Outer retinal tubulation and inner retinal pseudocysts in a patient with maternally inherited diabetes and deafness evaluated with optical coherence tomography angiogram.

A 47-year-old lady (index case) with diabetes and deafness showed multiple oval circumferential areas of perifoveal atrophy in both eyes. Autofluorescence revealed areas of hypoautofluorescence. Optical coherence tomography (OCT) showed depression of inner retinal surface, inner retinal hyporeflective spaces (pseudocysts), disorganization/thinning of outer retina, outer retinal tubulation, loss of external limiting membrane, ellipsoid and interdigitation zone, and thinning of the retinal pigment epithelium and choriocapillaris. The patient was evaluated using OCT angiogram. Retinal lesions of her mother (68-year-old) were very obvious on autofluorescence imaging. The result of A3243G mutation in MTTL1 gene was positive in the index case confirming the diagnosis of maternally inherited diabetes and deafness (MIDD).

Panscleritis masquerading as an attack of primary acute angle closure glaucoma.

Purpose: To report a female who presented with acute angle closure glaucoma and was found to have panscleritis on further evaluation. Method: Case report. Case description: A 50-year-old female was referred to us as a case of primary acute angle closure attack in the right eye and for laser peripheral iridotomy. She had severe pain, redness, a very shallow anterior chamber, and an intraocular pressure of 38 mmHg in the right eye. However, the fellow eye had a deep anterior chamber and the right eye also had severe chemosis, lid edema, scleral tenderness, choroidal folds, and pain during ocular movements which was limited. Ultrasound biomicroscopy showed a ciliochoroidal effusion with anterior rotation of the ciliary body. The ultrasound of the eye revealed an increased thickness of the ocular coats and subtenon fluid. A diagnosis of panscleritis causing secondary angle closure glaucoma was made. She responded well to topical atropine, and topical with systemic steroids. Conclusions: Secondary angle closure glaucoma due to panscleritis may mimic primary acute angle closure attack in a clinical setting. It is important to differentiate the two as treatment is opposite and may worsen the condition if misdiagnosed.

Recurrent retinal and choroidal ischemia in a case of ocular ischemic syndrome.

A 62 year-old male patient presented with fluctuating vision in both eyes. On fundus fluorescein angiogram, there was an area of choroidal and retinal ischemia in the left eye. The carotid evaluation revealed complete stenosis of the right internal carotid artery and 90% stenosis of the left internal carotid artery for which he underwent left modified endarterectomy. The vision and angiographic features improved after the procedure. Eleven months after the procedure, he again presented with choroidal ischemia due to recurrent left internal carotid artery blockage which improved gradually after carotid stenting.

Acquired myopia followed by acquired hyperopia due to serous neurosensory retinal detachment following topiramate intake.

PURPOSE:: To report a patient with fluctuating refraction following the use of oral topiramate. CASE REPORT:: A 38-year-old male patient was diagnosed elsewhere with sudden-onset-acquired myopia, high intraocular pressure, and bilateral angle closure glaucoma for which he underwent laser peripheral iridotomy in both eyes and was started on topical antiglaucoma medications and topical steroids following laser peripheral iridotomy. He was referred for ultrasound biomicroscopy, which showed bilateral ciliary effusion. Ultrasound of eyes revealed choroidal thickening. On further questioning, he was noted to have taken oral topiramate for 7 days, which he stopped a week before the ocular symptoms. He was started on atropine, on which the acquired myopia resolved, the anterior chamber deepened, and the intraocular pressure came down. After 4 days, he developed acquired hyperopia in the left eye. Neurosensory retinal detachment at the posterior pole was documented with optical coherence tomography. The fluorescein angiography showed few ink-blot leaks and one smokestack leak in the left eye. The neurosensory detachment resolved spontaneously with an uncorrected visual acuity of 6/6 in either eye. CONCLUSION:: A unique case of central serous chorioretinopathy following oral intake of topiramate is presented. This patient had also received laser peripheral iridotomy and topical steroids following the peripheral iridotomy.

Central retinal arterial occlusion in a patient with pyoderma gangrenosum.

A 74-year-old male presented to us with a history of vision loss for 36 hours in the right eye (RE). The RE had a visual acuity of hand movements. The fundus revealed a pale retina, cattle tracking in the retinal vessels, and a cherry-red spot at the macula. The patient was a known case of pyoderma gangrenosum (PG) and had received intravenous methylprednisolone and cyclophosphamide at the onset of visual symptoms. An emergency anterior chamber paracentesis was performed following unsuccessful attempts of ocular massage. The patient improved to 6/9 in the RE 4 months after paracentesis. The patient had an aggressive course of PG, for which he needed a combination of oral steroid, immunomodulator therapy and biologicals. An association between central retinal arterial occlusion and PG has not been reported before, according to the best of authors' knowledge.

Pearls and pitfalls of optical coherence tomography angiography in the multimodal evaluation of uveitis.

BACKGROUND: Optical coherence tomography angiography (OCTA) employs a novel imaging algorithm that detects the amplitude or phase decorrelation of blood cell movement. It thus provides a flow map with depth-resolved visualization of the various vascular layers in the posterior pole of the eye including the retina capillary plexus and the choroid. In the past 3 years, the number of research papers on the subject of OCTA in retinal diseases has grown exponentially including important applications in the field of uveitis. While the study of OCTA in uveitic diseases has gained remarkable relevance worldwide, interpretation can be challenging, and many limitations exist in optimally using this advanced system in uveitic eyes. The aim of this review is to describe the many significant applications of OCTA in uveitis disorders and to outline the various limitations that can confound interpretation and support uveitis specialists in the integration of OCTA in the multimodal imaging approach to inflammatory diseases. MAIN BODY: Unlike conventional angiography that can dynamically detect inflammation and leakage of dye from retinal vessels, OCTA provides other important biomarkers of inflammation. Detailed microvascular reconstruction of normal and abnormal blood vessels and quantitative evaluation are advantages of OCTA analysis. OCTA can therefore non-invasively detect choroidal neovascularization that may complicate inflammatory disorders, and with remarkable depth-resolved capability, OCTA can identify and quantitate flow loss as a manifestation of ischemia and/or inflammation. The areas of flow deficit on OCTA at the level of the inner choroid often co-localize with hypofluorescent lesions with indocyanine green angiography. These regions of presumed choriocapillaris ischemia may occur in placoid disorders. Space-occupying granulomas may occur in disorders such as sarcoid and may or may not co-localize with choriocapillaris ischemia on ICG angiography. Blocking or shadowing artifacts should be excluded when evaluating inner choroidal abnormalities with OCT angiography. Fundus autofluorescence may assess the metabolic function of the retinal pigment epithelium (RPE) and the viability of the overlying photoreceptors and thus the activity of inflammation associated with uveitic lesions. The photoreceptors are physiologically maintained by the diffusion of oxygen from the choriocapillaris below and, to a lesser extent, from the deep retinal capillary plexus above. The depth-resolved capability of OCTA may therefore provide additional significant microvascular information about these vascular layers that may be driving the development of hyper-autofluorescent RPE inflammation and photoreceptor loss. CONCLUSIONS: The implementation of OCTA in the evaluation and management of uveitis disorders is being spurred by our greater knowledge and understanding of its application. In order to take full advantage of this exciting new imaging modality, however, uveitis specialists must understand the limitations of interpretation and potential artifact-related pitfalls in assessment and should continue to support evaluation with multimodal imaging to best optimize diagnoses and treatment of inflammatory diseases.