End-stage cytomegalic inclusions retinitis and disseminated intravascular coagulation

Areas of necrosis of the retinal pigment epithelium developed in each eye of a 40-year-old renal transplant recipient. Myocardial infarctions supervened and eventually caused his death. Autopsy demonstrated cytomegalic inclusion bodies in the lungs and liver, and areas of retinal scarring and fibrin thrombi in the choriocapillaris of both eyes. The ocular features likely resulted from the resolved retinitis and preterminal disseminated intravascular coagulation.     

Adult cytomegalovirus inclusion retino-uveitis.

A 27-year-old male renal transplant recipient acquired cytomegalovirus (CMV) infection of both eyes. This was characterized early by a distinctive necrotic retinitis with discrete advancing edges. A large exudative retinal detachment and hypopyon developed in one eye, and cultures from the anterior chamber aspirate grew CMV. Cytomegalovirus was also isolated from the urine and throat; after serial negative base-line titers, CMV antibody titer became positive. At autopsy CMV was isolated from lung, esophagus, and kidney tissue. Light and electron microscopic studies demonstrated extensive necrosis and disruption of the sensory retina and retinal pigment epithelium with exudative retinal detachments. The diseased retinal cells contained a multiplicity of viral particles in various stages of maturity. Cytomegalic inclusion-bearing cells were demonstrated in the choroid of the more severely affected eye.     

Foveomacular retinitis.

A group of patients is described who developed the clinical features of foveomacular retinitis. No causative factors were isolated, and all patients strongly denied any type of sun gazing. It is possible that there is a group of patients who have the features of foveomacular retinitis but have not had any direct exposure to the sun. These patients would then constitute a primary type of foveomacular retinitis, as opposed to a secondary type which has a known cause and is synonymous with solar retinopathy.     

Syphilitic retinitis. A cause of necrotizing retinitis

A healthy-appearing male patient presented with signs of unilateral fibrinous iritis, necrotizing retinitis, retinal vasculitis, and vitritis, suggestive of a herpesvirus retinitis or acute retinal necrosis syndrome (ARN). The patient, an active homosexual, withheld the details of his sexual history, portraying himself as exclusively heterosexual. With the exception of a positive VDRL and FTA-ABS, the workup was negative. Examination of the cerebrospinal fluid confirmed the presence of neurosyphilis. The patient was successfully treated with intravenous penicillin, which resulted in a complete visual recovery. Syphilitic retinitis must be considered in evaluating patients with necrotizing retinitis.     

X-linked retinitis pigmentosa.

Of 107 consecutive patients with genetically-determined retinitis pigmentosa, 23 were provisionally diagnosed as having inherited the disease in an X-linked fashion. 42 affected males and 61 females were examined, and from the data obtained the following conclusions were drawn: (1) X-linked retinitis pigmentosa exists and is distinct from choroideremia. (2) In contrast to the results of previous surveys, X-linked retinitis pigmentosa is a common form of this disease and over 20 per cent. of retinitis pigmentosa is probably transmitted in an X-linked manner. (3) (a) In contradistinction to the findings of previous investigators, most if not all adult heterozygous females have detectable degenerative changes in the ocular fundus. (b) The ocular changes in heterozygous females are most easily detected by fundus examination, visual field testing, dark adaptation measurements, and estimation of retinal rhodopsin concentration. The single most frequent abnormality is peripheral retinal pigment epithelial atrophy, which is found in all adult heterozygous females. (c) The pattern of retinal dysfunction in heterozygous females, and in particular preservation of the ocular electrical responses, suggests that the disease in women is qualitatively different from that in men and in other genetic forms of retinitis pigmentosa. There is some evidience that the disease in heterozygous women is patchy. (d) Degeneration in heterozygous females is usually symmetrical, but great variation was found in the severity of degeneration amongst heterozygotes of similar ages. No non-genetic influences were found to account for this. No evidence came to light by which the importance of X-chromosome inactivation could be assessed in determining the phenotype of heterozygous women. (4) No evidience is available to determine the number of X-linked genes transmitting the disease.     

Aggressive toxoplasma retinitis.

Toxoplasma infection is a common cause of infectious uveitis. It usually produces a characteristic fundal appearance, with evidence of previous inflammation. However, it may occur in an atypical and aggressive form. Steroids administered to salvage vision may then worsen the clinical course. Retinal biopsy may be diagnostic in cases where doubt exists. We illustrate these points with two cases.     

Sector retinitis pigmentosa.

BACKGROUND: Retinitis pigmentosa (RP) is one of the most common hereditary retinal dystrophies and causes of visual impairment affecting all age groups. The reported incidence varies, but is considered to be between 1 in 3,000 to 1 in 7,000. Sector retinitis pigmentosa is an atypical form of RP that is characterized by regionalized areas of bone spicule pigmentation, usually in the inferior quadrants of the retina. CASE REPORT: A 57-year-old Hispanic man with a history of previously diagnosed retinitis pigmentosa came to the clinic with a longstanding symptom of decreased vision at night. Bone spicule pigmentation was found in the nasal and inferior quadrants in each eye. He demonstrated superior and temporal visual-field loss corresponding to the areas of the affected retina. Clinical measurements of visual-field loss, best-corrected visual acuity, and ophthalmoscopic appearance have remained stable during the five years the patient has been followed. DISCUSSION: Sector retinitis pigmentosa is an atypical form of RP that is characterized by bilateral pigmentary retinopathy, usually isolated to the inferior quadrants. The remainder of the retina appears clinically normal, although studies have found functional abnormalities in these areas as well. Sector RP is generally considered a stationary to slowly progressive disease, with subnormal electro-retinogram findings and visual-field defects corresponding to the involved retinal sectors. CONCLUSION: Management of RP is very difficult because there are no proven methods of treatment. Studies have shown 15,000 IU of vitamin A palmitate per day may slow the progression, though this result is controversial. Low vision rehabilitation, long wavelength pass filters, and pedigree counseling remain the mainstay of management.     

Photoaversion in retinitis pigmentosa.

Photoaversion, or light-induced interference with visual comfort and performance, has been a recognised but poorly documented symptom in retinitis pigmentosa (RP). We found that a majority of our RP patients complained of photoaversion even in the absence of significant cataract. RP patients had reduced contrast sensitivity relative to normal people, but the decrement in their visual performance as a result of glare or photostress was only slight. RP patients had raised short-term adaptation and increment threshold levels, but their rate of short-term or photopic adaptation was normal. Photoaversion in RP may result because a small interference with contrast sensitivity or adaptation can place patients in a range of functional disability, or it may derive from a combination of minor aberrations.     

Infectious retinitis. Diagnostic modalities

Advances in surgical techniques have made vitreoretinal and sclerochorio-retinal biopsies part of the available diagnostic armamentarium of the ophthalmologist. Special studies can be performed on tissue obtained in this way in order to provide a specific etiologic diagnosis of virus associated pathology. The advantages and disadvantages of immunopathologic and ultrastructural methods, tissue culture, and nucleic acid hybridization techniques for viral identification are compared and discussed.     

Cytomegalovirus retinitis in AIDS.

Cytomegalovirus (CMV) retinitis is the most common retinal opportunistic infection in people with the acquired immune deficiency syndrome (AIDS). Ordinarily, CMV causes little morbidity in an immunocompetent person. However, in the immunosuppressed patient this virus is responsible for progressive retinal destruction with necrosis leading to a devastating loss of vision. This paper will provide the practitioner with a clinical approach to the presentation, diagnosis and management of patients with CMV retinitis secondary to AIDS.     

Zidovudine and cytomegalovirus retinitis.

A 38-year-old bisexual man with acquired immunodeficiency syndrome (AIDS) who was being treated with oral acyclovir for herpetic stomatitis had a history of blurred vision OS that was diagnosed as cytomegalovirus retinitis. The patient refused ganciclovir administration. Two additional lesions developed OS in the succeeding four months. All clinical evidence of active retinitis cleared after zidovudine was administered, and the patient has remained free of any clinically active retinal lesions for 28 months while continuing to receive acyclovir and zidovudine. Although ganciclovir and foscarnet are the drugs of choice to treat cytomegalovirus retinitis, this observation may be fortuitous for patients whose other AIDS manifestations suggest using zidovudine rather than ganciclovir or for patients whose cytomegalovirus retinitis appears to be resistant to agents currently used to treat this infection.     

Motion thresholds in retinitis pigmentosa.

Minimum displacement thresholds, or dmin, were measured in 29 subjects with retinitis pigmentosa (RP) and 10 subjects with normal vision. The results showed that RP can affect an observer's ability to judge the correct direction of motion in a random-dot pattern. The majority of RP subjects had elevated dmin. They required a larger displacement to perceive the correct direction of motion. Only 5 of the 29 RP subjects had thresholds within two standard deviations of the mean of the normal-observer distribution. Moreover, three RP subjects were unable to detect the correct direction of motion regardless of the displacement magnitude, and four RP subjects consistently reported motion in the opposite direction at small displacements. The results cannot be explained by abnormal temporal processing or a reduction in the effective luminance. There was a statistically significant correlation (r = 0.72, P less than 0.001) between log threshold and log MAR, consistent with the hypothesis that a reduction in the spatial density of the photoreceptors contributes to the motion-threshold elevation. Motion thresholds also were measured in subjects with normal vision under conditions of simulated "photoreceptor" dropout. The results showed that a random elimination of information from over 25% of the image positions significantly elevates motion thresholds. These results also support the spatial-density reduction hypothesis.     

Immune complexes in retinitis pigmentosa.

In a group of 46 patients with retinitis pigmentosa (RP) we studied the presence of circulating immune complexes (CIC) and the alterations in the complement system. Our results showed the presence of CIC in 43.5% of the patients studied, reduced levels of the complement components C3 and C4 (p less than 0.001), and of the haemolytic activity CH50 (p less than 0.001) when compared with a control group consisting of a 100 healthy subjects. We found a statistically significant correlation between the values of C3 and CIC (p less than 0.01), C4 and CIC (p less than 0.01), and between CH50 and CIC (p less than 0.001). These findings indicate that the CIC may play a role in the pathogenesis of primary retinitis pigmentosa.     

Subretinal hemorrhage in cytomegalovirus retinitis.

A case of subretinal hemorrhage associated with cytomegalovirus (CMV) retinitis in a human immunodeficiency virus-positive woman is described. The patient was found to have an exudative retinal detachment involving the fovea, subretinal hemorrhage, cotton wool spots, and active CMV retinitis temporal to the exudative detachment. No evidence of systemic coagulopathy was found. Although unusual, subretinal hemorrhage may be observed in association with CMV retinitis in the absence of a systemic coagulopathy.