Complete Ring Cyst of Iris Pigment Epithelium Documented by Ultrasound Biomicroscopy

The authors report a complete ring cyst of the iris pigment epithelium confirmed by ultrasound biomicroscopy. In this case, ultrasound biomicroscopy effectively excluded the differential diagnoses of ring melanoma of the iris and multiple separate cysts of the iris pigment epithelium. [Ophthalmic Surg Lasers Imaging 2009;40:495-497.

Peripheral Cone Dystrophy: Expanded Clinical Spectrum, Multimodal and Ultrawide-Field Imaging, and Genomic Analysis

Purpose. To present new clinical features, multimodal and ultrawide-field imaging characteristics of peripheral cone dystrophy (PCD), and results of laboratory and genetic investigation to decipher the etiology. Methods. Retrospective observational case-series. Results. Three patients with PCD presented with bilateral paracentral scotomas and a mean visual acuity of 20/25. All exhibited confluent macular hyperautofluorescence with a central bull’s eye lesion. Spectral-domain optical coherence tomography revealed loss of outer retinal elements, particularly the inner segment ellipsoid band and external limiting membrane, within the area of macular hyperautofluorescence. This area corresponded with a lightened fundus appearance and variable retinal pigment epithelium (RPE) abnormalities. Full field and multifocal electroretinography distinguished PCD from other photoreceptor dystrophies. Ultrawide-field imaging revealed irregular peripheral retinal lesions in a distribution greater nasally than temporally and not contiguous with the macular lesion. Functional and anatomic testing remained stable over a mean follow-up of 3 years. Laboratory investigation for causes of uveitis was negative. Whole exome sequencing identified rare variants in genes associated with macular or cone dystrophy or degeneration. Conclusions. In contrast to the original description, the funduscopic and fluorescein angiographic appearance of PCD is abnormal, although the defects are subtle. Peripheral lesions may be observed in some patients. Bilateral, symmetric, macular hyperautofluorescence associated with outer retinal atrophy that spares the fovea is a characteristic of PCD. Pathogenic variants in the same gene were not shared across the cohort, suggesting genetic heterogeneity. Further evaluation is warranted

Bartonella Henselae-Associated Neuroretinitis with Orbital Intraconal and Optic Nerve Sheath Involvement

"We present clinical, radiological and histopathological findings of a patient with neuroretinitis, with positive Bartonella henselae IgM and an indeterminate QuantiFERON-TB Gold In-Tube (QFT-GIT) test, who developed orbital intraconal and optic nerve sheath inflammation. This is the first report of intraconal inflammation prompting optic nerve sheath fenestration (ONSF) in association with B. henselae infection.