Richard H. Roe

Ocular bartonella infections.

What is now commonly known as cat scratch disease was first reported by Parinaud in 1889, when he described 3 patients with follicular conjunctivitis, regional lymphadenopathy, chronic fever, and previous contact with pets. Initially termed Parinaud’s oculoglandular syndrome (POGS), subsequent reports eventually bore out the intimate relationship between this condition and exposure to cats. Only recently has the etiologic agent for cat scratch disease been identified as Bartonella henselae, in part because of the development of biomolecular techniques allowing for the identification and diagnosis of previously unknown infectious organisms. This review discusses the basic and clinical biology of the various Bartonella species, and also summarizes the ocular complications that can accompany infection by these relatively common pathogens.

Retinal pigment epitheliopathy, macular telangiectasis, and intraretinal crystal deposits in HIV-positive patients receiving ritonavir.

Purpose: The purpose of this study was to describe the occurrence of a retinal pigment epitheliopathy associated with macular telangiectasis and intraretinal crystal deposits in three human immunodeficiency virus-positive patients receiving long-term ritonavir as part of highly active antiretroviral therapy. Methods: The patients records were reviewed. Results: The CD4 T-cell counts at presentation were 163 cells per microliter, 464 cells per microliter, and 349 cells per microliter, and viral loads were undetectable in all patients. None of the patients had a concurrent AIDS-defining illness. Other significant medical history included hyperlipidemia in one patient and a remote history of lymphoma and tuberculosis in a second patient. Initial visual acuity ranged from 20/32 to 20/400, with a median of 20/150. Anterior segment examination and intraocular pressures were normal in all eyes. Posterior segment examination revealed bilateral macular retinal pigment epitheliopathy with intraretinal crystalline deposits. No hemorrhage or cotton wool spots were seen consistent with human immunodeficiency virus retinopathy, and there was no evidence of previous or active cystomegalovirus retinitis. Fluorescein angiography revealed parafoveal telangiectasis with late leakage in two of the three patients. Optical coherence tomography showed thickening of the macula in three eyes and inner foveal cysts in two eyes. Autofluorescence performed on one patient revealed complete loss of normal retinal pigment epithelium autofluorescence corresponding to the area of retinal pigment epitheliopathy bilaterally. The only medicine common to all 3 patients was ritonavir, and the duration of ritonavir therapy before presentation was 19 months in one patient, 30 months in the second patient, and 5 years in the third patient. Conclusion: Retinal changes characterized by retinal pigment epitheliopathy, parafoveal telangiectasias, and intraretinal crystal deposits occurred in three human immunodeficiency virus-positive patients on long-term ritonavir as part of highly active antiretroviral therapy.

SOLITARY CIRCUMSCRIBED "PEARL WHITE" RETINAL MASS (SO-CALLED RETINAL ASTROCYTIC PROLIFERATION) RESIDES IN DEEP RETINA OR BENEATH RETINA: FINDINGS ON MULTIMODAL IMAGING IN 4 CASES.

Purpose: To report novel observations of previously described solitary circumscribed retinal astrocytic proliferation using spectral domain optical coherence tomography that suggests this tumor does not arise in the nerve fiber layer as initially believed, but arises within deep retinal or retinal pigment epithelial structures. Methods: Retrospective review of four cases. Results: Patient age ranged from 46 to 75 years. The tumor was pearl white or yellow-white (n = 4, 100%), located in the macula (n = 1, 25%) or macula to equator (n = 3, 75%) regions, and with mean tumor base of 1.2 mm and thickness of 0.8 mm. There were no feeding vessels, intrinsic vessels, subretinal fluid, or vitreoretinal traction. Mild surrounding retinal pigment epithelial hyperplasia and atrophy rimmed each tumor (n = 4, 100%). Fluorescein angiography depicted the mass with early hypofluorescence (n = 3/3, 100%) and late hypofluorescence (n = 2/3, 67%). Spectral domain optical coherence tomography demonstrated the mass with an abruptly elevated “snowball” configuration (n = 4, 100%), with smooth or slightly irregular surface (n = 4, 100%), and originating from deep retina or retinal pigment epithelial (n = 4, 100%), with overlying compression and draping of retinal tissue (n = 4, 100%). Conclusion: This previously described small yellow-white retinal tumor appears to arise in the outer retinal layers and not from the inner retinal layers as formerly believed. This tumor may not be astrocytic as initially believed since it arises deep within the retina, but it could represent a deep glial or pigment epithelial fibrous mass. The pathogenesis and pathology of this rare lesion remain unknown.

Angioid streaks and optic nerve head drusen in hyperphosphatemic familial tumoral calcinosis.

PURPOSE To describe a patient with angioid streaks and optic nerve head drusen associated with familial tumoral calcinosis (FTC). DESIGN Retrospective, observational case report. METHODS The patients records were reviewed. RESULTS After receiving blunt trauma to the head, the patient presented with loss of vision in the left eye. Fundus examination revealed bilateral angioid streaks, optic nerve head drusen, and choroidal ruptures associated with subretinal hemorrhages. Subsequent physical, laboratory, and radiologic examinations revealed periarticular calcification, elevated serum phosphate levels, and normal calcium levels, consistent with FTC. CONCLUSIONS FTC should be considered in the differential diagnosis for patients presenting with angioid streaks and optic nerve head drusen.

Obliterative idiopathic juxtafoveolar telangiectasis: A 25-year follow-up

PURPOSE To report findings over 25 years of follow-up on a case of bilateral obliterative idiopathic juxtafoveolar retinal telangiectasis. METHOD Case report. RESULTS A 25-year-old white man with obliterative idiopathic juxtafoveolar retinal telangiectasis presented with a visual acuity of 20/30 in the right eye and 20/40 in the left. Fluorescein angiography revealed bilateral enlarged foveal avascular zones and late perifoveal leakage. Six years after presentation, the foveal avascular zone had enlarged only slightly in both eyes and the leakage had almost entirely subsided. For the next 19 years, the visual acuity and clinical findings have remained unchanged. His most recent visual acuity was 20/25 in the right eye and 20/40 in the left. No treatment was given throughout the entire follow-up period of 25 years. CONCLUSION Obliterative idiopathic juxtafoveolar retinal telangiectasis is a rare disorder that may initially progress with subsequent stabilization and good long-term visual outcome.

Resolution of a giant pigment epithelial detachment after treatment with intravitreal bevacizumab.

PURPOSE To describe a patient with a giant pigment epithelial detachment (PED) successfully treated with intravitreal bevacizumab. DESIGN Retrospective, observational case report. METHODS The patients records were reviewed. RESULTS An 81-year-old man with history of age-related macular degeneration was seen on routine follow-up examination with an asymptomatic PED temporal to the macula in the right eye and visual acuity of 20/40. The patient was observed. Ten months later, the patient was found to have decreased vision to 20/80 and massive enlargement of the PED, with extension from the temporal equator into the macula. Optical coherence tomography of the macula showed a PED with overlying intraretinal cysts. The patient was given injections of intravitreal bevacizumab for every 4 months (1.25 mg) with complete resolution of the PED and return to baseline visual acuity of 20/40. CONCLUSION Giant PED secondary to exudative age-related macular degeneration can be successfully treated with intravitreal bevacizumab.