L. El Matri

Pediatric Penetrating Keratoplasty: Indications and Outcomes

PURPOSE This report evaluates patient characteristics, indications, and outcomes of pediatric keratoplasty, and identifies variables that help to predict poor surgical outcomes. METHODS We undertook a retrospective review of all cases in our department of primary penetrating keratoplasty performed in children 14 years of age or younger between January 2003 and December 2009. RESULTS Sixteen primary penetrating keratoplasties were performed during the study interval. Mean age was 11.2 years (3 to 14 years) and the gender ratio was 2. The mean duration of follow-up was 16 months (2 to 36 months).The surgical indications were acquired traumatic opacities in 6 cases, keratoconus in 5 cases, corneal perforation secondary to infectious keratitis in 3 cases, hereditary corneal dystrophy in 1 case, and acquired non-traumatic opacities secondary to congenital glaucoma in one case. The initial visual acuity was less than 1/20 in 68% of cases and the mean final visual acuity after 1 year was 2/10. The graft was clear in 52% of cases after 1 year of follow-up. Postoperative complications were graft failure (24%), ocular inflammation (5%), and ocular trauma (19%). CONCLUSION Penetrating keratoplasty in children has been documented to have a higher rate of graft failure and a worse visual prognosis than adult keratoplasty. Poor prognosis outcomes were especially caused by noncooperation of parents and postoperative ocular trauma.

Les décollements de rétine rhegmatogènes bilatéraux simultanés. À propos de 7 observations

PURPOSE Simultaneous bilateral rhegmatogenous retinal detachments are rare. The purpose of our study was to examine the incidence, predictive factors, surgical results and prognosis of these retinal detachments. PATIENTS AND METHODS A retrospective analysis of the medical records of 468 consecutive patients, 7-89 years of age (mean, 45.7 years), undergoing surgery for rhegmatogenous retinal detachment between 1993 and 2000. RESULTS During this period, a total of 497 operations for rhegmatogenous retinal detachment were done. Thirty-two patients had bilateral rhegmatogenous retinal detachment. Simultaneous detachments were observed in seven patients (1.5%). In four cases, the diagnosis of retinal detachment was fortuitous. In fact, most patients presented with unilateral symptoms. The mean age (35 years) of patients suffering from simultaneous bilateral rhegmatogenous retinal detachment was younger than that of patients with unilateral or consecutive bilateral retinal detachments. Five patients were myopic. Multiple round retinal holes were the most frequent lesions responsible for retinal detachment. A preoperative proliferative vitreoretinopathy was found in 57% of cases. The retina was reattached in nine cases (81%). CONCLUSION Simultaneous bilateral rhegmatogenous retinal detachment is usually found in relatively young myopic patients with atrophic retinal holes. They are rare but severe because of their frequent association with preoperative proliferative vitreoretinopathy.But Les decollements de retine rhegmatogenes bilateraux simultanes sont rares. Le but de notre etude est d’etudier leur incidence, les facteurs favorisants leur survenue, leurs resultats operatoires et leur pronostic. Patients et methodes Il s’agit d’une etude retrospective portant sur des patients âges de 7 a 89 ans (45,7 ans) operes de decollements de retine rhegmatogenes durant la periode allant de 1993 a l’an 2000. Resultats Durant cette periode 497 interventions pour decollement de retine rhegmatogene ont ete pratiquees. Trente-deux patients avaient un decollement de retine rhegmatogene bilateral dont 7 (1,5 %) etaient simultanes. Dans 4 cas, la decouverte du decollement de retine rhegmatogene bilateral etait fortuite. En effet, la symptomatologie etait le plus souvent unilaterale. L’âge moyen (35 ans) des patients porteurs de decollements de retine rhegmatogenes bilateraux etait inferieur a celui des patients presentant un decollement de retine rhegmatogene unilateral ou bilateralise. Le plus souvent, les trous atrophiques etaient a l’origine du decollement de retine. En preoperatoire, la frequence de la proliferation vitreoretinienne etait de 57 %. La retine a ete reappliquee dans 9 cas sur 11 operes (81 %). Conclusion Les decollements de retine rhegmatogenes bilateraux surviennent le plus souvent chez des sujets jeunes, myopes, porteurs de lesions degeneratives de la peripherie retinienne a type de trous atrophiques. Ils sont rares mais graves car souvent associes a une proliferation vitreoretinienne.

Les abcès graves de la cornée : à propos de 100 cas

But Etudier les facteurs favorisants, les aspects cliniques, les elements du diagnostic microbiologique et discuter la conduite therapeutique et le pronostic des abces corneens graves. Patients et methodes Nous avons realise une etude retrospective des patients hospitalises dans le service B a l’institut Hedi Raies d’ophtalmologie (Tunis, Tunisie) pour abces grave de la cornee sur une periode allant d’aout 1996 a novembre 2004. Resultats Cent patients presentant un abces corneen ont ete hospitalises : 55 hommes et 45 femmes, âges de 11 ans a 87 ans. Les principaux facteurs predisposant retrouves etaient une pathologie de la surface oculaire (30 %), un traumatisme oculaire (28 %), une chirurgie oculaire (17 %), le port de lentilles de contact (8 %). Dans 9 % des cas, nous n’avons trouve aucun facteur de risque. La culture apres grattage corneen a ete positive dans 42 % des cas, identifiant des bacteries Gram- (48,6 %), des bacteries Gram− (29,7 %) et des mycoses (21,6 %). La majorite des patients (93 %) ont recu une antibiotherapie a large spectre, avec un succes therapeutique dans 53,8 % des cas. Les antifongiques par voie parenterale ont ete indiques dans 17 cas. Le recours a la chirurgie a ete necessaire dans 14 cas (25,8 %) : une keratoplastie therapeutique dans 5 cas et une keratoplastie a froid dans 9 cas. L’acuite visuelle finale a ete superieure ou egale a l’acuite visuelle initiale dans 81 cas. Une perte anatomique du globe a ete observee dans 8 cas : une evisceration dans 6 cas, et une enucleation dans 2 cas. Conclusion L’abces corneen est une pathologie grave et frequente pouvant entrainer la cecite. Seule une prise en charge precoce et adaptee aux resultats microbiologiques permet d’ameliorer le pronostic de laabces corneen.

Épaisseur choroïdienne fovéolaire au SD-OCT dans la myopie forte avec ou sans néovaisseaux choroïdiens

PURPOSE To measure macular choroidal thickness (CT) using spectral-domain optical coherence tomography (SD-OCT) in eyes with myopic macular choroidal neovascularization (CNV), and to compare choroidal thickness in these eyes with highly myopic eyes without CNV. PATIENTS AND METHODS Sixty-four eyes with myopic CNV matched with 64 highly myopic eyes without CNV by age and axial length (AL) were examined between January 2010 and November 2011. OCT scans were performed with spectral-domain OCT (TOPCON OCT 2000). The reference position was changed from the vitreous to the choroid. OCT scan patterns consisted of seven sections; the subfoveal CT was measured manually between Bruchs membrane and the internal portion of the sclera in eyes with CNV and from the pigment epithelium to the scleral interface in eyes without CNV. RESULTS In the subgroup with CNV, the mean subfoveal CT was 51.71 μm ± 17.35. A statistically significant negative correlation was found between CT and AL (r=-0.615, P=0.0001). Regression analysis demonstrated a decrease of 8.4 μm per mm of AL. In the subgroup without CNV, matched with the CNV subgroup by age (P=0.597), and AL (P=0.813), the mean subfoveal CT was 93.35 μm ± 34.81 μm. The difference between the two subgroups was statistically significant (P<10(-4)). DISCUSSION Macular choroidal thickness is reduced in high myopia, especially when complicated by CNV. It has not yet been shown that choroidal thinning may be a risk factor for choroidal neovascularization, but our results may suggest that macular choroidal thinning may lead to hypoxic retinal changes resulting in secretion of VEGF and thus CNV. CONCLUSION Macular choroidal thinning observed in high myopia with CNV. These findings may suggest that choroidal changes may play a role in the pathogenesis of choroidal neovascularization.

712 Tumeur fibreuse solitaire de l’orbite ; une cause rare d’exophtalmie unilatérale : à propos d’une observation

Introduction Initialement decrite au niveau de la plevre, la tumeur fibreuse solitaire est une tumeur mesenchymateuse ubiquitaire exceptionnellement retrouvee dans l’orbite. Materiels et Methodes Cas clinique. Observation Nous presentons l’observation d’un patient âge de 38 ans ayant consulte pour une protrusion du globe oculaire gauche d’evolution tres rapide (deux semaines). L’examen de l’œil gauche a montre une exophtalmie de grade III indolore associee a un chemosis important, une keratite d’exposition avec un ulcere corneen de la moitie inferieure de la cornee, une limitation de la motilite oculaire et une acuite visuelle reduite a la perception lumineuse mal orientee. L’imagerie tomodensitometrique a montre une volumineuse formation intra-conique bien limitee spontanement dense prenant le contraste, englobant le nerf optique evoquant un meningiome ou une pseudotumeur inflammatoire de l’orbite. L’imagerie par resonnance magnetique nucleaire a mis en evidence cette volumineuse masse a double composante kystique et tissulaire, avec un iso signal el T1 et hypo-signal en T2, sans signe de specificite. L’examen anatomopathologique de la piece operatoire obtenue apres exenteration a permis de poser le diagnostic de tumeur fibreuse solitaire de l’orbite. Discussion Le diagnostic de tumeur fibreuse solitaire est anatomopathologique. Il s’agit d’une tumeur de nature mesenchymateuse avec une forte expression de l’antigene CD34 et de la vimentine en immunohistochimie. Conclusion La tumeur fibreuse solitaire de l’orbite est une tumeur rare habituellement benigne. Elle doit etre differenciee immuno-histo-chimiquement des autres types de tumeurs mesenchymateuses de l’orbite. Sa prise en charge repose sur l’exerese chirurgicale complete et un suivi clinique prolonge.

Caractéristiques cliniques et angiographiques de la dystrophie cristalline de Bietti: À propos d’une patiente âgée de 8 ans

Introduction La dystrophie cristalline de Bietti est une degenerescence tapeto-retinienne caracterisee par la presence de petits cristaux refringents au niveau du limbe corneen et de la retine associes a une atrophie de l’epithelium pigmentaire retinien et a une sclerose des vaisseaux choroidiens. Nous rapportons le cas d’une dystrophie cristalline de Bietti survenue chez une fillette âgee de 8 ans. Observation Une fillette, âgee de 8 ans, issue d’un mariage consanguin, nous fut adressee pour un strabisme convergent intermittent. L’acuite visuelle etait reduite a 4/10 e a droite et a 3/10 e a gauche. L’examen biomicroscopique de la cornee au fort grossissement trouvait de petits cristaux refringents sous-epitheliaux prelimbiques bilateraux. L’examen du fond d’œil mit en evidence des alterations de l’epithelium pigmentaire maculaire avec de petits points refringents predominants au pole posterieur et a la moyenne peripherie. L’angiographie retinienne a la fluoresceine confirma les alterations de l’epithelium pigmentaire. L’angiographie au vert d’infracyanine montrait des plages d’atrophie choriocapillaire. Le champ visuel de Goldman trouvait un scotome paracentral bilateral. L’electroretinogramme montrait une reduction du nombre des photorecepteurs affectant aussi bien les cones que les bâtonnets. Discussion Les lesions ophtalmoscopiques dans la dystrophie cristalline de Bietti surviennent generalement entre 20 et 30 ans. La particularite de notre observation est leur apparition a un âge plus precoce (soit a l’âge de 8 ans). L’evolution se caracterise par l’extension centrifuge des lesions.INTRODUCTION Biettis crystalline corneoretinal dystrophy is a tapetoretinal degeneration, characterized by the presence of refringent crystals in the corneal limbus and the retina with sclerosis of choroidal vessels. We report the clinical and angiographic features of an 8-year-old girl affected with Biettis crystalline dystrophy. CASE REPORT This 8-year-old girl was a sporadic case, born of consanguineous parents. She was referred to our hospital for intermittent strabismus. Her visual acuity was 4/10 at the right eye and 3/10 at the left eye. Biomicroscopy revealed very fine crystals in the limbal area bilaterally. Mydriatic funduscopic examination showed bilateral macular pigment mottling and depigmentation, numerous tiny refractile yellow dots scattered throughout the posterior pole and the mid-periphery associated with diffuse retinal pigment epithelial atrophy and pigment accumulation. Fluorescein angiography revealed retinal pigmentary epithelium alterations. Indocyanine green (Infracyanin) angiography showed areas of choroidal atrophy. The electroretinogram noted a reduction in the number of both types of photoreceptors. COMMENTS Ophthalmological lesions normally occur between 20 and 30 years of age. The particularity of our case report is the manifestation of the disease at an earlier age (8 years). The progression is characterized by a centrifuge expansion of lesions.

Corrélations phénotype–génotype de la rétinopathie pigmentaire non syndromique : à propos de dix familles tunisiennes ☆

PURPOSE To evaluate the clinical phenotype of ten Tunisian families with non-syndromic retinitis pigmentosa (RP), to characterize genes and mutations causing these conditions, and to elaborate phenotype-genotype correlations. METHODS Descriptive clinical genetic study of 114 individuals, of whom 27 are affected by non-syndromic RP. Ophthalmic examination and various visual tests were performed. DNA was analyzed using single nucleotide polymorphism, microsatellite genotyping and direct sequencing to determine the genes and mutations involved. RESULTS We identified seven mutated genes: RPE65, RDH12, USHER 2A, PDE6a, PDE6b, CRB1, and NR2E3. Analysis of phenotype-genotype correlation indicated that some genes were associated with specific phenotypes. In RPE65 mutations, we found early onset dystrophy, nystagmus, keratoconus, white dot deposits in earlier stages and clumped pigment in later stages. The RDH12-associated phenotype (juvenile RP) showed severe and early-onset dystrophy, diffuse spicule pigmentation, macular edema and thickening, and tomographic re-organization of retinal layers. The CRB1 mutation was characterized by preserved para-arteriolar retinal pigment epithelium and no hemeralopia. CONCLUSION RP is clinically and genetically heterogeneous. The two ultimate goals of research are to provide efficient clinical diagnostic of affected gene by phenotype-genotype correlation and to design novel treatment regimens. Our goal is to create a specific chip for our population, and then future research will focus on the identification of the remaining causal genes, the elucidation of the molecular mechanisms of disease in the retina and the development of gene therapy approaches.

Aspects tomographiques et en autofluorescence du fond d’œil dans la maladie de Best☆

Best vitelliform macular dystrophy is the second most frequent hereditary maculopathy, with bilateral involvement and juvenile onset. It is clinically characterized by bilateral deposits of lipofuscin-like autofluorescent material in the subretinal space, with a typical phenotypic manifestation taking the form of a vitelliform macular lesion evolving gradually into more advanced stages. The purpose of our study was to describe fundus autofluorescence patterns and OCT findings in three patients (6 eyes) with several stages of Best vitelliform macular dystrophy. Optical coherence tomography (OCT) has become the first imaging technique to order when confronted with a hereditary maculopathy suggesting Best disease. Fundus autofluorescence combined with OCT allow for better diagnosis and management, which are necessary for any genetic analysis.

Retinal dystrophy and congenital glaucoma as major causes of vision loss in students attending two institutions for the visually disabled in Tunis city, Tunisia.

PURPOSE To assess vision loss, identify affected anatomical sites, determine etiologies and potentially avoidable causes in students attending two institutions for the visually disabled in Tunis city. METHODS A visit for a complete ophthalmological examination was performed. All students attending these schools were recruited in our study. The World Health Organisation Programme for the Prevention of Blindness (WHO/PBL) examination record for children was used. Data was analysed by the SPSS version 17 statistical software. RESULTS A total of 172 students were recruited with mean age of 11.9±3.3 years (between 6 and 18 years). One hundred and thirty-seven (79.6%) were under 16 years. The sex-ratio was 1.17. Ninety students (52.3%) had low vision and eighty-two (47.7%) were blind. We reported retina (29%), whole globe (29%), globe appears normal (11%) and optic nerve (9.8%) as the common sites of ocular abnormalities. Retinal dystrophy (22.7%) and congenital glaucoma (22.7%) were the most reported ocular diseases. The main etiologies were hereditary (54.1%) and unknown (30.8%). Consanguinity was reported in 108 students (62.8%), and fifty-five students (32%) had a positive family history. Overall, 50.5% (87/172) of ocular diseases were potentially treatable or preventable. CONCLUSION Retinal dystrophy and congenital glaucoma were the most common eye diseases. Heredity was the main etiology, and consanguinity was high. To decrease their incidence, awareness of the family members of the risks of consanguinous marriage and appropriate therapy for congenital glaucoma/cataract may significantly improve the childs visual prognosis.

652 - Occlusion de l’artère ophtalmique révélant une thrombophlébite cérébrale.

But Rapporter le cas d’un patient ayant presente un syndrome clinique d’occlusion de l’artere ophtalmique gauche revelant une thrombophlebite cerebrale infectieuse compliquant une pansinusite Materiels et Methodes Patient âge de 48 ans, diabetique consulte en urgence pour baisse brutale de l’acuite visuelle et ptosis de l’œil gauche. L’examen revele a cet œil des perceptions lumineuses negatives, une ophtalmoplegie totale, une anesthesie corneenne et un reflexe photomoteur aboli. Le fond d’œil objective un œdeme blanc retinien ischemique etendu avec un retrecissement arteriel diffus et une atrophie optique. L’examen de l’œil droit est sans particularite. L’angiographie a la fluoresceine montre un retard manifeste du remplissage choroidien ainsi qu’un retard de perfusion arterielle. Le scanner orbitocerebral est normal, l’angio-IRM revele une occlusion des arteres ophtalmique et carotide interne gauches ainsi qu’une thrombose des sinus transverse et caverneux. La TDM des sinus de la face montre une pansinusite a predominance sphenoidale. Un drainage du sinus sphenoidal, une antibiotherapie et une heparinotherapie ont ete entrepris entrainant l’amelioration clinique et biologique avec persistance du deficit visuel. Discussion L’occlusion de l’artere ophtalmique implique des repercussions a la fois sur les circulations retinienne et choroidienne avec des consequences fonctionnelles extremement severes. En effet, la perte visuelle est permanente dans la plupart des cas. Conclusion L’occlusion de l’artere ophtalmique est un accident rare mais grave qui impose un bilan etiologique exhaustif afin d’eviter qu’une urgence fonctionnelle visuelle ne se transforme en une urgence vitale.