Cases reported "Facial Hemiatrophy"

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1/9. Misdiagnosis of silent sinus syndrome.

    PURPOSE: To review cases of spontaneous enophthalmos erroneously diagnosed as silent sinus syndrome to identify other inflammatory disorders with a similar clinical presentation. methods: Retrospective observational case series. Chart reviews were performed covering encounters over a 5-year period, searching for patients referred to two physicians with an incorrect diagnosis of silent sinus syndrome. Only cases of acquired enophthalmos of reported nontraumatic or nonneoplastic cause were included. Particular attention was directed toward noting clinical features of upper eyelid position and periocular atrophy. RESULTS: Nineteen cases of erroneous referral for silent sinus syndrome were identified. Fourteen of these cases were due to tumor, trauma, congenital facial asymmetry, or diffuse facial lipodystrophy. Among the remaining cases, four were diagnosed as Parry-Romberg syndrome and one as linear scleroderma. CONCLUSIONS: Parry-Romberg syndrome and linear scleroderma must be distinguished from silent sinus syndrome as causes of inflammatory-mediated, spontaneous enophthalmos.
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keywords = enophthalmos
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2/9. Parry-Romberg syndrome.

    The authors report an unusual case of Parry-Romberg syndrome (PRS). magnetic resonance imaging and computed tomography scan of the craniofacial region and surgical correction of enophthalmos were performed. Results after the operative intervention included persistent palpebral edema and ecchymosis and transient choroid vasculitis of the right eye, highlighted with echography and fluorescein angiography. The fundus oculi examination showed retinal choroid folds. Immunological test results were weakly positive. The authors conclude enophthalmos, associated with right side hemi atrophy, and the transient choroids vasculitis support the diagnosis of PRS. Furthermore, it is suggested the case had an autoimmune etiology, rather than a hemi facial asymmetry caused by a facial trauma that occurred in puberty.
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keywords = enophthalmos
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3/9. Progressive hemifacial atrophy (Parry-Romberg syndrome): report of two cases.

    We describe 2 cases of progressive hemifacial atrophy (Parry-Romberg syndrome) with typical facial and ocular involvement. Ocular manifestations in these 2 cases include enophthalmos, due to fat atrophy, decreased corneal sensitivity, episcleritis, and band keratopathy. The slowly progressive changes were documented in one case over a 40 year period. Both cases were unilateral with limitation of the lesions at the midline.
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keywords = enophthalmos
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4/9. Progressive hemifacial atrophy. A natural history study.

    PURPOSE: To describe two very different natural history courses in 2 patients with hemifacial atrophy. Progressive hemifacial atrophy (Parry-Romberg syndrome, Romberg syndrome, PHA) is characterized by slowly progressive atrophy, frequently involving only one side of the face, primarily affecting the subcutaneous tissue and fat. The onset usually occurs during the first 2 decades of life. The cause and pathophysiology are unknown. Ophthalmic involvement is common, with progressive enophthalmos a frequent finding. Pupillary disturbances, heterochromia, uveitis, pigmentary disturbances of the ocular fundus, and restrictive strabismus have also been reported. Neurologic findings may be present, but the natural history and progression of ocular findings are often not described in the literature. methods: We studied the records and present findings of 2 patients with progressive hemifacial atrophy who were observed in our institution over a 10-year period. RESULTS: Both patients showed progression of ophthalmic findings, primarily on the affected side. One patient has had chronic uveitis with secondary cataract and glaucoma, in addition to retinal pigmentary changes. She also had a third-nerve paresis of the contralateral eye and mild seizure activity. The other patient had mild uveitis, some progression of unilateral retinal pigmentary changes, and a significant increase in hyperopia in the affected eye, in addition to hypotony at age 19 without a clear cause, but with secondary retinal and refractive changes. CONCLUSION: Ocular manifestations of progressive hemifacial atrophy are varied, but can progress from mild visual impairment to blindness.
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keywords = enophthalmos
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5/9. Hemifacial atrophy: an unusual cause of upper eyelid retraction.

    Progressive hemifacial atrophy or Parry Romberg syndrome may present with variable ocular features, the commonest being enophthalmos. We report a mild case in a 62-year-old man who presented with upper eyelid retraction, nocturnal lagophthalmos, and symptoms of corneal exposure. Recession of the levator muscle corrected the lid retraction and abolished the symptoms of corneal exposure.
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keywords = enophthalmos
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6/9. Progressive hemifacial atrophy: ten-year observation of a case.

    The authors describe the case of a 14-year-old girl who experienced progressive hemifacial atrophy at the age of three-and-a-half years. The patient's early age at appearance of the condition resulted not only in atrophy of skin, subcutaneous tissue, and muscles, but also in very severe deformation of the right side of the face due to disorders in growth of cartilaginous and osseous tissue. Changes in her brain case and craniofacial skeleton included enophthalmos, underdevelopment of the eyelids, nose, hard plate, and dental process of the jaw including the premolar teeth, and a lack of the second molar. These changes caused an irregular line of bite. The differential diagnosis excluded hemifacial microsomy and sclerodermal hemifacial atrophy.
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keywords = enophthalmos
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7/9. Correction of enophthalmos in progressive hemifacial atrophy: a case report.

    This case report describes a technique used for correction of enophthalmos secondary to progressive hemifacial atrophy (Parry-Romberg syndrome). The only previous described technique utilized an orbital floor implant, but this method was apparently only partially successful in correcting the conditions, i.e., it did not correct both the enophthalmos and the pseudoptosis that occurs secondary to intraorbital fatty atrophy. In the present technique, the periorbita was cut at two equators, the globe and anterior periorbita advanced forward, and the resulting empty spaces filled with thin slices of radiated cartilage. Both the enophthalmos and the pseudoptosis were corrected in a single operation. There were no long-term complications and the correction has been maintained 3 years postoperatively.
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keywords = enophthalmos
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8/9. coloboma in progressive hemifacial atrophy.

    Over a seven-year period, a 22-year-old woman developed clinical linear scleroderma with associated ipsilateral coloboma of the upper eyelid, blepharoptosis, enophthalmos, and impairment of extraocular motility. Reconstructive surgery included correction of the enophthalmos by Silastic block implant, closure of the coloboma, and resection of the levator palpebrae superioris muscle for correction of the blepharoptosis.
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keywords = enophthalmos
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9/9. Parry-Romberg syndrome associated with intracranial vascular malformations.

    We describe a 23-year-old woman with iridocyclitis, enophthalmos, facial hemiatrophy, and transient numbness of her contralateral upper and lower extremities. The patient was found to have white matter densities in the right hemisphere in magnetic resonance T2-weighted images and vascular malformations involving right vertebral, right carotid, and right anterior cerebral arteries. Histopathologic evaluation of a biopsy specimen of anterior orbital fat and lacrimal gland revealed fibrosis and chronic inflammation. These findings were consistent with the diagnosis of progressive facial hemiatrophy (Parry-Romberg syndrome) in association with iridocyclitis and intracranial vascular malformations.
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keywords = enophthalmos
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