Cases reported • Disease Progression

1/35. Tumor growth of a choroidal malignant melanoma and aqueous flare. Report of a case.

We report the use of the laser flare-cell meter (LFCM) in monitoring blood-ocular barrier breakdown induced by a choroidal malignant melanoma in an 88-year-old white male, who refused enucleation or radiation treatment. During a follow-up of 16 months, aqueous flare values measured with the LFCM increased from 12.8 to 26.5 photon counts/ms with continuous tumor growth from a height of 7.9 to 18 mm. Our finding of increasing flare values with tumor growth appears to confirm the observation that aqueous flare is influenced by the size of uveal malignant melanomas. Quantification of aqueous flare with the LFCM may be helpful in the follow-up of eyes with intraocular tumors.

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2/35. Sorsby's fundus dystrophy: a case report of 24 years follow-up with electrodiagnostic tests and indocyanine green angiography.

PURPOSE: Five families with dominantly inherited macular dystrophy were originally described by Sorsby et al. in 1949. Key features include early bilateral central visual loss secondary to either choroidal neovascularisation or central geographical atrophy and late progressive chorioretinal atrophy. We report a member of one of the original families who has been studied with a series of investigations over a long time, providing important information on differences in the phenotype and natural history of a rare genetically determined macular dystrophy. methods: The patient has been followed up for the last 24 years, from asymptomatic to full manifestation of Sorsby's fundus dystrophy. Series of fundus photographs, colour vision, dark adaptation and electrodiagnostic tests were performed. The disease was also studied with fundus fluorescein angiography and indocyanine green angiography. RESULTS: Unlike her other family members, who were reported in other studies as all having rapid loss of vision secondary to disciform macular disease, our patient has a unique clinical course in that she has a progressive bilateral central and generalised chorioretinal atrophy with a well-preserved minute central island of fovea. Nyctalopia was her early and only symptom. There was evidence of central scotoma, tritanopia and mild abnormality in dark adaptation. Rod function was affected earlier and to a larger degree than cone function. CONCLUSIONS: The overall features suggest phenotypic variability within a family in this autosomal dominant macular dystrophy. The findings from indocyanine green angiography and a consecutive series of electrodiagnostic tests in this condition support the theory of partial choroidal hypoperfusion and an interesting progressive rod-cone dystrophy as part of the pathophysiology.

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3/35. Central retinal vein occlusion: report of two familial cases.

The authors report a 46-year-old father and 17-year-old son who each presented with unilateral central retinal vein occlusion (CRVO) and bilateral abnormalities of retinal vascular perfusion. The son presented with a nonperfused CRVO in the left eye, developed traction-rhegmatogenous retinal detachment treated with vitreous surgery, and developed prolonged arteriovenous filling in the retina of the fellow eye. The father presented with progressive CRVO in the right eye, developed choroido-vitreal neovascularization following laser treatment to create a chorioretinal anastomosis, underwent vitrectomy for retinal detachment and vitreous hemorrhage in that eye, and developed prolonged arm-eye and retinal arteriovenous circulation times in the fellow eye. An extensive evaluation (including hematological studies and imaging of the major vessels of the neck) failed to reveal a predisposing cause in either patient although echocardiography disclosed a mitral valve thrombus in the father. After institution of coumadin therapy, the circulatory parameters in the fellow eye of each patient improved.

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4/35. Ectopic choroid plexus within a juvenile arachnoid cyst of the cerebellopontine angle: cause of cyst formation or reason of cyst growth.

The unusual and rare case of a 6-year-old boy is reported who presented with an arachnoid cyst located in the cerebellopontine angle incorporating an ectopic piece of choroid plexus tissue. A microneurosurgical cyst wall resection was performed and the plexus tissue identified and removed. The rare occurrence of ectopic choroid plexus tissue within cysts of the CNS is discussed.

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5/35. Pigmented paravenous retinochoroidal atrophy: a literature review supported by a unique case and insight.

Pigmented paravenous retinochoroidal atrophy (PPRCA) is a rare disorder of unknown origin characterised by bone corpuscle pigmentation accumulation along the distribution of the retinal veins. In addition there are peripapillary pigmentary changes as well as areas of chorioretinal atrophy adjacent to the perivenular pigmentary changes. The finding of PPRCA is usually incidental and does not affect vision. The literature regarding this condition is reviewed. Its natural course has been considered controversial. observation of the initial insult leading to PPRCA has not previously been reported. A case that provides a photographic record over 20 years of the development of this condition from the initial insult, including the gradual development of the characteristic fundus appearance, is described. The initial presentation was with a sudden reduction of vision and gross diffuse macular oedema in one eye, which was rapidly followed by similar involvement of the fellow eye despite treatment. Thus it was possible to examine the patient at the stage of the initial insult, 5 years before the development of the typical and pathognomonic retinal changes of PPRCA. The clinical and electrophysiological findings are discussed, as also is the relevance of this case to the literature reviewed.

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6/35. Immunosuppressive therapy in serpiginous choroiditis--case report and brief review of the literature.

BACKGROUND: At present there is no consensus regarding the efficacy of the immunosuppressive therapy in serpiginous choroiditis. PATIENT AND methods: We describe the case of a patient with a macular serpiginous choroiditis. Despite corticosteroid therapy the choroiditis was progressive and threatened the central vision. Therefore a triple immunosuppressive therapy with prednisone 1 mg/kg body weight/day (mg/kg/d), azathioprine 1.5 mg/kg/d, and low-dose cyclosporine A 4.5 mg/kg/d was introduced. RESULTS: Under this triple immunosuppressive therapy a prompt stabilisation of the chorioretinal process could be observed in fluorescein angiography. After five months of therapy the examination of the visual field shows an impressive recovery. No significant side effects have been noted. CONCLUSIONS: In the literature, until now only six patients with serpiginous choroiditis treated with a triple agent immunosuppressive therapy have been reported. In all cases this regimen seemed to have a positive influence on the disease. Our present observation corroborates the beneficial effect of a triple agent immunosuppressive therapy on the course of serpiginous choroiditis. However, the beneficial effect of such a therapeutic approach needs to be confirmed by further observations.

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7/35. choroid plexus carcinoma in an adult.

choroid plexus carcinomas are rare in adults. They can behave aggressively and their optimal management is uncertain. An adult patient with choroid plexus carcinoma who was treated with an incomplete surgical resection and postoperative radiotherapy is reported. Despite an identifiable local response to radiotherapy, disease progression resulted in death 4 years after presentation. This report reviews the current literature and discusses the management issues regarding this uncommon adult malignancy.

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8/35. gyrate atrophy of the choroid and retina: further experience with long-term reduction of ornithine levels in children.

OBJECTIVE: To determine whether the long-term reduction of plasma ornithine levels by way of an arginine-restricted diet in patients with gyrate atrophy will slow the progression of this chorioretinal degeneration. DESIGN: natural history study of 2 pairs of siblings with gyrate atrophy treated with an arginine-restricted diet. MAIN OUTCOME MEASURES: Fundus photography and electrophysical and psychophysical retinal function tests. RESULTS: After 16 to 17 years of receiving an arginine-restricted diet, the younger sibling in each pair, who was prescribed the diet at an earlier age than the older sibling, demonstrated a slower progression of lesions compared with the older sibling. CONCLUSIONS: If started at an early age, long-term substantial reduction of plasma ornithine levels may appreciably slow the progression of the chorioretinal lesions and, to a lesser extent, the progressive loss of retinal function in patients with gyrate atrophy.

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9/35. Successful treatment of serpiginous choroiditis with alkylating agents.

OBJECTIVE: To describe the management and long-term outcomes of patients with serpiginous choroiditis treated with alkylating agents. DESIGN: Retrospective, noncomparative case series. PARTICIPANTS: Nine patients with active, vision-threatening serpiginous choroiditis who had progressive inflammation while on steroids and/or immunosuppressive agents other than alkylating agents treated at three tertiary care uveitis referral centers. methods: patients received systemic immunosuppression with an alkylating agent, either chlorambucil or cyclophosphamide. prednisone also was given initially and was tapered and discontinued. MAIN OUTCOME MEASURES: visual acuity, clinical disease activity, duration of treatment, duration of drug-free disease remission, and side effects of alkylating agent therapy. RESULTS: No patients had recurrences while on therapy. No further visual loss was encountered after starting the therapy. Six of the patients regained vision. All but two patients achieved prolonged drug-free remissions, ranging in duration between 15 and 96 months (median, 78 months). Side effects included transient bone marrow suppression, nausea, and fatigue. Secondary malignancy was encountered in one patient, whose carcinoma of the urinary bladder was treated successfully. CONCLUSIONS: Adequate immunosuppression with alkylating agents may favorably alter the long-term prognosis of patients with serpiginous choroiditis.

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10/35. indocyanine green angiography in pigmented paravenous retinochoroidal atrophy.

PURPOSE: To report long-term follow-up of patients with pigmented paravenous retinochoroidal atrophy (PPRCA) and to assess the involvement of the choroid and retinal pigment epithelium (RPE) in PPRCA. methods: Clinical features of PPRCA were studied retrospectively in four patients followed for 6-26 years. Retinal pigment epithelium and choroidal changes were analysed with fluorescein and indocyanine green (ICG) angiography. RESULTS: The two younger patients, aged 16 and 28 years and followed for 6 and 18 years, respectively, showed stationary RPE atrophy and pigmentation. indocyanine green angiography visualized slight to modest atrophy of the choriocapillaris. The two older patients, aged 69 and 70 years and each followed for 26 years, showed slow progression of disease during follow-up. indocyanine green angiography revealed choriocapillaris atrophy partly extending into the areas shown as hyperfluorescent in fluorescein angiography. CONCLUSION: Pigmented paravenous retinochoroidal atrophy is probably a slowly progressive disease, particularly in older patients. The choriocapillaris atrophy in this disease is more properly evaluated by ICG angiography and can be underestimated by fluorescein anigiography.

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