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Biomedical subjects

A R Irvine

Publications and source records attributed to A R Irvine.

At least 19 recordsLinked to original sources

Ocular manifestations of leukemia: leukemic infiltration versus infectious process.

OBJECTIVE: To determine whether specific guidelines can be developed to distinguish whether retinal infiltration in leukemia patients represents infection or neoplasia. DESIGN: Retrospective noncomparative interventional case series. PARTICIPANTS: Six patients recently seen at University of California San Francisco with retinal infiltrates in a setting of leukemia, for which adequate written and photographic information of disease course was available. INTERVENTION: Observation consisted of retrospective review of clinic charts, hospital medical records, and fundus photographs. MAIN OUTCOME MEASURES: Determination of whether retinal infiltrates represented neoplasia or infection was made by review of medical records. RESULTS: In this series, neoplastic retinal infiltrates were found in patients who had newly diagnosed leukemia and those who were in blast crisis. In contrast, the two patients who were in complete remission, but had undergone bone marrow transplantation, had retinal infiltrates attributable to infection. CONCLUSIONS: Every patient with retinal infiltrates in the setting of newly or previously diagnosed leukemia requires a systemic and central nervous system workup before the initiation of ophthalmologic treatment. The systemic status of the patient is highly informative in determining whether infection or neoplasia is responsible for the infiltration.

Adult↗

Spontaneous central retinal artery occlusion in hemoglobin sickle cell disease.

PURPOSE: To describe a case of spontaneous central retinal artery occlusion in a young man with hemoglobin sickle cell disease. METHOD: Case report. RESULTS: A 31-year-old African-American man with a history of hemoglobin sickle cell disease developed sudden painless loss of vision in the right eye. Medical history was remarkable for the recent history of a mild painful crisis, but no other systemic illness or contributing factors. Central retinal artery occlusion was diagnosed with retinal whitening, cherry red spot, and delayed arteriovenous transit on fluorescein angiography. Over the ensuing week, the patient had visual recovery to 20/60 in the absence of therapeutic intervention. CONCLUSION: Central retinal artery occlusion has been reported in sickle cell hemoglobinopathies (ie, SS, S-thal, sickle trait, and sickle cell), but the association with sickle cell disease is rare. Most reports have described additional contributing factors, such as trauma or concomitant systemic illness, to help account for the central retinal artery occlusion. The present case suggests that sickle cell disease alone is sufficient for the development of central retinal artery occlusion.

Adult↗

Visual and anatomic outcomes associated with posterior segment complications after ganciclovir implant procedures in patients with AIDS and cytomegalovirus retinitis.

PURPOSE: To determine anatomic and visual acuity outcomes of posterior segment complications after ganciclovir implant surgery. METHODS: We reviewed the medical records of 63 patients with acquired immunodeficiency syndrome who had active cytomegalovirus retinitis in 82 eyes and who underwent 110 consecutive ganciclovir implant procedures. Preoperative and postoperative visual acuity, type of postoperative complication, treatment, and lines of visual acuity change were determined. RESULTS: Thirty-eight eyes of 19 patients had bilateral ganciclovir implant procedures, and 25 eyes of 19 patients underwent two or more ganciclovir implant procedures. Thirteen (12%) of 110 ganciclovir implant procedures developed posterior segment complications: rhegmatogenous retinal detachment in six, vitreous hemorrhage in four, endophthalmitis in two, and cystoid macular edema with epiretinal membrane in one. Treatment included pars plana vitrectomy with silicone oil in two cases and without silicone oil in three cases, scleral buckling in one, intravitreal antibiotic injection in two, and laser photocoagulation in two. Overall, median visual acuity was 20/25 preoperatively. Median follow-up was 6 months for all eyes and 7 months for eyes with complications. Postoperative median visual acuity was 20/25 for eyes without complications vs 20/40 at 1 month, 20/60 at 3 and 6 months, and 20/100 at 12 months in eyes with complications (P < .001). More eyes with than without complications lost 2 or more lines of visual acuity (P < .001). CONCLUSION: Postoperative complications occurred in 12% of the ganciclovir implant procedures and were associated with decreased visual acuity despite treatment.

AIDS-Related Opportunistic Infections↗

Norrie disease in a family with a manifesting female carrier.

OBJECTIVES: To show that Norrie disease can occur in a girl and to describe her ophthalmologic and genetic features. METHODS: Amplification of DNA polymerase chain reaction and sequencing of asymmetric polymerase chain reaction for exon 3 were performed on the blood specimen obtained from a girl born with bilateral retinal detachments. PATIENT: A female child with bilateral retinal detachment who had 2 uncles in whom Norrie disease had already been diagnosed. RESULTS: The child had a mutation in the third exon (T776-->A; Ile 123-->Asn) identical to the mutation found in her uncles. CONCLUSIONS: Norrie disease can occur in girls. The most likely explanation is nonrandom or unfavorable X inactivation, although timing of development of the peripheral retina and its blood supply could render it vulnerable to effects of the mutant allele at a critical developmental phase.

Blindness↗

A polymerase chain reaction-based assay for diagnosing varicella-zoster virus retinitis in patients with acquired immunodeficiency syndrome.

PURPOSE: To develop a rapid, sensitive, and specific laboratory assay based on the polymerase chain reaction for the diagnosis of varicella-zoster virus retinitis in patients with acquired immunodeficiency syndrome (AIDS). METHODS: We developed and tested a polymerase chain reaction-based assay for the detection of varicella-zoster virus DNA in vitreous samples. We attempted to detect varicella-zoster virus DNA in 14 vitreous samples from patients with AIDS and a clinical diagnosis of progressive outer retinal necrosis syndrome. For controls, we also attempted to detect varicella-zoster virus DNA in vitreous samples from 75 immunocompetent patients with vitreoretinal disease and 88 patients with AIDS and vitreoretinal inflammatory disease not related to progressive outer retinal necrosis syndrome. RESULTS: Varicella-zoster virus DNA was detected in 11 of 14 vitreous samples from AIDS patients with progressive outer retinal necrosis syndrome. All three samples that scored negative for varicella-zoster virus DNA came from eyes that had been treated aggressively with antiviral drugs and had clinically inactive disease at the time of vitreous biopsy. Varicella-zoster virus DNA was detected in only two of 75 control vitreous samples from immunocompetent patients with vitreoretinal disease and two of 88 control vitreous samples from patients with AIDS and vitreoretinal inflammatory disease not related to progressive outer retinal necrosis syndrome. CONCLUSION: We have developed a rapid, sensitive, and specific polymerase chain reaction-based diagnostic assay for varicella-zoster virus DNA that will assist in the diagnosis of varicella-zoster virus retinitis in patients with AIDS.

Acquired Immunodeficiency Syndrome↗

Nocardia scleritis.

PURPOSE: To describe a case of Nocardia scleritis, an unusual ocular infection. METHODS: Case report and review of pertinent literature. RESULTS: An 83-year-old man with leukocytoclastic vasculitis was initially examined for infectious necrotizing scleritis after explantation of an extruded scleral buckle. The patient was successfully treated with sulfonamides. CONCLUSIONS: Nocardia asteroides may cause infectious scleritis in the absence of cataract surgery or trauma. Treatment with sulfonamides can result in a satisfactory outcome.

Aged↗

Retinal detachment in AIDS: long-term results after repair with silicone oil.

AIMS: To study the long-term results of vitrectomy and silicone oil injection in AIDS patients with retinal detachment due to viral retinitis. METHODS: A consecutive series of 83 eyes in 75 AIDS patients who were treated with vitrectomy and silicone oil for retinal detachment due to viral retinitis was studied prospectively and followed until the last patient in the series had died. RESULTS: Median postoperative survival was 6 months, but 20% of patients survived 12 months or more. Whereas best corrected visual acuity was 20/100 or better in over half the patients 1-2 months after operation, there was a distressing decrease in acuity by 6 months, owing to a variety of factors. Patients operated on before macular detachment did not have significantly better postoperative vision than those operated within a week after macular detachment. Almost all patients who had already lost vision in the fellow eye and many who had cytomegalovirus retinitis in the fellow eye with retention of good vision had their quality of life improved by the surgery in that the operated eye eventually became the better seeing eye. In no patient whose fellow eye was normal and free of retinitis, however, did the operated eye ever become the better eye. CONCLUSIONS: Although the majority of patients recovered macular vision in the first 1-2 months after operation, there was a gradual decline in acuity thereafter, sometimes without obvious cause. The results of this series suggest that it may be reasonable to postpone surgery until the macula detaches and that patients whose fellow eye is free of retinitis with normal vision are unlikely to have their quality of life improved significantly by the surgery.

AIDS-Related Opportunistic Infections↗

Fundus changes in Behcet's disease.

OBJECTIVE: To describe the fundus findings in Behcet's Disease with emphasis on characteristic small white patches of retinitis. METHODS: Case study, utilizing review of clinical charts and fundus photographic files. RESULTS: Search of the University of California San Francisco ophthalmic photography files revealed 6 patients coded as Behcet's Disease between 1989 and 1996. Examination of the patients and their clinical records indicated that all 6 met the International Diagnostic Criteria for Behcet's Disease. The single most common fundus finding in these cases was the presence of transient, white patches of retinitis often with a small adjacent hemorrhage. Retinal vasculitis and optic neuritis were also seen, but neither with the frequency of the white patches. CONCLUSION: The presence of small patches of retinal whitening is the most characteristic finding in Behcet's Disease. These lesions are different from cotton wool spots, and their recognition can aid in the diagnosis of Behcet's Disease and of episodes of reactivation.

Adult↗

Acquired immunodeficiency syndrome--associated herpes simplex virus retinitis. Clinical description and use of a polymerase chain reaction--based assay as a diagnostic tool.

OBJECTIVES: To describe 2 patients with acquired immunodeficiency syndrome who experienced a rapidly progressive, bilateral retinitis due to herpes simplex virus (HSV) (1 case due to HSV type 1 [HSV-1] and 1 case due to HSV type 2 [HSV-2] and to present a novel diagnostic polymerase chain reaction (PCR)-based assay. METHODS: The presentation, clinical course, and diagnostic PCR-based assay used to make the diagnosis of HSV retinitis in 2 patients with acquired immunodeficiency syndrome are described. RESULTS: Both patients experienced a rapidly progressive, bilateral retinal necrosis associated with intraretinal hemorrhages and a diffuse vasculitis. The PCR-based assays demonstrated HSV DNA in the vitreous specimens from the 2 patients. Restriction analysis on the amplified DNA showed HSV-1 in 1 patient and HSV-2 in the second patient. The diagnosis was supported in both patients by the occurrence of a herpes simplex-like encephalitis, and in 1 patient by a positive vitreous culture. The HSV-1-associated vasculitis affected primarily the retinal arterioles, with marked capillary dropout and occlusion of larger arcade vessels. In contrast, the HSV-2-associated vasculitis affected the retinal veins more than the arterioles, and was associated with an exudative retinal detachment. CONCLUSIONS: To our knowledge, these are the first 2 patients with acquired immunodeficiency syndrome in whom HSV has been implicated as the sole cause of a rapidly progressing, necrotizing retinitis. Combined PCR and restriction analysis of vitreous samples from such patients is a useful and highly specific means of diagnosing HSV-1 and HSV-2 retinitis.

AIDS-Related Opportunistic Infections↗

Bone marrow transplantation retinopathy in the absence of radiation therapy.

PURPOSE: To describe a case of bone marrow transplantation retinopathy in a patient in the absence of prior radiation therapy. METHODS: Case report. RESULTS: Bilateral scotomata developed in a 26-year-old woman because of a retinal microvasculopathy two years after receiving an autologous bone marrow transplantation for acute promyelocytic leukemia. No prior radiation therapy had been given. CONCLUSIONS: Certain combinations of antimetabolites used during induction and transplantation may themselves be toxic to the retinal microvasculature. Patients who undergo bone marrow transplantation would benefit from regular and complete eye examinations, even in the absence of prior radiation therapy.

Adult↗

Central serous chorioretinopathy in patients with systemic lupus erythematosus.

PURPOSE: To describe three patients with systemic lupus erythematosus in whom ophthalmoscopic and fluorescein angiographic evidence of central serous chorioretinopathy developed. METHODS: The authors retrospectively reviewed the clinical and photographic records of three patients with systemic lupus erythematous in whom central serous chorioretinopathy developed. RESULTS: Ophthalmoscopic changes observed in these patients with systemic lupus erythematosus included discrete areas of clumping and mottling of the retinal pigment epithelium (RPE), focal RPE detachments, serous elevations of the neurosensory retina, and late subretinal fibrosis with scar formation. Fluorescein angiographic findings included transmission hypofluorescence and hyperfluorescence corresponding to focal RPE alterations, early punctate intense hyperfluorescence corresponding to RPE leaks with progressive filling of sub-RPE detachment spaces, and slow late filing of subretinal detachment spaces. CONCLUSION: Patients with systemic lupus erythematosus are at increased risk to have central serous chorioretinopathy develop. The pathogenetic implications for an association between systemic lupus erythematosus and central serous chorioretinopathy as well as the similarity to the chorioretinopathy seen with accelerated hypertension, pregnancy, hemodialysis, organ transplantation, and exogenous and endogenous hypercortisolism are discussed. Focal choroidal vasculature compromise with secondary dysfunction of overlying RPE cells is the proposed common mechanism.

Adult↗

Treatment of presumed fungal endophthalmitis with oral fluconazole.

Amphotericin B is the usual treatment for fungal endophthalmitis, but its toxicity and lack of oral bioavailability may limit its use in some patients. The authors report the successful management of two cases of presumed metastatic fungal endophthalmitis with oral fluconazole, a bis-triazole compound with a broad antifungal spectrum.

Administration, Oral↗

Vogt-Koyanagi-Harada syndrome in a 4-year old child.

PURPOSE: We studied a case of severe bilateral Vogt-Koyanagi-Harada syndrome in a 4-year-old boy. METHODS: We evaluated the patient's clinical course. RESULTS: The patient had severe bilateral, nongranulomatous uveitis and mild uveitic glaucoma. Initial examination and laboratory evaluation failed to provide a diagnosis. The patient subsequently developed areas of vitiligo, alopecia, and poliosis, suggesting the diagnosis of Vogt-Koyanagi-Harada syndrome. This diagnosis was confirmed by the eventual development of bilateral neurosensory retinal detachments. Vision was lost despite aggressive therapy with corticosteroids and chlorambucil. CONCLUSION: Although uncommon, Vogt-Koyanagi-Harada may affect young children, and may be severe.

Alopecia↗

A sensitive and specific polymerase chain reaction-based assay for the diagnosis of cytomegalovirus retinitis.

PURPOSE: To develop a sensitive and specific laboratory assay for the diagnosis of cytomegalovirus retinitis. METHOD: We used a polymerase chain reaction-based assay for detection of cytomegalovirus DNA in vitreous samples. We attempted to detect cytomegalovirus DNA in 19 vitreous samples from patients with the acquired immunodeficiency syndrome (AIDS) who had untreated cytomegalovirus retinitis and in 40 vitreous samples from patients with AIDS who had been treated with systemic ganciclovir or foscarnet, or both. We also attempted to detect cytomegalovirus DNA in vitreous samples from 54 immunocompetent patients, including 32 with retinal detachment or macular hole, 11 with vitreous inflammation, and 11 with vitreous hemorrhage. Additionally, we attempted to detect cytomegalovirus DNA in 15 vitreous samples from patients with AIDS who had vitreoretinal inflammation not caused by cytomegalovirus. RESULTS: Cytomegalovirus DNA was detected in 18 of 19 eyes with untreated cytomegalovirus retinitis. We detected cytomegalovirus DNA in 19 of 40 vitreous samples from patients with previously treated cytomegalovirus retinitis. Cytomegalovirus DNA was not detected in any of 69 patients who did not have a clinical diagnosis of cytomegalovirus retinitis. Thus, the assay had an estimated sensitivity of 95% in detecting untreated cytomegalovirus retinitis and a sensitivity of 48% in detecting cytomegalovirus retinitis that had been treated with systemic ganciclovir or foscarnet, or both. The assay did not give false-positive results in patients with vitreous hemorrhage or vitreous inflammation. Most important, the assay did not give false-positive results in AIDS patients with vitreous inflammation from causes other than cytomegalovirus retinitis. CONCLUSION: We have developed a sensitive and specific diagnostic assay that will assist in the diagnosis of cytomegalovirus retinitis.

AIDS-Related Opportunistic Infections↗

Subretinal fibrosis in central serous chorioretinopathy.

PURPOSE: To report unusual and heretofore unreported visually damaging manifestations of severe central serous chorioretinopathy. METHODS: Case studies. RESULTS: Each of six male patients (average age, 40 years) had a form of severe central serous chorioretinopathy with at least one eye containing fibrin in the subretinal space that then developed into a subretinal fibrotic scar. Scar formation was followed by a tenting up of the macula, vascularization of the fibrosis (subretinal neovascularization), or a retinal pigment epithelial rip. Four of the seven eyes with subretinal fibrosis had severe visual loss (20/400 or worse). CONCLUSION: Subretinal fibrin and other extracellular matrix molecules appear to stimulate the retinal pigment epithelium to undergo fibrous metaplasia, which results in subretinal fibrotic scar formation and other sequelae, all of which can lead to severe visual loss.

Adult↗

Retinal distortion and cotton-wool spots associated with epiretinal membrane contraction.

PURPOSE AND BACKGROUND: Several articles on surgery for macular pucker have noted the presence of intraretinal white spots, presumably cotton-wool spots, associated with epiretinal membranes. It was proposed that membrane contraction and resultant nerve fiber layer distortion might lead to blockage of axoplasmic flow and thus a cotton-wool spot. To see whether the observations supported this concept, the authors studied patients with epiretinal membranes associated with intraretinal white spots involving the center of the fovea, where there are normally no retinal vessels and the only axons are in Henle fiber layer. METHODS: Four patients with epiretinal membranes associated with prominent areas of intraretinal whitening involving the center of the fovea were examined before and after surgery. Observations and measurements were made from projected fundus photographs. RESULTS: The intraretinal white spots, which appeared preoperatively to lie in the center of the fovea, proved to have been pulled in from more peripheral retina by the epiretinal membrane. These white areas were associated with surprisingly great distortion of the inner retina. Stretching of the inner retina seemed to play a larger role in the retinal distortion than full-thickness retinal folding. CONCLUSION: The appearance and time course of these intraretinal white lesions were consistent with cotton-wool spots caused by mechanical disruption of axonal flow. The marked retinal stretching and distortion of the innermost layers of the retina were the most striking findings in this study and also seemed consistent with the concept that membrane contraction and resultant nerve fiber layer distortion might lead to blockage of axoplasmic flow and thus a cotton-wool spot.

Axonal Transport↗