1/265. Acute corneal necrosis after excimer laser keratectomy for hyperopia.OBJECTIVE: To describe a new, rare clinical complication after routine excimer laser photorefractive keratectomy to correct hyperopia. DESIGN: Case report with clinicopathologic correlation. MAIN OUTCOME MEASURES: Four weeks after treatment with excimer laser, a perforating keratoplasty was performed for persistent corneal opacities. The corneal button was examined using light and electron microscopy. Special immunohistochemical stains were used to detect apoptosis. RESULTS: The patient developed corneal opacities, endothelial precipitates, and a fibrinous exudate in the anterior chamber after the laser treatment. The changes did not respond to therapy directed against bacteria, fungi, and acanthamoeba. All examinations and special stains were negative for micro-organisms. By light microscopy, an anterior zone of corneal necrosis was present with a moderate amount of acute inflammatory cells. At the interface between necrotic and viable corneal stroma, keratocytes with typical features of apoptosis were detected by immunohistochemistry and electron microscopy. CONCLUSION: This is the first full histopathologic report of a case of acute corneal necrosis with signs of apoptosis after excimer laser therapy of the cornea. Surgeons should be aware of this rare but potentially severe complication.- - - - - - - - - - ranking = 1keywords = vi (Clic here for more details about this article) |
2/265. Double anterior chamber deep lamellar keratoplasty: case report.PURPOSE AND methods: We report a case in which, although we planned to perform a penetrating keratoplasty for corneal stromal opacity with normal corneal endothelium, the host's Descemet's membrane became inadvertently detached and the operation resulted in double anterior chamber deep lamellar keratoplasty (DLKP). RESULT: After surgery, the patient's corrected visual acuity was 20/30. CONCLUSION: Double anterior chamber DLKP is safe and valuable.- - - - - - - - - - ranking = 1keywords = vi (Clic here for more details about this article) |
3/265. The use of contact B mode ultrasound in pediatric ophthalmology.It has been shown that an ultrasound system such as the Bronson-Turner Contact B Mode can be added, fruitfully, to the armamentarium of the individual pediatric ophthalmologist. It can be used quickly and precisely with an ophthalmological training and without patient discomfort or anesthesia no matter what the age. Valuable anterior segment information can be readily gained without a waterbath. Examples are shown of children with opaque media who were found to have congenital cataracts and congenital aphakia, microphthalmos, PHPV, retinal anomalies and ectatic coloboma.- - - - - - - - - - ranking = 1keywords = vi (Clic here for more details about this article) |
4/265. Ocular changes in mucopolysaccharidosis iv A (Morquio A syndrome) and long-term results of perforating keratoplasty.BACKGROUND: The mucopolysaccharidoses (MPS) are an inhomogeneous group of disorders of errors in the carbohydrate metabolism with severe ocular involvement (corneal opacification, retinal degeneration, optic atrophy). PATIENT PRESENTATION: We report on a boy aged 12 years, with Morquio A (MPS IV A) syndrome. Ocular findings: progressive pseudoexophthalmus due to shallow orbits, increasing corneal stromal clouding, intermittent dissociated manifest nystagmus of the left eye, nyctalopia. visual acuity OD cc = 0.16, OS cc = 0.05. electrophysiology: changes suggesting a symptomatic tapetoretinal degeneration and optic atrophy. TREATMENT AND COURSE OF DISEASE: OS: perforating keratoplasty. Postoperative improvement of visual acuity to 0.25 for nearly a year, followed by progressive reopacification of the corneal graft. Both eyes: progressive signs of tapetoretinal degeneration and optic atrophy. visual acuity now reduced to OD 0.05, OS 0.1. CONCLUSIONS: Success of a keratoplasty is limited by (1) reopacification of the cornea, (2) visual impairment due to (a) retinal degeneration and (b) optic atrophy. The indication for perforating keratoplasty has to be thought about very carefully in these multimorbid patients. In our patient, beside progressive visual impairment there is a progressive deafness which dominates his social and school life. Attending school is severely complicated by the double handicap. Perforating keratoplasty enabled the boy to attend a school for physically handicapped without a special low-vision care for another year. Progressive visual loss without further treatment options now renders optical and electronic low-vision aids necessary. Although the time of improved visual acuity lasted less than a year, we think patients with a life expectancy of less than 20 years should have every possible improvement of their situation - even if it does not last permanently. We therefore propose perforating keratoplasty in spite of insufficient long-term results.- - - - - - - - - - ranking = 1081.2989941102keywords = mucopolysaccharidosis, vi (Clic here for more details about this article) |
5/265. Severe nonimmune hydrops fetalis and congenital corneal opacification secondary to human parvovirus B19 infection. A case report.BACKGROUND: In parvovirus infections in animals, congenital anomalies are seen, but the teratogenic potential in humans seems fairly low. CASE: A fetus with hydrops, ascites and pleural effusion was seen at a prenatal ultrasound examination. Fetal cordocentesis was performed, and fetal blood was positive for parvovirus antibodies. Intravascular fetal blood transfusion was given at 21 and 23 weeks of gestation. At 39 weeks labor started spontaneously, and a 2,960-g, female infant was delivered. The newborn had bilateral opacification of the cornea. CONCLUSION: In this case a combination of fetal parvovirus B19 infection and congenital corneal opacification was seen. This case also demonstrates that blood transfusions in hydropic fetuses may reverse the hydrops and prevent intrauterine death.- - - - - - - - - - ranking = 7keywords = vi (Clic here for more details about this article) |
6/265. Vanished twin and fetal alcohol syndrome in the surviving twin. A case report.BACKGROUND: The diagnosis of twin pregnancy can be made early in pregnancy by ultrasonography (US). Follow-up examination occasionally demonstrates the disappearance of one of the twins. CASE: A twin disappeared on US examination; the surviving twin exhibited signs of fetal alcohol syndrome and other congenital anomalies, accompanied by a placental form of fetus papyraceus. CONCLUSION: Early US examination is useful for diagnosing multiple gestation. However, a follow-up examination is required to alert the clinician to the disappearance of one twin. Careful examination of the placenta may document fetal remnants. In this case a small, atretic nodule on the placental surface was evidence of the vanished twin.- - - - - - - - - - ranking = 11keywords = vi (Clic here for more details about this article) |
7/265. A first British case of fish-eye disease presenting at age 75 years: a double heterozygote for defined and new mutations affecting LCAT structure and expression.Fish-eye disease is a familial syndrome with corneal opacification, major high density lipoprotein (HDL) deficiency in plasma, significant cholesterol esterification in plasma on non-HDL lipoproteins, generally without premature coronary disease. This first British male case from unrelated British parents had infarcts when aged 49 and 73 years but was asymptomatic at age 81 years, with plasma cholesterol 4.3-7.1 mmol/litre, triglycerides 1.8-2.2 mmol/litre, HDL cholesterol < 0.1 mmol/litre, apolipoprotein a-i < 0.16 g/litre, lipoprotein(a) 0.61 g/litre. cholesterol esterification was impaired using HDL-3 and A-I proteoliposomes but not using VLDL/IDL/LDL. The findings are those of LCAT deficiency with the classic fish-eye disease defect. Most of the 22 reported cases were homozygous or heterozygous for a Thr-Ile mutation at codon 123 of the lecithin:cholesterol acyltransferase (LCAT) gene. This patient was a double heterozygote for this mutation and a second new incompletely defined mutation affecting LCAT expression as defined by reduced mass and activity in plasma.- - - - - - - - - - ranking = 1keywords = vi (Clic here for more details about this article) |
8/265. Classical LCAT deficiency resulting from a novel homozygous dinucleotide deletion in exon 4 of the human lecithin: cholesterol acyltransferase gene causing a frameshift and stop codon at residue 144.Lecithin: cholesterolacyltransferase (LCAT) transacylates the fatty acid at the sn-2 position of lecithin to the 3beta-OH group of cholesterol forming lysolecithin and the majority of cholesteryl ester found in plasma. LCAT participates in the reverse cholesterol transport pathway in man where it esterifies tissue-derived cholesterol following efflux from peripheral cells into HDL. Only 38 unique mutations in the human LCAT gene have been reported worldwide. Our French female proband presented with corneal opacity and no detectable plasma LCAT activity using either endogenous or exogenous assays. Her total plasma cholesterol and HDL cholesterol were low (2.34 mmol/l and 0.184 mmol/l, respectively) with a very high cholesterol/cholesteryl ester molar ratio (10.9:1). plasma triglycerides were 0.470 mmol/l with low apo B (40.5 mg/dl), apo A-I (14.7 mg/dl), apo A-II (6.8 mg/dl) and apo E (2.1 mg/dl) levels. plasma lipoprotein analysis by ultracentrifugation showed very low HDL concentrations and a characteristic shift of the lipoprotein profile towards larger, less dense particles. No proteinuria, renal dysfunction or signs of atherosclerosis were noted at age 45. sequence analysis of her LCAT gene showed a novel homozygous TG-deletion at residues 138-139 that resulted in a frameshift causing the generation of a stop codon and premature termination of the LCAT protein at amino acid residue 144. Western blotting of the patient's plasma using a polyclonal IgY primary antibody against human LCAT failed to demonstrate the presence of a truncated LCAT protein. A 53 bp mismatched PCR primer was designed to generate an Fsp 1 restriction site in the wild type sequence of exon 4 where the mutation occurred. The 155 bp PCR product from the wild type allele produced a 103 bp and 52 bp fragment with Fsp 1 and no cleavage products with the mutant allele thus permitting rapid screening for this novel mutation.- - - - - - - - - - ranking = 1keywords = vi (Clic here for more details about this article) |
9/265. Corneal changes in spondyloepiphyseal dysplasia tarda.BACKGROUND: A new type of corneal opacity with prominent corneal nerve fibers as an ocular complication of spondyloepiphyseal dysplasia tarda (SEDT). CASE: A 58-year-old woman, diagnosed with SEDT at 5 years of age, underwent a complete ophthalmological examination. OBSERVATIONS: The patient had no complaints and no history of eye disease. No relatives were reported to have suffered from SEDT. Slit-lamp examination disclosed a diffuse opacity in the central cornea in both eyes, which was localized in the middle to deep stroma. Dot opacities in the central and paracentral cornea were located in the middle of the stroma in both eyes. Optically clear regions were observed in the peripheral cornea of both eyes. More interestingly, corneal nerve fibers were visible passing from the limbus to the central cornea in both eyes. RESULTS: The etiology of the corneal opacities of this patient with SEDT is unknown. However, collagen and proteoglycan abnormalities in the skin of patients with SEDT have been reported. Therefore, such abnormalities may also be present in the cornea and these alterations may lead to corneal complications.- - - - - - - - - - ranking = 1keywords = vi (Clic here for more details about this article) |
10/265. Exogenous fungal endophthalmitis caused by paecilomyces.A 17-year-old white boy developed a fulminating corneal infection and endophthalmitis in his left eye after trauma. He was treated with antibiotics and corticosteroids for one week prior to diagnosis. A saprophytic fungus, paecilomyces viridis, was cultured from corneal scrapings and was demonstrated in the vitreous cavity by histopathologic examination.- - - - - - - - - - ranking = 3keywords = vi (Clic here for more details about this article) |
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