1/68. The atrophic variant of dermatofibrosarcoma protuberans in childhood: a report of six cases.dermatofibrosarcoma protuberans (DFSP) is typically diagnosed during early adult life at a tumoral stage. It occurs only rarely in children. We report six childhood cases of DFSP which presented initially with the misleading clinical appearance of atrophic plaques, and we review over 140 cases of DFSP in childhood. As compared with adult forms, DFSP in children does not show distinctive features except for a tendency for acral localization. The diagnosis is difficult because of the slow course of the lesions, which present initially as apparently benign atrophic morphoeaor keloid-like plaques. We believe that DFSP in childhood is probably under-estimated, as a significant proportion of patients diagnosed as young adults had an onset several years earlier. Better knowledge of the initial appearance is important for making an early diagnosis and for an easier surgical treatment.- - - - - - - - - - ranking = 1keywords = coma (Clic here for more details about this article) |
2/68. Extensive chorioretinal atrophy in Vogt-Koyanagi-Harada disease.PURPOSE: To report extensive chorioretinal atrophy during the long-term course of Vogt-Koyanagi-Harada (VKH) disease not treated properly in the initial phase. CASES: Four patients with VKH disease were examined more than 10 years after onset of the disease. OBSERVATIONS: They presented initially with classic features of VKH disease, except 1 patient who had developed bilateral, acute angle-closure glaucoma as the initial sign. Two patients received systemic corticosteroid therapy at the acute phase of the disease. During the follow-up of 13-34 years subsequent to onset, these patients had chronic recurrent anterior uveitis with apparently stable depigmented fundus. Eventually, they developed diffuse, extensive chorioretinal atrophy that resulted in severe visual loss. One patient had an unusual familial occurrence of the disease. CONCLUSIONS: Failure to prescribe proper corticosteroid therapy in the initial phase of VKH disease may lead to chronic recurrent uveitis. Long-standing uveitic reactions may eventually result in severe visual loss due to extensive chorioretinal degeneration.- - - - - - - - - - ranking = 0.2keywords = coma (Clic here for more details about this article) |
3/68. Osteogenic sarcoma and phthisis bulbi: a case report.PURPOSE: To describe a case of osteogenic sarcoma (osteosarcoma) that developed within a phthisical eye. METHOD: Case report. RESULTS: An 86-year-old woman with a 20-year history of phthisis bulbi developed pain and proptosis. Tumor was identified by computed tomography. An exenteration was performed, and osteogenic sarcoma was identified. CONCLUSION: Osteogenic sarcoma is the most common primary malignant tumor of bone. In the orbit it frequently is associated with prior irradiation for retinoblastoma. We describe the first case of osteogenic sarcoma that developed de novo from bone within a phthisical eye.- - - - - - - - - - ranking = 1.8keywords = coma (Clic here for more details about this article) |
4/68. zoster sine herpete with bilateral ocular involvement.PURPOSE: To report a case of zoster sine herpete with bilateral ocular involvement. METHOD: Case report. RESULTS: A 65-year-old man showed bilateral iridocyclitis with sectoral iris atrophy and elevated intraocular pressure unresponsive to steroid treatment. No cutaneous eruption was manifest on the forehead. A target region of varicella-zoster virus dna sequence was amplified from the aqueous sample from the left eye by polymerase chain reaction. Bilateral iridocyclitis resolved promptly after initiation of systemic and topical acyclovir treatment. Secondary glaucoma was well controlled by bilateral trabeculectomy. CONCLUSIONS: zoster sine herpete should be considered and polymerase chain reaction performed on an aqueous sample to detect varicella-zoster virus dna for rapid diagnosis whenever anterior uveitis accompanies the characteristic iris atrophy, even in the case of bilateral involvement.- - - - - - - - - - ranking = 0.2keywords = coma (Clic here for more details about this article) |
5/68. Delayed onset of hemidystonia and hemiballismus following head injury: a clinicopathological correlation. Case report.The authors report the case of a young man who suffered multiple injuries in a motor vehicle accident, the most significant of which arose in the brain, creating an unusual clinical syndrome. After experiencing an initial coma for several days, the patient was found to have a right-sided homonymous hemianopsia and a right hemiparesis, which was more marked at the shoulder and was accompanied by preservation of finger movement. Dystonic movements appeared 2 months later and progressed, along with increased spasticity on volition, to severe uncontrolled arm movements at 2 years postinjury. This motor disorder continued to worsen during the following 6 years prior to the patient's death. At autopsy, the left side of the brain was observed to have marked atrophy of the optic tract, a partial lesion of the posterior portion of the medial segment of the globus pallidus (GP), and a reduction in the size of the internal capsule at the level of the GP, suggesting impaired circulation to these areas at the time of injury. The isolated lesion of the internal segment of the GP was the presumed cause of the dystonia, acting through an alteration in thalamic inhibition. The atrophic subthalamic nucleus was the probable cause of the hemiballismus. The authors speculate that this and other delayed and progressive features of this case were the result of an active, but disordered, adaptive process that failed to compensate and, instead, caused even greater problems than the original injury.- - - - - - - - - - ranking = 0.2keywords = coma (Clic here for more details about this article) |
6/68. Cerebral atrophy and convulsive seizures after recovery from cerebral edema and coma in a patient with fulminant hepatitis b.We report a 48-year-old woman who developed convulsive seizures and cerebral atrophy after recovery from fulminant hepatitis b with coma and cerebral edema at the acute stage. Neurological disturbances and cerebral signs are rare sequelae of fulminant hepatic failure (FHF); only a few cases have reported in the literature. Cortical laminar necrosis secondary to cerebral edema or delayed neuronal death due to toxic substances may have caused delayed brain atrophy and epileptogenesis.- - - - - - - - - - ranking = 1keywords = coma (Clic here for more details about this article) |
7/68. Wide clinical variability in a family with a CACNA1A T666m mutation: hemiplegic migraine, coma, and progressive ataxia.We report a Japanese family carrying a T666M missense mutation of CACNA1A. Affected members demonstrated a strikingly wide clinical spectrum including migraine, hemiplegia, coma, and progressive cerebellar ataxia. Despite such variability of the clinical features, they demonstrated similar magnetic resonance imaging findings demonstrating cerebellar atrophy predominantly of the cerebellar vermis. These magnetic resonance images appeared not to correlate with clinical severity. Our findings should indicate that a T666M mutation of CACNA1A may be associated with more variable clinical features and that paroxysmal hemiplegic migraine attacks and progressive cerebellar atrophy should have distinct mechanisms of pathogenesis.- - - - - - - - - - ranking = 1keywords = coma (Clic here for more details about this article) |
8/68. The histopathology of pigmentary dispersion syndrome with glaucoma.iris tissue and trabecular meshwork, obtained at time of trabeculectomy, was studied using the light and electron microscope in a 54-year-old woman with pigmentary dispersion syndrome with glaucoma. The specific defect was a loss of the outer epithelial cells of the iris with marked thinning of the remaining outer layers so that the two-cell architecture of the iris epithelium was maintained. In addition, the radial muscle layer was increased in both number and size of muscle fiber. It would appear that the pigmentary dispersion syndrome may represent a congenital or developmental abnormality of the iris epithelium, or both, and that the glaucoma which occasionally occurs in conjunction with this syndrome is of the usual open-angle type.- - - - - - - - - - ranking = 1.2keywords = coma (Clic here for more details about this article) |
9/68. Acute haptic-induced pigmentary glaucoma with an AcrySof intraocular lens.A 49-year-old man had uneventful endocapsular phacoemulsification with in-the-bag implantation of an AcrySof SA60AT single-piece intraocular lens (IOL) (Alcon) in the right eye. Twenty-seven days postoperatively, he presented with ocular pain, intraocular pressure of 48 mm Hg, 360 degrees of hyperpigmentation of the trabecular meshwork, and iris pigment epithelial atrophy in the region of the upper temporal haptic, which had dislocated into the sulcus. The patient made an excellent recovery following IOL removal and exchange. Scanning electron microscopy of the explanted IOL demonstrated that the haptic had a rough lateral surface and anterolateral edge. We do not think this IOL should be implanted in the sulcus placement of the heptics. In this article, we report the case of a patient with an AcrySof SA60ATIOL (Alcon) who developed acute pigmentary glaucoma when the inferior haptic slipped out of the bag and came into contact with the pigmented iris and ciliary body.- - - - - - - - - - ranking = 1keywords = coma (Clic here for more details about this article) |
10/68. Atrophic dermatofibroma: a case report and review of the literature.BACKGROUND: Atrophic dermatofibroma is an uncommon variant of dermatofibroma. It lacks the classic clinical features of a dermatofibroma and is often misdiagnosed. OBJECTIVE: To describe the clinical and histologic features of atrophic dermatofibroma. methods: Case report and review of the literature. RESULTS: A 45-year-old white woman was found to have a 7 mm x 5 mm atrophic, depressed lesion in the right axilla. A clinical diagnosis of anetoderma was made and the lesion was excised with minimal margins. Histopathologic examination revealed findings consistent with dermatofibroma. However, focal CD34 staining and involvement of superficial subcutaneous tissue raised concern regarding an early dermatofibrosarcoma protuberans developing in a dermatofibroma. The patient underwent Mohs micrographic surgery (MMS) for definitive treatment. The final diagnosis is thought to be atrophic dermatofibroma. Reported cases in the English language literature are reviewed and the clinical and histopathologic findings are described. CONCLUSION: Atrophic dermatofibroma is a well-described, yet uncommon, variant of dermatofibroma. It is often clinically misdiagnosed, and histopathologic evaluation can be misleading. The clinician and pathologist should consider this diagnosis in the evaluation of atrophic, depressed lesions.- - - - - - - - - - ranking = 0.2keywords = coma (Clic here for more details about this article) |
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