[Gillespie syndrome: an uncommon presentation of congenital aniridia]. / Syndrome de Gillespie: un cas rare d'aniridie congénitale.

We report here the case of a patient suffering from Gillespie syndrome (GS) (partial aniridia, mental retardation, and cerebellar ataxia). The typical presentation is based on fixed dilated pupils in a hypotonic child. Iris abnormalities distinguish GS from other forms of aniridia. At the time of initial presentation, neurological involvement and radiologic abnormalities could be absent or delayed. Genetic determinism is still unknown. The pattern of inheritance seems to be heterogeneous, according to the autosomal recessive and autosomal dominant forms previously described.

Is manual counting of corneal endothelial cell density in eye banks still acceptable? The French experience.

AIM: To examine the differences in manual endothelial cell counting methods in French eye banks and to analyse whether these differences could explain some substantial discrepancies observed in endothelial cell density (ECD) for corneas made available for transplant. METHODS: A questionnaire was sent to the 22 eye banks asking for details of the technical features of the light microscopes used, the microscope calibration, strategy for cell counting, the technical staff, and the method of presenting endothelial data. RESULTS: All eye banks responded and 91% (20/22) used only manual counting methods, in real time, directly through a microscope, and 62 different technicians, with varying experience, were involved in such counting. Counting of cells within the borders of a grid that were in contact with two adjacent borders was the most common method (17/22, 77%). Of the eight banks (8/22, 36%) that did not calibrate their microscopes, six reported the highest ECD values. Of the 14 others (64%), six applied a "magnification correcting factor" to the initial cell counts. In five of these cases, the corrected ECD was lower than estimated on initial count. Most of the banks (12/22, 55%) counted 100 cells or less in one to six non-adjacent zones of the mosaic. 14 of the banks (14/22, 64%) also graded cell polymegethism while seven (7/22, 32%) also graded pleomorphism ("hexagonality"). CONCLUSIONS: Lack of microscope calibration appears to be the leading cause of variance in ECD estimates in French eye banks. Other factors such as differences in counting strategy, the evaluation of smaller numbers of cells, and the different extent of experience of the technicians may also contribute to intraobserver and interobserver variability. Further comparative studies, including cross checking and the outcome of repeated counts from manual methods, are clearly needed with cross calibration to a computer based image archiving and analysis system.

[Optimizing microbiological controls of corneal organ culture media]. / Optimisation du contrôle microbiologique des milieux d'organoculture des greffons cornéens.

BACKGROUND/AIMS: To compare the efficiency of an automated method using blood bottles with conventional microbiological tests for controlling sterility in cornea organ culture media. METHODS: Two complementary studies were conducted. Experimental study: standard organ culture media were contaminated with four different inocula of 14 bacteria and 3 fungi. The bactericidal activity of organ culture media were evaluated after 48 hours of incubation at 31C. Observational study: 357 samples of organ culture media were collected over 1 year in our cornea bank. For both studies, media were inoculated in three blood bottles (aerobic, anaerobic, fungal) placed in an automat with automated detection every 10 minutes, and in three conventional microbiological media as a control. Changes in organ culture medium color and growth on conventional broth were checked daily by visual inspection. All samples were observed experimentally for 14 days. The sensitivity and rapidity of contamination detection were compared across the three methods: blood bottles, conventional method, and visual inspection of medium color. RESULTS: Experimental study: organ culture medium eradicated five bacteria: S. pneumoniae, B. catarrhalis, E. coli, P. acnes and H. influenzae. For the others, (Methicillin-resistant S. aureus, Methicillin-sensitive S. aureus, S. epidermidis, S. haemolyticus, P. aeruginosa, A. baumannii, B. subtilis, K. pneumoniae, E. faecalis, C. albicans, C. kruzei, A. fumigatus) the blood bottle method, the conventional microbiological method, and the visual inspection detected microbiological growth respectively in 100%, 76.5%, and 70% of cases. Mean detection time using blood bottles was 15.1 hours (standard deviation, 13.8; range, 2-52). In cases of detection by the blood-bottle method and the conventional method, the former was always faster: 95.5% versus 65.2% detection within 24 hours (p=0.022). Observational study: the global contamination rate was 8% (29/357 analysis). The gain in sensitivity with blood bottles was 25% compared with the conventional method. Five bacteria (three coag. neg Staphylococcus, one E. faecalis, one P. paucimobilis) were detected only by the blood bottles. In addition, these were always detected more quickly with, respectively, 66.6% versus 26.6% detection with 24 hours (p=0.028). CONCLUSIONS: Blood bottles detect contaminations of cornea organ culture media more efficiently and faster than conventional microbiological methods. They make it possible to reduce the quarantine period with an equally high security level. Consequently, they should be recommended in cornea preservation guidelines.

[Management of traumatic cataracts]. / Les cataractes traumatiques: conduite à tenir pratique.

Traumatic cataract is the severe result of an accident often concerning young patients, sometimes children. Anatomical and clinical presentations vary depending on whether the lens is luxated, the wound involves perforation, or there is an intraocular foreign body. The associated wounds and the late complications, particularly retinovitreous ones, can impair the visual prognosis sometimes over the very long term. The correction of aphakia with an intraocular lens should be decided on a case by case basis.

[Facial anesthetic blocks in the treatment of acute pain during ophthalmic zoster]. / Blocs anesthésiques faciaux dans le traitement de la douleur aiguë du zona ophtalmique.

BACKGROUND: Ophthalmic zoster is frequently accompanied by severe pain in the frontal and nasal divisions of the ophthalmic nerve. Treating this pain is often difficult, particularly in elderly patients, owing to iatrogenic effects and to interactions with the pre-existing diseases and treatments frequently present in this age group. The aim of our study was to consider the efficacy and toxicity of the frontal and nasal nerve blocks in the treatment of severe pain during acute ophthalmic zoster in the elderly. MATERIAL AND METHODS: A prospective study was conducted on 20 patients (mean age, 76 +/-7 years; range, 63-88) presenting with acute ophthalmic zoster with severe pain (less than 1 month since onset), which had resisted analgesic medication. All patients had a visual analogue score for pain (VAS) of 4 or more and received one or more anesthetic blocks of a compound of bupivacaine with adrenaline associated with clonidine at the frontal branch and sometimes the nasal branch levels of the ophthalmic nerve. Pain was measured daily by VAS for 5 days, and the blocks were repeated if the VAS was still 4 or higher. Patients were checked for local or systemic side effects. RESULTS: The number of anesthetic blocks per patient ranged from one to four (mean: 2.3 +/-0.7). All patients experienced less pain after the first injection. The mean preinjection VAS was 7.4 +/-1 and fell to 4.8 +/-1.0, 4.1 +/-1.1, 3.5 +/-1.0, 3.2 +/-0.6 and 2.8 +/-0.9 at day 1, day 2, day 3, day 4 and day 5, respectively (p<0.001). It was possible to reduce analgesic medication permanently in all patients. No local or systemic side effect was observed. CONCLUSION: Anesthetic blocks of the frontal and nasal branches, repeated if necessary, give fast and effective relief from the severe pain of acute ophthalmic zoster. They are fully tolerated and simple to administer, making them an excellent indication in the complementary treatment of the pain of hyperalgic acute zoster in the elderly.

Sensitivity and rapidity of blood culture bottles in the detection of cornea organ culture media contamination by bacteria and fungi.

AIMS: To test the bactericidal activity of standard organ culture medium, and to compare the sensitivity and rapidity of blood culture bottles with conventional microbiological methods for detection of bacteria and fungi inoculated in a standard cornea organ culture medium. METHODS: The bactericidal activity of contaminated standard organ culture medium containing 100 IU/ml penicillin, 0.1 mg/ml streptomycin, and 0.25 micro g/ml amphotericin B was evaluated after 48 hours of incubation at 31 degrees C with five inocula of 14 bacteria. Two yeasts (Candida spp) and one Aspergillus were also tested. Contaminated media were then inoculated in three blood bottles (aerobic, anaerobic, fungal) placed in a Bactec 9240 automat; three conventional microbiological broths were the control. Changes in colour of organ culture medium and growth on conventional broth were screened daily by visual inspection. The sensitivity and rapidity of detection of contamination were compared between the three methods: blood bottle, conventional, and visual. RESULTS: Organ culture medium eradicated five bacteria irrespective of the starting inoculums: Streptococcus pneumoniae, Branhamella catarrhalis, Escherichia coli, Propionibacterium acnes, and Haemophilus influenzae. For micro-organisms where the medium was ineffective or bactericidal only (methicillin resistant Staphylococcus aureus, methicillin sensitive Staphylococcus aureus, Staphylococcus epidermidis, Staphylococcus haemolyticus, Pseudomonas aeruginosa, Acinetobacter baumannii, Bacillus subtilis, Klebsiella pneumoniae, Enterococcus faecalis, Candida albicans, Candida kruzei, Aspergillus fumigatus), the blood bottle, conventional, and visual methods detected microbial growth in 100%, 76.5%, and 70% of cases respectively. Mean detection time using blood bottles was 15.1 hours (SD 13.8, range 2-52). In cases of detection by the blood bottle method and the conventional method, the former was always faster: 95.5% against 65.2% detection within 24 hours (p=0.022) respectively. CONCLUSIONS: Blood bottles detect more efficiently and more rapidly a wider range of bacteria and fungi than the conventional microbiological method and the visual inspection of organ culture media.

[Cornea donation consent by telephone]. / Recueil du consentement au don de cornée par téléphone.

BACKGROUND: The cornea donation process often runs into problems of obtaining family consent. A face-to-face interview is often not possible for logistical reasons. We carried out a prospective study on the effectiveness of telephone contact in obtaining donation consent. MATERIAL AND METHODS: Consent was obtained by a single, non medical, hospital coordinator. He contacted families selected on good staff-family relations during the patient's stay. If a face-to-face interview was not possible, a telephone interview was conducted using a standardized procedure. RESULTS: Over 21 months, 334 families were contacted, either in a face-to-face interview (142, 42.5%) or by telephone (192, 57.5%). Donation consent was obtained in 66.5% of cases, 106 times by telephone (47.7%) and 116 times in the face-to-face interview (52.3%). The acceptance rate was 55.2% by telephone and 81.6% face to face (p<0.001). CONCLUSIONS: The telephone interview was an effective method for obtaining consent for cornea donation. Although the acceptance rate using this method is lower than the face-to-face interview, using the telephone should not be overlooked as this enabled procurement of nearly half the corneas in our hospital.

[Automated analyser of organ cultured corneal endothelial mosaic]. / Mise au point d'un analyseur automatique de l'endothélium des greffons cornéens conservés.

PURPOSE: Until now, organ-cultured corneal endothelial mosaic has been assessed in France by cell counting using a calibrated graticule, or by drawing cells on a computerized image. The former method is unsatisfactory because it is characterized by a lack of objective evaluation of the cell surface and hexagonality and it requires an experienced technician. The latter method is time-consuming and requires careful attention. We aimed to make an efficient, fast and easy to use, automated digital analyzer of video images of the corneal endothelium. METHODS: The hardware included a PC Pentium III ((R)) 800 MHz-Ram 256, a Data Translation 3155 acquisition card, a Sony SC 75 CE CCD camera, and a 22-inch screen. Special functions for automated cell boundary determination consisted of Plug-in programs included in the ImageTool software. Calibration was performed using a calibrated micrometer. Cell densities of 40 organ-cultured corneas measured by both manual and automated counting were compared using parametric tests (Student's t test for paired variables and the Pearson correlation coefficient). RESULTS: All steps were considered more ergonomic i.e., endothelial image capture, image selection, thresholding of multiple areas of interest, automated cell count, automated detection of errors in cell boundary drawing, presentation of the results in an HTML file including the number of counted cells, cell density, coefficient of variation of cell area, cell surface histogram and cell hexagonality. The device was efficient because the global process lasted on average 7 minutes and did not require an experienced technician. The correlation between cell densities obtained with both methods was high (r=+0.84, p<0.001). The results showed an under-estimation using manual counting (2191+/-322 vs. 2273+/-457 cell/mm(2), p=0.046), compared with the automated method. CONCLUSIONS: Our automated endothelial cell analyzer is efficient and gives reliable results quickly and easily. A multicentric validation would allow us to standardize cell counts among cornea banks in our country.

Automated tri-image analysis of stored corneal endothelium.

BACKGROUND: Endothelial examination of organ culture stored corneas is usually done manually and on several mosaic zones. Some banks use an image analyser that takes account of only one zone. This method is restricted by image quality, and may be inaccurate if endothelial cell density (ECD) within the mosaic is not homogeneous. The authors have developed an analyser that has tools for automatic error detection and correction, and can measure ECD and perform morphometry on multiple zones of three images of the endothelial mosaic. METHODS: 60 human corneas were divided into two equal groups: group 1 with homogeneous mosaics, group 2 with heterogeneous ones. Three standard microscopy video images of the endothelium, graded by quality, were analysed either in isolation (so called mono-image analysis) or simultaneously (so called tri-image analysis), with 50 or 300 endothelial cells (ECs) counted. The automated analysis was compared with the manual analysis, which concerned 10 non-adjacent zones and about 300 cells. For each analysis method, failures and durations were studied according to image quality. RESULTS: All corneas were able to undergo analysis, in about 2 or 7.5 minutes for 50 and 300 ECs respectively. The tri-image analysis did not increase analysis time and never failed, even with mediocre images. The tri-image analysis of 300 ECs was always most highly correlated with the manual count, particularly in the heterogeneous cornea group (r=0.94, p<0.001) and prevented serious count errors. CONCLUSIONS: This analyser allows reliable and rapid analysis of ECD, even for heterogeneous endothelia mosaics and mediocre images.

[Use of a pair of blood culture bottles for sterility testing of corneal organ culture media]. / Intérêt de l'utilisation de flacons à hémoculture pour le contrôle de stérilité microbiologique des greffons cornéens.

PURPOSE: To test the effectiveness and rapidity of a pair of blood culture bottles in the diagnosis of bacterial and fungal contamination of corneal organ culture media. MATERIAL: and methods: Seven hundred and sixty one microbiological analysis of storage media (Inosol(R) and Exosol(R), Opsia, Toulouse, France), sampled in all phases of the organ culture at 31 degrees C of 410 consecutive corneas, were analyzed. Each medium was inoculated in a pair of Bactec Plus Aerobic/F(R) and Bactec Lytic/10 Anaerobic/F(R) blood bottles (Becton Dickinson, Cockeysville, MD) and placed in a Bactec 9240 incubator for 14 days at 37 degrees C and in a Sabouraud broth at 20 degrees C. Changes in color or turbidity of storage media were evaluated daily at the corneal bank. Recipients were screened after graft for signs of infection. RESULTS: The overall contamination rate was 2.4% (18/761). Contamination was detected in less than 1 day in 78% (14/18) and in less than 2 days in 94% (17/18). Positivity of the microbiological controls of starting media preceded medium color changes in 10 out of 14 cases. Bactec blood bottles allowed detection of bacteria as well as Candida sp. yeasts. DISCUSSION: The use of a pair of aerobic and anaerobic blood culture bottles is a simple, effective and rapid method for the diagnosis of a wide range of microbiological contaminations of organ-cultured corneas during banking. CONCLUSION: The validation of this protocol will require a prospective study to compare it with the conventional microbiological method.

[Corneal harvesting from donors over 85 years of age: cornea outcome after banking and grafting]. / Prélèvements de cornées après 85 ans: issue des greffons après organoculture et devenir des greffés.

PURPOSE: To study the suitability of corneas from very old donors for graft after organ culture and their clinical and endothelial outcomes in recipients after perforating keratoplasty. METHODS: We stored 419 corneas at 31 degrees C for 13.1 +/- 4 days (mean +/- SD) and then divided them according to donor age: group 1, donors under 85 years of age (n=3 3 0, 79%, 16-84 years old), and group 2, donors over the age of 85 (n=8 9, 21%, 85-100 years old). Endothelial density at the time of harvest and before and after organ culture, rates of suitability for grafting, and clinical and endothelial outcomes of the 196 keratoplasty procedures were compared in a prospective longitudinal study of the 2 groups, with a mean follow-up of 25 months. The corneas were grafted with no pre-established policy on matching with the age of the receiver. Statistical analysis was carried out on SPSS 10.0: Chi(2), Student t test, and Kaplan Meier survival curves. RESULTS: The average age of the donors was 72.1 +/- 16.7 years. The macroscopic aspect of the corneas was judged to be of slightly lower quality in group 2. No statistically significant difference was found in overall suitability for transplantation (group 1, 45% vs group 2, 54%, p=0.17) but elimination for low endothelial density was more frequent in group 2 (67% vs 39%, p=0.001). Cell density at the beginning of organ culture was lower in very old corneas than in younger corneas (respectively, 2116 +/- 368 vs 2 311 +/- 360 cell/mm(2), p=0.002) but no difference was apparent at the end of organ culture (respectively, 2 011 +/- 285 vs 2 090 +/- 296, p=0.12) because very old corneas lost fewer cells than younger ones (respectively, 5.6% vs 10.0%, p=0.001). There was no correlation between donor/receiver age (r=0. 337) but group 1 corneas were slightly more frequently allotted to receivers with normal endothelium (p=0.019). During surgery, the two groups did not differ in terms of the macroscopic aspect of the grafts. In the 196 grafted patients, and without age-matching, overall graft survival (86% vs 79%, p=0.275), visual acuity, and endothelial density (1 194 +/- 469 vs 1098 +/- 545 cells/mm(2), p=0.387) did not differ at the completion of the study. DISCUSSION: The corneas from very old donors were macroscopically of poorer quality and had a lower cellular endothelial density at harvesting, but these differences disappeared after organ culture because of greater cell loss in corneas from younger donors. Selection by organ culture ensures that functional, anatomical, and cellular results are not influenced by very old donor age. CONCLUSION: Considering the aging population in countries with a high standard of living, the techniques available for selecting corneas based on endothelial quality, and the increasing need for corneal grafts, the very old age should not be deemed off-limits for corneal harvesting.

Cornea procurement from very old donors: post organ culture cornea outcome and recipient graft outcome.

AIM: To study the suitability of corneas from very old donors for graft after banking and their clinical and endothelial outcomes in recipients. METHODS: 419 corneas stored in organ culture were divided into group 1, donors under 85 years (330 corneas) and group 2, "very old" donors aged 85 years and over (89 corneas). Endothelial cell density (ECD) before and after organ culture, discard rate before and after storage, and clinical and endothelial outcomes of the 196 penetrating keratoplasties (PKP) (158 in group 1 and 38 in group 2) were compared in a prospective longitudinal study. RESULTS: Initial ECD was lower in group 2 than in group 1 and elimination for low ECD was more frequent in group 2 (respectively 38% v 20.2%, p=0.001). At the end of storage, because very old corneas lost fewer ECs than younger ones (respectively 4.2% v 9.5%, p=0.022), ECD was comparable between the two groups. The corneas of very old donors had a poorer macroscopic appearance at procurement and during surgery. Despite this, in grafted patients, overall graft survival in groups 1 and 2 (respectively 87.4% v 80.6%, p=0.197), visual acuity, and ECD did not differ at completion of the study (mean follow up 25 months). CONCLUSION: This study suggests that endothelial cell count during banking ensures that functional and cellular results of PKPs are not dramatically influenced by very old donor age. Considering Europe's ageing population, the very elderly should not be deemed off limits for corneal procurement.

Use of a pair of blood culture bottles for sterility testing of corneal organ culture media.

AIMS: To test the effectiveness and rapidity of a pair of blood culture bottles in the diagnosis of bacterial and fungal contamination of corneal organ culture media. METHODS: 761 microbiological analyses of storage media (Inosol and Exosol, Opsia, Toulouse, France), sampled in all phases of the organ culture at 31 degrees C of 410 consecutive corneas, were analysed. Each medium was inoculated in a pair of Bactec Plus Aerobic/F and Bactec Lytic/10 Anaerobic/F blood bottles and placed in a Bactec 9240 incubator for 14 days at 37 degrees C and in a Sabouraud broth at 20 degrees C. Changes in colour or turbidity of storage media were evaluated daily at the corneal bank. Recipients were screened post-graft for infectious signs. RESULTS: Overall contamination rate was 2.4% (18/761). Contamination was detected in less than 1 day in 78% (14/18) and less than 2 days in 94% (17/18). Positivity of the microbiological controls of starting media preceded changes medium colour in 10 out of 14 cases. Bactec blood bottles allowed detection of bacteria as well as yeasts. CONCLUSION: The use of a pair of Bactec blood culture bottles appears reliable for the rapid diagnosis of a wide range of microbiological contaminations of organ cultured corneas during banking.

[Epithelial invasion of the corneal button. Good techniques for human corneal harvesting]. / L'invasion epithéliale du bouton cornéen. A propos des bonnes pratiques de prélèvement des cornées humaines.

Corneoscleral excision is the most common harvesting technique used in France before keratoplasty. We report two cases of corneal button epithelial invasion during organ culture caused by an excision that was too small. Two corneas stored for 23 days in organ culture at 31 degrees C had a cellular proliferation on the surface of Descemet's membrane. Cornea button diameters were respectively 11 and 13mm. The epithelial origin of the invasion was confirmed by examining the flat mount of Descemet's membrane by inverted microscopy and standard histopathology. The authors discuss the origin and the potential consequences of the epithelial invasion and emphasize the importance of good corneal harvesting recommendations before keratoplasty.

[Complications of radiotherapy in patients with age-related macular degeneration (AMD). A study of 48 cases]. / Complications de la radiothérapie des néovaissaux de la dégénérescence maculaire liée à l'âge.

PURPOSE: This study describes the ocular complications after teletherapy indicated for patients with age-related macular degeneration (AMD). MATERIAL: and methods: The study concerned 48 patients suffering from an exudative form of age-related macular degeneration, non suitable for laser photocoagulation, and irradiated by high-energy electron beam therapy. RESULTS: With a mean follow-up time of 2 years, late effects were observed in 16 (33. 3%) patients: 9 (18.75%) radiation optic neuropathies, 9 radiation retinopathies (18.75%) and 2 neovascular glaucomas (4.1%). CONCLUSION: The frequent and serious complications observed led us to adapt the technique and dose of radiation therapy indicated in patients with age-related macular degeneration.

[Myxoma of the left atrium: a cause of retinal artery embolism not to be ignored. Apropos of a case]. / Myxome de l'oreillette gauche: une cause d'embolie artérielle rétinienne à ne pas méconnaître. A propos d'une observation.

We report a case of retinal artery obstruction in a 56-year-old woman which proved to be a first symptom of a left atrial myxoma. The patient also had an inflammatory syndrome and a transient embolism of the cerebellum and the left leg. Two dimensional echocardiography showed an atrial mass compatible with myxoma. This diagnosis was confirmed by histological analysis of the surgically-removed tumour.

[Iris tuberculosis. A propos of a case diagnosed by iridectomy]. / Tuberculose irienne. A propos d'une observation diagnostiquée par iridectomie.

Ocular tuberculosis is currently rare in developed countries. We report a case of tuberculous nodular anterior uveitis which revealed primary tuberculosis in a 2-year old girl. This diagnosis was established on microscopic examination of a surgical iridectomy specimen. Thus, a metastatic retinoblastoma was eliminated. The subsequent clinical investigations showed that the girl's father had active pulmonary tuberculosis.

[Eye manifestations of Horton's disease]. / Manifestations ophtalmologiques de la maladie de Horton.

Ocular complications occurring in temporal arteritis are of different severities. These manifestations of the disease occurred in a population of 57 patients with diagnosis of temporal arteritis, in 16 of them (28%). The diagnosis was suggested by finding an inflammatory syndrome (high erythrocyte sedimentation rate) and confirmed by temporal artery biopsy. In making this diagnosis, it was helpful to find a history of characteristic temporal headaches, neckache, jaw claudication, fever and malaise or weight loss in addition to polymyalgia and polyarthralgia. 6 patients presented with diplopia and different ocular muscle or nerve palsies. The 10 others came for sudden loss of vision due to ischemic anterior or posterior neuropathy and in one case, central artery obliteration. Prompt treatment with steroids gave good results on oculomotor troubles and in preventing the risk of involvement of the fellow eye. But loss of vision did not regress with this treatment. That means the importance in making this diagnosis and starting very promptly this treatment.