1/12. Sarcomatoid carcinoma and orbital apex syndrome.PURPOSE: To report a case of sarcomatoid carcinoma and orbital apex syndrome in a previously healthy adult. methods: A previously healthy 45-year-old man presented with exophthalmos of the left eye and a mass visible through his left nostril. A biopsy was performed and immunohistochemistry was used to confirm the diagnosis of the tumor. The patient was treated with chemotherapy and radiotherapy. RESULTS: After treatment, we observed a substantial reduction in the size of the mass, but side effects of treatment developed. The visual acuity of the left eye was no light perception. Eight months later, the patient presented with bone and liver metastases, and he died 4 months later. CONCLUSIONS: Sarcomatoid carcinoma is an aggressive tumor that can produce compressive symptoms with very poor visual and survival prognoses. A cranio-orbital computed tomography scan should be performed when patients present with symptoms of conjunctivitis and orbital apex syndrome.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
2/12. Difficulty differentiating Leber's from dominant optic neuropathy in a patient with remote visual loss.A 31-year-old man who inexplicably lost vision as a child was referred for evaluation of bilateral optic atrophy. Other family members had also suffered unexplained visual loss. He had asymmetric impairment of visual acuity, central scotomas, and optic disc pallor. He also had a tritan color vision defect and excavation of the temporal portion of his optic discs, two features that were consistent with autosomal dominant optic atrophy. However, examination of the mitochondrial dna of the proband and of two of his relatives revealed a mutation at nucleotide 11778, known to be associated with Leber's hereditary optic neuropathy. This case illustrates the difficulty physicians may encounter when trying to clinically differentiate Leber's from dominant optic atrophy in patients with remote visual loss, and it emphasizes the importance of obtaining a molecular assay for a mitochondrial mutation in cases of ambiguously classified hereditary optic neuropathy.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
3/12. Mucolipidosis IV.Five new cases of Mucolipidosis IV are related and the seven cases in the literature reviewed. Neurological signs of the disorder and the value of skin and conjuncitival biopsies for its diagnosis are emphasized. Arguments in favour of a recessive autosomic inheritance are brought forth. The disease does not affect exclusively Jewish people. In two cases absence of oligosacchariduria was demonstrated.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
4/12. optic nerve decompression in osteopetrosis.osteopetrosis is a rare disorder characterized by generalized increased skeletal density with abnormalities of bone modeling. The skull base is usually involved. Loss of vision with optic nerve atrophy is the most common neurological finding and is traditionally believed to be the result of optic nerve compression within the compromised optic canal. However, retinal degeneration has recently been described and is hypothesized to be the etiology of the visual loss (thus challenging the value of surgical decompression). The authors report their experience with six children suffering from osteopetrosis and severe visual loss. All six patients underwent bilateral microsurgical optic nerve decompression through a supraorbital craniotomy. Improvement in visual acuity occurred postoperatively in five patients, and none had complications. Two technical points are emphasized: 1) optic nerve decompression should be wide and include not only unroofing of the canal but also drilling along both sides of the optic nerve, and 2) the thick, irregular, and highly domed orbital roof should be smoothed down by high-speed drilling to facilitate surgical exposure with minimal retraction of the frontal lobe.- - - - - - - - - - ranking = 2keywords = size (Clic here for more details about this article) |
5/12. The meningioma of the sphenoidal plane.The present paper reports on meningioma of the sphenoidal plane. Four women and two men were investigated. All cases examined presented with impairment of visual acuity, optic atrophy, and visual field defects. The interval between onset of the disease and a noticeable space-occupying growth was 11.5 years. Frequent false diagnoses and the importance of clinical-roentgenological follow-ups at regular intervals are especially emphasized.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
6/12. optic neuritis in inflammatory bowel disease.It is not unusual for various ocular diseases to be associated with inflammatory bowel disease. Ocular involvement includes the inflammatory entities of keratitis, conjunctivitis, episcleritis, orbital pseudotumor, iritis and neuroretinitis , as well as those states in which the etiology remains undefined, i.e., scleromalacia perforans, peripheral corneal ulceration and furrow, retinal artery occlusion, and optic neuropathy. We report five cases of optic neuropathy associated with ulcerative colitis to emphasize that this may be the sole ocular manifestation of inflammatory bowel disease.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
7/12. Uveal melanoma: case report of extension through the optic nerve to the surgical margin in the orbital apex.An unsuspected uveal melanoma arising in a phthisical eye extended through the optic nerve to the surgical margin in the orbital apex without involvement of other orbital structures. Step sections showed the continuous extension through the nerve. Factors likely to predispose to optic nerve extension include epithelioid cell type, large tumour size, peripapillary involvement, and glaucoma. optic nerve extension carries the risks noted with orbital extension of intraocular tumour, but may in addition lead more directly to intracranial invasion, including a chiasmal syndrome. The role of exenteration and radiotherapy in these cases remains uncertain.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
8/12. Orbital hydatid cyst.A six-year-old Bantu child presented with a mass in the right orbital region, proptosis, and moderate pallor of the optic disk. Orbital exploration revealed an unsuspected hydatid cyst. The characteristic histopathologic features of the organism echinococcus granulosus are emphasized.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
9/12. Behcet's disease: an unusual case with bilateral obliterating retinal panarteritis and ischemic optic atrophy.Report of an unusual case of Behcet's disease with bilateral obliterating retinal panarteritis and ischemic optic atrophy. The rarity of the primary optic nerve involvement and chiefly the exceptional full acute bilateral occlusion of the retinal arteries are emphasized. This ophthalmoscopic picture is further clinical evidence for the major importance of the vascular involvement in the pathogenesis of Behcet's disease.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
10/12. Infantile cerebello-optic atrophy. Neuropathology of the progressive encephalopathy syndrome with edema, hypsarrhythmia and optic atrophy (the PEHO syndrome).Uniform neuropathological changes are described in eight cases of the progressive encephalopathy syndrome with edema, hypsarrhythmia and optic atrophy (PEHO syndrome). Two of the autopsied patients were sisters and two other cases were familial. Macroscopically, cerebral and pronounced cerebellar atrophy was seen, the essential histopathological lesions being confined to the cerebellar cortex and the optic nerve. There was a severe neuronal loss in the inner granular layer of the cerebellum. The purkinje cells were relatively preserved in number although reduced in size, deformed and slightly disaligned. Their dendrites were horizontally oriented and the proximal axons contained abundant torpedoes. The molecular layer was narrow. The optic nerves were atrophic. Serial neuroimaging studies showed that the disease process is operative during the postnatal period, although a prenatal onset cannot be excluded. An aberrant expression of immunoreactivity against the 200-kDa neurofilament polypeptide in Purkinje cell perikarya indicated disorganization of the cytoskeleton of these cells. The combination of clinical and pathological features of our patients differs from that observed in the few published cases of so-called primary degeneration of the granular layer. Infantile cerebello-optic atrophy, clinically characterized by seizures, blindness and early arrest in psychomotor development, thus seems to constitute a new autosomal recessive disorder.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
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