Conjunctival swabs were collected during surgery and transported using Stuart transport broth (Oxoid, UK). Antibiotic susceptibility of conjunctival isolates was determined using the Kirby-Bauer disc-diffusion method. Results: Non-chlamydia pathogenic bacteria were recovered from conjunctiva of 438 (31%) participants before treatment. The isolated conjunctival bacteria were Staphylococcus aureus, coagulase-negative Staphylococci, Streptococcus group (A, C, F and G), Enterococci, Streptococcus pneumoniae, Moraxella spp., Escherichia coli, Citrobacter spp., Proteus spp., Klebsiella spp., Pseudomonas spp. and Enterobacter spp. Overall, resistance rates of 57.8% to azithromycin and 68.5% to chloramphenicol were found. However, 86-94.4% sensitivity was demonstrated to ciprofloxacin and norfloxacin. Moderate sensitivity rates (61.8-78.4%) were observed to ceftriaxone, tetracycline and cotrimoxazole. Conclusion: Fluoroquinolones that have activity against the majority of bacterial isolates were potent at in vitro. However, unacceptably high levels of resistance to azithromycin and chloramphenicol in rural community indicated a need for further study and antimicrobial resistance surveillance.

Purpose: This study was aimed at reporting the outcomes of trabeculectomy in primary juvenile open angle glaucoma (JOAG). Design: This study was a retrospective noncomparative case series. Materials and Methods: We included 60 eyes of 41 JOAG patients who underwent primary trabeculectomy without mitomycin-C (MMC) between 1995 and 2007. The primary outcome was success, defined as complete, if intraocular pressure (IOP) was >5 and ≤21 mmHg without medications or qualified if IOP was >5 and ≤21 mmHg with or without antiglaucoma medications. Secondary outcome measures were mean and percentage IOP reduction, complications, and risk factors for the failure of trabeculectomy. Results: The mean (±standard deviation) age at presentation was 24.1 ± 6.8 years (range, 12-35). Mean follow-up was 67 ± 41 months (range, 12-156). At 1 year, the probability of complete success was 92% (n = 56, 95% CI: 81-96%), at 3 years it was 89% (n = 47, 95% CI: 78-95%), and at the end of 5 years, it was 80% (n = 34, 95% CI: 65-89%). The probability of qualified success was 100% (n = 60) at 1 year, 98% (n = 51, 95% CI: 87-100%) at 3 years, and 96% (n = 36, 95% CI: 84-99%) at the end of 5 years. The mean IOP reduced from 35 ± 10 to 13 ± 2.5 mmHg (P < 0.001) after trabeculectomy. There was no serious postoperative complication. Young age was the only significant risk factor associated with the failure (odds ratio = 0.89, P = 0.03). Conclusion: Primary trabeculectomy without MMC has good success rates in JOAG.

In this retrospective case series, we report the spectrum and outcomes of aggressive posterior retinopathy of prematurity (APROP) in infants ≥1500 g birth weight. Twenty-nine eyes of 15 infants are included. All infants were referred from level I or II nurseries, received supplemental unmonitored oxygen for prolonged duration (>1 week) and had multiple systemic co-morbidities. Of the 29 eyes, 10 (34.5%) had zone 1 and 19 (65.5%) had posterior zone 2 disease. Twenty-five (86.2%) eyes had flat neovascularization and 4 (13.8%) eyes had brush like proliferation. We noticed large vascular loops in 10 (34.5%) eyes. After confluent laser photocoagulation, 22 (75.9%) eyes had a favorable outcome. The study concludes that APROP in heavier (≥1500 g birth weight) premature infants occurs mostly in posterior zone 2 with flat neovascularization and atypical features like large vascular loops. Supplemental unmonitored oxygen for prolonged duration and multiple systemic co-morbidities could be a contributing factor.

Purpose: The purpose of this study is to evaluate the lateral rectus periosteal fixation and partial vertical rectus transpositioning (VRT) as treatment modalities to correct exotropic Duane retraction syndrome (Exo-DRS). Materials and Methods: Prospective interventional case study of cases of Exo-DRS with limitation of adduction. A total of 13 patients were subdivided into two groups. Six patients underwent only lateral rectus periosteal fixation (group A) and seven patients also underwent partial VRT (group B). Assessment involved prism bar cover test, abduction and adduction range, extent of binocular single visual field and exophthalmometry. These tests were repeated at 1 week, 1 month and 3 months post-operatively and data analyzed. Results: The pre-operative mean values and ranges were 26.2 Δ (22-35) exotropia for group A and −21.3 Δ (14-30) exotropia for group B. The post-operative mean and range was +0.6 Δ esotropia (+20 to −8) for group A and 8 Δ (−2 to −20) exotropia for group B. Mean grade of limitation of abduction changed from −3.8 to −3.6 versus −3.6 to −2.8 and mean grade of limitation of adduction changed from −1.9 to −0.7 versus −1.5 to −0.5 in the groups A and B respectively. Mean binocular single visual field changed from 14.7° to 23.3° in group A and 11.8° to 26.4° in the group B respectively. Conclusion: Lateral rectus periosteal fixation is an effective surgery to correct the exodeviation, anomalous head posture and improving adduction in Exo-DRS and partial VRT in addition is effective in improving abduction and binocular single visual fields.

Purpose: To compare the outcome of Collagen cross-linking (CXL) with that following topography-guided customized ablation treatment (T-CAT) with simultaneous CXL in eyes with progressive keratoconus. Materials and Methods: This was a prospective, non-randomized single centre study of 66 eyes with progressive keratoconus. Of these, 40 eyes underwent CXL and 26 eyes underwent T-CAT + CXL. The refractive, topographic, tomographic and aberrometric changes measured at baseline, 1, 3 and 6 months post-operatively were compared between both groups. Results: After a mean follow-up of 7.7 ± 1.3 months, the mean retinoscopic cylinder decreased by 1.02 ± 3.16 D in the CXL group ( P = 0.1) and 2.87 ± 3.22 D in the T-CAT + CXL group ( P = 0.04). The Best corrected visual acuity increased by 2 lines or more in 10% of eyes in the CXL group and in 23.3% of eyes in the T-CAT + CXL group. The mean steepest-K reduced by 0.40 ± 3.71 D ( P = 0.77) in the CXL group and by 2.91 ± 2.01D ( P = 0.03) in the T-CAT + CXL group. The sag factor and surface asymmetry index showed no significant change in the CXL group but reduced by 3.59 ± 5.94 D ( P = 0.01) and 0.72 ± 1.18 ( P = 0.02) respectively in the T-CAT + CXL group. There was a significant increase in the highest posterior corneal elevation in both groups (9.57 ± 14.93 μ in the CXL group and 7.85 ± 9.25 μ in the T-CAT + CXL group, P ≤ 0.001 for both). There was significantly greater reduction of mean coma ( P < 0.001) and mean higher-order aberrations ( P = 0.01) following T-CAT + CXL compared to CXL. Conclusions: CAT + CXL is an effective approach to confer biomechanical stability and to improve the corneal contour in eyes with keratoconus and results in better refractive, topographic and aberrometric outcomes than CXL alone.

We performed endothelial keratoplasty (EK) in three eyes of two siblings (2.5 years, male and 3.5 years, female) with congenital hereditary endothelial dystrophy (CHED) and report the intraoperative and postoperative difficulties. Repeated iris prolapse, apprehension of crystalline lens touch due to positive vitreous pressure, and need for frequent air injections to attach the graft were intraoperative challenges in all three eyes. These were addressed by use of Sheet′s glide instead of Busin′s glide during graft insertion and suturing of main and side ports before air injection. One eye had graft dislocation on second postoperative day due to eye rubbing by the child. Graft was repositioned with air and a venting incision was created. Postoperative examination required repeated general anesthesia. Corneal edema resolved completely in all three eyes. Present case series highlights the possible intraoperative and postoperative challenges and their solutions in pediatric EK for CHED.

Purpose: To analyze the complications and their managements in Descemet′s stripping endothelial keratoplasty (DSEK) in consecutive 430 cases by single surgeon in a tertiary eye hospital. Materials and Methods: 430 eyes of 366 patients with endothelial dysfunctions scheduled for DSEK, were analyzed retrospectively. In all cases donor dissection was performed manually, and ′Taco′ insertion and unfolding technique was used. Intra-operative and postoperative complications with their managements and outcomes were reviewed retrospectively. Periodic endothelial cell density was analyzed for each patient till the last visit. Follow-up period was between 3 to 60 months (mean 18.7 months). Results: 13 (3.0%) eyes had operative complications during donor dissection and 16 (3.7%) had during recipient procedure. In 7 (1.6%) eyes, donor lenticule was replaced with a new one during the surgery. In early postoperative period, 21 (4.9%) eyes had donor dislocation and 12 (2.8%) eyes had air-induced pupillary block; and they were managed immediately. 2 cases had primary graft failure and in 1 case had postoperative bacterial endophthalmitis requiring evisceration. In late postoperative period, 48 (11.3%) eyes had secondary glaucoma and 14 (3.3%) eyes had late secondary graft failure. Endothelial rejection occurred in 5 (1.2%) cases. Mean endothelial cell loss was 19.7% after 3 months and 54.2% after 5 years. Total graft failure in this series was 31 (7.2%) and in 17 cases re-DSEK was performed successfully. Conclusions: Both operative and postoperative complications do occur in DSEK. Most of these complications can be managed by medical or appropriate surgical means. Some of the complications can be avoided and reduced with experience.

Context: The provision of ocular surgical interventions for poorer, less educated populations is increasing as a result of increased globalization and outreach. However, these populations still have trouble understanding surgical concepts and are not always fully informed decision makers. Aims: We aimed to test the effect that a multimedia addition to a traditional verbal informed consent would have on patient comprehension of relatively difficult cataract surgical concepts. Settings and Design: We conducted a randomized controlled trial with relatively uneducated patients reporting to a private surgical hospital in Chennai, India. 47 patients were placed into the intervention group and 50 patients were placed into the control group. Materials and Methods: The intervention group was presented with a scripted verbal informed consent as well as a 3-fold pamphlet and a presentation with a 3-dimensional model of the eye. The control group was only presented with a scripted verbal informed consent. The two groups were tested using an 11 item "True/False/I don′t know" quiz directly before the informed consent, directly after the informed consent, and one-day postoperatively. Statistical Analysis Used: Scores on the quiz were compared across groups and time-points using paired t-tests. Results: Patients in the both groups showed a significant improvement in scores between pre- and post-informed consent quizzes (P value on the order of 10 -6 ) and the improvement in scores was significantly greater in the intervention group than the control group (P value on the order of 10 -16 ). There was no significant difference observed in either group with regards to the change in scores between post-informed consent and post-operative quizzes. Conclusion: Multimedia aids in addition to a standard informed consent process are effective in improving patient comprehension even for patients with low literacy and limited knowledge of surgical interventions.

Background : Konkan coast of India is geographically distinct and its pattern of blindness has never been mapped. Aim : To study the prevalence and causes of blindness and cataract surgical services in Sindhudurg district of West Coast. Subjects : Individual aged > 50 years. Materials and Methods: Rapid assessment of avoidable blindness used to map blindness pattern in the district. Statistical analysis: SPSS version 19. Results: Amongst those examined 1415 (51.7%) had visual acuity (VA) >20/60, 924 (33.8%, confidence interval (C.I) 30.5%-36.8%) had VA 20/200-

Aim : To study long term outcome of bilateral congenital and developmental cataract surgery. Subjects: 258 pediatric cataract operated eyes of 129 children. Materials and Methods: Children who underwent pediatric cataract surgery in 2004-8 were traced and examined prospectively in 2010-11. Demographic and clinical factors were noted from retrospective chart readings. All children underwent visual acuity estimation and comprehensive ocular examination in a standardized manner. L. V. Prasad Child Vision Function scores (LVP-CVF) were noted for before and after surgery. Statistics: Statistical analysis was done with SPSS version 16 including multi-variate analysis. Results: Children aged 9.1 years (std dev 4.6, range 7 weeks-15 years) at the time of surgery. 74/129 (57.4%) were boys. The average duration of follow-up was 4.4 years (stddev 1.6, range 3-8 years). 177 (68.6%) eyes had vision 6/18 and 157 (60.9%) had BCVA >6/60 3-8 years after surgery. 48 (37.2%) had binocular stereoacuity

Aim: To report our experience of brachytherapy using ′BARC I-125 Ocu-Prosta seeds′ for the management of intraocular tumors with regard to tumor control, globe preservation visual outcome, and patient survival at Sankara Nethralaya, Chennai, India between September 2003 and May 2011. Materials and Methods: We reviewed records of 35 eyes of 35 patients who underwent ophthalmic brachytherapy between September 2003 and May 2011. Twenty-one cases had choroidal melanoma, nine had childhood retinoblastoma, two had adult-onset retinoblastoma, and there were one case each of vasoproliferative tumor, retinal angioma, and ciliary body melanoma. Brachytherapy was administered using a 15- or 20-mm gold plaque with or without a notch. Brachytherapy was the primary treatment modality in all tumors other than retinoblastoma, wherein brachytherapy was done post chemoreduction for residual tumor. Results: For choroidal melanomas, the mean radiation dose was 68.69 ± 15.07 (range, 47.72-94.2) Gy. The eye salvage rate was 13/20 (65%) and tumor control rate was 16/20 (80%) at an average follow-up of 24.43 ± 24.75 (range, 1.5-87.98) months. For retinoblastoma, the mean dose was 45.85 ± 3.90 (range, 39.51-50.92) Gy. The eye salvage rate and tumor control rate was 5/6 (83.3%) at an average follow-up of 38.36 ± 31.33 (range, 4.14-97.78) months. All eyes with retinoblastoma needed additional focal therapy for tumor control and eye salvage. Conclusion: The results of this retrospective study confirms that the use of ′BARC I-125 Ocu-Prosta seeds′ in episcleral plaques to treat intraocular tumors offers a viable option for the management of intraocular cancers.

Context: Contrast sensitivity (CS) function is one of the most important tests available for evaluating visual impairment. Multiple sclerosis (MS) can produce highly selective losses in visual function and psychophysical studies have demonstrated CS deficits for some spatial frequencies. Aims: This work studies the differences in CS between a group of controls and a group of MS patients, focusing on the location of the maximum sensitivity peak, shape of the curve, and determination of the most affected spatial frequencies. Materials and Methods: Using a sinusoidal stimulus the authors assessed CS function in 28 subjects with definitive relapsing remitting MS, and in 50 controls with acuities of 20/25 or better. The peaks of the CS curves were studied by fitting third degree polynomials to individual sets of data. Results: Compared with the control group, the CS function curve for MS subjects showed more deficits in extreme points (low- and high-spatial frequencies). Our results display significant CS losses, at the high-frequencies band level, in the beginning of the disease. When the disease progresses and the disabilities appear, there are greater losses at the low-frequencies band level. In average, the CS curve peaks for the MS group were shifted in relation to the control group. Conclusions: CS losses in the MS group suggest an association with ageing and disability level in the expanded disability status scale. The position of the CS function peak is influenced by MS, age, and degree of disability.

Purpose: To compare clarity, exposure and quality of anterior chamber angle visualization in congenital glaucoma patients, using RetCam and indirect gonioscopy images. Design: Cross-sectional study Participants. Congenital glaucoma patients over age of 5 years. Materials and Methods: A prospective consecutive pilot study was done in congenital glaucoma patients who were older than 5 years. Methods used are indirect gonioscopy and RetCam imaging. Clarity of the image, extent of angle visible and details of angle structures seen were graded for both methods, on digitally recorded images, in each eye, by two masked observers. Outcome Measures: Image clarity, interobserver agreement. Results: 40 eyes of 25 congenital glaucoma patients were studied. RetCam image had excellent clarity in 77.5% of patients versus 47.5% by gonioscopy. The extent of angle seen was similar by both methods. Agreement between RetCam and gonioscopy images regarding details of angle structures was 72.50% by observer 1 and 65.00% by observer 2. Conclusions: There was good agreement between RetCam and indirect gonioscopy images in detecting angle structures of congenital glaucoma patients. However, RetCam provided greater clarity, with better quality, and higher magnification images. RetCam can be a useful alternative to gonioscopy in infants and small children without the need for general anesthesia.

Purpose: This work was conducted to study the ability of bacterial and fungal isolates from keratitis cases in Upper Egypt to produce enzymes, toxins, and to test the isolated fungal species sensitivity to some therapeutic agents. Materials and Methods: One hundred and fifteen patients clinically diagnosed to have microbial keratitis were investigated. From these cases, 37 bacterial isolates and 25 fungal isolates were screened for their ability to produce extra-cellular enzymes in solid media. In addition, the ability of fungal isolates to produce mycotoxins and their sensitivity to 4 antifungal agents were tested. Results: Protease, lipase, hemolysins, urease, phosphatase, and catalase were detected respectively in 48.65%, 37.84%, 59.46%, 43.24%, 67.57%, and 100% out of 37 bacterial isolates tested. Out of 25 fungal isolates tested during the present study, 80% were positive for protease, 84% for lipase and urease, 28% for blood hemolysis, and 100% for phosphatase and catalase enzymes. Thirteen fungal isolates were able to produce detectable amounts of 7 mycotoxins in culture medium (aflatoxins (B1, B2, G1, and G2), sterigmatocystin, fumagillin, diacetoxyscirpenol, zearalenone, T-2 toxin, and trichodermin). Among the antifungal agents tested in this study, terbinafine showed the highest effect against most isolates in vitro. Conclusion: In conclusion, the ability of bacterial and fungal isolates to produce extracellular enzymes and toxins may be aid in the invasion and destruction of eye tissues, which, in turn, lead to vision loss.

We describe the ocular alterations and the management after stings from Hymenopteran insects. In all the five patients, the insect was identified as bee. The patients presented with significant corneal edema, which resolved dramatically in three of them after removal of stingers. Among the other two one went for permanent corneal decompensation and the other developed Intumuscent cataract with increased intraocular pressure. Although a rare occurrence, ocular trauma caused by Hymenopteran insects has a potential to cause severe ocular damage in humans. A high level of clinical suspicion and immediate removal of the stingers along with administration of high doses of topical and systemic steroids is a must to prevent chances of permanent corneal damage and intraocular complications.

Objective: To investigate the clinicopathological correlation of parapapillary atrophy. Materials and Methods: The study included 16 eyes of rhesus monkeys (Macaca mulatta) - 4 eyes with experimental glaucoma, 11 eyes after experimental temporary occlusion of the central retinal artery, and 1 normal eye. On histological sections, we measured zones with different histological characteristics.On fundus photographs, alpha zone and beta zone of parapapillary atrophy were measured and correlated with the histological data. Results: The size of the clinical alpha zone of parapapillary atrophy was significantly correlated with the size of the histological region with irregularities of the retinal pigment epithelium (P = 0.05; correlation coefficient r = 0.49) and with the size of the histological region with a decreased density of retinal photoreceptors (P = 0.01; r = 0.60). The size of clinical beta zone of parapapillary atrophy significantly correlated with the size of the histological region with complete loss of the retinal pigment epithelium (P

Hemangioblastomas are benign vascular neoplasms which originate almost exclusively from central nervous system (CNS). They account for 2 % of all intracranial neoplasms, 7 %–12 % of posterior fossa tumours. 60–75 % cases of hemangioblastomas occur sporadically and rest 25 % to 40 % occur in genetically inherited in the autosomal dominant neoplasia syndrome known as Von Hippel-Lindau (VHL) disease with mutation occurring on chromosome 3p. We, hereby, report a case of one such rare case of a 30-year old male who presented to us with the complaint of holocranial headache for past 2 months associated with vomiting along with bilateral cerebellar signs and symptoms. MRI Brain revealed multiple cysts with enhancing mural nodules present on both cerebellar hemispheres. Due to recurrence and multi-focality of lesions, VHL syndrome was suspected. VHL is a rare autosomal dominant disease. MRI is the gold standard test. It demonstrates specific appearances depending on the tumour anatomy. Hemangioblastoma of the CNS in VHL usually develop from childhood at an age of <10 years or early teen until the age of 30 years. The most common locations for hemangioblastomas in VHL are cerebellum and spinal cord as seen in our patient. VHL being a lifelong disease with no cure till date, the patients should be frequently screened for lesions at various potential sites in his/her lifetime so as to provide required intervention at the earliest. Hence, a genetic screening for VHL (VHL tumour suppressor gene), should be performed in cases of hemangioblastoma. • VHL is a rare autosomal dominant disease that has been recognised for about 70 years, accounting only for 2% of all intracranial neoplasms • Our patient was a case of recurrent cerebellar hemangioblastomas with retinal angiomas with bilateral cerebellar involvement which is uncommon . • The case was managed by surgical tumour excision which included the cystic component and the mural nodule • Microsurgical resection is curative and most craniospinal hemangioblastomas can be completely and safely resected. Recurrence is rare if mural nodule is completely resected. • VHL being a lifelong disease with no cure till date. Hence, a genetic screening for VHL tumour suppressor gene should be performed in cases of hemangioblastoma. [ABSTRACT FROM AUTHOR]

Progressive anterior segment fibrosis syndrome (ASFS), after intraocular surgery in older children (≥9 years) and adults with congenital aniridia, is described in the literature. In this report, we describe an unique case of ASFS in an infant with congenital aniridia following a combined trabeculotomy-ectomy and its recurrence after a descemet stripping endothelial keratoplasty. The ophthalmologists should be well aware of this entity and warn the parents about its possibilities. Use of immunomodulators or prolonged anti-inflammatory therapy may be considered to prevent its occurrence.

Aims: von Hippel-Lindau (VHL) disease is caused by mutations in the VHL tumor suppressor gene. As tumors that develop in the context of VHL also occur in a sporadic context, the frequency of this syndrome may be underestimated. Our aim was to identify VHL gene mutations in Argentinian patients who fulfilled the clinical criteria for type 1 VHL disease and in patients with VHL-associated manifestations that did not meet these criteria. Methods: We performed a retrospective cohort study, including patients who met current diagnostic criteria for type 1 VHL (Group 1, n = 19) and patients with VHL-associated manifestations that did not meet these criteria (Group 2, n = 21). Genomic DNA was extracted from peripheral blood leukocytes. Mutation analysis involved DNA sequencing, while large deletions were determined by universal primer quantitative fluorescent multiplex polymerase chain reaction (UPQFM-PCR) and multiplex ligation-dependent probe amplification (MLPA) analysis. Results: VHL mutations were detected in 16/19 (84.2%) patients in Group 1 and included: gross deletions (4/16); nonsense mutations (6/16); frameshift mutations (4/16); missense mutations (1/16); and splicing mutations (1/16). Three of these mutations were novel. No alterations were found in 3 of 19 VHL patients. In Group 2, one nonsense VHL mutation was detected in a young patient with a solitary central nervous system hemangioblastoma without familial history. A study of 30 first-degree relatives revealed four carriers with VHL mutations. Conclusions: We found three novel mutations in the VHL gene in our population. Our results emphasize the importance of a complete genetic study of VHL to confirm type 1 VHL disease, not only in patients with clinical diagnostic criteria but also in those presenting a single typical manifestation. [ABSTRACT FROM AUTHOR]

Aim: To report our experience of brachytherapy using 'BARC I-125 Ocu-Prosta seeds' for the management of intraocular tumors with regard to tumor control, globe preservation visual outcome, and patient survival at Sankara Nethralaya, Chennai, India between September 2003 and May 2011. Materials and Methods: We reviewed records of 35 eyes of 35 patients who underwent ophthalmic brachytherapy between September 2003 and May 2011. Twenty-one cases had choroidal melanoma, nine had childhood retinoblastoma, two had adult-onset retinoblastoma, and there were one case each of vasoproliferative tumor, retinal angioma, and ciliary body melanoma. Brachytherapy was administered using a 15- or 20-mm gold plaque with or without a notch. Brachytherapy was the primary treatment modality in all tumors other than retinoblastoma, wherein brachytherapy was done post chemoreduction for residual tumor. Results: For choroidal melanomas, the mean radiation dose was 68.69 ± 15.07 (range, 47.72-94.2) Gy. The eye salvage rate was 13/20 (65%) and tumor control rate was 16/20 (80%) at an average follow-up of 24.43 ± 24.75 (range, 1.5-87.98) months. For retinoblastoma, the mean dose was 45.85 ± 3.90 (range, 39.51-50.92) Gy. The eye salvage rate and tumor control rate was 5/6 (83.3%) at an average follow-up of 38.36 ± 31.33 (range, 4.14-97.78) months. All eyes with retinoblastoma needed additional focal therapy for tumor control and eye salvage. Conclusion: The results of this retrospective study confirms that the use of 'BARC I-125 Ocu-Prosta seeds' in episcleral plaques to treat intraocular tumors offers a viable option for the management of intraocular cancers. [ABSTRACT FROM AUTHOR]

Although a large kindred with familial pheochromocytoma (Pheo) and paraganglioma (PGL) was discovered in 1962 and later found to represent von Hippel-Lindau (VHL) type 2A (mutation Y112H), the phenotype lacks current characterization. Branch-specific expressivity was suspected based on oral family history. Family pedigree analysis, prospective interviews, and extensive record review were used to extend the pedigree, determine phenotype, examine branch-specific expression, and analyze mortality rates over 5 decades. In its 3 known affected branches the kindred now comprises 107 people with or at-risk for VHL, of whom 49 have been diagnosed and 35/49 (71%) are clinically affected. Phenotypic cumulative lifetime risk was 71% for Pheo/PGL, 15% for hemangioblastoma, 33% for retinal angioma, 3% for renal cell carcinoma, and 3% for pancreatic cysts. The mean ages for VHL and Pheo/PGL diagnosis were younger in successive generations. Branch II-4 predominately expressed RA, while branch II-5 predominantly expressed Pheo/PGL. Disease-specific mortality occurred early and was less frequent in successive generations. This analysis of Y112H VHL confirms a high cumulative risk for pheochromocytoma/paraganglioma. Over time, both age at diagnosis and disease-specific mortality have decreased. The observed branch-specific expressivity prompts further study of genetic and environmental disease modifiers in this large family. [ABSTRACT FROM AUTHOR]

Copyright of Canadian Journal of Ophthalmology is the property of Elsevier B.V. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Der Ophthalmologe is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

This survey reviews selected medical disorders in which the ophthalmologist has a unique opportunity of anticipating the existence of asymptomatic, life-threatening disease simply by recognizing that certain benign conditions involving the eye or adnexa represent clues to occult non-ocular cancer and other neoplasias. Having knowledge that these clues are associated with specific types of neoplasia, the ophthalmologist can then initiate a goal-directed oncologic investigation designed to identify the underlying neoplasm with the hope of providing potentially life-saving therapy. The survey is organized to feature each clue separately. The clinical characteristics of the clue are described and illustrated with color photographs when possible. A discussion reviews associated malignancies and other important medical features. The survey begins with clues expressed anteriorly in the lids, progressively reviewing those clues located more posteriorly, concluding with clues involving the retinal pigment epithelium and choroid. [Copyright &y& Elsevier]

Wittebol-Post D, Hes FJ, Lips CJM (University Hospital Utrecht, Utrecht, The Netherlands). The eye in von Hippel–Lindau disease. Long-term follow-up of screening and treatment: recommendations (Minisymposium: MEN & VHL). J Intern Med 1998; 243: 555–561. Von Hippel–Lindau disease (VHL) is an autosomal dominant tumour syndrome caused by germline mutations of the VHL tumour suppressor gene located on chromosome 3p25–26. In VHL tumours may occur in 14 different target organs, including the eye. Retinal angiomas are considered the first manifestation of VHL disease in 43% of cases, and the cumulative probability of developing a retinal angioma in one or both eyes rises during each decade of life, reaching 80% for patients over 80 years old. Since 1976 patients with VHL at the University Hospital of Utrecht and their at-risk relatives have been screened periodically by a multidisciplinary team. Long-term follow-up ophthalmological data were analysed with special attention to natural course and results of treatment. In addition, we looked for a genotype-phenotype correlation. Retinal angiomas were found in all families. In one large family with a missense mutation (V170D) of the VHL gene, in which the complete spectrum of visceral- and central nervous system (CNS) features of VHL is present, macular, parapapillary, optic disc and ora serrata angiomas were also found. In general, however, a clear-cut genotype-phenotype correlation could not be found. Only early detection and treatment of peripheral retinal angiomas can be expected to decrease the percentage of patients with decreased visual acuity. Therefore, early detection and treatment of these tumours is of paramount importance. Ophthalmological screening of patients and persons at risk should start as early as possible. In patients with apparently sporadic retinal angiomas it is advisable to perform germline DNA analysis, since the risk of developing VHL is high, especially if the angiomas are bilateral, or unilateral and multifocal, if the patient is young, or if there is a family history suggestive of VHL. [ABSTRACT FROM AUTHOR]