1/71. Anterior segment ischemia and sector iris atrophy: after strabismus surgery in a patient with chronic lymphocytic leukemia.A 69-year-old woman with chronic lymphocytic leukemia developed segmental iris atrophy and iridocyclitis after routine surgery for exotropia. Both the clinical picture and fluorescein angiogram indicated anterior segment ischemia. It is postulated that this was related to hyperviscosity of the blood caused by a high white blood cell count (114,000/cu mm). The possibility of anterior segment ischemia should be kept in mind when contemplating strabismus or retinal detachment surgery in the presence of hematologic disorders likely to increase blood viscosity. In these cases a minimal amount of surgery should be done with proper supportive therapy. strabismus surgery should be done in stages allowing for hemodynamic compensation between procedures.- - - - - - - - - - ranking = 1keywords = white, blood cell (Clic here for more details about this article) |
2/71. A case in the spectrum of the oculo-encephalo-hepato-renal syndrome.An 18-year-old male is presented with unprecedented central nervous system findings (cerebral dysplasia and sacral meningocele) possibly in the spectrum of the oculo-encephalo-hepato-renal syndrome. He had severe mental retardation, triplegia, epilepsy, retinitis pigmentosa, and chronic renal failure. magnetic resonance imaging demonstrated cerebral dysplasia (left dominant abnormal gyri, hypoplastic white matter, basal ganglia, and thalamus, and absence of the septum pellucidum) and the hypoplastic cerebellum and brainstem. A sacral meningocele was observed first at 16 years of age. His renal function gradually worsened after 11 years of age. His liver function was normal. The previously reported 72 cases with the oculo-encephalo-hepato-renal syndrome are reviewed.- - - - - - - - - - ranking = 0.7245561490023keywords = white (Clic here for more details about this article) |
3/71. Orbital infarction and melting in a patient with systemic lupus erythematosus.OBJECTIVE: To present a patient with systemic lupus erythematosus who developed infarction and melting of the orbit secondary to her systemic disease. DESIGN: A case report. PARTICIPANT: A 61-year-old white woman with a 5-year history of systemic lupus erythematosus. methods: The patient presented with left orbital pain, limitation of extraocular movements, and a fistula from the ethmoid sinus to the upper eyelid. A detailed examination with computerized tomography, ultrasound, and a comprehensive medical evaluation with laboratory testing was performed. Histopathologic analysis with special stains of the orbital tissues was also performed. RESULTS: Histopathologic examination of the biopsy specimens revealed the features of an inflammatory process involving the orbit, similar to a panniculitis. These include a lymphocytic reaction with a predominance of plasma cells, vasculitis with occlusion, and thickening of the vessel walls, necrosis, and hyalinization of fat. CONCLUSION: This is a unique case in which infarction and melting of the entire orbital structures occurred in the presence of systemic lupus erythematosus. The underlying disease process is a lupus-related panniculitis. The authors stress that this is a very rare entity and that other diseases should be ruled out before entertaining this diagnosis.- - - - - - - - - - ranking = 0.7245561490023keywords = white (Clic here for more details about this article) |
4/71. A case of juvenile myelomonocytic leukemia with ocular infiltration.We present a case of juvenile myelomonocytic leukemia (JMML) with ocular infiltration. A 1-month-old boy presented with myeloid precursors in peripheral blood and a white blood cell count >10x10(9)/l. His peripheral blood monocyte count was >1x10(9)/l, bone marrow blasts were <20%, and no Ph chromosome was identified. The boy also presented with hepatosplenomegaly, pallor, fever, and skin rash. We diagnosed this case as JMML, although hemoglobin F was within the normal range and no spontaneous colony growth was observed from peripheral blood mononuclear cells. Neither Epstein-Barr (EB) virus nor cytomegalovirus was detected by PCR in bone marrow aspirate or peripheral blood. The patient had several lesions into which JMML cells might have infiltrated, including skin, liver, spleen, oral cavity, right lung, sigmoid colon, and both eyes. To our knowledge, this is the first reported case of JMML with ocular involvement. Since infiltration of JMML cells into both eyes causes blindness, further consideration of the timing of bone marrow transplantation (BMT) in JMML is necessary.- - - - - - - - - - ranking = 1keywords = white, blood cell (Clic here for more details about this article) |
5/71. Chronic infantile neurological cutaneous and articular/neonatal onset multisystem inflammatory disease syndrome: ocular manifestations in a recently recognized chronic inflammatory disease of childhood.OBJECTIVE: To report on the ocular manifestations of the Chronic Infantile Neurological Cutaneous and Articular/Neonatal Onset Multisystem Inflammatory disease (CINCA/NOMID) syndrome, a rare, recently identified, pediatric multisystem inflammatory disease with chronic cutaneous, neurological, and articular manifestations. DESIGN: Descriptive case-report study. SETTING: International collaborative study based on a questionnaire. RESULTS: We included 31 patients. The mean age at onset of eye manifestations was 4.5 years. Optic disc changes were the most common feature, occurring in 26 patients (83%), including optic disc edema, pseudopapilledema, and optic atrophy. Anterior segment manifestations varying from mild to severe were seen in 13 patients (42%); chronic anterior uveitis, in 17 patients (55%). Moderate to severe visual acuity loss in at least 1 eye was seen in 8 patients (26%) as a consequence of the disease. Posterior synechia, glaucoma, and white iritis were not observed in any patient. CONCLUSION: Ocular manifestations with potentially sight-threatening complications occur commonly in the CINCA/NOMID syndrome. The distinctive nature of these complications may assist the ophthalmologist in recognizing this rare disorder and distinguishing it from juvenile rheumatoid arthritis.- - - - - - - - - - ranking = 0.7245561490023keywords = white (Clic here for more details about this article) |
6/71. phenotype of a patient with pure partial trisomy 2p(p23-->pter).We present the case of a 7-month-old girl with the karyotype 46,XX, der(13) t(2;13)(p23;p11.2).ish der(13)(wcp2 ) de novo. Painting confirmed that the additional segment on 13p was of chromosome 2 origin, resulting in trisomy 2p23 -->2pter. The child had a prominent forehead with a flat hemangioma, depressed nasal bridge, protruding tongue, posteriorly angulated ears, esotropia with poor abduction of the right eye, bilateral severe myopia (-5.5 D), retinal hypopigmentation, foveal hypoplasia, and striking left optic nerve hypoplasia. She also had pectus excavatum, a protruding abdomen with diastasis recti, generalized hypotonia, delayed fine and gross motor development, grade II reflux on the left side, and grade III-IV reflux on the right side. An EEG showed epileptiform discharges. Computed tomographic scan of the brain showed decreased white matter, but magnetic resonance imaging showed normal results.- - - - - - - - - - ranking = 0.7245561490023keywords = white (Clic here for more details about this article) |
7/71. Tubercular endophthalmitis simulating retinoblastoma.PURPOSE: To report a case of tubercular endophthalmitis simulating retinoblastoma. methods: Case report. An 8-year-old female presented with a history of complete loss of vision and a white pupillary reflex in the left eye of 3 month's duration. RESULTS: retinoblastoma could not be excluded on the basis of clinical examination and relevant investigational studies. In the left eye, a computed tomography (CT) scan demonstrated a large vitreous mass with foci of calcification. Enucleation in the left eye was performed, and histopathological examination revealed a chronic granulomatous endophthalmitis and acid-fast bacilli consistent with tubercular pathology. CONCLUSION: This case illustrates that tubercular endophthalmitis with leukocoria and a vitreous mass containing focal calcification may simulate retinoblastoma.- - - - - - - - - - ranking = 0.7245561490023keywords = white (Clic here for more details about this article) |
8/71. Ocular melanocytosis and melanoma.The hypothesis that ocular melanocytosis is a precancerous condition that may lead to a choroidal melanoma should be seriously questioned for the following reasons: The incidence of malignant degeneration in a hyperpigmented eye is unknown and overreported. If the hypothesis were correct a bilateral melanoma would occasionally occur in patients with bilateral melanosis. No such case has been reported. In patients with unilateral melanosis the blue, unaffected eye may also develop a melanoma. A 67-year-old white woman with one dark and one blue eye provided the first such instance, although previous cases may not have been reported if their histologic picture was not unusual. The incidence of melanoma in our patients with unilateral melanosis was rare compared with the many melanomas developing in normal pigmented eyes (4/418). I found no statistically significant difference in the incidence of choroidal melanomas originating in the hyperpigmented or in the blue eye in patients with unilateral ocular melanocytosis.- - - - - - - - - - ranking = 0.7245561490023keywords = white (Clic here for more details about this article) |
9/71. Atypical anterior optic neuropathy caused by toxoplasmosis.PURPOSE: To report atypical anterior optic neuropathy due to toxoplasmosis. methods: Interventional case report. A 33-year-old male presented with sudden painless loss of vision and floaters in the right eye. Examination demonstrated a best-corrected visual acuity of 20/200, optic nerve head edema, retinal hemorrhages, and vitreous opacities. RESULTS: Nine days later, a granuloma at the optic nerve head was apparent, and the patient was treated with pyrimethamine, sulfadiazine, folinic acid, and prednisone. Six weeks after initiating therapy, best-corrected visual acuity had improved to 20/25. CONCLUSION: optic nerve involvement in toxoplasmosis is uncommon and, when it occurs, usually presents with a white inflammatory mass on the optic disk. The current case demonstrates the importance of including toxoplasmosis in the differential diagnosis of unilateral anterior optic neuropathy, even if a focal inflammatory mass is not apparent.- - - - - - - - - - ranking = 0.7245561490023keywords = white (Clic here for more details about this article) |
10/71. Transthyretin Ser-44 mutation in a case with vitreous amyloidosis.PURPOSE: To report a case of vitreous amyloidosis associated with a transthyretin Ser-44 mutation. methods: Interventional case report. A 44-year-old Japanese woman had a 2-month history of visual disturbance in both eyes. The vitreous and conjunctival specimens were subjected to histopathological examination. dna was isolated from peripheral blood cells of the patient. The transthyretin gene was amplified and directly sequenced. RESULTS: The vitreous and conjunctiva specimens showed typical light microscopic features of amyloidosis. Direct sequencing of the transthyretin gene revealed a single base-pair substitution, which results in an amino acid substitution at position 44, phenylalanine to serine (transthyretin Ser-44). CONCLUSION: Transthyretin Ser-44 may cause vitreous amyloidosis.- - - - - - - - - - ranking = 0.2754438509977keywords = blood cell (Clic here for more details about this article) |
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