Keratoconus and granular dystrophy

Concurrent bilateral keratoconus and granular dystrophy is reported in a 32 year old patient with decreased vision. Initially contact lenses were attempted unsuccessfully to treat the conditions. There are a handful of other reports of these combined pathologies in the literature, and the likelihood of a chance cause or possible genetic linkage between the conditions is discussed

Supplementary Material for: Acute Myeloid Leukaemia Presenting as Gaze Palsy

The most frequent initial ocular manifestation of acute myeloid leukaemia (AML) is retinal involvement. Here, we report an unusual case of AML associated with a pontine chloroma presenting with gaze palsy as the initial symptom. A 77-year-old Caucasian man presented to the Eye Casualty complaining of a one-day history of blurred vision. On examination, his face was turned to the left, both eyes were fixed in dextroversion and the patient demonstrated left gaze palsy associated with left motor neurone VII palsy. Baseline blood investigations revealed leucocytosis with 60% circulating myeloblasts. A bone marrow biopsy confirmed the diagnosis of myelomonocytic leukaemia. A CT scan showed a well-circumscribed lesion in the dorsal pons, most likely representing a chloroma. Chloromas or myeloblastomas related to AML are localised extramedullary tumours composed of leukaemic myeloid cells. Chloromas may be present at the time of the initial diagnosis of leukaemia or may precede the diagnosis by 1 month to 2 years; however, their occurrence in the central nervous system is rare, comprising 1–6% of all chloromas. This case illustrates the many different ways that AML can manifest itself in the eyes, and ophthalmologists should be aware of the great variety of presenting symptoms in undiagnosed AML

Interventions for the treatment of uveitic macular edema: a systematic review and meta-analysis

Rushmia Karim,1 Evripidis Sykakis,2 Susan Lightman,3 Samantha Fraser-Bell4 1Faculty of Medicine, University of Sydney, Camperdown, NSW, Australia; 2Department of Ophthalmology, Whipps Cross University Hospital, 3UCL Institute of Ophthalmology and Moorfields Eye Hospital, London, UK; 4University of Sydney, Clinical Ophthalmology and Eye Health, Sydney Adventist Hospital Clinical School, Sydney, NSW, Australia Background: Uveitic macular edema is the major cause of reduced vision in eyes with uveitis. Objectives: To assess the effectiveness of interventions in the treatment of uveitic macular edema. Search strategy: Cochrane Central Register of Controlled Trials, Medline, and Embase. There were no language or data restrictions in the search for trials. The databases were last searched on December 1, 2011. Reference lists of included trials were searched. Archives of Ophthalmology, Ophthalmology, Retina, the British Journal of Ophthalmology, and the New England Journal of Medicine were searched for clinical trials and reviews. Selection criteria: Participants of any age and sex with any type of uveitic macular edema were included. Early, chronic, refractory, or secondary uveitic macular edema were included. We included trials that compared any interventions of any dose and duration, including comparison with another treatment, sham treatment, or no treatment. Data collection and analysis: Best-corrected visual acuity and central macular thickness were the primary outcome measures. Secondary outcome data including adverse effects were collected. Conclusion: More results from randomized controlled trials with long follow-up periods are needed for interventions for uveitic macular edema to assist in determining the overall long-term benefit of different treatments. The only intervention with sufficiently robust randomized controlled trials for a meta-analysis was acetazolamide, which was shown to be ineffective in improving vision in eyes with uveitic macular edema, and is clinically now rarely used. Interventions showing promise in this disease include dexamethasone implants, immunomodulatory drugs and anti-vascular endothelial growth-factor agents. When macular edema has become refractory after multiple interventions, pars plana vitrectomy could be considered. The disease pathophysiology is uncertain and the course of disease unpredictable. As there are no clear guidelines from the literature, interventions should be tailored to the individual patient. Keywords: uveitic macular edema, uveiti

An in depth analysis of histopathological characteristics found in keratoconus

AIMS: The aims of this study were to re-assess the histopathology of the disease by introducing more modern measuring techniques and to determine if axial stromal thinning, which is the most apparent change, is related to the other alterations observed.METHODS: Recipient keratoconic corneas from 36 patients following corneal transplantation were studied. Haematoxylin and eosin (H&amp;E) and periodic acid-Schiff (PAS) staining were used to identify breaks in Bowman's layer and Descemet's membrane. Thickness of corneal layers was measured by Leica QWin software. Epithelial and stromal thickness were measured in each sample at the periphery of the corneal button and at the area of maximal stromal thinning. The presence of apoptotic cells in Bowman's layer breaks was detected by terminal deoxynucleotidyl transferase mediated dUTP-X nick end labelling.RESULTS: In all 36 corneal samples the central stroma, at the apex of the cone, was thinner than the peripheral. There was a negative correlation between central stromal and central epithelial thickness (p = 0.009). Bowman's layer breaks were found in 92% of corneas. Apoptotic cells were detected at the level of Bowman's breaks membrane. We found a positive correlation between epithelial thickness and the number of Bowman's layer breaks (p = 0.009 for central epithelial thickness and p = 0.003 for peripheral epithelial thickness). Descemet's membrane deformities were observed in 19% of corneas and central stromal thickness of these corneas was significantly less than corneas without breaks (p = 0.006).CONCLUSIONS: There are various different histopathological features associated with keratoconus and some of them are very subtle and not very well studied. Accurate measurements also suggest some correlations between them. Stromal thinning is associated with the number of breaks in Descemet's membrane, but it is the thickening of the epithelium which is associated with breaks in Bowman's layer.</p