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Aim: To describe the multimodal imaging findings of pigmented paravenous retinochoroidal atrophy. Methods: A 23-year-old female presented to us for a routine ocular examination. She had a best-corrected visual acuity of 6/6 in both eyes. Anterior segment examination was unremarkable. Fundus examination showed pigmentary changes along the retinal vasculature extending from mid periphery to post-equatorial retina suggesting a diagnosis of pigmented paravenous retinochoroidal atrophy. Swept-source optical coherence tomography of the macula showed choriocapillaris thinning at the mid periphery whereas coherence tomography angiography at the mid periphery showed a relatively normal choriocapillaris vasculature in the early stage of the disease. Conclusion: A relatively normal choriocapillaris structure was seen on ocular coherence tomography angiography which could have been due to a milder form of the disease in a young patient.

Background: Pigmented Paravenous Chorioretinal Atrophy (PPCRA) is a rare and predominantly sporadic form of chorioretinal atrophy. Ocular and systemic inflammation has been considered a pos...

Purpose To report an atypical case of unifocal, unilateral pigmented paravenous retinochoroidal atrophy (PPRCA) characterized by thickening and cystic degeneration of the retinal nerve fiber layer (RNFL). Observations A 79-year old Asian woman presented with a large area of atrophic, pigmented change along the inferior arcade of her right eye. She denied nyctalopia and any other visual complaints. Visual acuity was 20/40 in both eyes and visual fields were significant for a large absolute peripheral scotoma superiorly in the affected eye corresponding to the atrophic area. Spectral domain optical coherence tomography through the lesion showed loss of choroid except for largest Haller's layer vessels, significant retinal pigment epithelium atrophy with migration and pigment clumping, outer retinal layer loss and RNFL thickening with cystic degeneration. Fundus autofluorescence imaging showed a large area of hypoautofluorescence corresponding to the area of atrophy. Full field electroretinogram demonstrated normal scotopic response and reduced photopic response in the right eye. Conclusions and importance PPRCA is typically bilateral and symmetric, affecting primarily the outer retina and choroid. However, in rare cases, this disease can present unilaterally and/or unifocally, with degeneration extending to the inner retinal layers.

As the name suggests, in pigmented paravenous chorioretinal atrophy (PPCRA), patches of chorioretinal atrophy and pigment clumping are distributed along the veins (Figs. 22.1 and 22.2). In most cases, the retinal vessels, macula, and optic discs are normal, and the disease is usually nonprogressive.

Amblyopia screening is often either costly or laborious. We evaluated the Canon Powershot TX1 (CPTX1) digital camera as an efficient screener for amblyogenic risk factors (ARF). We included 138 subjects: 84-amblyopes and 54-normal. With the red-eye-reduction feature off, we obtained Bruckner reflex photographs of different sized crescents which suggested anisometropia, while asymmetrical brightness indicated strabismus; symmetry implied normalcy. Eight sets of randomly arranged 138 photographs were made. After training, 8 personnel, marked each as normal or abnormal. Of the 84 amblyopes, 42 were strabismus alone (SA), 36 had anisometropia alone (AA) while six were mixed amblyopes (MA). Overall mean sensitivity for amblyopes was 0.86 (95% CI: 0.83-0.89) and specificity 0.85 (95% CI: 0.77-0.93). Sub-group analyses on SA, AA and MA returned sensitivities of 0.86, 0.89 and 0.69, while specificities were 0.85 for all three. Overall Cohen's Kappa was 0.66 (95% CI: 0.62-0.71). The CPTX1 appears to be a feasible option to screen for ARF, although results need to be validated on appropriate age groups.

In this retrospective case series, we studied the predisposing factors, causative organisms, clinical spectrum, and outcomes of 12 cases of culture-proven infectious scleritis. Nine of 12 patients had a history of preceding trauma (surgical or accidental). Past surgical history included small-incision cataract surgery (4), pterygium surgery (1), and trabeculectomy (1). Six patients had multifocal scleral abscesses due to Pseudomonas, Klebsiella, or Nocardia. Only 2 patients retained useful vision (>6/18). A poor visual acuity at presentation usually resulted in a worse visual outcome (P = 0.005). Four eyes developed phthisis. The addition of surgical intervention did not result in a significantly better visual outcome than medical management alone (P = 0.209), but resulted in a higher globe preservation rate (P = 0.045). Therefore, we concluded that infection must be ruled out in cases of scleritis with preceding history of trauma, and aggressive surgical intervention improves the anatomical outcome but does not change the visual outcome.

Congenital eyelid imbrication syndrome (CEIS) is an extremely rare, benign, transient, self-limiting eyelid malposition disorder. The classic triad of signs in patients with a CEIS consists of bilateral upper eyelids overriding the lower eyelids when child was in sleep, bilateral medial and lateral canthal tendon laxity and tarsal conjunctival hyperemia. We report a third case of congenital combined eyelid imbrication and floppy eyelid syndrome in healthy neonate that was resolved within a week with conservative treatment.

The aim of this study is to investigate fundus autofluorescence (FAF) as well as fluorescein angiography (FA), indocyanine green angiography (IA), and optical coherence tomography (OCT) in a patient with pigmented paravenous chorioretinal atrophy (PPCRA). A funduscopic examination revealed chorioretinal atrophy along the paravenous area in both eyes. A marked bone spicule pigment clumping together with the atrophy was noted left eye. FA and IA showed a window defect and hypofluorescence, respectively, which exclusively corresponds to the atrophic area along the retinal vein area and the optic disc both eyes. FAF revealed geographic hypofluorescence along the paravenous and supranasal retinal areas. Hyperfluorescence was noted, which comparatively surrounded the hypofluorescence in the peripheral paravenous distribution. Hypofluorescence detected by FAF corresponded to the areas of retinal thinning and atrophy detected by OCT and FA. FAF is a useful examination in PPCRA, which can noninvasively demonstrate the distribution of deficit and dysfunction of retinal pigment epithelium.

Purpose: To describe a case of bilateral pigmented paravenous chorioretinal atrophy. Methods: Observational case report. Results: A 50-year-old female patient complained of right eye pain and decreased near visual acuity. She had a best-corrected visual acuity of 20/20 in both eyes. In the posterior segment, there were atrophic changes along retinal vessels, and bony spicule pigmentation was observed in a paravenous distribution. There were some abnormal changes in the electroretinogram and electrooculogram in both eyes. Conclusions: A diagnosis of bilateral pigmented paravenous chorioretinal atrophy was made.

Two patients with pigmented paravenous chorioretinal atrophy are presented. Both patients had chorioretinal atrophy with pigment clusters located in the paravenous areas, and one of the patients had macular affection. This patient had exotropia and juvenile cataract, the other patient had senile cataract. Electroretinography showed decreased, but not totally extinguished potentials. Automatic and manual perimetry revealed relative and absolute scotomas corresponding to the atrophic paravenous areas. To our knowledge this is only the sixth report of pigmented paravenous chorioretinal atrophy with macular affection.

Pigmented paravenous chorioretinal atrophy (PPCRA) is an uncommon retinal disorder of unknown etiology that is neither well understood nor classified. We report an atypical case of PPCRA, associated with Coat's like response (CLR) in a 64-year-old man of Asian origin. Both the eyes were involved, though asymmetrically.

Systemic imaging of patients with suspect ocular tuberculosis include chest X-rays and computed tomography (CT) scans. Reports have suggested a role for 18 fluorodeoxyglucose-positron emission tomography/CT (FDG-PET/CT) scans. We report on the clinical utility of 18 FDG PET/CT in two patients. Case 1: A 38-year-old female patient presented with recurrent anterior uveitis. A 18 FDG-PET scan revealed metabolically active supraclavicular and chest lymph nodes. An aspiration cytology of the cervical lymph node revealed caseating granulomas suggestive of tuberculosis. Case 2: A 58-year-old female patient presented with recurrent anterior uveitis. A 18 FDG-PET scan revealed metabolically active lymph nodes in the neck. A biopsy of the cervical lymph node revealed epithelioid granulomas suggestive of tuberculosis. Both patients were started on standard antitubercular therapy with a subsequent marked reduction of activity. PET/CT scans may suggest the sites of safe high-yield biopsies.

We are presenting the initial results of inverted internal limiting membrane (ILM) flap technique for large macular hole. Five eyes of five patients with large diameter macular hole (>700 μm) were selected. All patients underwent inverted ILM flap technique for macular hole. Anatomical closure and functional success were achieved in all patients. There was no loss of best-corrected visual acuity in any of the patients. Inverted ILM flap technique in macular hole surgery seems to have a better hole closure rates, especially in large diameter macular holes. Larger case series is required to assess the efficacy and safety of this technique.

WOS: 000330854300019, PubMed ID: 24088642, Unilateral extensive myelination of the peripapillary nerve fibers may be associated with anisometropic myopia, strabismus, and reduced vision. Myelination of optic nerve fibers terminate at lamina cribrosa. Yet in some patients, myelination progresses into the peripapillary retinal nerve fibers and may affect the visual acuity. In this report, we described 4 patients. All patients presented extensive peripapillary myelinated nerve fibers associated with myopic anisometropia. After routine ophthalmic and orthoptic examinations, all patients underwent treatment for amblyopia through correction with spectacles, contact lenses, and the occlusion of the good eye. Corrected visual acuity improved in 1 patient, but 3 patients had no increase in visual acuity despite treatment with full cycloplegic refraction and appropriate patching. Probably because of structural abnormalies of the macula, visual results are often disappointing with appropriate correction of the refractive error and occlusion.

Unilateral retinitis pigmentosa in a woman and pigmented paravenous chorioretinal atrophy in her daughter and son

We investigated a case of unilateral optic nerve hypoplasia using spectral domain optical coherence tomography (SDOCT). Optical coherence tomography was done on both eyes using 5-line Raster scan for the fovea to analyze the retinal nerve fiber layer thickness, inner retinal layer thickness, outer retinal layer thickness, and optic disc cube scan for the disc. Retinal nerve fiber layer thickness, inner retinal layer thickness, and outer retinal layer thickness were manually measured at 21-points of each five lines, and results were compared between both eyes. Retinal nerve fiber layer thickness and inner retinal layer thickness of optic nerve hypoplasia were significantly thinner than the opposite eye, but there was no significant difference in the thickness of the outer retinal layer between both eyes.

Rhinosporidium seeberi , till recently known as a fungus, has been reclassified as a protistan parasite. It infects humans and many animal species. The authors describe a rare case of oculosporidiosis with involvement of the lacrimal sac exhibiting features of idiopathic orbital inflammatory disease in a young male patient. Clinical features, pathophysiology, and management of lacrimal sac rhinosporidiosis have been discussed.

This report describes an atypical case of pigmented paravenous chorioretinal atrophy, associated with focal progressive peripheral retinal microangiopathy, in a 51-year-old black female. The eyes were asymmetrically involved. Although several cases have been reported with typical features of this uncommon entity, none of them have been known to be associated with retinal microangiopathy. The occurrence of such microangiopathy supports the hypothesis that damage to the retinal photoreceptors may induce retinal microangiopathy, as suggested in other clinical and experimental studies.

Six patients (three men and three women, ranging in age from 19 to 65 years) with pigmented paravenous chorioretinal atrophy, a rare disorder of unknown origin, were studied for three- to 21-year periods. The diagnosis was made on the basis of the distinctive fundus appearance of bilateral, symmetrical bone corpuscular pigment accumulation exclusively along the distribution of the retinal veins. Although the fundus abnormalities can be mild or severe, retinal function tests indicated that this is a geographic and not a generalized disorder. Central visual acuities were normal. Follow-up studies showed no evidence of ophthalmoscopic or functional deterioration in five of the patients. In the sixth (Patient 6, the most severely affected), there was a mild progression of the fundus abnormality during the 21-year follow-up period.

A 30-year-old man with pigmented paravenous chorioretinal atrophy showed, within a relatively short time, changes that documented the progressive nature of this disease. These changes included: further constriction of peripheral visual fields; more extensive and frequently confluent areas of retinochoroidal atrophy; a scalloped appearance of lesions resembling posterior gyrate atrophy; peripheral pigment clumping; and the presence of localized atrophic areas with crystal deposition in the peripheral retina.