The evaluation and surgical management of cyclodialysis clefts that have failed to respond to conservative management.

PURPOSE: To investigate factors that may influence successful correction of hypotony in a consecutive series of patients with cyclodialysis clefts repaired surgically over a 10-year period. DESIGN: Retrospective interventional case series. METHODS: Interventional case series of consecutive patients with cyclodialysis clefts and hypotony treated surgically after failure of conservative treatment. RESULTS: Eighteen patients (18 eyes) of mean (SD) age 48.3 (15.8) years at the time of surgery were included (16 male, 2 female). All were diagnosed using gonioscopy, usually assisted with intracameral viscoelastic injection. Imaging used in three cases was not found to be sufficiently precise to plan surgical intervention, without prior gonioscopic cleft visualisation. The intraocular pressure (IOP) was restored in nine cases (50%) after one procedure with a postoperative IOP (mean ± SD) of 13.6 ± 4.5 mm Hg (6/11 who had cyclopexy as a first procedure and 3/6 who had cryopexy). 2-3 procedures were required in the remaining nine patients. There was a trend towards the use of cyclopexy for larger clefts and cryopexy for smaller clefts (NS). We observed a trend for a lower likelihood of successful closure of larger clefts after one intervention. Two eyes that had cyclopexy required later IOP-lowering surgery to achieve IOP control. CONCLUSIONS: Most clefts were closed with one procedure. A trend towards larger cleft size as a preoperative risk factor for failure to achieve closure with one procedure was observed. In this series, imaging was not found to be sufficiently precise to replace viscoelastic-assisted gonioscopy in the diagnosis and evaluation of cyclodialysis clefts.

Computer assisted evaluation of retinal vessels tortuosity in Fabry disease.

PURPOSE: Fabry Disease (FD) is a rare X-linked metabolic disorder characterized by diffuse deposition of sphingolipids in many tissues. Retinal vessel tortuosity is a common ocular manifestation in FD and may represent a useful marker for the disease. Unfortunately its clinical evaluation is poorly reproducibile and alternative means of evaluation may be of interest. We tested a new semi-automatic software measuring retinal vessel tortuosity from eye fundus digital images in a group of FD patients. METHODS: Observational case-control study evaluating four mathematical parameters describing tortuosity (relative length, sum of angle metric [SOAM], product of angle distance [PAD], triangular index) obtained from fundus pictures of 35 FD patients and 35 age-matched controls. Only the right eye was considered in order to reduce bias. Mann-Whitney test was used to compare the FD group versus the control group, males versus females and patients with versus without clinically identified retinal vessels tortuosity in the FD group. Linear regression analysis was performed on a subgroup of patients to evaluate the possible association of retinal vessels tortuosity parameters with age and with markers of systemic disease's progression. RESULTS: Three parameters (SOAM, PAD and triangular index) were significantly higher in FD patients in comparison with the controls (p < 0.0001, p = 0.001, p = 0.002 respectively). In the FD group the same three parameters showed higher values in hemizygous males than in heterozygous females ((p < 0.0001, p = 0.002, p < 0.0001 respectively). CONCLUSION: A computer assisted analysis of retinal vasculature demonstrated an increased vessels tortuosity in FD patients. The technique might be useful to establish disease severity and monitor its progression.

A non-healing corneal ulcer as the presenting feature of type 1 diabetes mellitus: a case report.

INTRODUCTION: Diabetic keratopathy is a rare complication of diabetes mellitus. This case illustrates the importance of checking blood sugar levels of patients with non-healing corneal ulcers to rule out the possibility of undiagnosed diabetes mellitus. CASE PRESENTATION: We report the unusual case of a 24-year-old southeast Asian woman who presented with a sterile corneal ulcer to our hospital and later was found to be diabetic after a prolonged hospital stay. Despite all efforts, the corneal ulcer had failed to heal until treatment for previously undiagnosed diabetes was started. The sterile corneal ulcer began to heal once blood sugar levels began to normalize. CONCLUSIONS: Diabetic keratopathy is a rare complication of diabetes mellitus and needs to be considered as a diagnosis in younger patients with non-healing sterile corneal ulcers. Blood sugar levels should be checked in these cases for undiagnosed diabetes mellitus.

Cyclodialysis cleft: causes and repair.

PURPOSE OF REVIEW: To report the most recent developments in the diagnosis and management of cyclodialysis clefts. RECENT FINDINGS: Cyclodialysis clefts are rare. The most common reason for presentation is blunt-ocular trauma followed by various iatrogenic interventions. Diagnosis is particularly challenging and various new noninvasive techniques have been described to facilitate this process, such as ultrasound biomicroscopy (UBM) and the anterior segment OCT (AS-OCT). The management of cyclodialysis clefts should be conservative initially followed by a variety of nonsurgical and surgical modalities to achieve closure. SUMMARY: The management of cyclodialysis clefts requires a step-wise approach. Initially, it is of particular importance to identify the full extent and location of the cleft as in some cases more than one cleft may be present requiring a variety of nonsurgical and surgical interventions. Nonincisional interventions include the application of various lasers and cryotherapy in the vicinity of the cleft. The traditional approach of direct cyclopexy has more recently been complemented by recent reports of employing modified external plombage procedures, vitrectomy and gas assisted endotamponade. There are insufficient studies formally evaluating these techniques to be able to assess their safety and efficacy.

Exposure to Euphorbia lathyris latex resulting in alkaline chemical injury: a case report.

INTRODUCTION: We report the case of a patient with extreme pain following accidental exposure to the latex of Euphorbia lathyris. CASE PRESENTATION: A 76-year-old Caucasian woman attended the ophthalmology department with acute severe bilateral eye pain. This occurred immediately after having pulled a weed out of her garden with her bare hands. She recalled having subsequently rubbed her eyes. The offending plant, was brought into hospital and was identified as the Caper Spurge (Euphorbia lathyris). Her ocular pH was alkaline (pH 9). After copious irrigation, the pH normalised. She was treated with topical steroids, cycloplegics, lubricants and opioid oral analgesia. Three days later, she was symptom-free and her vision had returned to normal. CONCLUSION: Exposure to Caper spurge latex is a rare cause of keratoconjunctivitis. It can, however, potentially lead to corneal ulceration, anterior uveitis and rarely blindness. Treatment remains largely empirical. Exposure to the milky latex can result in extreme pain requiring prompt treatment. The use of goggles and gloves is recommended when handling this plant.

Ocular metastases from HER2 positive breast carcinoma and the response to combination therapy with Paclitaxel and Trastuzumab: a case report.

PURPOSE: Breast cancer is the most common tumour to metastasize to the uveal tract. The mean survival period after diagnosis of metastasis to the eye, ranges from 10 to 32 months. However, recent advances in therapy including the use of monoclonal antibody therapy, will hopefully improve treatment outcomes and prolong survival rates. METHODS: We report a case of a 45 year old woman with a HER2 positive breast cancer, who developed two metastatic lesions in the left choroid, and the left optic nerve sheath. She underwent treatment with a combination of chemotherapy (Paclitaxel) and anti-HER2 monoclonal antibodies (Trastuzumab). RESULTS: Nine months after treatment, a B-scan showed resolution of the superior choroidal focus, as well as absence of blood flow within the optic nerve sheath. The inferonasal lesion was still present but the dimensions were reduced. CONCLUSION: The patient underwent a combined treatment of chemotherapy and Trastuzumab to increase the response rate. Trastuzumab is a humanized monoclonal antibody, which binds to the extracellular segment of the HER2/neu receptor. Nine months following the therapy her vision was stable, whilst one focus of the tumour in the affected eye, had regressed. The favourable response highlights the significant impact of this new therapy, as an alternative to external beam radiotherapy in patients with ocular metastasis from HER2 (+) breast cancer.

Juxtapapillary cytomegalovirus retinitis with optic neuritis.

A 49-year-old man with AIDS developed acute monocular visual loss and an ipsilateral swollen optic disc with a large right relative afferent pupillary defect, a nerve fiber bundle visual field defect, and a peripapillary retinal infiltrate. Lumbar puncture disclosed cytomegalovirus (CMV) DNA on polymerase chain reaction (PCR). Treatment with oral valganciclovir produced complete resolution of the visual deficits and the fundus abnormality. This case differs from previously reported cases of CMV optic neuritis in which visual function has been irreversibly lost.

Optic atrophy, necrotizing anterior scleritis and keratitis presenting in association with Streptococcal Toxic Shock Syndrome: a case report.

INTRODUCTION: We report a case of optic atrophy, necrotizing anterior scleritis and keratitis presenting in a patient with Streptococcal Toxic Shock Syndrome. CASE PRESENTATION: A 43-year-old woman developed streptococcal toxic shock syndrome secondary to septic arthritis of her right ankle. Streptococcus pyogenes (b-haemolyticus Group A) was isolated from blood cultures and joint aspirate. She was referred for ophthalmology review as her right eye became injected and the pupil had become unresponsive to light whilst she was in the Intensive Therapy Unit (ITU). The iris appeared atrophic and was mid-dilated with no direct or consensual response to light. Three zones of sub-epithelial opacification where noted in the cornea. There where extensive posterior synechiae. Indirect ophthalmoscopy showed a pale right disc. The vision was reduced to hand movements (HM). A diagnosis of optic atrophy was made secondary to post-streptococcal uveitis. She subsequently developed a necrotizing anterior scleritis. CONCLUSION: This case illustrates a previously unreported association of optic atrophy, necrotizing anterior scleritis and keratitis in a patient with post-streptococcal uveitis. This patient had developed Streptococcal Toxic Shock Syndrome secondary to septic arthritis. We recommend increased awareness of the potential risks of these patients developing severe ocular involvement.

Ocular manifestations of Fabry's disease: data from the Fabry Outcome Survey.

BACKGROUND: Fabry's disease is an X-linked lysosomal storage disorder characterised by deficient activity of the enzyme alpha-galactosidase A. AIM: To study eye abnormalities in patients with Fabry's disease in the Fabry Outcome Survey (FOS). METHODS: This is the largest study of ocular manifestations in patients with Fabry's disease. In all, 173 of the 688 patients enrolled in FOS underwent a detailed ophthalmic examination, with a special focus on abnormalities of the cornea, lens, conjunctival and retinal vessels. RESULTS: Cornea verticillata was reported in 76.9% of females and 73.1% of males; vessel tortuosity was observed in 21.9% of females and in 48.7% of males. Fabry cataract was recorded in 9.8% of females and in 23.1% of males. Cornea verticillata was therefore the most frequently reported ophthalmic abnormality in Fabry's disease in both hemizygotic males and heterozygotic females. Tortuous conjunctival and retinal vessels and Fabry cataract were more frequently found in males than in females. Vessel tortuosity was more frequently observed in patients with a higher severity score and greater impairment of renal and cardiac function, suggesting that it may be associated with a more severe disease. The youngest patient with ocular changes was 3 years old. The prevalence of tortuous vessels in males increased with age. CONCLUSION: The presence of cornea verticillata is a useful aid in the diagnosis of Fabry's disease, as it is often present at the time of diagnosis. Vessel tortuosity may have some predictive value for systemic involvement.

Lamellar macular hole as the presenting feature in a child with Coats' disease.

We report a case of Coats' disease and lamellar macular hole in a 10-year-old boy who presented with blurring of vision in his left eye. This is the first reported case of Coats' disease presenting with a lamellar macular hole in a child.

The value of visual evoked potentials in the evaluation of periorbital hemangiomas.

PURPOSE: To present a case of a child with an extensive facial hemangioma in whom the suspicion of intracranial involvement was raised by visual evoked potential (VEP) evaluation. DESIGN: Observational case report. METHODS: VEP analysis was performed using flash and pattern stimuli to determine whether treatment intervention other than occlusion therapy was needed. RESULTS: VEP analysis indicated a marked transoccipital asymmetry in the flash and pattern VEP suggestive of right hemispheric dysfunction. Neuroimaging showed intracranial extension of the hemangioma. CONCLUSION: The VEP evaluation is a noninvasive technique performed in the awake child. This case highlights the usefulness of VEP analysis in the management of extensive periocular capillary hemangiomas and its use as a modality to identify potential intracranial involvement. Large plaquelike facial capillary hemangiomas may have possible underlying dural involvement suggestive of PHACES syndrome.

Conjunctival lymphoid hyperplasia presenting with bilateral panuveitis.

PURPOSE: To report on a case of bilateral conjunctival lymphoid hyperplasia presenting with bilateral panuveitis in a female patient. DESIGN: Observational case report. METHODS: In this case excisional biopsy indicated lymphoid tissue. Immunohistochemistry showed a follicular architecture with appropriate zoning of B and T lymphocytes. RESULTS: Causes of panuveitis were considered and excluded based on a detailed history, physical examination, and laboratory investigations. The bilateral panuveitis resolved on topical corticosteroids and visual acuity returned to normal. CONCLUSIONS: Ocular adnexal lymphoproliferative lesions consist of a spectrum of disease entities, including reactive lymphoid hyperplasia, atypical lymphoid hyperplasia, and lymphoma. There are no established clinical criteria to differentiate between these lesions. This case highlights the importance of a thorough ophthalmic examination in these patients, as an intra-ocular inflammatory process may accompany the conjunctival findings. Furthermore, we advocate periodic follow-up examinations because of the small potential risk of developing ocular or systemic lymphoma in these patients.

Unilateral keratoconus in a child with chronic and persistent eye rubbing.

PURPOSE: To report a case of unilateral keratoconus in a 7- year-old female, secondary to chronic persistent eye-rubbing in the absence of any systemic condition. DESIGN: Observational case report. METHODS: In the case of this child, no organic cause was found to explain the onset of keratoconus other than persistent eye-rubbing in the affected eye. RESULTS: Nocturnal eye padding was instigated and the condition stabilized. She was reviewed over a 2-year period of follow-up. CONCLUSIONS: Eye-rubbing has been implicated in the pathogenesis of keratoconus. Eye-rubbing is also often a feature of a number of conditions linked to keratoconus such as Down syndrome, atopic keratoconjunctivitis, mental retardation, and Lebers' congenital amaurosis. There are a number of reports linking eye-rubbing and keratoconus in children, typically in relation to these conditions. However, in this case the keratoconus was secondary to chronic and persistent eye-rubbing in a healthy child.