Intraocular inflammatory reactions without focal necrotizing retinochoroiditis in patients with acquired systemic toxoplasmosis

PURPOSE: To describe the occurrence of intraocular inflammatory reactions as the sole ophthalmic manifestation of acquired systemic toxoplasmosis. METHODS: Review of medical records for 10 patients with uveitis and evidence of recent Toxoplasma gondii infection. RESULTS: Patient ages ranged from 3 to 51 years. Ocular symptoms were present in each of eight adult patients. Inflammation was unilateral in nine patients; it manifested as vitreous humor cells and haze (10 patients), anterior chamber cells (seven patients), and retinal vasculitis (seven patients). No patient had necrotizing retinochoroiditis upon initial examination. Inflammation resolved in each of nine patients who had follow-up examinations. Foci of retinitis or inactive retinochoroidal scars were seen in four of these nine patients during follow-up examinations, at intervals of 2.0 weeks to 2.5 years after initial examination. CONCLUSIONS: Retinal vasculitis and associated inflammatory reactions may be the only ophthalmic disorder during the early stages of a newly acquired T. gondii infection. Later development of retinitis or scars consistent with toxoplasmic retinochoroiditis in the same eyes suggests that the initial, isolated inflammation may be caused by the presence of parasites in retinal tissue. These cases may have implications for understanding the original source of retinal infection in patients who have recurrent toxoplasmic retinochoroiditis and for treatment of newly acquired T gondii infection.     

Posterior scleritis and its association with HLA B27 haplotype

BACKGROUND: Posterior scleritis is most commonly idiopathic but is also found in association with systemic disorders like rheumatoid arthritis. HLA B27 spondyloarthropathy manifests itself mainly in the form of anterior uveitis and anterior scleritis. Posterior scleritis has not been previously reported in patients with HLA B27 haplotype. We document the likely association between posterior scleritis and HLA B27 haplotype in a series of 5 patients who presented to the Ocular Inflammation and Immunology Services of the Singapore National Eye Centre. METHODS: Retrospective observational case series. The medical records and ultrasound B scans of 5 patients with a diagnosis of posterior scleritis were reviewed. RESULTS: The medical records of 5 patients (4 Chinese and 1 Malay) in the age range of 22-64 years were reviewed. All had unilateral disease (4 in the left eye and 1 in the right eye) at presentation. The most common presenting symptoms were pain (5) and blurring of vision (5); diplopia and proptosis was present in 2 patients. Two patients had anterior scleritis and all had an associated anterior uveitis. Posterior segment findings included optic disc edema (5), macular edema (5), choroidal folds (3), exudative retinal detachment (2) and choroidal effusion (1). All patients had a thickened sclera on serial ultrasound B scan. 2 patients had joint involvement preceding ocular signs and symptoms and were diagnosed to have ankylosing spondylitis. HLAB27 was positive in all our patients. Systemic workup for other diseases was negative. The ocular inflammation responded to a varying combination of topical, periocular and systemic steroids. One of them developed a recurrence of posterior scleritis which resolved with oral steroids. Visual outcome was satisfactory in all but 1 patient who had retinal pigment epithelium atrophy at the macula. CONCLUSION: Although posterior scleritis has been reported in association with ankylosing spondylitis, it is not clear whether it is the HLAB27 haplotype that is associated with posterior scleritis or ankylosing spondylitis. All our patients were HLAB27 positive but 2 of them did not have an underlying spondyloarthropathy. This suggests the possible association of posterior scleritis with HLAB27 haplotype.     

Anterior chamber infiltrates associated with systemic lymphoma: report of two cases and review of the literature

PURPOSE: To describe the clinicopathologic features of two patients with systemic lymphoma who developed anterior chamber (AC) infiltrates of lymphoma cells. DESIGN: Two case reports and literature review. METHODS: The clinical and pathologic findings in two patients with AC infiltrates secondary to systemic B-cell lymphoma are reviewed. MAIN OUTCOME MEASUREMENTS: Clinical observation and cytologic/flow cytometric examination of the infiltrate after AC aspiration. RESULTS: One patient presented with uveal infiltration, an exudative retinal detachment and an AC infiltrate. Systemic evaluation revealed a follicular lymphoma involving several groups of lymph nodes. The second patient with a known history of abdominal lymphoma was found to have blurred vision, photophobia and an AC infiltrate. Flow cytometric analysis of the AC infiltrate in both patients showed phenotypes consistent with the patients' systemic lymphomas. CONCLUSIONS: A pseudohypopyon in an adult may represent either the initial manifestation or a later complication of systemic lymphoma, similar to what has been reported in acute leukemia.     

A 25-Year-Old Man with Exudative Retinal Detachments and Infiltrates without Hematological or Neurological Findings Found to Have Relapsed Precursor T-Cell Acute Lymphoblastic Leukemia

Background

Precursor T-cell acute lymphoblastic leukemia (pre-T-ALL) may cause ocular pathologies such as cotton-wool spots, retinal hemorrhage, and less commonly, retinal detachment or leukemic infiltration of the retina itself. However, these findings are typically accompanied by the pathognomonic hematological signs of acute leukemia.

Case Presentation

In this case report and review of the literature, we describe a particularly unusual case of a 25-year-old man who presented to our hospital with bilateral exudative retinal detachments associated with posterior pole thickening without any hematological or neurological findings. The patient, who had a history of previously treated pre-T-ALL in complete remission, was found to have leukemia cell infiltration on retinal biopsy.

Conclusion

Our case underscores the fact that the ophthalmologist may be the first provider to detect the relapse of previously treated leukemia, and that ophthalmic evaluation is critical for detecting malignant ocular infiltrates.Key Words: Leukemia, Retinal detachment, Ophthalmology     

New corneal findings in human T-cell lymphotrophic virus type 1 infection

PURPOSE: Human T-cell lymphotrophic virus type 1 is a RNA retrovirus that primarily affects CD4+ T-cells. Human T-cell lymphotrophic virus type 1 infection is the established cause of adult T-cell leukemia/lymphoma, an aggressive malignancy of CD4+ T-cells, and two nonneoplastic conditions: human T-cell lymphotrophic virus type 1-associated myelopathy/tropical spastic paraparesis and human T-cell lymphotrophic virus type 1 uveitis. Other reported ophthalmic manifestations of human T-cell lymphotrophic virus type 1 infection include lymphomatous and leukemic infiltrates in the eye and ocular adnexa in patients with adult T-cell leukemia/lymphoma, retinal pigmentary degeneration, and neuro-ophthalmic disorders in patients with human T-cell lymphotrophic virus type 1-associated myelopathy/tropical spastic paraparesis and keratoconjunctivitis sicca, episcleritis, and sclerouveitis in asymptomatic human T-cell lymphotrophic virus type 1 carriers. This report describes the ocular findings in three Jamaican patients with human T-cell lymphotrophic virus type 1 infection and adult T-cell leukemia/lymphoma. METHODS: The clinical records of three patients with human T-cell lymphotrophic virus type 1 infection and adult T-cell leukemia/lymphoma examined at the National Eye Institute were reviewed. Each patient had one or more complete ophthalmic evaluations. RESULTS: All three patients had corneal abnormalities, including corneal haze and central opacities with thinning; bilateral immunoprotein keratopathy; and peripheral corneal thinning, scarring, and neovascularization. All three patients had elevated serum immunoglobulin levels. CONCLUSIONS: We believe that the novel corneal findings in these patients are most likely a consequence of the hypergammaglobulinemia induced by the human T-cell lymphotrophic virus type 1 infection or the T-cell malignancy.     

Analysis of 135 autopsy eyes for ocular involvement in leukemia

To explain the marked variation in the reported incidence of how often leukemic cells infiltrate the eye in fatal cases of leukemia, we tested the hypothesis that ocular leukemic infiltration is related to the peripheral leukocyte count during the final hours of life. We reviewed tissue sections, as well as autopsy and clinical records, from 135 patients who had fatal leukemia and had their eyes examined after death at Duke University Medical Center. Infiltrates of leukemic cells were found in the eyes of 42 of 135 patients (31.1%), with the choroid being the most frequently involved site. We detected a significant positive correlation between the ocular leukemic infiltration and an agonal leukocyte count as well as the severity of systemic disease. Differences in the agonal circulating leukocyte count may partly explain variations in the incidence of leukemic infiltrates in different postmortem studies.     

Flecked retina in systemic large-cell non-Hodgkin's lymphoma: case report

We report on a patient with a past medical history of successfully treated systemic large-cell non-Hodgkin's lymphoma (SNHL), who presented with multifocal yellowish retinal infiltrates (flecked retina) involving the post-equatorial retina of one eye. Fluorescein angiography revealed that the retinal infiltrates were hypofluorescent throughout the examination. The correct diagnosis of this ocular picture was important because the retinal lesions indicated central nervous system recurrence of systemic large-cell non-Hodgkin's lymphoma.     

Outcome and cost analysis of scheduled versus emergency scleral buckling surgery.

BACKGROUND: Retinal detachments are usually considered to be a surgical emergency. However, there are additional risks and costs for unnecessary emergency surgeries. The purpose of this study is to evaluate whether the conventional wisdom for treating all retinal detachments as emergencies needs to be re-examined. METHODS: Forty-eight patients who had an emergency scleral buckle and 89 patients who had a scheduled procedure were randomly selected from 884 consecutive patients who had a primary scleral buckling procedure during a 4 1/2-year period. The medical records of each patient were used to obtain detailed information related to prognosis. The visual acuity measurements of each patient, taken 6 months after the procedure, were obtained from the records of the ophthalmologist following the patient. Linear regression analysis was used to compare the final visual outcome for patients who had emergency surgery with patients who had scheduled surgery after taking into account patient factors related to prognosis. RESULTS: Patients selected for emergency surgery had better visual prognoses than scheduled patients but had the same risk of systemic complications and the same extent of detachment if the macula was not involved. None of the 18 patients with an attached macula experienced macular involvement while awaiting scheduled surgery. There were no differences between emergency and scheduled patients in ocular or systemic complications, rate of reattachment, rate of decreased visual acuity after surgery, visual outcome adjusted for prognosis, or, since 1985, length of hospital stay. A greater cost was incurred for the patients having emergency surgery due to difference in pay scales for support personnel. CONCLUSIONS: Because the study is not large and the patients were not randomized to treatment, the results are not definitive. However, they suggest that emergency surgery is unnecessary for many patients with a detached retina.     

Acquired toxoplasmic retinitis in an immunosuppressed patient: diagnosis by transvitreal fine-needle aspiration biopsy

BACKGROUND: Acquired multifocal white retinal lesions in an immunosuppressed patient are diagnostically challenging. METHODS: Case report of a 34-year-old woman who underwent bone marrow transplantation for chronic myelogenous leukemia. Four months after the transplant, while on relatively high doses of immunosuppressive drugs, she developed bilateral multifocal retinitis versus leukemic retinal infiltration. Fine-needle aspiration biopsy was performed on one eye in an attempt to establish a cytological diagnosis. RESULTS: The aspirate was found to contain individual crescent-shaped intraretinal organisms and cysts, consistent with the diagnosis of toxoplasmic retinitis. The patient was started immediately on an anti-toxoplasmosis regimen consisting of sulfadiazine, pyrimethamine, and folinic acid. Follow-up examinations revealed complete inactivation of the retinitis and no delayed complications of the biopsy. CONCLUSION: Fine-needle aspiration biopsy can be a useful diagnostic tool in selected patients with acquired retinal infiltrates.     

Primary intraocular lymphoma: another great masquerader

PURPOSE: To describe diverse and atypical presentations of the most common masquerader in neoplastic masquerade syndromes. METHODS: Retrospective interventional case series. The authors identified three patients who presented with atypical and diagnostically challenging masquerading manifestations. These patients were eventually found to have primary intraocular lymphoma (PIOL). Their case histories, presenting signs and symptoms, diagnostic tests, and treatments are described. RESULTS: Patient 1 masqueraded as viral retinitis and branch retinal vein occlusion but was resistant to 5 weeks of oral and intravenous acyclovir. Patient 2 presented with choroidal infiltrates and vision loss. This patient had had breast carcinoma for the last 25 years and secondary metastasis was suspected. Patient 3 had chronic uveo-retinitis and a chronic Propionibacterium acnes infection was suspected. All three patients were diagnosed with PIOL. CONCLUSIONS: PIOL is an aggressive masquerader and not only presents clinical diagnostic difficulties but also requires expert tissue handling and analysis, so that early diagnosis can be made and therapy can be instituted.     

Posterior scleritis: clinical features, systemic associations, and outcome in a large series of patients

OBJECTIVE: To document the clinical features, systemic associations, and visual outcome in a large number of patients with posterior scleritis. DESIGN: Retrospective, noncomparative case series. PARTICIPANTS: There were 137 patient records showing patients with a diagnosis of posterior scleritis who were attending or had attended the scleritis clinic at Moorfields Eye Hospital between 1974 and 1996. Ninety-nine records were suitable for detailed analysis. METHODS: The medical records and B-mode ultrasound examinations were reviewed. MAIN OUTCOME MEASURES: The clinical features, systemic associations, treatment, and outcome of each patient were determined. RESULTS: Posterior scleritis occurred at all ages. The mean age at onset was 49.3 years. Posterior scleritis began before age 40 in 30% of patients and was twice as common in women as in men. The B-mode ultrasound examination showed diffuse and nodular changes in the posterior sclera. Necrotizing posterior scleritis was not identified. Twenty-nine percent of patients had an associated systemic disease that included systemic vasculidites, autoimmune diseases, and lymphoma. Such patients more commonly had nodular changes on B-mode ultrasound examination. Early treatment controlled posterior scleral inflammation and limited visual loss. Thirty-one percent of patients lost two or more lines of vision. Statistical analysis revealed that patients older than age 50 had an increased risk of having an associated systemic disease and were more likely to experience visual loss. Patients with associated systemic disease required more aggressive immunosuppressive therapy and more frequently had accompanying anterior scleritis. There was no association between unilateral, bilateral, or recurrent disease and the presence of systemic disease or visual loss from posterior scleritis. CONCLUSIONS: The B-mode ultrasound examination reveals that posterior scleritis occurs far more often than previously thought and can lead to rapid and permanent visual loss. All patients with posterior scleritis must be assumed to be at risk of visual loss. Forty percent of patients had no anterior scleral inflammation, and 9% had no detectable physical signs. All patients need to be investigated for an associated systemic disease and all require early treatment to minimize loss of vision.     

Ocular toxoplasmosis: clinical features and prognosis of 154 patients

PURPOSE: To ascertain the clinical features, visual outcome, and recurrence rates of ocular toxoplasmosis (OT) in a large series of patients. To determine the efficacy of various treatment strategies and identify the patients at risk of visual loss. DESIGN: Retrospective noncomparative observational case series. PARTICIPANTS: One hundred fifty-four consecutive patients with active lesions of OT (first attack and/or recurrence) were identified in a cohort of 1300 consecutive patients with uveitis. Mean follow-up was 5.8 years. INTERVENTION: A review of the medical records of 154 patients with active OT. MAIN OUTCOME MEASURES: Patients were subdivided according to clinical and laboratory criteria. Numerous variables were compared per patient and group, including age and gender distribution, onset and course of infection, clinical ocular features, laboratory data, therapeutic strategies and their outcomes, number of recurrences, complications, final visual acuity, and features associated with poor visual outcome. RESULTS: Primary retinal lesions were observed in 28% and a combination of active lesions and old retinochoroidal scars in 72% of the patients at first presentation to the ophthalmologist. Mean age at first presentation with an active OT lesion was 29.5 years. Patients with primary OT were older than those with a combination of active lesions and old scars (P < 0.001). Serologic characteristics of the acute phase of systemic infection were found in 11% of the patients. Ocular involvement in these patients was associated with advanced age at onset (P < 0.001) and was characterized by severe intraocular inflammation. Most (82%) of the patients with serologic characteristics of the acute phase of systemic infection had primary lesions (compared with 23% of OT in the chronic phase of systemic infection; P < 0.001). Extensive retinal lesions were more frequently observed during the acute phase of systemic infection (P = 0.02) and in patients with primary OT (P < 0.04). Recurrences, which developed in 79% of all patients followed for more than 5 years, were located predominantly in previously affected eyes (with old scars) in contrast to the sporadic cases of recurrence in the healthy contralateral eye (P < 0.0001). Standard short-term therapeutic modalities had no effect on visual outcome or future recurrence rates. Legal blindness in one or both eyes was confirmed for 24% of the patients. Blindness of both eyes was more frequent in patients with congenital OT (P < 0.001). Risk factors for visual loss included congenital infection, OT manifesting during the acute phase of systemic infection, central location and/or extensive retinal lesions, and the administration of corticosteroids without a shield of antiparasitic drugs. CONCLUSIONS: Legal blindness in at least one eye developed in 24% of the patients with OT. Recurrences, which developed in 79% of the patients with long-term follow-up, were located predominantly in eyes with toxoplasmic scars. Various short-term therapeutic modalities had no effect on visual outcomes or future recurrence rates, with the exception of a poor visual outcome for patients who received corticosteroids without a shield of antiparasitic drugs.     

Prevalence of systemic and ocular disease in infantile exotropia: comparison with infantile esotropia.

OBJECTIVE: Exotropia in infancy is believed to be associated with an increased prevalence of neurologic, ocular, and craniofacial abnormalities; however, the prevalence of coexisting ocular and systemic disease in these patients is unknown. In this study, the prevalence of ocular disease and systemic illness was determined in patients diagnosed with exotropia in infancy. DESIGN: Observational comparative case series. PARTICIPANTS: Medical records of 70 patients diagnosed with exotropia in the first year of life were reviewed and compared with records of 136 patients diagnosed with esotropia before 1 year of age. INTERVENTION: Patients with no disorders (other than latent nystagmus, dissociated vertical deviation, or oblique muscle overaction) were grouped as "simple" strabismus. Patients with systemic disorders (including prematurity, neurologic disease, and genetic disease) and patients with ocular disorders (including congenital nystagmus, other strabismus, ptosis, and any condition associated with loss of vision [except amblyopia]) were grouped as "complex" strabismus. MAIN OUTCOME MEASURES: Prevalence of coexisting systemic and ocular disorders. The demographics, strabismus measurements, and types of coexisting disease in the simple and complex groups were compared. RESULTS: A high percentage of both exotropia (67%) and esotropia (49%) patients had a coexisting ocular or systemic abnormality. Exotropia patients with a constant strabismus were more likely to have coexisting ocular or systemic disease than those with an intermittent strabismus. Smaller angles of exotropia or esotropia were associated with a higher likelihood of coexisting ocular or systemic diseases. Systemic disorders were found more frequently than ocular disorders in both the exotropia and esotropia groups. In 25% of all patients referred for evaluation of strabismus, an additional ocular or systemic abnormality was discovered by the ophthalmologist. CONCLUSION: Patients presenting to a university hospital-based practice in the first year of life with exotropia were more likely than those presenting with esotropia to have coexisting ocular and systemic disease. Both groups had a notably high prevalence of associated disorders. The percentages measured in this population may not be applicable to other practices because of referral bias. However, clinicians should consider that children presenting with infantile exotropia and esotropia appear to be at risk for coexisting ocular or systemic disease.     

Optic disk edema associated with peripapillary serous retinal detachment: an early sign of systemic Bartonella henselae infection

PURPOSE: To describe optic disk edema associated with peripapillary serous retinal detachment as an early sign of systemic Bartonella henselae infection. METHODS: Multicentered, retrospective case series. RESULTS: Five women and two men presented with optic disk edema producing peripapillary serous retinal detachment. Each patient had a markedly elevated serum anti-B. henselae antibody titer. Patient age ranged from 11 to 44 years, with a mean and median of 26.6 and 28 years, respectively. The time from the onset of systemic symptoms to the onset of visual symptoms varied from 3 days to 1 month. The peripapillary serous retinal detachment resolved within 1 to 3 weeks in each case, producing a macular star in four of seven patients. Initial vision was 20/200 or worse in five of seven patients and improved in four of these five patients to 20/30 or better. CONCLUSIONS: Systemic B. henselae infection should be considered in patients who develop optic disk edema associated with a peripapillary serous retinal detachment, even in the absence of classic neuroretinitis with a macular star.     

Evaluation of patients with scleritis for systemic disease

OBJECTIVE: To evaluate the relationship between associated medical conditions and scleritis-particularly, the timing of the diagnosis of these diseases. DESIGN: Retrospective case series. PARTICIPANTS: Patients with scleritis presenting to a single center over an 18-year period. METHODS: Medical records were reviewed for the presence of an associated infectious or rheumatic disease and for the timing of the diagnosis of the systemic disease relative to the presentation for evaluation of the scleritis. MAIN OUTCOME MEASURES: Presence of an associated medical condition and timing of diagnosis relative to that of scleritis. RESULTS: In a series of 243 patients with scleritis, 44.0% had an associated medical condition: 7.0%, an infection, and 37.0%, a rheumatic disease. The most frequent infection was herpes zoster, and the most frequent rheumatic disease was rheumatoid arthritis, present in 4.5% and 15.2% of patients, respectively. Of the 107 patients with an underlying disease, 77.6% had a previously diagnosed disease, 14.0% had their conditions diagnosed as a result of the initial evaluation, and 8.4% developed a systemic disease during follow-up. Systemic vasculitis was less likely to have been previously diagnosed than other rheumatic diseases (59.1% vs. 83.8%, P = 0.015) and more likely to be diagnosed by the initial diagnostic evaluation (27.3% vs. 8.8%, P = 0.027). Ten patients (4.1%) had a positive antineutrophil cytoplasmic antibody (ANCA) test result without clinical evidence of a systemic vasculitis. Four of 5 patients with a positive cytoplasmic ANCA test result but no clinical evidence of systemic vasculitis required immunosuppressive drugs for control of the scleritis, whereas 1 of the 5 patients with a positive perinuclear ANCA test result required immunosuppressive drugs. Among patients with no evident systemic disease after the initial diagnostic evaluation, the rate of occurrence of a rheumatic disease was 4% per person-year. CONCLUSIONS: Although associated systemic diseases are frequent among patients with scleritis, the majority are previously diagnosed. Systemic vasculitis is less likely than other rheumatic diseases to have been previously diagnosed. Because vasculitis is a potentially life-threatening disorder, it should be a focus of the diagnostic evaluation.     

Retinal manifestations of ocular lymphoma (reticulum cell sarcoma).

BACKGROUND: Diagnosis and treatment of ocular large cell lymphoma may lessen visual loss and prolong life. Although reports in the literature have described retinal infiltrates in eyes with large cell lymphoma, they have focused on the more prominent vitreous and subretinal pigment epithelial findings. Eyes with retinal infiltrates and hemorrhagic retinal necrosis are usually believed to harbor a microbial infection. The authors describe 5 patients, aged 57 to 85 years, with ocular lymphoma in whom the most prominent initial findings were in the retina. METHOD: Patients presented with findings suggestive of an infectious retinal necrosis. When the initial therapy failed, investigators performed a vitreous biopsy. Two patients had a concomitant retinal biopsy. Radiation therapy was given to four patients. RESULTS: All five patients had vitreous cells. Three patients had prominent perivascular exudate. Four patients had grayish-white retinal infiltrates, and three patients had associated retinal hemorrhage. Three patients had subretinal small white spots. An edematous thickened retina developed in three patients, and hemorrhagic retinal necrosis developed in three patients. The initial diagnosis was believed to be acute retinal necrosis (ARN) in three patients, toxoplasmosis in one patient, and frosted branch angiitis in one patient. Vitreous biopsy was positive in two patients but negative in three patients. In two of these three patients, the diagnosis was established by retinal biopsy. CONCLUSION: Ocular lymphoma should be considered in the differential diagnosis of retinal vasculitis or necrotizing retinitis in a middle-aged or older patient. Retinal biopsy may be helpful in establishing the diagnosis.     

Orbital Richter Syndrome

Abstract

We report two patients with previously diagnosed chronic lymphocytic leukemia who developed Richter syndrome in the orbit as the sole extranodal site. The medical history, clinical findings, orbital imaging and histopathological features of the patients were reviewed. Treatment protocols and the outcomes were also assessed. The first patient developed Richter syndrome at the age of 64 years, 3 years after the diagnosis of chronic lymphocytic leukemia. The tumor was located at the inferotemporal quadrant of the orbit. The second patient was 59 years old when Richter syndrome arose in the lacrimal gland, 4 years after the diagnosis of chronic lymphocytic leukemia. Incisional biopsy from the orbital tumors were performed. Histopathological findings included diffuse CD20, CD 23, CD5, bcl2, bcl6 positive lymphocytic infiltration. Both patients were treated with chemotherapy and rituximab. During 3 years of follow-up, there was no orbital or systemic recurrence of the disease. Richter syndrome may develop in the orbital soft tissue and the lacrimal gland, and the orbital disease appears to have a better prognosis compared to patients with systemic involvement.     

Fluocinolone Acetonide Intravitreal Implants in Vogt-Koyanagi-Harada Disease

Purpose: To describe the use of fluocinolone acetonide implants (Retisert) in Vogt-Koyanagi-Harada disease (VKH).

Design: Interventional case series.

Methods: Retrospective review of medical records.

Results: Two patients with VKH requiring high-dose systemic corticosteroid therapy to control their inflammation and bilateral serous retinal detachments received bilateral fluocinolone acetonide implants. Upon tapering of systemic corticosteroids, one patient had recurrent serous retinal detachments and the other patient’s anterior chamber and vitreous inflammation returned.

Conclusions: The authors’ experience with fluocinolone acetonide implants in VKH has been mixed with an inability to fully taper off of systemic corticosteroids.     

Peripheral retinal nonperfusion associated with chronic myeloid leukemia

PURPOSE: To report a case of peripheral retinal nonperfusion and chronic myeloid leukemia in a 23-year-old woman. DESIGN: Observational case report. METHODS: A complete ophthalmic and systemic evaluation was performed. RESULTS: Ophthalmic examination revealed peripheral retinal nonperfusion with retinal neovascularization in both eyes. Fluorescein angiography of both eyes showed a marked midperipheral and peripheral avascular retina temporally with arteriovenous anastomosis and seafan neovascularizations. Blood work showed no abnormalities, although marked leucocytosis (up to 750 x 10(9)/l) and thrombocytosis (646 x 10(9)/l) were present in 1998 when the patient was diagnosed with leukemia. Following treatment, the patient has been in remission. CONCLUSIONS: Peripheral retinal nonperfusion with retinal neovascularization may occur as a complication of chronic myeloid leukemia. In contrast to other studies describing this association, our patient had a bilateral peripheral retinal nonperfusion with seafan neovascularizations without relapse of the myeloid leukemia and without any of the other retinal signs associated with chronic myeloid leukemia, such as tortuosity of veins, intraretinal or preretinal hemorrhages, and cotton-wool exudates.     

Retinal Vasculitis Caused by Adult T-cell Leukemia/Lymphoma

Background To report a case of lymphomatous infiltration and bilateral retinal vasculitis observed among 83 cases of adult T-cell leukemia (ATL) treated in the University Hospital Center in Fort-de-France (Martinique, French West Indies) between 1984 and 2003.Case A complete clinical ophthalmologic examination was performed in this patient along with fluorescein angiography.Observations After being checked for diffuse adenopathies, myodesopsias, and phosphenes, the 35-year-old patient was diagnosed with ATL. The ocular impairment, present since the onset of ATL as peripheral subretinal infiltrates, spread progressively and afferently to the rest of the retina in the form of an essentially venous vasculitis. Impairment of the vitreous was noted only in the end stages of disease progression. As ocular lesions progressed, the general state of the patient degraded at the same time despite chemotherapeutic measures.Conclusion Among the more than 300 seropositive for human T-cell lymphotropic virus type 1 (HTLV-1) or patients with HTLV-1-associated myelopathy/tropical spastic paraparesis treated at our hospital in the last 20 years, and among the 83 cases of ATL, only this single case of retinal vasculitis associated with HTLV-1 was observed (1/83, 1.2%) in Martinique, confirming the geographic variability of the clinical phenotype of HTLV-1 infection. The incidence of retinal vasculitis in ATL patients may signify an even worse prognosis than initially indicated. Jpn J Ophthalmol 2005;49:41–45 © Japanese Ophthalmological Society 2005