Cases reported "Orbital Diseases"

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1/11. Bilateral microphthalmos with colobomatous orbital cyst and de-novo balanced translocation t(3;5).

    A term Caucasian male infant, born to a healthy non-related couple, was noted at birth to have bilateral edema and bluish discoloration of the lower eyelids. On physical examination, the eye globes were not visualized and hypertelorism was noted. Radiological imaging revealed large bilateral orbital cysts, microphthalmos, and severe optic nerve hypoplasia. Histological study of the excised orbital masses showed cysts lined by primitive, immature retinal tissue which contained neuroglial elements and scattered dysplastic rosettes. Chromosome analysis revealed an apparent balanced reciprocal translocation between the long arm of chromosome 3 and 5, i.e. 46, XY, t (3; 5) (q27; q11.2).Chromosome studies in parents were normal. To our knowledge, the association of this balanced translocation and microphthalmos with cyst has not been previously described in the English literature.
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keywords = physical
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2/11. Superior orbital 'petrositis' and late ischaemic monocular blindness induced by intense UV radiation exposure.

    Many physical agents cause neuropathies. The most common are chronic pressure, vibration and temperature. In general, these lesions occur at work, as a result of accidents or through chronic exposure to the physical agent. radiation leading to peripheral neuropathy is also related to radiotherapy in cancer treatment, as an undesirable side-effect. We present here a case report of short, intense UV radiation exposure at work, leading to delayed-onset ocular neuropathy. A clear cause-effect relationship is shown, demonstrated using magnetic resonance imaging scans. We suggest that the mechanism was thermal and ischaemic.
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keywords = physical
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3/11. Awan's syndrome (primary orbital hypertelorism, narrow-angle glaucoma and lean physique) in two women.

    Awan's syndrome, characterized by a lean physique, orbital hypertelorism and angle-closure glaucoma, is a distinct clinical entity which should be more widely recognized. The patients, usually women in their sixth decade, may suffer several self-limiting and misdiagnosed episodes of angle-closure before a definite diagnosis is made. The general physical appearance and hypertelorism in two women, one aged 56 and the other 59 years, with Awan's syndrome aroused the suspicion of angle-closure glaucoma. The timely confirmation of diagnosis and proper management prevented further visual loss in both patients. Some patients with Awan's syndrome may show advanced damage to the optic nerve without ever having had an acute attack of glaucoma or any other symptoms, which makes it important that all patients with orbital hypertelorism and suspected glaucomatous optic discs should undergo gonioscopic evaluation to rule out glaucoma.
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keywords = physical
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4/11. Periorbital edema: an important physical finding in dermatomyositis.

    Periorbital edema is frequently present in dermatomyositis and can be seen in the absence of other skin lesions. Sometimes, as in the two cases reported here, it can be the dominant physical sign.
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keywords = physical
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5/11. exophthalmos and basilar impression. A contribution to differential diagnosis of endocrine orbitopathy.

    We report on a male patient with exophthalmos of unclear etiology, basilar impression, syringohydromyelia and type II arnold-chiari malformation. Two diseases involving the orbital region were to be considered in differential diagnostic terms: endocrine orbitopathy and osseous orbit dysplasia. The typical physical appearance associated with basilar impression as well as suppurative keratitis in Lagophthalmos was striking. Tetraspasticity with pareses, bulbar symptoms, proximally pronounced muscular atrophy as well as a left hemihypesthesia was shown neurologically. Although the orbit CT was normal, sonography revealed thickened ocular muscles. There was euthyroidism in diffuse goiter with negative thyroid autoantibody findings. Because of lack of definitive detection of muscular swelling, tumor, or vascular anomaly in the various images, orbital dysplasia in the context of a malformation syndrome affecting several organs is suggested as the cause of the exophthalmos.
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keywords = physical
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6/11. Periocular granuloma annulare.

    A 2-year-old boy presented with a 3-month history of nontender masses involving the right superotemporal orbit and scalp. Over the three-week period before presentation he also developed nodules on his wrist, abdomen, and buttocks. The remainder of the ocular and general physical exam was unremarkable. A biopsy of the right orbital mass demonstrated stellate areas of collagen necrosis surrounded by pallisading epithelioid histiocytes and scattered multinucleated giant cells. Special stains for connective tissue mucin were positive in the areas of necrosis. The diagnosis reached pathologically and clinically were that of pseudorheumatoid nodules and granuloma annulare syndrome respectively. These two diagnostic terms have been used independently in the ophthalmologic and dermatologic literature, despite the identical histologic and clinical behaviors seen by both medical groups. A unified nomenclature system of pseudorheumatoid nodule for the histopathology of an individual lesion and granuloma annulare for the syndrome is suggested.
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keywords = physical
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7/11. Orbital abscess following blepharoplasty.

    Orbital abscess following blepharoplasty is surprisingly rare. The diagnosis may not be obvious from the physical signs alone. ultrasonography is a helpful diagnostic tool in such problems. The abscess cavity can be visualized. A case of orbital abscess is presented.
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keywords = physical
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8/11. Acquired orbital retraction syndrome.

    Four patients with infiltrative orbital disease involving an extraocular muscle demonstrated a characteristic retraction-motility pattern. The cardinal feature was retraction of the globe on attempted gaze opposite the field of action of the involved muscle. Variable eye movement limitation was present in the field of action and opposite to the field of action of the involved muscle. When motility was restricted, the force-duction test was positive. Modest proptosis and episcleal vascular congestion over the extraocular muscle insertion were associted physical findings. Dysthyroid eye disease, inflammatory myositis, and neoplasms are among the infiltrative myopathies that may produce an acquired orbital retraction symdrome.
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keywords = physical
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9/11. Anophthalmic socket pain.

    We examined and treated four patients with anophthalmic socket pain. Conditions responsible for this problem in this series included scleritis after evisceration, amputation neuroma, pain from a skull-base meningioma, and chemical dependency with drug-seeking behavior. The pain associated with the scleritis after evisceration responded to removal of the scleral remnant. The pain associated with the amputation neuroma responded to removal of the orbital implant and its pseudocapsule in which the amputation neuroma was embedded. The pain associated with the meningioma was intractable. The pain associated with the chemical dependency remained a persistent problem. A careful history and physical examination are critical in the evaluation of anophthalmic socket pain. Computed tomography or magnetic resonance imaging may be helpful in some cases.
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ranking = 1
keywords = physical
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10/11. Ophthalmologic examination in the diagnosis of proteus syndrome.

    PURPOSE: To describe the clinical features of proteus syndrome, a rare recently recognized hamartoneoplastic malformation, with emphasis on the ocular findings. methods: Complete physical and ocular examination of two new patients with proteus syndrome. RESULTS: The two reported cases illustrate the wide clinical polymorphism of proteus syndrome and the overlap of its clinical manifestations with those of other overgrowth syndromes. Both patients had periorbital exostoses and epibulbar tumors. The ocular findings are compared with those in the literature. CONCLUSION: Considering the paucity of information in the ophthalmic literature, this article explores the role of the ophthalmologist in diagnosing this rare entity.
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keywords = physical
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