[Corneal edema in pseudophakia. A case report]. / Oedème cornéen du pseudophake. A propos d'une observation anatomo-clinique.

Pseudophakic bullous keratopathy is one of the most common complications of intraocular lenses, which explains the high rate of this condition in series of patients experiencing corneal grafts. It is caused by irreversible damage to the endothelial layer of the implanted eye involving the artificial lens, and occurs either during cataract extraction or after the operation. Intraoperative endothelial protection and the choice of an appropriate lens may contribute to decreasing this secondary disease, which can have severe consequences for many patients whose corneal graft may not restore their visual potential as well as expected. We report a clinicopathological case of a 59-year-old male, previously operated on for cataract in both eyes with an anterior chamber lens. A bilateral corneal edema occurred a few years after the operation, with a bilateral decrease in vision as a consequence. A corneal graft was performed in 2000 on the left eye in order to improve the patient's visual autonomy. The best treatment of this condition remains prevention, avoiding the use of this type of intraocular lens, whose main complication is corneal edema.

[Why are aphakic anterior chamber intraocular lenses still implanted? Five-year incidence and implantation circumstances at the Hôtel-Dieu in Paris]. / Pourquoi pose-t-on encore des implants de chambre antérieure à support angulaire chez l'aphaque? Incidence et circonstances des implantations sur 5 ans à l'Hôtel-Dieu de Paris.

PURPOSE: To report incidence and circumstances of aphakic anterior chamber intraocular lens implantation. METHODS: Retrospective study of medical charts of anterior chamber intraocular lens implantation in the Ophthalmology Department, Hôtel Dieu, Paris, from 1996 to 2000, investigating the number of anterior intraocular lenses (ACIOL) implanted, circumstances, and incidence during cataract extraction. RESULTS: One hundred and forty-three ACIOLs (139 patients, 60 men, 79 women) were implanted. The mean age was 75.98+/-10.6 years [range, 35-96 years]. All ACIOLs were of open-loop design: open C-loop or Kelman lens. Four circumstances leading to ACIOL implantation were reported: extracapsular cataract extraction with intraoperative complications (94 cases), intracapsular cataract extraction (13 cases), secondary implantation (20 cases), and penetrating keratoplasty with ACIOL (9 cases). Since 1996, the number of ACIOLs implanted each year has decreased (p<0.01): 1.09% of cataract extractions performed in 1996 were implanted with ACIOLs and 0.43% in 2000. At the end of the 5-year period, 12,580 cataract extractions had been performed in our department, with a mean incidence of ACIOL implantation at 0.85%. CONCLUSION: ACIOLs continue to be implanted in some cases in the absence of capsular support. Because of endothelial complications, they must be reserved for elderly patients. Endothelial surveillance using specular microscopy remains indispensable.

[Bilateral keratoconus: a case report]. / Kératocône bilatéral évolué

Among corneal dystrophies, the keratoconus is one of the most frequently observed among young adults. A clinico pathological case is reported in a 13-year-old-girl of African origin. The diagnosis of bilateral keratoconus was established based on the obvious changes of the corneal curvature and thickness. After an unsuccessful attempt to improve vision with contact lenses, a keratoplasty was finally performed on one side to remove the pathological cornea. Its histopathological study found the characteristic changes of keratoconus: breaks of Bowman's layer and corneal thinning.

[Followup of chicken pox keratitis. Anatomic-clinical case report]. / Kératite varicelleuse séquellaire.

Chicken pox is a very common infectious disease in children. Its corneal involvement is less serious than with measles, which may lead to blindness in numerous developing countries. However, with occasional cases occur. A case of a 59-year-old male patient whose left cornea was involved during a chicken pox infection at the age of 7 is reported. More recently, the vision of the right eye was normal at 20/20 and reduced to visual perception in the affected left eye. Corneal sensitivity was maintained in the left eye, which, however exhibited a central epithelial defect. A central round opacity of the left corneal stroma was believed to be the scar resulting from a previous disciform keratitis. The left central cornea was thinned and there was neither an anterior chamber flare nor new corneal vessels. This corneal condition required a corneal allograft, performed quickly because of the potential risk of perforation. Histopathological study of the corneal button showed a central corneal thinning with an increase in epithelial thickness. The corneal stroma was disorganized, with irregular collagen bundles. No inflammatory cells could be observed, however. All the histopathological changes observed were those of a corneal scar.

Amniotic membrane transplantation for partial limbal stem cell deficiency.

AIM: To examine the efficacy, safety, and long term outcomes of amniotic membrane transplantation for corneal surface reconstruction in cases of partial limbal stem cell deficiency. METHODS: 17 eyes of 15 patients with partial limbal stem cell deficiency underwent superficial keratectomy of the conjunctivalised corneal surface followed by amniotic membrane transplantation. Cases were followed up for at least a year. RESULTS: All eyes exhibited a stable, intact corneal epithelial surface after a mean follow up period of 25.8 months with no eyes developing recurrent erosion or persistent epithelial defect. The mean time to re-epithelialisation was 22.8 days. Overall improvement in visual acuity was observed in 92.9% of 14 eyes with visual potential. Of those, five eyes gained six or more lines, two eyes gained between four and five lines, six eyes gained between one and three lines, and one eye lost three lines of Snellen acuity. Pain and photophobia were abolished in 86% of cases and substantially reduced in 14%, with all eyes exhibiting decreased vascularisation and inflammation at final follow up. CONCLUSIONS: Amniotic membrane transplantation appears to be a safe and effective method of restoring a stable corneal epithelium for cases of partial limbal stem cell deficiency and can be considered as an alternative to limbal autograft or allograft.

Histologic phenotype-genotype correlation of corneal dystrophies associated with eight distinct mutations in the TGFBI gene.

PURPOSE: To establish a phenotype-genotype correlation of various autosomal-dominant corneal dystrophies among French subjects. DESIGN: Retrospective molecular genetic study and clinicopathologic correlation. PARTICIPANTS: Forty-four subjects from 26 unrelated French families were included in this study, and 60 corneal buttons could be examined at the histologic and ultrastructural levels. METHODS: Light microscopy and transmission electron microscopy were performed on corneal specimens obtained during keratoplasty. Blood samples were collected for DNA analysis. MAIN OUTCOME MEASURES: After genomic DNA extraction from peripheral blood leukocytes of each family member, exons of the TGFBI gene were amplified by polymerase chain reaction (PCR), and the PCR products were directly sequenced on both strands. RESULTS: Four different mutations were found to be responsible for dystrophy of granular type (R555W, R124L, R124H, and R124L+delT125-delE126), three other different mutations produced a lattice type (R124C, H626R, and A546T), and the last mutation identified was associated with the honeycomb-shaped dystrophy (R555Q). Each subtype of dystrophy showed, histologically and ultrastructurally, specific characteristics that are easily recognizable. However, besides these stereotyped forms, differential histologic diagnosis of atypical forms remains difficult, and these forms could be misdiagnosed. CONCLUSIONS: The characteristic biomicroscopic appearance and histopathologic features of each "classic" dystrophy present a significant degree of specificity and generally provide an accurate diagnosis. However, atypical forms in which clinical and histologic data alone could be misleading, are unequivocally diagnosed after DNA analysis.

[Avellino dystrophy. Current diagnostic criteria]. / La dystrophie d'Avellino. Critères diagnostiques modernes.

We report a French family suffering from an Avellino corneal dystrophy diagnosed by using clinical, histological, ultrastructural and genetics findings. Our results indicate that direct corneal examination and routine histological examinations must always be associated with an assay for BIGH3 gene mutations to establish a modern and unambiguous diagnosis of a corneal dystrophy.

Clinical, histologic, and ultrastructural features of the corneal dystrophy caused by the R124L mutation of the BIGH3 gene.

OBJECTIVE: This study was designed to describe the clinical, histologic, and ultrastructural features of the corneal dystrophy associated with the R124L mutation of the BIGH3 gene. DESIGN: Retrospective clinical and histologic review of a new genetic mutation. PARTICIPANTS: Thirty-four patients from five unrelated French families with corneal dystrophy caused by the R124L mutation of the BIGH3 gene were studied at the clinical, histologic, and ultrastructural levels. Records of patients carrying this mutation were compared with those from three unrelated patients with corneal dystrophy of Bowman's layer (CDB) type 2 (R555Q mutation) and from three unrelated patients with classic corneal granular dystrophy (R555W mutation). INTERVENTION: The mutational genetic status of the BIGH3 gene was determined for each patient, and the histologic and ultrastructural data available after corneal graft were analyzed. MAIN OUTCOMES MEASURES: Genomic DNA was extracted from peripheral blood leukocytes. Exons 4 and 12 of the BIGH3 gene were amplified by the polymerase chain reaction (PCR), and the PCR products were directly sequenced. RESULTS: All 34 patients with the R124L mutation displayed the clinical, histologic, and electron microscopic features of the dystrophy previously described as a superficial variant of corneal granular dystrophy. Combining molecular genetics with clinical and histologic findings established a clear distinction between the R555Q and R555W dystrophies. CONCLUSIONS: The R124L mutation of the BIGH3 gene is associated with specific clinical and morphologic criteria. This indicates that molecular studies are needed for an adequate classification of corneal dystrophies. All criteria are presently available to segregate the dystrophy caused by the R124L mutation (known as CDB1) from the dystrophy caused by the R555Q mutation (known as CDB2).

Macular hemorrhage after laser in situ keratomileusis for high myopia.

We describe 2 women with high myopia of -12.0 and -18.0 diopters who presented with myopic macular hemorrhages 1 and 4 days, respectively, after being treated by laser in situ keratomileusis (LASIK). One hemorrhage was related to a pre-existing choroidal neovascularization and the other to the presence of lacquer cracks. The hemorrhages resolved but resulted in a permanent decrease in vision. A careful fundus examination should be conducted before performing LASIK in highly myopic patients. In cases of similar macular pathology, fluorescein angiography should be done before LASIK.

[Therapeutic photokeratectomy for the treatment of band keratopathy]. / La photokératectomie thérapeutique dans le traitement de la kératopathie en bandelettes.

PURPOSE: This retrospective study was designed to investigate the therapeutic potential of phototherapeutic keratectomy (PTK) for the treatment of band keratopathy. PATIENTS AND METHODS: PTK was performed with the Excimed UV 200, (Summit Technology, Inc) on 27 eyes of 22 patients and with the Nidek EC5000 on 11 eyes of 10 patients with band keratopathy. Mean patient age was 57 years. The mean follow-up period was 19 months (ranged from 4 to 54 months). When possible, the change in best corrected visual acuity and spherical equivalent was evaluated at 6, 12, 18 and 24 months. RESULTS: Almost 90% of patients with smooth bands achieved visual improvement and 85% of patients with rough bands improved ocular discomfort. The mean hyperopic shift caused by tissue ablation was less than +2.0D after one year, for smooth bands. CONCLUSION: Excimer laser PTK is a safe and effective outpatient treatment and should be used as initial treatment for band keratopathy.

A new mutation (A546T) of the betaig-h3 gene responsible for a French lattice corneal dystrophy type IIIA.

PURPOSE: To characterize the betaig-h3 gene defect in a French family affected with lattice corneal dystrophy type IIIA (LCDIIIA). METHODS: Histologic examination was performed from corneal buttons of two patients. Genomic DNA was extracted from leukocytes, and exons of the betaig-h3 gene were amplified by polymerase chain reaction to be directly sequenced. RESULTS: Numerous deposits were evident in the stroma and beneath the Bowman membrane, which had all the features of amyloid deposits. Analysis of exon 12 revealed a heterozygous G to A transition on codon 546. CONCLUSION: In contrast to Japanese patients, these French patients affected with LCDIIIA carry a distinct mutation of the betaig-h3 gene (A546T instead of P501T). Therefore, it is unclear whether different mutations could result in the same dystrophy or whether we are dealing with clinical heterogeneity of LCDIIIA.

[Phototherapeutic keratectomy in the treatment of Groenouw's type I corneal dystrophy]. / La photokératectomie thérapeutique dans le traitement de la dystrophie de Groenouw I.

PURPOSE: This study was designed to investigate the therapeutic potential of phototherapeutic keratectomy (PTK) for the treatment of corneal granular dystrophy. PATIENTS AND METHODS: PTK was performed with the Excimed UV 200, (Summit Technology, Inc) on a series of 27 eyes of 22 patients with corneal granular dystrophy. Mean patients' age was 34.6 years. The mean follow-up period was 31 months (ranged from 6 to 52 months). The changes in best corrected visual acuity and spherical equivalent were evaluated at 6, 12, 18 and 24 months. RESULTS: All of patients achieved visual improvement. Mean preoperative best corrected visual acuity (BCVA) was 20/100 and mean postoperative BCVA was 20/30 at one year. The mean hyperopic shift caused by tissue ablation was +/- 2.8 D after one year. CONCLUSION: Corneal grafting was the standard treatment for visually disabling granular dystrophy, but PTK has significant advantages over this procedure and must now be the standard method of managing corneal granular dystrophy when intervention is required.