Imported pythium insidiosum keratitis after a swim in Thailand by a contact lens-wearing traveler.

A 30-year-old woman with a history of contact lens wear and exposure to swimming pool water in Thailand presented with a non-responsive, progressive corneal ulcer of the right eye. Confocal microscopy evidenced septate linear branching structures, raising suspicion of fungal keratitis. She was promptly treated with topical antibiotics and both topical and intravenous caspofungin plus voriconazole. Worsening of the clinical picture after 1 month of intensive medical therapy led to a large therapeutic penetrating keratoplasty being performed. Corneal cultures grew a mold-like organism, which was identified by sequencing as Pythium insidiosum, an aquatic oomycete. After 4 years of follow-up, the graft exhibits no infection relapse, but graft transparency has been lost after two rejection episodes. Keratoplasty combined with antifungal treatment may offer a cure to P. insidiosum keratitis, although long-term preservation of corneal transparency is difficult to obtain.

Appearance of regressing drusen on optical coherence tomography in age-related macular degeneration.

OBJECTIVE: To describe and interpret a multilaminar sub-retinal pigment epithelium (RPE) intense hyper-reflectivity observed in vivo in eyes clinically diagnosed with regressing drusen. DESIGN: Observational case series. PARTICIPANTS: Twenty-three consecutive patients clinically diagnosed with regressing calcific drusen due to nonneovascular age-related macular degeneration (AMD). METHODS: Patients were submitted to confocal scanning laser ophthalmoscopy (cSLO) fundus imaging and "eye-tracked" spectral-domain optical coherence tomography (SD-OCT). MAIN OUTCOME MEASURES: Localization and possible origin and composition of the multilaminar sub-RPE hyperreflectivity. RESULTS: Thirty eyes of 23 consecutive patients (8 male and 15 female; mean age, 82.7±10.1 years) showing on SD-OCT an intense multilaminar sub-RPE hyperreflectivity, which matched with regressing calcific drusen as visualized by cSLO infrared (IR) and MultiColor (Heidelberg Engineering, Heidelberg, Germany) images, were included in this study. The multilaminar hyperreflectivity was found to localize to beneath the RPE and above the outer Bruch's membrane (oBM) layer. A mean of 1.2 multilaminar sub-RPE hyperreflectivities per SD-OCT scan were identified by 2 readers. The SD-OCT analysis allowed the 2 readers to describe 3 different types of sub-RPE hyperreflectivity. "Type 1" laminar/multilaminar hyperreflectivity (found in 24 scans of 12 eyes) was characterized by an intense signal originating from what we interpreted as the inner Bruch's membrane (iBM) layer. "Type 2" multilaminar hyperreflectivity (found in 130 scans of 27 eyes) was characterized by an intense signal originating from the oBM layer. "Type 3" multilaminar fragmented hyperreflectivity (found in 22 scans of 11 eyes) was characterized by an intense signal originating from what we interpreted as both the iBM and the oBM, showing different degrees of fragmentation. CONCLUSIONS: We describe a novel SD-OCT finding appearing as multilaminar sub-RPE intense hyper-reflectivity observed in vivo in eyes with regressing drusen. This multilaminar sub-RPE hyperreflectivity could be interpreted as layers of lipid mineralization (membranous debris also called "lipoprotein-derived debris" developing calcification), internal and external to the basement membrane, with different degrees of fragmentation.

Cystoid macular degeneration in exudative age-related macular degeneration.

PURPOSE: To investigate the prevalence and clinical significance of cystoid macular degeneration in eyes that underwent intravitreal ranibizumab injections for exudative age-related macular degeneration. DESIGN: Retrospective, interventional case series. METHODS: We reviewed the charts of 56 consecutive patients (19 male, 37 female; mean age ± standard deviation, 80.81 ± 4.8 years) with exudative age-related macular degeneration who received the last intravitreal ranibizumab injection at least 6 months before and were judged to have a fibroatrophic scar without signs of progression by fluorescein angiography or spectral-domain optical coherence tomography. Main outcome measures were the estimated prevalence and clinical significance of cystoid macular degeneration. RESULTS: Twenty-two eyes showed various combinations of degenerative pseudocysts, whereas 34 eyes did not show any pseudocysts. The 95% confidence interval for the prevalence estimate was 36.98% to 41.02%. Degenerative pseudocysts appeared square-shaped, did not change their overall appearance over time, and were located just below the internal limiting membrane in 11 eyes (50%), in the inner nuclear layer in 16 eyes (72.7%), in the outer nuclear layer in 8 eyes (36.3%), and in all the retinal layers in 6 eyes (27.2%). Best-corrected visual acuity improved in eyes with and without degenerative pseudocysts and decreased significantly in eyes with degenerative pseudocysts (P = .03). Mean central macular thickness decreased significantly (P < .001) to 324.1 µm and to 328.2 µm in eyes and without degenerative pseudocysts, respectively. CONCLUSIONS: Cystoid macular degeneration represents a well-distinguished clinical entity that may be detected in exudative age-related macular degeneration eyes showing a posttreatment fibroatrophic scar and should not be considered as a manifestation of choroidal neovascularization activity.

Angiography features of early onset drusen.

AIMS: Drusen are rarely observed in patients < 50 years of age. Two types of early onset drusen (EOD) are commonly described: basal laminar drusen (BLD) and drusen associated with Malattia Leventinese (ML). Our purpose was to classify the phenotype of EOD on the basis of fundus examination, and fluorescein angiography (FA) and indocyanine green angiography (ICGA) features. METHODS: We performed a prospective study including 48 consecutive EOD patients. All of them had a complete ophthalmologic examination including FA and ICGA. RESULTS: BLD (67%) were extremely hyperfluorescent on FA and ICGA. ML (10%) was characterised by a combination of small radial and large round drusen with differences in staining in both FA and ICGA. We evidenced a third type of EOD (23%) harbouring an aspect of large colloid drusen (LCD), mildly hyperfluorescent in the early phases of FA, with a progressive staining in late phases. In intermediate and late phases of ICGA, LCD presented as hypofluorescent dot surrounded by a hyperfluorescent halo bordered by a thin hypofluorescent ring. CONCLUSION: Three types of EOD are distinguished by their FA and ICGA features. We report a new kind of juvenile drusen, distinct from BLD and ML, named LCD, associated with a good vision and absence of complications.

Primate model of uveoretinitis and vasculitis/experimental autoimmune uveoretinitis induced in cynomolgus monkeys by retinal s antigen.

PURPOSE: We aimed to describe the clinical and angiographic changes in an experimental model of autoimmune uveoretinitis and vasculitis in primates. METHODS: Six cynomolgus monkeys received a single subcutaneous immunization with 100 microg of human S antigen with complete Freund's adjuvant. RESULTS: All the animals had a bilateral long-term disease occurring usually in 1 eye approximately 4 weeks after immunization, the second eye being involved 1-5 weeks later. A cyclic course of the disease could be demonstrated by repeated fundus fluorescein angiograms. The initial and principal manifestation consisted in retinal vascular sheathing affecting veins and venules. The more severe forms showed areas of posterior uveoretinitis, dense vitritis and anterior uveitis. CONCLUSION: A single systemic injection of pure human retinal S antigen could induce a chronic and recurrent ocular disease similar to human retinal vasculitis.

Familial uveal melanoma: a report on two families and a review of the literature.

PURPOSE: A total of 69 families affected by uveal melanoma have been reported in the literature. This report describes two additional families. In addition to presenting these cases, which constitute exceptions, the paper reviews the literature. MATERIAL AND METHODS: Two families, each with two affected members, were analysed in this retrospective study. The pedigree of each family has been pieced together. RESULTS: Considering the low incidence of familial uveal melanoma in the general population, it seems unlikely that inherited genetic factors are responsible for the condition; this question remains difficult to resolve. DISCUSSION: The characteristics of each family history are described and compared with the literature data. The mode of possible inheritance is discussed. Both the histopathology and anatomical location are studied, after which we discuss the body of evidence to establish whether there is an inherited cancer predisposition syndrome in patients with familial uveal melanoma. CONCLUSION: The statistical likelihood of such an uncommon tumour occurring independently in two or more family members leads us to believe that some cases of familial uveal melanoma may go unrecognized, and that reports on too few families have been published worldwide to prove the existence of a single mendelien gene. However, appropriate tissue samples, such as blood and tumour samples, should be obtained and conserved for present or future cytogenetic and molecular genetic studies.