1/379. Von Hippel's disease in association with von Recklinghausen's neurofibromatosis.Ten members of a large family who showed manifestations of either von hippel-lindau disease or von Recklinghausen's neurofibromatosis were examined. Three of 10 members were found to have retinal angiomas which had not been present on fundus examination 3 years previously. These angiomas were associated with ocular and systemic signs of neurofibromatosis. These cases show overlapping manifestations of different phakomatoses and provide support for the concept of a common aetiology for these diseases.- - - - - - - - - - ranking = 1keywords = ocular (Clic here for more details about this article) |
2/379. listeria monocytogenes endophthalmitis with a black hypopyon.A 68-year-old woman had a marked decrease in visual acuity, increased intraocular pressure, and acute iridocyclitis. She developed a pigmented hypopyon simulating an occult intraocular melanoma. Two anterior chamber paracenteses showed growth of listeria monocytogenes. The patient received systemic intravenous penicillin, topical fortified gentamicin sulfate drops, and intraocular injections of cephaloridine. On discharge from the hospital after a two-week stay, visual acuity had improved and intraocular pressure had decreased.- - - - - - - - - - ranking = 4keywords = ocular (Clic here for more details about this article) |
3/379. blindness as a consequence of a paraneoplastic syndrome in a woman with clear cell carcinoma of the ovary.BACKGROUND: paraneoplastic syndromes are rare conditions associated with cancer that result in serious disease states at unique sites. In 1982, a report of bilateral diffuse uveal melanocytic proliferation associated with nonocular cancers which resulted in blindness was reported. We present a case of a woman with recurrent ovarian cancer who developed this paraneoplastic syndrome. CASE: A 55-year-old woman had been diagnosed in 1990 with an ocular melanoma of her right eye and in 1994 with clear cell carcinoma of the ovary. With recurrence of ovarian cancer, new eye lesions were identified in both eyes. After enulcleation of her right eye, an ocular melanoma and diffuse bilateral melanocytic proliferation (BDUMP) were found. The sight in her left eye continued to deteriorate as other signs of BDUMP occurred in the eye. Within 1 month of diagnosis, the patient was blind. She subsequently succumbed to progression of ovarian cancer. CONCLUSION: Recurrent ovarian cancer is usually an intraabdominal disease that results in gastrointestinal dysfunction. This case illustrates a rare paraneoplastic syndrome associated with ovarian cancer that mimics metastatic disease to the eye, but has a different pathophysiology.- - - - - - - - - - ranking = 3keywords = ocular (Clic here for more details about this article) |
4/379. Use of the polymerase chain reaction to detect B- and T-cell gene rearrangements in vitreous specimens from patients with intraocular lymphoma.OBJECTIVE: To determine whether the polymerase chain reaction for B- and T-cell gene rearrangements could be applied to vitreous specimens to aid in the diagnosis of intraocular lymphoma. methods: Vitreous washing specimens from 4 patients were received in balanced saline solution and centrifuged, and a portion of the pellet was used to make routine cytospins. The remainder was used to make a crude extract of dna that was amplified for immunoglobulin heavy chain and T-cell receptor gamma gene rearrangements and the 14;18 translocation by polymerase chain reaction. RESULTS: One patient had 2 specimens 2 years apart. In each, there was an identical band corresponding to the minor cluster region breakpoint of the bcl-2 oncogene, indicating the presence of a 14;18 translocation. One patient showed an immunoglobulin heavy chain gene rearrangement indicating a B-cell lymphoma. Two patients showed rearrangements of the T-cell receptor gamma gene, indicating the presence of a T-cell lymphoma. CONCLUSIONS AND CLINICAL RELEVANCE: Vitreous washing specimens can be used successfully to detect B- and T-cell gene rearrangements by polymerase chain reaction. This may be useful to confirm the diagnosis of intraocular large cell lymphoma in cases suggestive of the diagnosis. Prompt handling of the specimens is necessary to prevent degradation of the dna.- - - - - - - - - - ranking = 6keywords = ocular (Clic here for more details about this article) |
5/379. Squamous cell carcinoma with necrotizing scleritis.PURPOSE: To report on a case of limbal squamous cell carcinoma (SCC) with necrotizing scleritis in a young, previously healthy, white Australian male. methods: A 31-year-old man presented with a left limbal lesion intermittently causing a red eye and foreign body sensation. He had enjoyed surfing for many years. Repeat hiv tests were negative and the lesion was biopsied. RESULTS: biopsy showed a well-differentiated SCC apparently arising in an intra-epithelial (in situ) carcinoma of the conjunctiva. The lesion was excised and a corneoscleral graft repair was performed. CONCLUSION: The present case highlights the potential for a significant increase in the prevalence of ocular surface neoplasia in healthy young people who have had excessive UV-B exposure.- - - - - - - - - - ranking = 1keywords = ocular (Clic here for more details about this article) |
6/379. Benign pleomorphic adenoma arising from the palpebral lobe of the lacrimal gland associated with elevated intraocular pressure.A 46-year-old man complained of a painless mass in the left upper eyelid. At age 51 years, the patient complained of dull pain in the left eye and diplopia. His left intraocular pressure had increased to 33 mm Hg. No proptosis was noted. The nontender mass in the eyelid was palpable. Computed tomography showed a mass anterior to the orbital rim compressing the globe. The lesion was removed en bloc by a transconjunctival approach. Histopathologic examination of the excised mass showed myxomatous stroma and highly cellular epithelial areas. Postoperatively, the left intraocular pressure normalized. To our knowledge, benign pleomorphic adenomas arising from the palpebral lobe of the lacrimal gland associated with elevated intraocular pressure, as demonstrated in our patient, may be rare.- - - - - - - - - - ranking = 7keywords = ocular (Clic here for more details about this article) |
7/379. Presence of bilateral limbal dermoids and choroidal osteomas in a family with inherited limbal dermoids.We report a case of bilateral limbal dermoids and bilateral choroidal osteomas in a 14-year-old girl with no extraocular anomalies. Histopathological examination of a limbal lesion confirmed the clinical diagnosis of dermoid. Computerized tomography and ultrasonography were compatible with a diagnosis of choroidal osteoma. Limbal dermoids were present in the patient's mother, in a brother with down syndrome, and in an aunt with no choroidal osteoma. The present pedigree is compatible with autosomal dominant inheritance of bilateral limbal dermoids. The same gene may be involved in the pathogenesis of ocular choristomas in same patients.- - - - - - - - - - ranking = 2keywords = ocular (Clic here for more details about this article) |
8/379. Intravitreal chemotherapy for the treatment of recurrent intraocular lymphoma.AIM: To develop and assess a protocol for the treatment of intraocular lymphoma by intravitreal injection of methotrexate and thiotepa. methods: A patient with intraocular non-Hodgkin's lymphoma which recurred after radiotherapy and repeated systemic chemotherapeutic regimens underwent repeated intravitreal injections of methotrexate and thiotepa. The patient was closely monitored by cytology, anterior chamber flare measurements, IL-10 and IL-6 levels. methotrexate drug clearance studies were performed on vitreous samples taken before each injection. RESULTS: Complete tumour clearance was achieved by the third week of therapy. IL-10 and IL-6 levels quickly dropped to barely detectable levels as the tumour was cleared from the eye. Flare measurements decreased from 500 to 15 photons/s over the same time. A plot of the methotrexate levels over time revealed a first order kinetic rate of elimination with an effective tumoricidal intravitreal dose persisting for 5 days after injection. CONCLUSION: Intravitreal chemotherapy for the treatment of recurrent intraocular lymphoma appears effective in prolonging local remission of ocular disease even in the presence of an aggressively growing tumour. A single intravitreal injection of methotrexate can lead to a prolonged tumoricidal concentration lasting for a longer period than that achieved by systemic administration.- - - - - - - - - - ranking = 8keywords = ocular (Clic here for more details about this article) |
9/379. T-cell and T/natural killer-cell lymphomas involving ocular and ocular adnexal tissues: a clinicopathologic, immunohistochemical, and molecular study of seven cases.OBJECTIVE: Lymphomas of the eye and its adnexa are frequently of B lineage. This study aims to characterize the clinical and histopathologic features of the rare non-B-cell non-Hodgkin lymphomas (NHL) of these locations. DESIGN: Retrospective, noncomparative case series. PARTICIPANTS: Seven cases of T- and T/NK-cell lymphomas involving the ocular and ocular adnexal tissues. methods: A morphologic, immunohistochemical, and molecular analysis (polymerase chain reaction) of each of the tumors was undertaken. The lesions were classified according to the Revised European-American Lymphoma (REAL) classification. The clinical and follow-up data were collected. RESULTS: The patients included four women and three men ranging in age from 32 to 88 years (mean, 63 years). The presenting ophthalmic symptoms varied from a small nodule on the upper eyelid and conjunctival swellings to dramatic loss of vision associated with gross protrusion of the globe. Five of the cases presented were secondary manifestations of a systemic lymphoma in ocular tissues; two cases represented primary disease. Three cases were "peripheral T-cell lymphomas (PTCL), unspecified" with positivity for CD3, CD8, and betaF1 and negativity for CD56. Two cases were CD3 , CD30 , and CD56- and were classified as "anaplastic large-cell lymphomas of T-cell type" (T-ALCL). The remaining two cases showed an immunophenotype of CD3 , CD56 , and betaF1- and proved to contain Epstein-Barr virus (EBV) by in situ hybridization, consistent with "T/natural killer (NK)-cell lymphoma of nasal type." Clonal T-cell populations were shown in all three of the PTCLs by Southern blot (n = 1) and polymerase chain reaction (n = 2) for the T-cell receptor gamma and beta genes in one case of ALCL but not in the T/NK-cell lymphomas. Five patients died within 2 years; only two patients (one primary PTCL and one cutaneous T-ALCL) were disease free at 4 and 5 years' follow-up, respectively. CONCLUSION: This study shows that a heterogeneous group of T-cell lymphomas can involve the eye and its adnexal tissue. Most T-cell neoplasms are secondary manifestations of systemic disease and carry a poor prognosis. These findings, in conjunction with published data on ocular B-NHL, also indicate that immunophenotypic differentiation between T- and B-NHL in these locations is of clinical importance.- - - - - - - - - - ranking = 12keywords = ocular (Clic here for more details about this article) |
10/379. Posterior scleral choristoma in the organoid nevus syndrome (linear nevus sebaceus of Jadassohn).PURPOSE: To highlight the association of posterior osseous and/or cartilaginous ocular choristomas with epibulbar choristomas and the nevus sebaceus of Jadassohn. DESIGN: Small case series. PARTICIPANTS: Four patients with the organoid nevus syndrome. methods: Clinical and histopathologic studies in four patients with epibulbar lesions and nevus sebaceus of Jadassohn. MAIN OUTCOME MEASURES: Ophthalmoscopic findings of peripapillary lesions. Computed tomographic and ultrasonographic characteristic of posterior scleral lesions. Ocular histopathologic findings in one globe from one of the study subjects. RESULTS: Three patients had the triad of posterior osseous/cartilaginous ocular choristomas, anterior epibulbar choristomas, and nevus sebaceus of Jadassohn and one patient had anterior epibulbar choristomas and posterior osseous/cartilaginous ocular choristomas. ultrasonography and computed tomography were valuable in detecting scleral ossification or epibulbar cartilage or both. The ophthalmoscopic findings were similar to those of a choroidal osteoma. CONCLUSIONS: The presence of posterior osseous/cartilaginous ocular choristomas in a patient with epilepsy or epibulbar lesions or both suggests the diagnosis of nevus sebaceus of Jadassohn. Osseous/cartilaginous ocular choristomas should be suspected in patients with nevus sebaceus of Jadassohn and peripapillary hypopigmented fundus lesions.- - - - - - - - - - ranking = 5keywords = ocular (Clic here for more details about this article) |
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