[Nodular melanoma on primary acquired conjunctival melanosis]. / Mélanome nodulaire sur mélanose acquise primitive de la conjonctive.

A 74-year-old woman consulted for bloody tears. The etiology was a large conjunctival nodular melanoma hidden in the left superior fornix that had developed quietly on an unknown primary acquired melanosis. In this report the clinical and histological features as well as the treatment are presented. A decisional tree summarizes the treatment for conjunctival melanosis.

[Ophthalmology and urban underprivileged. Experience of 150 patients]. / Ophtalmologie et situation de précarité en milieu urbain. À propos d'une expérience de 150 patients.

INTRODUCTION: An ophthalmology consultation was carried out at the Hôtel-Dieu to facilitate eye care in underprivileged patients referred by the general medicine PASS (socialized health care) of the same hospital. MATERIALS AND METHODS: The files of 150 consecutive patients examined by a single ophthalmologist between January 2012 and June 2013 were reviewed. A standard examination was performed in these patients, sent with a consult sheet, to which a response was sent to PASS. As necessary, prescriptions were also written for the patients. RESULTS: Most patients (89/150) were immigrants from sub-Saharan Africa. Additionally, there were 25 Europeans with only 5 French, and 17 Asians. The mean age was 41 years with 90 men (mean age 43) and 60 women (mean age 36). The most common systemic diseases observed were hypertension, (13), diabetes (6), and hepatitis (6). The mean best-corrected visual acuity (129 patients) was a slightly more than 8/10 (16/20) (measurable in 143 patients). Refractive state (measured in 129 patients) was myopia (46), hyperopia (41), presbyopia (isolated in 12 cases), astigmatism (26), and emmetropia (28). Optical correction was prescribed in 87 patients. The main pathological conditions included pingueculae (19), cataracts (19), trauma (13) of which 4 patients were monocular, and pterygia (9). DISCUSSION AND CONCLUSION: Refractive errors were the main abnormality observed in these underprivileged patients. Apart from cataract as a pathologic condition observed in the general population, more characteristic of this sub-Saharan population were pinguecula, pterygium and trauma. This study highlights the more general question of access to eye care for all underprivileged patients.

Decreased intraocular pressure induced by nitric oxide donors is correlated to nitrite production in the rabbit eye.

PURPOSE: To evaluate the effect of intraocular administration of nitric oxide (NO) donors in the rabbit eye on intraocular pressure (IOP), inflammation, and toxicity. METHODS: Intravitreal and intracameral injections of two NO donors, SIN-1 and SNAP, and SIN-1C and BSS were performed. Clinical examination, IOP measurements, protein evaluation in aqueous humor, and histologic analysis of the ocular globes were realized. Nitric oxide release was demonstrated by nitrite production in the aqueous humor and in the vitreous using the Griess reaction. RESULTS: The drastic decrease of IOP, observed after a single NO donor injection, was correlated directly with nitrite production and, thus, to NO release. Injection of inactive metabolite of SIN-1, SIN-1C, which is not able to release NO, did not modulate IOP. When administered in the aqueous humor or in the vitreous, NO did not diffuse from one segment of the eye to another. No inflammation or histologic damage was observed as a result of a single NO donor administration. CONCLUSIONS: Nitric oxide is implicated directly in the regulation of IOP and its acute, and massive release into the rabbit eye did not induce inflammation or other growth toxic effects on the ocular tissues.

A new poly(ortho ester)-based drug delivery system as an adjunct treatment in filtering surgery.

PURPOSE: Pharmacologic modulation of wound healing after glaucoma filtering surgery remains a major clinical challenge in ophthalmology. Poly(ortho ester) (POE) is a bioerodible and biocompatible viscous polymer potentially useful as a sustained drug delivery system that allows the frequency of intraocular injections to be reduced. The purpose of this study was to determine the efficacy of POE containing a precise amount of 5-fluorouracil (5-FU) in an experimental model of filtering surgery in the rabbit. METHODS: Trabeculectomy was performed in pigmented rabbit eyes. An ointmentlike formulation of POE containing 1% wt/wt 5-FU was injected subconjunctivally at the site of surgery, during the procedure. Intraocular pressure (IOP), bleb persistence, and ocular inflammatory reaction were monitored until postoperative day 30. Quantitative analysis of 5-FU was performed in the anterior chamber. Histologic analysis was used to assess the appearance of the filtering fistula and the polymer's biocompatibility. RESULTS: The decrease in IOP from baseline and the persistence of the filtering bleb were significantly more marked in the 5-FU-treated eyes during postoperative days 9 through 28. Corneal toxicity triggered by 5-FU was significantly lower in the group that received 5-FU in POE compared with a 5-FU tamponade. Histopathologic evaluation showed that POE was well tolerated, and no fibrosis occurred in eyes treated with POE containing 5-FU. CONCLUSIONS: In this rabbit model of trabeculectomy, the formulation based on POE and containing a precise amount of 5-FU reduced IOP and prolonged bleb persistence in a way similar to the conventional method of a 5-FU tamponade, while significantly reducing 5-FU toxicity.

In vivo inhibition of lens regrowth by fibroblast growth factor 2-saporin.

PURPOSE: To investigate the ability of fibroblast growth factor (FGF) 2-saporin to prevent lens regrowth in the rabbit. METHODS: Chemically conjugated and genetically fused FGF2-saporin (made in Escherichia coli) were used. Extracapsular extraction of the lens was performed on the rabbit, and the cytotoxin either was injected directly into the capsule bag or was administered by FGF2-saporin-coated, heparin surface-modified (HSM) polymethylmethacrylate intraocular lenses. The potential of the conjugate was checked by slit lamp evaluation of capsular opacification and by measuring crystallin synthesis. Toxin diffusion and sites of toxin binding were assessed by immunohistochemistry. Possible toxicity was determined by histologic analysis of ocular tissues. RESULTS: FGF2-saporin effectively inhibited lens regrowth when it was injected directly into the capsular bag. However, high concentration of the toxin induced transient corneal edema and loss of pigment in the iris. Intraocular lenses coated with FGF2-saporin reduced lens regrowth and crystallin synthesis without any detectable clinical side effect. After implantation, FGF2-saporin was shown to have bound to the capsules and, to a lesser extent, to the iris; no histologic damage was found on ocular tissues as a result of implantation of drug-loaded HSM intraocular lenses. CONCLUSIONS: Chemically conjugated (FGF2-SAP) and genetically fused FGF2-saporin (rFGF2-SAP) bound to HSM intraocular lenses can prevent lens regrowth in the rabbit.

A preliminary comparative treatment planning study for radiotherapy of age-related maculopathy.

PURPOSE: We present a comparative planning of different approaches for external radiotherapy in age-related maculopathies. MATERIALS AND METHODS: Calculated dose distributions and dose-volume histograms for (a) bilateral irradiation with 6 MV photons, (b) a single lateral-oblique beam using either photons, electrons or protons and (c) an anterior circular proton beam. RESULTS: For lateral photon or electron beams the dose to the lens is usually lower than 10% of the dose to the macula. The entrance doses for bilateral photon beams are about 50% which increase up to 100% at the orbital bone. About 5 mm of optic nerves are irradiated at the maximal dose while the optic chiasma is spared. A single photon beam gives 50% of the dose to the fellow eye. The electron beam spares the fellow eye but gives a rather inhomogeneous dose to the target volume. For a lateral proton beam, 4 mm of optic nerve receives 90% of the dose, the skin dose is at least 70% of the dose to the macula and the lens and the fellow eye are spared. An anterior proton beam gives 90% of the dose to 1 mm of optic nerve and the 50% isodose approaches the periphery of the lens. CONCLUSION: Doses to the critical structures can be dramatically diminished for all the techniques by reducing the beam size, but only if very precise set-up techniques are used. Proton beams are an attractive solution, but the impact of such a choice on the use of proton facilities and on the national health system should be carefully evaluated, as well as the risk of radio-induced secondary neoplasias.

A novel variant of granular corneal dystrophy caused by association of 2 mutations in the TGFBI gene-R124L and DeltaT125-DeltaE126.

OBJECTIVE: To characterize the molecular defect in the TGFBI gene in a French family affected with an atypical granular corneal dystrophy. PATIENTS: This family comprises 9 affected individuals across 3 generations without consanguineous marriage. METHODS: Light and electron microscopy were used to examine corneal buttons from patients. Exons of the TGFBI gene were amplified by polymerase chain reaction and sequenced directly using an automated method. Restriction digestion analysis and heteroduplex screening were performed to confirm that the mutations identified were not polymorphisms. RESULTS: Round or snow-flakes-like deposits that stained red with Masson trichrome and appeared as dense, rod-shaped structures were observed in the most anterior layers of the central stroma. All patients were heterozygous for the R124L mutation and a novel mutation predicting the deletion of 2 amino acid residues-threonine (T) and glutamic acid (E)-at codons 125 and 126. CONCLUSIONS: This French family is affected with a novel variant of granular dystrophy that is caused by a molecular defect in the TGFBI gene, reported here for the first time. CLINICAL RELEVANCE: These 2 mutations cause a novel variant of granular dystrophy that is intermediate in severity between the classical and superficial variant forms. Arch Ophthalmol. 2000;118:814-818

Lack of correlation between the location of choroidal melanoma and ultraviolet-radiation dose distribution.

Ocular melanomas arise from the choroid. The results of our study of a total of 92 ocular melanomas would indicate that there is no preferential location for tumors on the eye. We estimated the ultraviolet (UV) radiation dose distribution using data available in the literature. We then compared tumor location and UV-radiation dose distribution. UVC and UVB do not reach the choroid, and UVA is filtered by the cornea and the lens. Only a small percentage of the incoming rays reach the posterior and inferior part of the retina, but none reach the superior and anterior part of the eye. We concluded that it is therefore very unlikely that UV-radiation exposure is responsible for choroidal melanoma.

Neotropical echinococcosis in Suriname: Echinococcus oligarthrus in the orbit and Echinococcus vogeli in the abdomen.

This paper reports two cases of neotropical echinococcosis caused by Echinococcus oligarthrus and E. vogeli, neither of which has been reported from Suriname. Case 1, a six-year-old boy, presented a 15 x 25 mm retro-ocular cystic tumor (observed by ultrasound, computed tomography scan, and magnetic resonance imaging) causing exophthalmia, chemosis, palpebral ptosis, and blindness of the left eye. Of two tentative diagnoses, Echinococcus cyst or dermoid tumor, the former was shown to be correct at surgery when a clear liquid and detached protoscoleces were aspirated. Rostellar hooks of the protoscolex were characteristic of E. oligarthrus. Case 2, a 41-year-old man, had polycystic masses excised from the liver and abdomen. A presurgery diagnosis of E. vogeli infection was made due to calcifications seen in the lesions, positive serology, residence of the patient in the tropical forest, and later by the size and shape of rostellar hooks. The presence of these two parasites in one of the former Guianas is not surprising; both species are endemic in tropical forest in Central and South America wherever people have not exterminated wild canids, especially the bush dog, (Speothos venaticus), and felids (wild cats of several species), along with pacas, agoutis, and other rodents that serve as intermediate host of these two cestodes. Eighty-six cases of polycystic echinococcosis are known in people from 11 countries from Nicaragua to Argentina: 32 due to E. vogeli, three to E. oligarthrus, and 51 for which determination of the species was not possible because the hooks of the protoscolex were not found or described. Research to elucidate aspects of transmission of E. vogeli and E. oligarthrus is of practical importance for defining measures for preventing the severe and frequently fatal illnesses caused by these two cestodes.

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.

Intraocular inflammation after proton beam irradiation for uveal melanoma.

AIM: To describe the inflammatory reaction that can occur following proton beam irradiation of uveal melanomas based on a large series of patients and to try to determine the risk factors for this reaction. METHODS: Data from a cohort of patients with uveal melanoma treated by proton beam irradiation between 1991 and 1994 were analysed. The presence of inflammation was recorded and evaluated. Kaplan-Meier estimates and statistical analysis of general and tumour related risk factors were performed. RESULTS: 28% of patients treated during this period presented with ocular inflammation (median follow up 62 months). Risks factors were essentially tumour related and were correlated with larger lesions (height > 5 mm, diameter > 12 mm, volume > 0.4 cm(3)). Multivariate analysis identified initial tumour height and irradiation of a large volume of the eye as the two most important risk factors. Ocular inflammation usually consisted of mild anterior uveitis, resolving rapidly after topical steroids and cycloplegics. The incidence of inflammation after proton beam irradiation of melanomas seems higher than previously reported and is related to larger lesions. Evidence of inflammation associated with uveal melanoma has been described and seems to be associated with tumour necrosis (spontaneous or after irradiation). The appearance of transient inflammation during the follow up of these patients may be related to the release of inflammatory cytokines during tumour necrosis. CONCLUSION: Inflammation following proton beam irradiation is not unusual. It is correlated with larger initial tumours and may be related to tumour necrosis.

Acute corneal edema in pellucid marginal degeneration or acute marginal keratoconus.

This article reports a case of bilateral corneal pellucid marginal degeneration. The right cornea had an acute hydrops. Both eyes underwent penetrating keratoplasty. A histopathological study of the corneal specimens was performed by light and electron microscopy. The histological changes observed on the right cornea showed breaks on Bowman's layer, edema and disorganization of the stromal collagen, and break of Descemet's membrane. The ultrastructural changes were similar to those observed in acute keratoconus, leading to the belief that these two corneal diseases are closely related.

A new fluorocarbon for keratoprosthesis.

Previous studies have demonstrated the potential use of microporous, biocompatible materials to improve the long-term stability of keratoprosthesis. To determine the factors that will influence corneal tissue ingrowth into biocompatible, microporous materials, we have compared three types of fluorocarbon polymers--Impra, Gore-Tex, and Proplast--after intrastromal implantation in rabbit corneas. Despite similar physicochemical structures, a great difference was observed in histologic and ultrastructural cross sections after 4- and 8-month follow-ups. For Gore-Tex, we observed extrusion of the implant and infiltration of necrotic and inflammatory cells. All implants of Proplast also led to significant corneal damage resulting in extrusion of the material. Through the use of electron and light microscopy and image analysis, this study demonstrates the presence of cell differentiation and collagen synthesis in the pores of the Impra implant. Apart from biocompatibility, this experiment demonstrates the influence of pore size, porous microorganization, and biomechanical factors on prosthetic corneal material. Only Impra offers satisfactory interface, allowing fibroblastic cells and neocollagen synthesis into its pores, and it can become transparent.

Nineteen years of penetrating keratoplasty in the Hotel-Dieu Hospital in Paris.

PURPOSE: To identify changing trends in penetrating keratoplasties (PKs) performed at the Hotel-Dieu Hospital in Paris between January 1980 and December 1999 and to explain the reasons for the changes. METHODS: We retrospectively reviewed 3,736 of the 3,836 PKs performed between January 1, 1980, and December 31, 1999, and classified them into diagnostic categories. RESULTS: The most common indications for PK were keratoconus (28.8%), herpetic infections (10.9%), graft failures (9.9%), aphakic and pseudophakic corneal edema (9.9%), Fuchs' endothelial dystrophy (9.4%), and nonherpetic leucoma (7.7%). Other indications represented 23.4% of the cases. The incidence of aphakic and pseudophakic corneal edema progressively increased between 1980 and 1991, became the most frequent indication in 1991 (21.4%), and then progressively decreased. The annual number of PKs increased between 1980 and 1986, decreased between 1987 and 1997, and increased again after September 1997. The decrease was caused by both a shortage of corneal buttons, and, in 1987, the fear of transmitting diseases through corneal transplantation, particularly human immunodeficiency virus. Beginning in 1992, decreases were also associated with stringent governmental regulations of eye bank tissue. CONCLUSION: Changes in the incidence and management of corneal disorders were the primary factors leading to modifications of grafting until 1987. After 1987, corneal button shortage probably corresponded to the acquired immune deficiency syndrome epidemic. Governmental regulations of eye banking led to a severe corneal button shortage between 1992 and 1997. Despite an increase in the number of PKs performed after 1997, corneal buttons are still preferentially allocated to patients in whom there is a high probability of graft success.

Blue light dose distribution and retinitis pigmentosa visual field defects: an hypothesis.

The hypothesis that retinitis pigmentosa (RP) is worsened by blue light has been raised a century ago. In order to check this hypothesis we calculated the theoretical dose distribution of light on the retinal surface. The relative dose to the different parts of the retina was calculated using a Monte-Carlo method. The changes in the peripheral isopters were calculated both degrading at a constant rate and degrading proportionally to light exposure. There is a considerably greater exposure to the superior compared to the inferior visual field. The maximum dose of UV and blue light is located on the superior field about 4mm above the macula. The dose received by the peripheral retina is markedly lower than the dose received by the central retina. The visual field defects most commonly described in RP are concentric, centered by the macula. These defects cannot, therefore, be explained by the impact of light on the retina. But some regional form of RP with a superior field defect can result from an abnormal genetically encoded sensitivity to ultraviolet and blue light.

[Initial results of proton therapy in choroidal melanoma at the d'Orsey Center for Proton Therapy; the first 464 cases]. / Résultats préliminaires de la protonthérapie du mélanome de la choroïde au centre de protonthérapie d'Orsay (CPO): les 464 premiers cas.

PURPOSE: Retrospective analysis of the treatment of choroidal melanoma with protontherapy at the Centre de protonthérapie d'Orsay, France. PATIENTS AND METHODS: Between September 1991 and September 1995, 612 patients presenting with choroidal melanoma were treated by protontherapy in Orsay. Following initial management of the first 464 patients, results were analyzed, as were results after a 1-year follow-up for 305 patients, a 2-year follow-up for 169 patients, and a 3-year follow-up for 59 patients. RESULTS: Univariate analysis showed that the actuarial local recurrence rate was 5%, the 3-year survival rate 88%, and the overall metastasic rate 5%. The initial tumor volume was the most significant predictive factor for visual results and metastases. Multivariate analysis revealed that visual results were significantly related to the initial tumor volume, initial retinal detachment, and total dose delivered to the optic nerve and macula. CONCLUSION: Protontherapy of choroidal melanoma allows in most cases conservation of the eye without modification of survival. Visual results mainly depend on the site and size of the tumor.

[Macroscopic study of choroidal and ciliary body melanoma]. / La macroscopie du mélanome du corps ciliaire et/ou de la choroïde.

Uveal melanoma mainly affects the choroid and to a lesser extent the ciliary body. Its clinical presentation, as well as the angiographic and microscopic features are often reported. Its macroscopic appearance is less often reported in publications, however. The macroscopic study of these tumors provides useful information and a series of photographs illustrating the macroscopic study of these tumors was gathered to be shown to ophthalmologists who have little experience with these intraocular tumors.

[Histiocytofibroma of the sclerocorneal limbus]. / Histiocytofibrome du limbe scléro-cornéen.

A case of epibulbar benign fibrous histiocytoma is presented, with an optical microscopic study. A greyish vascularised nodule developed at the corneoscleral limbus in a 65 year-old male. The clinical diagnosis was confirmed by histopathologic evaluation. Complete surgical excision had a curative effect.

[Orbital dysembryoplastic cyst at the conjunctival wall]. / Kyste dysembryoplasique de l'orbite à revêtement conjonctival.

An 18-year-old female patient developed a mass in the superomedial angle of her left orbit. After evaluation, this tumor was surgically removed. A dysembryoplastic cyst lined with conjunctival epithelium was diagnosed by light microscopic examination. Clinical and pathological data are detailed. The characteristics of dysembryoplastic orbital cysts are reviewed.

[Differential diagnosis of conjunctival melanoma: exteriorized uveal melanoma]. / Diagnostic différentiel du mélanome conjonctival: mélanome uvéal extériorisé.

Four years after unexplained left eye visual loss and 15 days after surgical procedure on the other eye, a 93-year-old female developed a pigmented left conjunctival lesion. Ultrasound examination demonstrated this lesion to be in fact an extraocular extension of uveal melanoma. The patient was treated by external radiotherapy after expanded enucleation.