Cases reported "Eye Diseases"

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1/59. Orbital infarction and melting in a patient with systemic lupus erythematosus.

    OBJECTIVE: To present a patient with systemic lupus erythematosus who developed infarction and melting of the orbit secondary to her systemic disease. DESIGN: A case report. PARTICIPANT: A 61-year-old white woman with a 5-year history of systemic lupus erythematosus. methods: The patient presented with left orbital pain, limitation of extraocular movements, and a fistula from the ethmoid sinus to the upper eyelid. A detailed examination with computerized tomography, ultrasound, and a comprehensive medical evaluation with laboratory testing was performed. Histopathologic analysis with special stains of the orbital tissues was also performed. RESULTS: Histopathologic examination of the biopsy specimens revealed the features of an inflammatory process involving the orbit, similar to a panniculitis. These include a lymphocytic reaction with a predominance of plasma cells, vasculitis with occlusion, and thickening of the vessel walls, necrosis, and hyalinization of fat. CONCLUSION: This is a unique case in which infarction and melting of the entire orbital structures occurred in the presence of systemic lupus erythematosus. The underlying disease process is a lupus-related panniculitis. The authors stress that this is a very rare entity and that other diseases should be ruled out before entertaining this diagnosis.
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2/59. Vascular anastomoses between the iris and persistent hyperplastic primary vitreous.

    Prominent radial iris blood vessels created small but definite pupillary notches in five children with persistent hyperplastic primary vitreous and persistence of the tunica vasculosa lentis. Detection of these abnormal iris vessels suggested the presence of persistent hyperplastic primary vitreous and related syndromes even when the diagnosis was obscured by the presence of an opaque lens. These vessels represented early developmental arrest and may have been associated with abnormal development of the macula and optic nerve. Surgical and amblyopia therapy, therefore, may not provide useful vision, and should be undertaken with caution.
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3/59. Documented acquired asteroid hyalosis in a case of early diagnosed diabetes mellitus.

    BACKGROUND: There have been many reports in the literature of the possible linkage of asteroid hyalosis (AH) to diabetes mellitus (DM). The controversy regarding an association between AH and DM has been one of the longest disputes in the ophthalmic literature. Here we present a case in which AH developed in a patient followed for 9 years after being diagnosed with DM. CASE REPORT: The patient had been examined on nine occasions (since his initial visit in June 1989) and asteroid hyalosis was not discovered until July 1996, when he came in with newly diagnosed diabetes mellitus. This suggests there may well be an association of asteroid hyalosis and diabetes mellitus. DISCUSSION: The patient in this case had a number of risk factors for the development of this ocular condition. He had a long history of systemic arterial hypertension, which has been reported to be linked to the formation of AH. He also had a chronic case of cystoid macular edema, which indicated a vascular compromise to the retinal vessels in the posterior pole, and this leakage may be responsible for serous constituents leaking into the vitreous, which may have caused AH. CONCLUSIONS: This may be the first time in the reported literature that AH was found to occur in a previously normal-appearing vitreous, which was documented over a 9-year period. We would suggest that asteroid hyalosis may be secondary to some form of vasculopathy in many incidences and that diabetes mellitus is one of the conditions that may be associated with the formation of AH.
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4/59. Temporal arteritis: a spectrum of ophthalmic complications.

    Seven patients of temporal arteritis with eye involvement have been presented. These cases represent a spectrum of disease from intermittent diplopia with minimal 6th nerve weakness through mild retinal ischemia with recovery to permanent bilateral blindness. Temporal arteritis should be suspected when any form of ocular ischemia is suspected by history or found on examination of an elderly person. An early diagnosis may protect the vision in both eyes if vision is normal at the time of diagnosis. If vision in one eye is decreased because of ischemia, the vision in the other eye can usually be retained if proper therapy is instituted. Furthermore, adequate therapy may even result in improvement in vision in the involved eye. patients with biopsy proven temporal arteritis should be continued on steroid therapy until the active disease is quiescent. Inactivity should be determined by carefully monitoring the ESR while steroids are being tapered. If the ESR rises, it is indicative of continued inflammation and if steroids are not continued, the eyes remain at risk as seen in Case 5. If the ESR remains elevated for a year or more despite continuation of high steroid levels, consideration should be given to repeating the temporal artery biopsy. Temporal arteritis should be considered in the differential diagnosis of any multisystem disease in older patients. Even central nervous system involvement may occur concomitantly, since the intracranial vessels are not immune from the disease process. tuberculosis, systemic syphilis and more recently the collagen vascular diseases have been dubbed the "great imitators" and "the protean diseases." We suggest that the same terminology can be applied to temporal arteritis. Temporal arteritis can affect any organ. Moreover, there is a wide spectrum of variation in the degree of involvement of any particular tissue as illustrated by these 7 cases of ocular involvement.
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5/59. Inadvertent injection of corticosteroid into the choroidal vasculature.

    A 21-year-old woman with pars planitis was treated with a subtenon's depot corticosteroid injection that was complicated by the appearance of the corticosteroid suspension within the choroidal vasculature. White, flocculent material was observed in the anterior chamber and 24 hours later this settled on the iris surface. Similar material was located in the major choroidal vessels. Two months after the incident, the choroid was normal appearing but the pars planitis was unchanged.
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6/59. Opacification of a hydrophilic acrylic intraocular lens with exacerbation of Behcet's uveitis.

    Behcet's disease is 1 of the most common causes of uveitis in the Eastern world. Its common ocular complications are uveitis, cataract, and obliteration of retinal vessels. phacoemulsification with intraocular lens (IOL) implantation in patients with Behcet's disease is known to be a safe procedure. We managed a patient with Behcet's disease who had aggravated uveitis and opacification of a hydrophilic acrylic IOL (ACRL-C160, Ophthalmed) 4 months after cataract surgery. Recalcitrant uveitis despite maximum tolerable medication and IOL opacification with vitreous opacity necessitated an IOL exchange and trans pars plana vitrectomy. After the procedure, the eye became quiescent. However, the visual acuity was 20/200 because of the obliteration of retinal vessels.
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7/59. Clinicopathologic correlation and pathogenesis of ocular and central nervous system manifestations in Hallervorden-Spatz syndrome.

    We have correlated the clinical and histopathologic features of the eyes and central nervous system in a patient with Hallervorden-Spatz syndrome who died at age 11 years. The main ocular findings included degeneration of photoreceptors, marked thinning of the outer nuclear and outer plexiform layers, retinal gliosis, narrowing and obliteration of blood vessels with a perivascular cuffing of pigment cells, and degenerative changes in the retinal pigment epithelial cells with accumulation of melanolipofuscin. The positive findings in the brain included a symmetrical, partially destructive lesion of the globus pallidus, especially in its internal fibers and neurons; in addition, we noted gliosis, widely disseminated axonal spheroidal bodies, which were most numerous in the globus pallidus and pars reticulata, as well as deposits of iron. Our histopathologic findings implicate three possible mechanisms, namely, lipid peroxidation, a deficiency of fatty acid membrane components, and increased cGMP which, either singly or in combination, are responsible for a pathogenesis that is common to the eye and brain in Hallervorden-Spatz syndrome.
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8/59. Epidermal nevus syndrome: a neurologic variant with hemimegalencephaly, gyral malformation, mental retardation, seizures, and facial hemihypertrophy.

    The epidermal nevus syndrome (ENS) is a sporadic neurocutaneous disorder that consists of epidermal nevi and congenital anomalies involving the brain and other systems. From among over 60 patients with ENS presenting with neurologic manifestations, we identified 17 who had hemimegalencephaly based on pathologic or radiologic studies. Associated brain and neurologic abnormalities included gyral malformations in 12 of 12, mental retardation in 13 of 14, seizures in 16 of 17 (including 9 with infantile spasms), and contralateral hemiparesis in 7 of 12. All had ipsilateral epidermal nevi of the head, and several had ipsilateral facial hemihypertrophy. We concluded that these abnormalities comprise a recognizable neurologic variant of ENS that we believe represents the full expression of primary brain involvement. Several patients also had evidence of acquired brain lesions such as infarcts, atrophy, porencephaly, and calcifications, which are best explained by prior ischemia or hemorrhage. Given repeated observations of blood vessel anomalies in ENS patients, we hypothesize that underlying vascular dysplasia predisposes to these acquired lesions. The same cause may be invoked to explain the wide variety of neurologic symptoms reported in ENS patients without hemimegalencephaly. While the cause of ENS remains unknown, several observations suggest a somatic mutation.
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9/59. The dural shunt syndrome. I. Management of glaucoma.

    The authors present four cases of the dural shunt syndrome in which shallowing of the anterior chamber or rubeosis developed. All patients were female, ranging in age from 66 to 79 years, exhibiting elevated intraocular pressure (IOP), decreased extraocular movements, injected tortuous episcleral vessels, and proptosis. The authors managed these four cases with laser iridotomy, gonioplasty, panretinal photocoagulation, or medical treatment. It is important to recognize associated findings in patients with shallow anterior chambers and elevated IOPs so that a diagnosis of a dural shunt is considered and appropriately treated. Theories on the mechanisms of increased IOP in the dural shunt syndrome and the management of various types of glaucoma in four different cases are reviewed.
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10/59. Clinicopathologic findings in anterior hyaloidal fibrovascular proliferation after diabetic vitrectomy.

    Two young patients (27 and 30 years old, respectively) with long-standing diabetes mellitus underwent vitreoretinal surgery for traction retinal detachment and vitreous hemorrhage. The postoperative course in each was characterized by early recurrence of the hemorrhage into the vitreous cavity, hypotony, and severe visual loss. Histopathologic examination of both operated on eyes showed anterior extraretinal fibrovascular proliferation extending along the anterior hyaloid to the posterior lens surface, causing traction detachments of the peripheral retina and ciliary body. The vessels originated from the anterior retina. There was no evidence of excess fibrous tissue at the well-healed sclerotomy wounds.
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