1/111. In vivo confocal microscopy of a family with Schnyder crystalline corneal dystrophy.OBJECTIVE: To analyze corneal morphology in Schnyder crystalline corneal dystrophy (SCCD) in vivo. DESIGN: Observational case series. PARTICIPANTS: Five eyes of four patients of various belonging to the same family were examined. methods: The eyes were examined using in vivo confocal microscopy (CM). MAIN OUTCOME MEASURES: The corneal morphology including keratocytes and stromal extracellular matrix, as well as basal epithelial/subepithelial nerves is, described. RESULTS: The right eye of a 48-year-old male patient had been treated with anterior keratectomy and the left eye with phototherapeutic keratectomy (PTK). The right eye presented with increased stromal reflectivity owing to accumulation of extracellular matrix and large subepithelial crystalline deposits. Far fewer crystals could be observed in the left eye. The haze, however, was increased, either because of the dystrophy or the excimer laser treatment. The anterior keratocytes appeared irregular, and the subepithelial nerves were undetectable in both eyes. His 78-year-old mother showed more advanced changes with dense crystals, highly fibrotic stroma, and severely damaged corneal innervation. The partly irregular anterior keratocytes of the 9- and 7-year-old children contained intracellular deposits, although the corneas were clinically clear with only subtle subepithelial crystalline formation. Accumulation of similar reflective material was also observed in association with the prominent subepithelial nerves. CONCLUSIONS: In the early stages of SCCD, highly reflective deposits accumulate intracellularly and around anterior keratocytes and along subepithelial nerves. With time, the normal corneal architecture becomes disturbed by large extracellular crystalline deposits and accumulation of highly reflective extracellular matrix resulting in central opacity and disruption of the subepithelial nerve plexus. Furthermore, neural regeneration after keratectomy appears delayed in SCCD.- - - - - - - - - - ranking = 1keywords = eye (Clic here for more details about this article) |
2/111. Phototherapeutic keratectomy for macular corneal dystrophy.PURPOSE: To report a case of early intervention with phototherapeutic keratectomy for treatment of macular corneal dystrophy. methods: We report a 21-year-old Saudi male with early macular corneal dystrophy, recurrent erosions, and decreased visual acuity, who underwent phototherapeutic keratectomy in the right eye and penetrating keratoplasty in the left eye with more than 2 years of follow-up. RESULTS: Following phototherapeutic keratectomy, uncorrected visual acuity in the right eye improved from 20/80 to 20/30. Following penetrating keratoplasty in the left eye, uncorrected visual acuity deteriorated from 20/80 to 20/120 due to irregular astigmatism; the eye was not amenable to improvement with spectacle correction, and the patient declined contact lens therapy. In the right eye, there has been no anterior recurrence, although some mid- to deep stromal haze, which is not visually significant, has developed. CONCLUSIONS: Early intervention for symptomatic, anterior macular corneal dystrophy with phototherapeutic keratectomy is relatively safe and preferable to observation or penetrating keratoplasty.- - - - - - - - - - ranking = 0.85714285714286keywords = eye (Clic here for more details about this article) |
3/111. Confocal microscopy in lattice corneal dystrophy.BACKGROUND: The purpose of the study was to assess the appearance of lattice corneal dystrophy by means of white-light confocal microscopy. methods: Two consecutive patients with lattice corneal dystrophy were prospectively examined. In vivo white-light tandem-scanning confocal microscopy was performed in the right eye of the first patient. Her left eye had undergone penetrating keratoplasty 4 years earlier. Histologic findings of the corneal button were compared with confocal microscopic findings of the right eye. The other patient was monocular and confocal microscopy was performed only in the non-seeing eye. RESULTS: In both patients, linear and branching structures with changing reflectivity and poorly demarcated margins were visualized in the stroma. The linear structures measured approximately 40-80 microm in width. CONCLUSION: Lattice corneal dystrophy presents characteristic linear images on confocal microscopy and should not be misdiagnosed as fungal hyphae in cases of corneal infection.- - - - - - - - - - ranking = 0.57142857142857keywords = eye (Clic here for more details about this article) |
4/111. Acute hydrops in the corneal ectasias: associated factors and outcomes.PURPOSE: To identify factors associated with the development of hydrops and affecting its clinical outcome. methods: Chart review of all patients with acute hydrops seen by a referral cornea service during a 2.5-year period between June 1996 and December 1998. RESULTS: Twenty-one patients (22 eyes) with acute hydrops were seen. Nineteen patients had keratoconus, 2 had pellucid marginal degeneration, and 1 had keratoglobus. Twenty-one of 22 (95%) eyes had seasonal allergies and 20 of 22 (91%) eyes had allergy-associated eye-rubbing behavior. Six of 22 (27%) had a diagnosis of Down's syndrome. Six patients were able to identify a traumatic inciting event: vigorous eye rubbing in 4 and traumatic contact lens insertion in 2. The affected area ranged from 7% to 100% of the corneal surface area and was related to disease duration and final visual acuity. Proximity of the area of edema to the corneal limbus ranged from 0 to 2.3 mm and was also related to prognosis. Three serious complications were observed: a leak, an infectious keratitis, and an infectious keratitis and coincidental neovascular glaucoma. Various medical therapies did not differ significantly in their effect on outcome, and ultimately 4 (18%) of 22 patients underwent penetrating keratoplasty. Best-corrected visual acuity was equal to or better than prehydrops visual acuity in 5 of the 6 patients in whom prehydrops visual acuity was known, without corneal transplantation. CONCLUSIONS: Allergy and eye-rubbing appear to be important risk factors in the development of hydrops. Visual results are acceptable in some patients without surgery. Close observation allows for the early detection and treatment of complications such as perforation and infection.- - - - - - - - - - ranking = 0.85714285714286keywords = eye (Clic here for more details about this article) |
5/111. Late onset lattice corneal dystrophy with systemic familial amyloidosis, amyloidosis V, in an English family.AIMS: To establish a clinical and molecular diagnosis in a family with late onset lattice corneal dystrophy. methods: Linkage analysis, single strand conformation polymorphism (SSCP) analysis, and direct sequencing of genomic dna were performed. A review of the patients' clinical symptoms and signs was undertaken. RESULTS: Linkage to chromosome 9q34 was established and a mutation in the gelsolin gene was found in affected individuals. Numerous symptoms experienced by the patients were attributable to this mutation. CONCLUSION: A diagnosis of amyloidosis type V (familial amyloidosis, Finnish type, FAF/Meretoja syndrome/gelsolin related amyloidosis) was made. This is the first case of amyloidosis type V described in the UK. This emphasises the importance of recognition of the extraocular manifestations of eye disease both in the diagnosis and management of the patient. In addition, these findings can help molecular geneticists in their search for disease-causing mutations.- - - - - - - - - - ranking = 0.14285714285714keywords = eye (Clic here for more details about this article) |
6/111. Senile pseudoexfoliation in aphakic eyes.In three eyes which underwent cataract extraction no evidence of senile pseudoexfoliation was found either before, or immediately after, surgery in spite of repeated biomicroscopical examination under full mydriasis. Years later pseudoexfoliative material was found on the anterior hyaloid and on the pupillary border. In another case, wherein the lens was traumatically dislocated and had lain in the inferior vitreous chamber for 53 years, pseudoexfoliative material was present on the anterior hyaloid, but not in the vitreous. Collating these clinical observations with data from electron and light microscopy studies, the authors refute the assumption that lens epithelium is the source of pseudoexfoliative material.- - - - - - - - - - ranking = 0.71428571428571keywords = eye (Clic here for more details about this article) |
7/111. Phototherapeutic keratectomy for Schnyder's crystalline corneal dystrophy.PURPOSE: To describe the treatment of Schnyder's crystalline dystrophy (SCD) with excimer laser phototherapeutic keratectomy (PTK). methods: Chart review of three patients (four eyes) with SCD treated with PTK between March 1992 and December 1998. Pre- and posttreatment visual acuity, subjective glare, photophobia, manifest refraction, ultrasound pachymetry, hyperopic shift, and biomicroscopic findings were studied. Brightness acuity testing (BAT) was evaluated in two eyes. RESULTS: Four eyes were treated during the study period. The average best corrected visual acuity (BCVA) improved from 20/175 to 20/40 under bright conditions. All patients reported subjective improvement in glare and photophobia. No visually significant recurrence was observed during the follow-up period which ranged from seven months to three years. The mean spherical equivalent refractive shift was 3.28 diopter (D). One patient developed irregular astigmatism from an eccentric ablation. No vision threatening complications were observed. CONCLUSION: PTK can be effective at improving visual symptoms in patients with SCD. It can be a useful therapeutic alternative to lamellar or penetrating keratoplasty in these patients.- - - - - - - - - - ranking = 0.42857142857143keywords = eye (Clic here for more details about this article) |
8/111. Corneal amyloidosis associated with congenital hereditary endothelial dystrophy.PURPOSE: To report the unusual occurrence of corneal amyloidosis indistinguishable from primary gelatinous drop-like dystrophy in three members of a family with congenital hereditary endothelial dystrophy (CHED). METHOD: Case study of three patients. RESULTS: Three patients, a 5-year-old girl, her 21-year-old maternal aunt, and a 16-year-old paternal uncle, presented with bilateral corneal edema and opacification secondary to CHED. All three underwent penetrating keratoplasty, cases 1 and 3 in one eye and case 2 in both eyes. Histopathology confirmed the diagnosis of CHED in all three patients but also revealed multiple subepithelial nodular deposits of amyloid, consistent with the diagnosis of primary gelatinous drop-like dystrophy, in all four specimens. Three other members of the same family underwent penetrating keratoplasty with histologic confirmation of CHED with no deposits of amyloid. CONCLUSION: To our knowledge, this is the first report of subepithelial corneal amyloid deposits in association with CHED. This may represent the concurrence of two primary dystrophies, although secondary amyloidosis cannot be ruled out. Early manifestation and absence of amyloid in three other family members with CHED lends more weight to a primary etiology.- - - - - - - - - - ranking = 0.28571428571429keywords = eye (Clic here for more details about this article) |
9/111. Band-shaped and whorled microcystic dystrophy of the corneal epithelium.OBJECTIVE: To report the clinical, histopathologic, and electron microscopic features of band-shaped and whorled microcystic corneal epithelial dystrophy. DESIGN: Two interventional case reports. PARTICIPANTS: Two patients, two eyes. INTERVENTION: The involved area of corneal epithelium was scraped from each cornea. RESULTS: Histopathologic examination showed microscopic vacuoles in the epithelial cytoplasm in both cases. Electron microscopic examination revealed mainly empty cytoplasmic vacuoles with scant nonspecific osmophilic material. The process recurred clinically in one patient. Changes in corneal topography are documented in one patient. CONCLUSION: Clinical findings and pathologic studies seem nearly identical to those in the original report. No pattern of systemic disorder or medication use was found. The cause of this condition remains unknown.- - - - - - - - - - ranking = 0.14285714285714keywords = eye (Clic here for more details about this article) |
10/111. Sloughing of corneal epithelium and wound healing complications associated with laser in situ keratomileusis in patients with epithelial basement membrane dystrophy.PURPOSE: To report sloughing of corneal epithelium during laser in situ keratomileusis and subsequent wound healing complications in patients with epithelial basement membrane dystrophy. methods: In a retrospective study, the surgical procedures, postoperative course, and visual acuities of 16 eyes of nine patients with epithelial basement membrane dystrophy who underwent laser in situ keratomileusis complicated with epithelial sloughing at three centers were reviewed. The mean follow-up period was 23 weeks (range, 4 to 52 weeks). RESULTS: In 13 (81%) of 16 eyes with epithelial basement membrane dystrophy, epithelial sloughing occurred during laser in situ keratomileusis. In eight of the 13 eyes, epithelial growth beneath the flap was observed. The flap was lifted and the interface epithelium scraped in six eyes. Flap melt or keratolysis occurred in four eyes. At the last follow-up visit, 13 of 16 eyes had an uncorrected visual acuity of 20/30 or better, and all eyes had a best-corrected visual acuity of 20/30 or better. CONCLUSIONS: patients with epithelial basement membrane dystrophy have poorly adherent corneal epithelium and are predisposed to epithelial sloughing during the microkeratome pass of laser in situ keratomileusis. This may lead to flap distortion, interface epithelial growth, flap keratolysis, and corneal scarring. It is not recommended that laser in situ keratomileusis be performed in patients with classic, symptomatic epithelial basement membrane dystrophy. In patients who present with mild and asymptomatic epithelial basement membrane dystrophy, laser in situ keratomileusis should be performed with caution, or photorefractive keratectomy may be the preferred refractive procedure.- - - - - - - - - - ranking = 1keywords = eye (Clic here for more details about this article) |
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