Cases reported "Corneal Diseases"

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1/19. corneal transplantation in a patient with mucopolysaccharidosis type VII (Sly disease).

    PURPOSE: To illustrate a good visual outcome following penetrating keratoplasty in a patient with Sly disease, a rare mucopolysaccharidosis (MPS) caused by a deficiency of beta-glucuronidase. methods: A 15-year-old male with progressive bilateral corneal opacification had a complete medical, genetic, and ophthalmic evaluation followed by a penetrating keratoplasty. RESULTS: The cornea has remained clear for two years following surgery. Histopathology of the corneal button demonstrated vacuoles and granular inclusions consistent with this lysosomal storage disease. CONCLUSION: While research is ongoing in the fields of enzyme replacement and bone marrow transplantation, these treatments may not alleviate or reverse the corneal clouding. This case illustrates that cornea transplantation may be a valuable treatment option for visually rehabilitating such patients.
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ranking = 1
keywords = bone
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2/19. Histopathologic findings in explanted osteo-odontokeratoprosthesis.

    PURPOSE: To report the histologic findings in three explanted osteo-odontokeratoprosthesis (OOKP) laminae. methods: The osteodental lamina of patient 1 with linear iga bullous dermatosis had to be explanted 15 months after OOKP surgery because of permanent leakage after the resorption of the inferior half of the bone/dentin complex. Patient 2 underwent surgery because of corneal blindness caused by ocular pemphigoid. The remnants of the osteodental lamina were removed after a spontaneous loss of the optic cylinder 30 months after surgery. Patient 3 showed decentration of the optic cylinder 14 years after OOKP surgery was performed because of severe bilateral chemical burn. After intraoperative detachment of the optical cylinder from the broken lamina, the keratoprosthesis had to be completely removed. All specimens were fixed, decalcified using electrolysis, sectioned, and stained with hematoxylin and eosin. RESULTS: All three laminae showed some degree of transformation and resorption of dentin and bone tissue. An inflammatory reaction could be observed in the laminae of patient 1 and 2. Downgrowth of keratinizing squamous epithelium and a reduction of bone tissue and dentin was found in patient 1. Most of the osteodental lamina of patient 2 had been replaced by connective tissue. No signs of acute or chronic inflammation could be found in patient 3. The optical cylinder was surrounded by a thin layer of connective tissue that was enclosed by a layer of woven bone. Dentine was found in just one half of the specimen, adjacent to a partly preserved alveolar-dental ligament. CONCLUSION: No conclusive explanation for the dissolution of the osteodental lamina in some of the patients can be offered at this time, although a few different mechanisms such as epithelial downgrowth and/or localized inflammation seem to play a pivotal role. Conversely, the preservation of the alveolar-dental ligament seems to be essential for the maintenance of the integrity of the OOKP.
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ranking = 4
keywords = bone
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3/19. Chronic endophthalmitis mimicking an endothelial immune reaction after penetrating keratoplasty.

    We describe a patient with chronic inflammation after combined penetrating keratoplasty and cataract surgery. This condition has been considered an unusual endothelial immune reaction. Cytopathological examination of the aqueous humor showed abundant neutrophil granulocytes, a few macrophages, and sparse lymphocytes. The predominance of neutrophil granulocytes but no macrophages or lymphocytes, as found in cases of an endothelial immune reaction, was interpreted as evidence of chronic endophthalmitis. Cytopathological evaluation of aqueous humor can be a helpful tool for differentiating between an endothelial immune reaction and chronic endophthalmitis after combined PKP and cataract surgery.
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ranking = 2385.2901624323
keywords = macrophage
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4/19. Bilateral enophthalmos associated with paget disease of the skull: a case report.

    PURPOSE: To describe a case of familial Paget disease of bone associated with bilateral enophthalmos and mild exposure keratopathy. methods: Case report and literature review. RESULTS: A 71-year-old woman with familial Paget disease of bone had a long history of "receding eyes," headaches, and moderately dry eyes. She had bilateral enophthalmos with mild exposure keratopathy. Radiologic and endocrinologic investigation confirmed Paget disease of bone affecting the skull. CONCLUSIONS: enophthalmos in association with Paget disease of bone is rare and is likely to result from differential expansion of the cranium compared with the orbital bones.
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ranking = 5
keywords = bone
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5/19. Lipofuscinosis of the cornea. A clinicopathologic study of three cases.

    BACKGROUND: lipofuscin pigments are the indigestible residue of lysosomal activity usually associated with normal aging. Abnormal amounts of lipofuscin also are associated with certain disease processes. The rarity of lipofuscin in the cornea and the similarities between its staining properties and those of intracellular micro-organisms caused great diagnostic problems in three cases. The correct diagnosis of corneal lipofuscinosis was made after extensive histochemical, autofluorescent, and ultrastructural studies. methods: Clinical histories of three patients are correlated with morphologic and histochemical findings on five corneal buttons with lipofuscinosis. RESULTS: The histopathologic features of one cornea with chronic keratitis and three corneas from two patients with bilateral opacities of undetermined origin were mostly similar. Large amounts of lipofuscin pigment were found within macrophages and stromal keratocytes. Other pathologic findings were nonspecific, including phagocytosis of degenerated collagen fibrils, scarring, and neovascularization of the stroma. CONCLUSION: The lipofuscin deposits are probably the consequence of a corneal degenerative process and not its cause, although their pathogenesis remains unclear. Familiarity with the morphologic appearance of corneal lipofuscinosis and its staining and autofluorescent properties is important because the small, 1- to 3-microns deposits may be mistaken for intracellular micro-organisms.
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ranking = 1192.6450812162
keywords = macrophage
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6/19. Bilateral recurrent calcareous degeneration of the cornea.

    PURPOSE: To report clinical, histologic, and electron microscopic findings in several consecutive keratoplasties with recurrent pancorneal calcification in a patient with chronic graft versus host disease and severe keratoconjunctivitis sicca following bone marrow transplantation for chronic myelogenous leukemia. methods: Altogether 5 penetrating keratoplasties were performed in both eyes for descemetocele formation and corneal perforation as well as pancorneal calcification associated with severe visual loss. Histologic examination and electron microscopy were performed on the corneal buttons obtained. RESULTS: histology and electron microscopy confirmed pancorneal extracellular calcium deposition with increasing severity in each consecutive keratoplasty. Calcification was associated with necrosis and inflammation of the corneal stroma. Clinically unsuspected fungal keratitis was observed in 1 specimen. CONCLUSIONS: keratoconjunctivitis sicca, epithelial defects, corneal inflammation, and infection are most probably responsible for the recurrent pancorneal calcification seen in our patient. The role of cytokines in this complication is discussed.
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ranking = 1
keywords = bone
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7/19. Corneal pseudomembrane from acute inflammatory response and fibrin formation to acute myeloid leukemic infiltrate.

    PURPOSE: To describe an unusual ocular manifestation of a patient with acute myeloid leukemia (AML). DESIGN: Observational case report. methods: A 59-year-old woman with a history of preleukemic myelodysplastic syndrome (MDS) and status post bone marrow transplant (BMT) complained of a sudden onset of poor vision associated with a corneal pseudomembrane. Ocular graft vs host disease was suspected, and the pseudomembrane was excised for histopathologic examination. RESULTS: The pseudomembrane showed myeloblasts admixed with an acute inflammatory response suggestive of the development of AML, a complication of MDS. bone marrow examination confirmed the diagnosis of relapsing AML. CONCLUSIONS: Acute myeloid leukemia could present as a pseudomembrane; thus, examination of relevant ocular tissue is recommended.
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ranking = 1
keywords = bone
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8/19. Bilateral eyelid ecchymoses and corneal crystals: an unusual presentation of multiple myeloma.

    PURPOSE: This report describes a case of multiple myeloma that presented as bilateral eyelid ecchymoses and corneal crystals in the absence of widespread signs of systemic disease. methods: A 55-year-old man was found to have the sudden appearance of bilateral eyelid ecchymoses after he flexed 90 degrees at the waist. On examination, amyloid deposition was found in the area of the lid ecchymoses, and corneal crystals were dispersed centrally and peripherally throughout all levels of the cornea on slit lamp examination. urinalysis revealed Bence-Jones proteins and free kappa light chains. Bone marrow plasmacytosis suggested multiple myeloma. RESULT: Ophthalmic and systemic findings led to a workup, which resulted in a diagnosis of multiple myeloma. CONCLUSION: Our case demonstrates that multiple myeloma may present clinically with ophthalmic findings such as bilateral eyelid ecchymoses and corneal crystals in the absence of widespread signs of disease. The ocular findings may be the first manifestations of disease. Therefore, a systemic workup should be performed on patients with this presentation, including urine and bone marrow analyses.
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ranking = 1
keywords = bone
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9/19. Recurrent corneal perforation and acute calcareous corneal degeneration in chronic graft-versus-host disease.

    keratoconjunctivitis sicca (KCS) is a common complication of graft-versus-host disease (GVHD), and may lead to corneal epithelial defect and melting. In contrast, recurrent corneal calcareous degeneration and perforation is rare. A 46-year-old woman developed chronic GVHD after bone marrow transplantation for aplastic anemia. Severe KCS with corneal melting and calcium deposits were noted in the left eye. Penetrating keratoplasty was performed because of corneal perforation, but poor re-epithelialization and calcium deposition recurred. Lamellar keratectomy and amniotic membrane transplantation (AMT) were performed, but acute calcareous degeneration developed with subsequent recurrence of corneal perforation. After regraft, AMT and tarsorrhaphy, the corneal graft remained clear for 3 months. However, breakdown of the corneal epithelium occurred 3 weeks after spontaneous separation of tarsorrhaphy. Six months later, corneal perforation recurred again along with exacerbation of GVHD. Regraft was performed, but the patient refused tarsorrhaphy and AMT. Poor re-epithelialization persisted after regraft. Corneal melting with impending corneal perforation ensued. Further corneal surgery was refused and the patient chose to undergo evisceration. This case demonstrates that the ocular complications of GVHD may be severe enough to lead to corneal perforation and calcareous degeneration that is recalcitrant to medical and surgical treatment.
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ranking = 1
keywords = bone
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10/19. Corneal and conjunctival crystals in paraproteinemia.

    A 64-year-old woman with bilateral corneal and conjunctival crystal deposition was evaluated. A biopsy of her conjunctiva showed intracytoplasmic inclusions of immunoglobulin crystals in fibrocytes, macrophages, and endothelial cells. serum protein electrophoresis showed elevated kappa and IgA light and heavy chains which corresponded with immunoperoxidase staining results of the conjunctival biopsy. Conjunctival and corneal crystal deposition may be indicative of paraproteinemia, and histopathologic examination of a conjunctival biopsy may be useful in diagnosing this condition.
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ranking = 1192.6450812162
keywords = macrophage
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