Cases reported "Retinal Detachment"

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1/33. Corneal epithelial toxic effects and inflammatory response to perfluorocarbon liquid.

    We report an unusual case of corneal epithelial toxic effects associated with perfluorocarbon liquids (PFCLs). The clinical and histopathologic findings are described. An elderly man underwent vitreoretinal surgery for a complicated retinal detachment. Perfluorodecalin was used to repair the retina. It was left in situ for 8 weeks and removed via the pars plana. One month after removal of heavy liquids the patient developed a nonhealing corneal epithelial defect associated with limbitis. Perfluorodecalin was found under the superior conjunctiva. A conjunctival biopsy revealed the presence of vacuoles in the conjunctival stroma surrounded by an inflammatory response that consisted of lymphocytes, macrophages, and giant cells. On surgical removal of the PFCL from the subconjunctival space, the epithelial defect healed. The histopathologic and clinical evidence suggest that the inflammatory response and corneal epithelial ulceration were caused by the prolonged presence of PFCL in the subconjunctival space. To the best of our knowledge, PFCLs have not previously been reported to cause corneal epithelial defects or incite an inflammatory response in the human eye.
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2/33. Bilateral necrotizing scleritis and blindness in the myelodysplastic syndrome presumably due to relapsing polychondritis.

    PURPOSE: The purpose of this study was to report a case of bilateral blindness, bilateral necrotizing scleritis, and bilateral deafness in a patient with myelodysplastic syndrome (MDS). In such a patient, the possibility of relapsing polychondritis (RPC) associated with MDS must be considered. CASE REPORT/methods: A 66-year-old patient suffered from myelodysplastic syndrome (MDS). Shortly before his death, he became bilaterally blind and deaf. A biopsy was taken from the conjunctiva and the bone marrow, and both eyes were obtained after death for further investigation. Findings of the clinical and laboratory work-up for the patient's hematologic disorder as well as an examination of the eyes by light microscopy and immunohistochemistry are presented. RESULTS: Ocular sections showed a diffuse necrotizing scleritis with moderate uveitis and no identifiable infectious agent. Neither was there any evidence of a leukemic infiltration. The deafness had been due to inner ear failure, and the patient died of a cardiac failure. CONCLUSIONS: Non-infectious scleritis associated with inner ear deafness is a strong indication of relapsing polychondritis (RPC). Furthermore, RPC can be associated with MDS. Thus, in addition to leukemic infiltration and infection involving ocular structures, ophthalmologists and otolargyngologists should be aware of the association between MDS and RPC and the potential complications.
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3/33. Clinicopathologic findings in eyes with retained perfluoro-n-octane liquid.

    OBJECTIVE: To describe the clinical and histopathologic findings in five eyes with retained perfluoro-n-octane (PFO) liquid after retinal reattachment surgery. DESIGN: Retrospective, noncomparative, clinicopathologic case series. PARTICIPANTS: Surgical specimens from five eyes were studied. methods: Surgical specimens from eyes with prior intraoperative PFO use submitted to the W. Richard Green eye pathology Laboratory at the Wilmer Ophthalmological Institute were identified and reviewed. MAIN OUTCOME MEASURES: Histopathologic analysis and energy dispersive spectroscopy identified intracellular vacuoles containing PFO. RESULTS: Five cases were identified. Three specimens were obtained at the time of further surgery for recurrent retinal detachment; one at repeat penetrating keratoplasty, and one at removal of retained PFO. Each eye had macroscopic white flake-like material on intraocular structures noted before or during surgery. Histopathologic analysis disclosed an inflammatory response featuring macrophages with intracellular vacuoles containing PFO. Removal of the PFO in all five eyes combined with repeat retinal reattachment surgery in three eyes resulted in resolution of the inflammatory response. CONCLUSIONS: Retention of PFO after surgery elicits an inflammatory response. We suspect that young patients, those with considerable residual vitreous gel, and eyes with larger amounts of retained PFO may be at higher risk for this complication.
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4/33. retinal detachment and cataract, facial dysmorphism, generalized osteoporosis, immobile spine and platyspondyly in a consanguinous kindred--a possible new syndrome.

    We report on a consanguineous family with 6 children (out of 7) affected by a spondylo-ocular syndrome. Clinical features include cataract, loss of vision due to retinal detachment, facial dysmorphism, facial hypotonia, normal height with disproportional short trunk, immobile spine with thorakal kyphosis and reduced lumbal lordosis. On ophthalmological examination of the index patient, a dense cataract and complete retinal detachment could be detected on the right eye. On the left eye, an absent lens nucleus was found, but no retinal detachment. On radiological examination, there was generalized moderate osteoporosis; the spine showed marked platyspondyly and the bone age was advanced. On laboratory investigations, a normal excretion of amino acids, mucopolysaccharides and oligosaccharides could be found. The phenotypical spectrum observed in the 6 affected individuals was rather uniform. The karyotype was normal in all affected children. This hitherto undescribed combination of oculo-skeletal symptoms shows most resemblance with connective tissue disorders, suggesting a range of candidate genes for mutation analysis.
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5/33. central serous chorioretinopathy after bone marrow transplantation.

    PURPOSE: To describe central serous chorioretinopathy after bone marrow transplantation. methods: The medical records of the patient were reviewed retrospectively. RESULTS: A 46-year-old Filipino man developed multifocal central serous chorioretinopathy affecting his left eye 4 months after bone marrow transplantation for acute myelogenous leukemia. Other co-existing medical problems at the time of presentation included systemic hypertension and graft-versus-host-disease (GVHD), for which the patient was using both systemic corticosteroids and cyclosporine. CONCLUSION: central serous chorioretinopathy is a rare cause of vision loss in patients after bone marrow transplantation. Previous descriptions of bone marrow transplantation-associated central serous chorioretinopathy in patients with thrombotic microangiopathy, as well as the occurrence of both systemic hypertension and the use of systemic corticosteroids and cyclosporine in our patient with bone marrow transplantation-associated central serous chorioretinopathy, support theories of choroidal vascular compromise in the pathogenesis of central serous chorioretinopathy.
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6/33. Perfluorodecalin-induced intravitreal inflammation.

    OBJECTIVE: To report an unusual case of intravitreal inflammation in a human eye caused by the presence of residual perfluorodecalin in a case of giant retinal tear and retinal detachment. methods: The posterior capsule of the lens, which was infiltrated with deposits, was collected during surgery. The specimen was stained with hematoxylin and eosin, with periodic acid-Schiff, and for melanin. Part of it was examined with electron microscopy. Immunohistochemical staining was performed to demonstrate CD68 antigens, cytokeratin, and glial fibrillary acid protein. RESULTS: Vacuolated macrophages and retinal pigment epithelial cells infiltrated the posterior capsule. Electron microscopy showed the presence of membrane-lined vacuoles within the macrophages. A monolayer of epithelial cells covered the cellular infiltration. CONCLUSION: Residual perfluorodecalin can induce an intraocular chronic macrophage response.
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7/33. Acute leukemia presenting as diabetes insipidus and bilateral exudative retinal detachment--a case report.

    To report an unusual case of leukemia presenting as both bilateral exudative retinal detachment (ERD) and central diabetes insipidus (DI), we evaluate the clinical hematological records including bone marrow aspirations and CSF tapping, both osmolarity and electrolytes concentration of the serum and urine, brain MRI, fundus photographs and fluorescein angiographs in this 25-year-old female patient. Examinations of peripheral blood and bone marrow aspiration confirmed the diagnosis of acute myelogenous leukemia (AML-M0). fluorescein angiography (FA) revealed bilateral ERD, dense choroidal leukemia cell infiltration with overlying retinal pigment epithelium (RPE) dysfunction and focal areas of choroidal infarction. Changes in both osmolarity and electrolytes concentration of the serum and urine from vasopressin test supported the diagnosis of central DI. Central DI and ERD may be presenting signs of acute leukemia and both may represent CNS involvement. In our case, dense choroidal leukemic cell infiltration results in devitalization of RPE and choroidal infarction. Leukemic disruption of hypothalamic pituitary area may lead to complete or partial deficiency of antidiuretic hormone (ADH). Rapid improvement in visual acuity and partial symptom relief of DI may ensue from appropriate chemotherapy and nasal DDAVP (1-desamino-8-D-arginine vasopressin) supply.
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8/33. Transmission electron microscopic study of a subretinal choroidal neovascular membrane due to age-related macular degeneration.

    From a patient with age-related macular degeneration we studied ultrastructurally a disciform scar that was removed from an eye with a vitreous hemorrhage. In cross section, the scar was divided by a retinal pigment epithelial (RPE) cell layer. The choroidal side consisted of fibrovascular tissue with active neovascular buds and inflammatory cells, including macrophages attached to the RPE basement membrane. Apart from the RPE, no components of Bruch's membrane could be identified. The retinal side contained organizing hemorrhage and a collagenous matrix with fibroblastlike cells probably of RPE and choroidal origin. The anatomy and the clinical findings at surgery suggest that such scars lie on (rather than within) the inner collagenous layer of Bruch's membrane and contain two components divided by the original RPE layer. The choroidal side is fibrovascular, including active neovascularization, and the retinal side is fibrous and formed by metaplastic RPE cells and choroidal fibrovascular ingrowth.
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9/33. Further evidence for proinflammatory nature of perfluorohexyloctane in the eye.

    BACKGROUND: The authors present a clinicopathological report of their initial experience with perfluorohexyloctane (F6H8), a novel semifluorinated liquid fluorocarbon developed as a long-term vitreous substitute. methods: A retrospective observational review was performed of five patients in whom F6H8 had been used for management of rhegmatogenous retinal detachment. Surgical specimens taken from two patients at the time of F6H8 removal were also submitted for histopathological, immunohistochemical and electron microscopic analysis. RESULTS: Clinical and histological analysis of the present small case series confirmed the propensity of F6H8 to emulsify, and suggested a probable biological reaction to F6H8. Surrounding and engulfing the F6H8 were numerous cells morphologically in keeping with macrophages. Immuno-histochemistry confirmed macrophage phenotype but electron microscopic evaluation showed epithelial ultra-structural features. It is suggested that the finding of macrophagic phenotype in cells with epithelial ultra-structure provides further evidence for a continuum of phenotypic differentiation of the pigment epithelial cells as part of the repair and regeneration that is the proliferative vitreo-retinopathy (PVR) response. CONCLUSIONS: The data do not indicate any benefit of F6H8 over other perfluorocarbons for use in short-term post-operative intraocular tamponade. Although early experience suggests that F6H8 use in primary vitrectomy with minimal PVR is acceptable and produces temporary inflammatory effects only, these cases can often be successfully managed by conventional scleral buckling techniques, or vitrectomy with standard tamponading agents, without the need for F6H8 and subsequent extra surgical procedures. Furthermore in eyes already predisposed to inflammation through prior surgery and/or presence of PVR, the inflammatory effects were not insignificant. The use of F6H8 is not recommended in the clinical setting, except as part of a controlled trial subject to the approval of an ethics committee and informed consent.
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10/33. Bilateral serous macular detachment as a presenting feature of acute lymphoblastic leukemia.

    PURPOSE: To report a case of bilateral serous maculopathy as an initial sign of acute lymphoblastic leukaemia in children. methods/RESULTS: A 13-year-old girl, who presented with symptoms of visual blurring, was found to have a bilateral serous maculopathy. Haematological abnormalities (thrombocytopenia with a mild lymphocytosis) prompted further investigation. A bone marrow aspirate revealed the presence of leukemic blasts and a diagnosis of acute lymphoblastic leukaemia was made. Her maculopathy completely resolved following systemic chemotherapy. CONCLUSIONS: Prompt recognition of disease led to early systemic treatment and restoration of visual function.
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