1/141. 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 = 1keywords = ocular (Clic here for more details about this article) |
2/141. Combined phacoemulsification and penetrating keratoplasty.To highlight indications, technique, and advantages of closed-chamber phacoemulsification and intraocular lens (IOL) implantation during penetrating keratoplasty for corneal opacities. case reports of 2 patients who underwent combined phacoemulsification, IOL implantation and penetrating keratoplasty. The technique described allowed controlled capsulorrhexis, cataract removal and in-the-bag IOL implantation. Complications due to increased posterior pressure during open-sky extracapsular cataract were not encountered. The surgical technique described in this report can only be used in selected patients undergoing combined corneal transplant and cataract surgery. In this group of patients, however, the technique offers many intra- and postoperative advantages.- - - - - - - - - - ranking = 1keywords = ocular (Clic here for more details about this article) |
3/141. 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 = ocular (Clic here for more details about this article) |
4/141. Chronic clinical course of two patients with severe corneal dystrophy caused by homozygous R124H mutations in the betaig-h3 gene.PURPOSE: To report the chronic clinical course of two patients with homozygous R124H mutations in the betaig-h3 gene.methods: case reports. RESULTS: Two patients with homozygous R124H mutations in the betaig-h3 gene developed severe juvenile corneal opacities that required keratoplasty. After surgery, corneal opacities recurred and limited the recovery of visual acuity in the chronic follow-up. CONCLUSION: In patients with homozygous R124H mutations in the betaig-h3 gene, recurrence of corneal opacities after keratoplasty limits the recovery of visual acuity in the chronic follow-up.- - - - - - - - - - ranking = 10.958149773031keywords = dystrophy (Clic here for more details about this article) |
5/141. corneal opacity and congenital glaucoma associated with massive heparan sulfaturia: report of one case.A four month-old male infant was noted to have had severe corneal opacity since birth. Buphthalmos, increased intraocular pressure and corneal opacity with neovascularization were noted during physical examination. There was neither dysmorphic face nor hirsutism and the liver and spleen were impalpable. In addition, hypotonia, poor head control, and absence of Moro and grasping reflexes were also noted. There was no evidence of congenital infection by TORCH study. Tests of both urine and plasma amino acids were within normal limits. However, excessive urinary excretion of heparan sulfate was detected by thin-layer chromatography. corneal transplantation was performed at 6 months old. Histopathological examination of the corneal button showed homogeneous thickening of Bowmen's membrane and intracytoplasmic pinkish substances in corneal stroma. The alcian blue stain was positive, which was consistent with mucopolysaccharidosis of cornea. The manifestation of this case may be a clinical variant of Sanfilippo's syndrome (Mucopolysaccharidosis type III).- - - - - - - - - - ranking = 1keywords = ocular (Clic here for more details about this article) |
6/141. Amniotic membrane transplantation in acute chemical and thermal injury.PURPOSE: To present a case of chemical injury and a case of thermal injury treated by amniotic membrane transplantation in acute phase. methods: case reports. An eye with sodium hydroxide injury, opaque cornea, and limbal ischemia of more than 180 degrees and an eye with hot tea injury, opaque cornea, stromal edema, and scarring were treated by amniotic membrane transplantation within the first few weeks of injury. RESULTS: In the eye with sodium hydroxide injury, 4 months after amniotic membrane transplantation, the ocular surface is stable, superficial corneal scarring with vascularization is present, and visual acuity is 20/25. In the eye with thermal injury, 6 months after amniotic membrane transplantation, the ocular surface is stable, but there is superficial scarring and vascularization, and visual acuity is 20/20. CONCLUSIONS: Amniotic membrane transplantation can be considered in chemical injury with severe limbal ischemia and in severe thermal injury in acute phase. Long-term studies are warranted to evaluate further the efficacy of amniotic membrane transplantation in these clinical situations.- - - - - - - - - - ranking = 2keywords = ocular (Clic here for more details about this article) |
7/141. Ocular findings in Fryns syndrome.PURPOSE: To demonstrate the ocular histopathologic findings in Fryns syndrome, a multiple congenital anomaly syndrome, with characteristic features including Dandy-Walker malformation, cleft palate, diaphragmatic hernia, lung hypoplasia, distal limb anomalies and polyhydramnios. The prevalence is about 0.7 per 10,000 births. Reported ocular features include microphthalmus, "cloudy cornea", irregularities of Bowman's layer, thickened posterior lens capsule and retinal dysplasia. methods: Case report. The ocular histopathologic and ultrastructural findings in a male fetus with Fryns syndrome who died immediately after his birth at 26th week of gestation are shown. RESULTS: An abnormal Descemet's membrane was found in addition to "cloudy corneae". Electron microscopy demonstrated absence of the banded collagen fibrils in Descemet's membrane, indicating corneal endothelial dysfunction. Otherwise, the eye was morphologically normal for its age; none of the other reported ocular features of Fryns syndrome were found. CONCLUSION: Corneal endothelial dysfunction might cause abnormal composition of anterior Descemet's membrane and could contribute to the "cloudy cornea" known to occur in Fryns syndrome.- - - - - - - - - - ranking = 4keywords = ocular (Clic here for more details about this article) |
8/141. Congenital corneal opacification in De Barsy syndrome.A newborn male was noted to have bilateral congenital corneal opacification. Findings from examination disclosed a variety of dysmorphic features, including cutis laxa, progeroid aspect, short stature, multiple hyperextensible subluxated joints, muscular hypotonia, and hyperreflexia. Bilateral penetrating keratoplasties were performed; histopathologic examination revealed diffuse epithelial thickening, loss of the Bowman layer, and stromal attenuation with anterior stromal scarring. Special stains showed no deposition of abnormal material in the corneas. Electron microscopy demonstrated absence of Bowman layer differentiation with a paucity of collagen fibers, as well as extensive small elastic fibers in the anterior stroma. The diagnosis of De Barsy syndrome was made, a rare progeroid syndrome associated with characteristic ocular, facial, skeletal, dermatologic, and neurologic abnormalities. De Barsy syndrome should be included in the differential diagnosis of congenital corneal opacification; its distinctive clinical features enable the clinician to easily differentiate it from other causes of congenitally cloudy corneas.- - - - - - - - - - ranking = 1keywords = ocular (Clic here for more details about this article) |
9/141. Corneal deposition after high-dose chlorpromazine hydrochloride therapy.PURPOSE: To report a case of dermal and ocular changes after prolonged use of high-dose chlorpromazine hydrochloride therapy. METHOD: This case report includes clinical history, clinical findings, and photographic images of ocular and dermal changes. RESULTS. chlorpromazine therapy in a cumulative dosage exceeding 1,100 g resulted in dramatic skin discoloration and multiple crystalline deposits in both corneas. Anterior capsular opacities were binocularly present. These changes were sufficient to cause reduction in visual acuity. CONCLUSIONS: chlorpromazine deposition at high levels can cause reduction in visual acuity and significant skin discoloration.- - - - - - - - - - ranking = 3keywords = ocular (Clic here for more details about this article) |
10/141. Pseudomonas corneal ulcer. The causative role of contaminated eye cosmetics.The clinical significance of contaminated ocular cosmetics is illustrated by the case of a 47-year-old woman in whom a Pseudomonas corneal ulcer developed immediately after she sustained minor corneal trauma with a mascara applicator. pseudomonas aeruginosa was cultured from the corneal ulcer and the mascara. In addition to the causative role in acute corneal ulcers, contaminated eye cosmetics contribute to chronic external eye infections. Retail eye cosmetics are typically free of contamination when purchased. The inoculation of the cosmetic occurs during normal use.- - - - - - - - - - ranking = 1keywords = ocular (Clic here for more details about this article) |
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