Treatment of cytomegalovirus retinitis with oral valganciclovir in an acquired immunodeficiency syndrome patient unresponsive to combination antiretroviral therapy.

BACKGROUND: Cytomegalovirus (CMV) retinitis was a much-feared visual complication of late-stage acquired immunodeficiency syndrome (AIDS) in the past. Its incidence has waned significantly owing to the benefits of potent antiretroviral combination therapy, which for many individuals has provided some degree of immune reconstitution and avoidance of opportunistic infections, particularly this blinding disease. CASE REPORT: A 45-year-old white man with long-standing, multidrug-resistant human immunodeficiency virus (HIV) infection and severe immunodeficiency despite multiple antiretroviral drug regimens, presented to the eye clinic reporting decreased vision and spider web patterns in his left eye for the past week. Best-corrected visual acuity was 20/20 in the right (O.D.) and 20/25 in the left eye (O.S.). Dilated funduscopic examination of the left eye found vasculitis of the midperipheral inferonasal arcade in the midperiphery, with surrounding intraretinal hemorrhage and granular retinal necrosis. Diagnosis of cytomegalovirus retinitis was made, and the patient began induction therapy with oral valganciclovir 900 mg twice a day for 3 weeks. Maintenance therapy after retinitis stabilization was 900 mg every day until any observed recurrence of infection. Three months after complete resolution of the active retinitis, the patient returned to the clinic reporting new floaters of recent onset. A reactivation of the CMV retinitis warranted a reinduction with valganciclovir 900 mg orally twice a day for 3 weeks. CONCLUSION: This case is illustrative of the efficacy and relative ease of administration of valganciclovir, the newest medication approved for treatment and maintenance of CMV retinitis. Despite his severe immunodeficiency, our patient tolerated the induction and maintenance therapy of oral valganciclovir well, and the CMV retinitis was stabilized and resolved with full recovery of visual acuity.     

Immune recovery uveitis in an iatrogenically immunosuppressed patient

PURPOSE: To report a case of immune recovery uveitis (IRU) in an iatrogenically immunosuppressed human immunodeficiency virus (HIV)-negative patient. METHODS: Interventional case report. One patient was diagnosed with cytomegalovirus retinitis in the left eye while receiving immunosuppressive treatment following renal transplantation. The retinitis resolved completely with systemic ganciclovir. Further reduction of immunosuppressive treatment, causing a rapid increase in CD4-T lymphocyte count, was associated in the same eye with the occurrence of IRU consisting of anterior uveitis, vitritis, and macular edema. RESULTS: Visual acuity at IRU presentation onset was 20/200 in the left eye. After 6 weeks of follow-up, the uveitis resolved with topical and periocular steroid treatment. Visual acuity restored to 20/40. CONCLUSIONS: IRU can occur in iatrogenically immunosuppressed HIV-negative patients.     

Coinfection of the retina by Epstein-Barr virus and cytomegalovirus in an AIDS patient

PURPOSE: To report an immunohistochemically proven case of a coinfection of the retina by Epstein-Barr virus and cytomegalovirus in a patient with acquired immunodeficiency syndrome (AIDS). DESIGN: Interventional case report. METHODS: Postmortem evaluation of retinal tissue of a 45-year-old female AIDS patient with atypical fibrinous iridocyclitis and vitritis in course of a cytomegalovirus retinitis in the left eye was performed for evidence of dual infection with cytomegalovirus and other herpes group viruses. Immunohistochemical examination with double-staining techniques and antibodies against Epstein-Barr virus and cytomegalovirus was used. RESULTS: In the retina of the left eye, cells reacting with antibodies against cytomegalovirus and cells stained with antibodies against Epstein-Barr virus were detected in the layer of ganglionic cells and the inner granular cell layer. CONCLUSION: Epstein-Barr virus coinfection should be taken into consideration in unusual cases of necrotizing cytomegalovirus retinitis in AIDS patients.     

Treatment of cytomegalovirus retinitis with intravitreal injection of liposome encapsulated ganciclovir in a patient with AIDS.

To study its safety and efficacy in treating cytomegalovirus (CMV) retinitis, an AIDS patient received an intravitreal injection of liposome encapsulated ganciclovir in the right eye. The left eye served as a control, receiving intravitreal free ganciclovir. The right eye showed no retinal haemorrhages or detachment; however, vision declined initially, stabilising later. Weekly examination showed neither progression of the CMV retinitis nor new lesions in the right eye. The left eye showed reactivation of old CMV retinitis. Liposome encapsulated ganciclovir reduced the number of intravitreal injections, stabilising CMV retinitis, and warrants further study.     

Herpes simplex virus type 2 acute retinal necrosis in an immunocompetent patient

PURPOSE: To report a case of acute retinal necrosis caused by herpes simplex virus 2 in an otherwise healthy patient. CASE REPORT: A 45-year-old man presented with one month's history of decreased vision in the right eye. He had previously received a course of intravenous gancyclovir because of a clinical suspicion of cytomegalovirus retinitis. The patient's ocular history was remarkable for a similar episode in the left eye thirty years earlier, resulting in important visual impairment. System and laboratory investigations were unremarkable. Ocular examination showed severe anterior granulomatous uveitis, vitreous haze, areas of necrosis and retinal exudates. The anterior chamber tap disclosed the presence of HSV type 2, and oral steroids and acyclovir were instituted. Two weeks after the patient had been discharged, a retinal detachment occurred in the right eye, necessitating surgical repair. The presence of HSV type 2 was confirmed in the vitreous. Visual acuity recovered completely after surgery and the patient was placed on a maintenance dose of oral acyclovir. CONCLUSIONS: HSV type 2 is a rare cause of acute retinal necrosis in healthy patients. Bilateral involvement can occur in the fellow eye, even with a long delay. Acute retinal necrosis is a severe ocular inflammatory syndrome associated with a very poor visual outcome. It is caused by VZV, HSV type 1 and, less commonly, by HSV type 2. The disease can affect healthy patients and cause bilateral involvement in the fellow eye, even with a long delay.     

Cytomegalovirus retinitis and FK 506

PURPOSE: To report a case of cytomegalovirus (CMV) retinitis in a patient immunosuppressed with FK 506. DESIGN: Interventional case report. METHODS: Retrospective institutional study. One patient, diagnosed with bilateral CMV retinitis while on immunosuppressive treatment with FK 506, received systemic ganciclovir and intravitreal, slow-release ganciclovir implant in one eye, combined with reduction in FK 506 dose. Main outcome measures were visual acuity and regression of CMV retinitis. RESULTS: Visual acuity at presentation was 20/20 in the right eye and 20/50 in the left eye. During the 6 months of follow-up, there was no change in visual acuity in either eye. The areas of CMV retinitis demonstrated progressive regression, leaving diffuse retinal atrophy. CONCLUSIONS: Cytomegalovirus retinitis can be a sight-threatening complication of immunosuppressive treatment with FK 506. Ganciclovir treatment together with a reduction in FK 506 dose was effective in preserving vision.     

Cytomegalovirus retinitis after fluocinolone acetonide (Retisert) implant

PURPOSE: To report a case of cytomegalovirus (CMV) retinitis after placement of a fluocinolone acetonide (Retisert) implant. DESIGN: Interventional case report. METHODS: Retrospective chart review. RESULTS: A 65-year-old man with a history of Adamantiades-Behcet disease and bilateral recurrent uveitis that was unresponsive to systemic corticosteroid-sparing immunosuppressive therapy developed clinical evidence of CMV retinitis after receiving his second intravitreal Retisert implant in the left eye, while on no systemic immunosuppression. He did not develop CMV retinitis in the right eye despite multiple intraocular and periocular steroid injections. The patient responded well to intravitreal foscarnet followed by placement of an intravitreal ganciclovir implant. CONCLUSIONS: Ophthalmologists should be aware of the potential risk for development of CMV retinitis after local ocular immunosuppressive therapy.     

Congenital iris ectropion associated with juvenile glaucoma

Congenital iris ectropion is an uncommon malformation of the anterior segment of the eye. The authors describe a case of bilateral congenital iris ectropion associated with juvenile glaucoma in an otherwise healthy 9-year-old girl. Intra-ocular pressure (IOP) measured by Goldmann applanation tonometry was 40 mmHg in the right eye and 32 mmHg in the left eye. Despite maximum IOP lowering therapy, the desired IOP levels needed to prevent further progression of the glaucoma could not be achieved. Visual field testing and confocal scanning laser tomography showed glaucomatous neuropathy. Trabeculectomy with antimetabolites (mitomycin C 0.2 mg/ml) was performed in both eyes and IOP was normalized without IOP lowering medications during a follow-up of 4 years. The authors point out the importance of recognizing congenital iris ectropion early to prevent blindness in young patients and improve their prognosis.     

Bilateral spontaneous anterior lens dislocation in a retinitis pigmentosa patient

PURPOSE: To report a case of bilateral spontaneous anterior lens dislocation associated with retinitis pigmentosa (RP). METHODS: A 45-year-old male with RP presented with elevated intraocular pressure (IOP) in the right eye and was treated with laser iridotomy (LI). After LI, complete crystalline lens dislocation into the anterior chamber occurred. Surgical intervention, including anterior vitrectomy, intracapsular cataract extraction (ICCE), and IOL scleral fixation was performed. Two years later, the same episode occurred in his left eye and a similar treatment was done. RESULTS: Surgery was successful in both eyes. CONCLUSIONS: This is the first report of bilateral spontaneous anterior lens dislocation in a RP patient.     

A case of uveal, palpebral, and orbital invasions in adult T-Cell leukemia

BACKGROUND: Patients with adult T-cell leukemia (ATL) may have eyelid lymphoma, uveitis, or cytomegalovirus retinitis due to being immunocompromised. However, there have been few reports on the invasion of multiple ocular lesions. We treated 1 unusual ATL patient with uveitis in whom multiple ocular invasions were suspected. CASE: A woman in whom ATL was diagnosed 10 years previously complained of blurred vision and decreased visual acuity in the right eye. Anterior uveitis of the right eye was suspected. One week later the cells increased in the anterior chamber, and fibrin exudates and hyphema appeared. She was admitted to our hospital. OBSERVATIONS: The visual acuity was 0.04 in the right eye and finger-counting from 30 cm in the left. She was treated with systemic steroid therapy. Inflammation disappeared, but both eyelids became swollen and multiple ocular lesions appeared. She was given carcinostatic therapy once more and the mass lesions decreased. Mass lesions appeared in the iris and in the bulbar conjunctiva. Computed tomography and magnetic resonance imagining (MRI) showed that the mass lesions extended to the right orbit and both nasal cavities. MRI also demonstrated choroidal thickening in the left eye. CONCLUSION: This case documents that ATL cells may cause severe uveitis and invade multiple ocular tissues such as the iris, eyelid, choroids, and orbit.     

Unilateral retinitis pigmentosa with amblyopia in the fellow eye

Purpose The purpose was to report a case of unilateral retinitis pigmentosa with amblyopia in the other eye.Methods Eight years ago, a 36-year-old woman complained of peripheral visual field loss in her left eye when she covered her right eye. Complete ophthalmological examinations including fundus photography and fluorescein angiography, full-field electroretinography, dark adaptation, and microperimetry were performed.Results Best-corrected visual acuity of the patient was 0.2 with +2.5 diopters in her right eye and 0.3 with +1.5 diopters in her left eye. Fundus examination did not reveal any abnormalities in the right eye, but narrow retinal vessels, optic disc pallor, and peripheral retinal pigment clumping was found in the left eye. The full-field electroretinography was normal in the right eye, but rod response, cone response, and combined response were non-recordable, and 30-Hz response was very small in the left eye. Dark adaptation thresholds were normal in the right eye, but were dramatically elevated in the left eye. Microperimetry was normal in the right eye, but the visual field was reduced to a 2×5° central field in the left eye.Conclusion Unilateral retinitis pigmentosa with amblyopia in the other eye is a rare condition. The patient presented here had a case of unilateral retinitis pigmentosa with the other eye with ametropic amblyopia. It is necessary to perform functional and morphological ophthalmological examinations to confirm the diagnosis.     

Cytomegalovirus retinitis in Wegener's granulomatosis: case report and review of the literature

PURPOSE: To present the first case of cytomegalovirus (CMV) retinitis in a Wegener's granulomatosis patient on systemic cyclosporine A (CsA) therapy. METHODS: A 41-year-old patient was treated with systemic CsA for c-ANCA positive Wegener's granulomatosis. Four weeks later he presented with blurred vision and a retinitis was diagnosed in the right eye. After detection of CMV DNA by polymerase chain reaction (PCR), the patient was treated with intravenous gancyclovir. Due to bone marrow toxicity, systemic antiviral therapy was discontinued and an intravitreal gancyclovir pellet was implanted. RESULTS: The retinitis lesions were scarified under this therapy and the patient's visual function was restored completely. CONCLUSIONS: Cytomegalovirus retinitis can occur under systemic monotherapy with CsA in patients with systemic vasculitis. Intraocular virus DNA detection by PCR is recommended to rule out retinal involvement of the underlying vasculitis in atypical cases and to preserve the patient's visual acuity.     

Longitudinal observations on mutations conferring ganciclovir resistance in patients with acquired immunodeficiency syndrome and cytomegalovirus retinitis: The Cytomegalovirus and Viral Resistance Study Group Report Number 8.

PURPOSE: Cytomegalovirus retinitis is the most common intraocular infection in patients with acquired immunodeficiency syndrome (AIDS). With prolonged suppressive anticytomegalovirus maintenance therapy, resistance occurs in over 25% of patients. We evaluated longitudinal changes in the cytomegalovirus genotype in patients with cytomegalovirus retinitis who developed ganciclovir resistance that was demonstrated in either the blood or urine. METHODS: Patients with AIDS and previously untreated cytomegalovirus retinitis were followed prospectively for the occurrence of resistance while on treatment. Blood and urine specimens were obtained periodically for cytomegalovirus culture according to a predetermined schedule. Positive isolates were tested for phenotypic susceptibility and for mutations in the UL97 and UL54 genes. RESULTS: A mutation conferring resistance to ganciclovir in either the UL97 or UL54 gene was detected in 18 patients. In general, patients with a genotypically resistant virus developed increasing phenotypic resistance over time. There was a suggestion that unless therapy was changed, UL97 mutations tended to persist. In seven of eight patients, the mutations identified in isolates from the blood and urine were identical. In selected patients, there was a suggestion that a mixed population of cytomegalovirus might be present. Progression of the retinitis in an involved eye (15 of 18), contralateral eye retinitis (10 of 11), and extraocular cytomegalovirus disease (5 of 18) occurred commonly among patients with resistant virus. CONCLUSION: Resistance-conferring mutations in the cytomegalovirus genome emerge and may persist when the selective pressure for resistance is maintained. Some patients appear to harbor complex subpopulations of virus with different mutations and different levels of phenotypic resistance. Changes in therapy may result in a shift in virus population and changes in the cytomegalovirus genotype identified.     

Spider keratouveitis. A case report

PURPOSE: To present a case report on a patient with keratouveitis after exposure to a tarantula. MATERIAL: A 15-year-old boy presented with ocular redness and discomfort after his pet tarantula (Chilean Rose) had walked over his face. METHODS: Clinical examination revealed inflammatory reactions in the corneas and the anterior chamber. After nine months, two subepithelial infiltrates of the cornea emerged, surrounding tarantula hairs of the stroma. The hairs were removed. RESULTS: Topical steroids relieved the symptoms, but ten months after exposure there was still a mild reaction in the anterior chamber of the right eye. CONCLUSION: Hairs from pet tarantulas might penetrate deeply into the eye and give keratouveitis. The hairs might be difficult to detect. Though rare, this phenomenon should be known by both ophthalmologists and the public.     

Two cases of retinitis pigmentosa associated with choroidal neovascularization

PURPOSE: To report two cases of retinitis pigmentosa in which choroidal neovascularization developed. CASES: The first case was a 50-year-old man with retinitis pigmentosa. In his left eye, choroidal neovascularization developed at the upper side of the fovea. Visual acuity in the eye was 0.3 at the first visit, and had decreased to 0.04 at a 14-year follow-up. The second case was a 60-year-old man with retinitis pigmentosa. Subfoveal choroidal neovascularization had developed in his right eye. Visual acuity in the eye was 0.03 at the first visit, and had decreased to hand motion at a 15-year follow-up. CONCLUSION: These cases are the first reports of the coincidence of retinitis pigmentosa and choroidal neovascularization in Japan. Although the etiology is unknown, genetic investigation might be useful to clarify the association between retinitis pigmentosa and choroidal neovascularization.     

CMV retinitis in a patient with good syndrome

PURPOSE: To describe cytomegalovirus (CMV) retinitis in a patient with Good syndrome. METHODS: A 48-year-old patient with Good syndrome presented with a necrotizing retinitis in the left eye. Quantitative touchdown real-time polymerase chain reaction (PCR) was performed on aqueous fluid. RESULTS: Quantitative PCR showed 152 copies of CMV per ml and was negative for varicella zoster virus (VZV), Epstein-Barr virus (EBV), herpes simplex virus (HSV-1), and HSV-2. The positive CMV PCR suggested CMV retinitis and the patient was treated with intravitreal ganciclovir injections (2.5 mg/0.05 ml), followed by ganciclovir implant. The retinal lesions showed decreasing activity two weeks after the onset of the therapy. A repeat PCR showed a decreasing number of CMV copies at one and two weeks (122 copies/ml and 0 copies/ml, respectively) that correlated clinically with the decreasing retinitis activity. CONCLUSIONS: Quantitative PCR can be useful in diagnosing as well as assessing the response to therapy of CMV retinitis in patients with Good syndrome.     

Reversible bull's-eye maculopathy associated with intravitreal fomivirsen therapy for cytomegalovirus retinitis

PURPOSE: To report two cases in which a bull's eye maculopathy developed after intravitreal injection of fomivirsen. METHODS: Case reports. RESULTS: A 50-year-old man with acquired immunodeficiency syndrome (AIDS) and refractory cytomegalovirus retinitis developed bull's-eye pigmentary changes in the macula of the right eye after initiating therapy with fomivirsen (Vitravene; CIBA Vision, Atlanta, Georgia) intravitreal injections. These pigmentary changes resolved upon cessation of treatment. A 36-year-old man with AIDS and refractory bilateral cytomegalovirus retinitis developed bull's-eye pigmentary changes in both eyes during bilateral intravitreal treatment with fomivirsen. Vision was not affected. These changes resolved after treatment with fomivirsen was stopped. CONCLUSION: Fomivirsen, a new medication for the treatment of refractory cytomegalovirus retinitis, may cause a bull's-eye maculopathy in some patients. The bull's-eye maculopathy is reversible and does not appear to affect vision.     

Weill Marchesani syndrome. Report of a case

Weill Marchesani syndrome is a congenital disease that combines microspherophakia and skeletal abnormalities. The authors report a 19-year-old male, born of a consanguineous marriage, with a progressive decrease in visual acuity. The general examination showed a squat look, dwarfism, muscle hypertrophy, short hands and feet, and joint stiffness. The ophthalmological examination showed that visual acuity was limited to hand motion in the right eye despite correction and no perception of light in the left eye. Intraocular pressure was 36 mmHg in the right eye and 40 mmHg in the left eye. The anterior chamber was irregular with iridophakodonesis and microspherophakia of both lenses. The zonula was partially ruptured in the right eye. The iridocorneal angle was narrow. Fundus eye examination showed a pale optic disc with an excavation of 9/10 on the right. In the left eye, the optic disc was totally excavated. Cardiovascular check-up revealed rheumatic aortic valvular cardiopathy. The therapy consisted of combined surgery: phakophagia with anterior vitrectomy plus trabeculectomy operated on the right eye. Weill Marchesani syndrome is a rare congenital affection with a recessive autosomal transmission. The visual prognosis is dominated by secondary glaucoma due to pupillary blockage by the mobile eye lens. This observation illustrates the seriousness of spontaneous progression in Weill Marchesani syndrome, justifying the necessity of lens extraction before the onset of complications.     

Acute angle-closure glaucoma from spontaneous massive hemorrhagic retinal detachment

PURPOSE: To report a case of acute angle-closure glaucoma resulting from spontaneous hemorrhagic retinal detachment. METHODS: An 81-year-old woman visited our emergency room for severe ocular pain and vision loss in her left eye. Her intraocular pressures (IOPs) were 14 mmHg in the right eye and 58 mmHg in the left eye. Her visual acuity was 0.4 in the right eye but she had no light perception in the left eye. The left anterior chamber depth was shallow and gonioscopy of the left eye showed a closed angle. In comparison, the right anterior chamber depth was normal and showed a wide, open angle. Computed tomography and ultrasonography demonstrated retinal detachment due to subretinal hemorrhage. After systemic and topical antiglaucoma medications failed to relieve her intractable severe ocular pain, she underwent enucleation. RESULTS: The ocular pathology specimen showed that a large subretinal hemorrhage caused retinal detachment and pushed displaced the lens-iris diaphragm, resulting in secondary angle-closure glaucoma. CONCLUSIONS: Prolonged anticoagulant therapy may cause hemorrhagic retinal detachment and secondary angle-closure glaucoma. If medical therapy fails to relieve pain or if there is suspicion of an intraocular tumor, enucleation should be considered as a therapeutic option.