[Atypical optic neuritis: the importance of a comprehensive diagnostic work-up]. / Atypische Neuritis nervi optici (NNO): Bedeutung einer umfassenden Abklärung.

A 65-year-old woman was referred for sudden bilateral loss of vision. She was vaccinated a few days earlier. The ophthalmological examination showed a massive optic disc swelling on both eyes. Magnetic resonance imaging (MRI) revealed suspected bilateral optic neuritis without cerebral involvement or transverse myelitis. After serological detection of anti-MOG (myelin oligodendrocyte glycoprotein) antibodies, the patient was treated with high-dose corticosteroid pulse therapy until vision recovered. Discussion: an atypical optic neuritis may indicate a neuromyelitis optica spectrum disorder (NMOSD), which should be further characterized by determination of Aquaporin 4(AQP4)-IgG and MOG-IgG.

[Floating membranes]. / Les membranes "flottantes."

INTRODUCTION: Floating membranes are epiretinal membranes that spontaneously detach from the retina and float in the vitreous cavity. PATIENTS AND METHOD: We studied a series of nine patients with a spontaneous separation of a premacular membrane. The majority of these detached membranes were secondary to vitreomacular traction syndrome. In four patients, the presence of peripheral retinal tears treated in the past by laser photocoagulation were found. None of the patients had complete vitreoretinal detachment before progressing to floating membranes. RESULTS: The separation of the membrane from the macular area occurred at the time of posterior vitreous detachment. The appearance of a bulky intravitreous floating body attached to the posterior vitreous cortex sometimes proved to be very awkward for vision, resulting in a vitrectomy in four patients for ablation of this large floating body. In five patients who were not operated, the presence of the membrane in the vitreous cavity was not responsible for significant functional disorders. DISCUSSION: The epimacular membranes concerned in this series were not very symptomatic before their detachment. They seemed to be integrated within the context of vitreomacular traction syndrome. The most invalidating cases that required surgery were those where the separation of the vitreous cortex remained incomplete, leaving an opaque membrane floating with a little mobility in front of the macular area (three out of four cases). CONCLUSION: The spontaneous detachment of a premacular membrane is an event that is quite seldom observed and generally follows the spontaneously favorable progression of vitreomacular traction syndrome.

Multinucleated giant cells in periretinal silicone granulomas are associated with progressive proliferative vitreoretinopathy.

PURPOSE: To determine the histologic features of granulomatous reactions in persilicone periretinal proliferation. PATIENTS AND METHODS: This retrospective study included 12 patients with recurrent retinal detachment and persilicone granulomatous proliferation after vitrectomy for proliferative vitreoretinopathy (PVR). All patients underwent reoperation for membrane surgery. Immunohistochemical study of the excised periretinal membranes was performed with cytokeratins, GFAP, vimentin, CD68, CD45, and lysozyme antibodies. RESULTS: The cellular characteristics of periretinal granulomas allow differentiation of two types of tissue. Spongy tissue (nine cases) showed an accumulation of mature vacuolated macrophages that contained silicone without multinucleated giant cells (MGC). The second type (three cases) consisted of an accumulation of sparsely vacuolated macrophages, epithelioid cells, and MGC. The MGC corresponded to transition forms of foreign body giant cells (FBGC). Spongy tissue was associated with anatomic success (58.3% of cases) and with stabilized PVR (66.7% of cases) at the time of the membrane surgery. MGC were associated with prolonged silicone oil tamponade, recurrent retinal detachment, and progressive PVR. CONCLUSIONS: Intraocular silicone oil can lead to periretinal foreign body granulomas. FBGC are occasionally observed and were associated with progressive PVR.

[Optic disk neovascularization in fungal endophthalmitis]. / Néovascularisation prépapillaire au cours d'une endophtalmie fongique présumée chez une toxicomane.

A case of optic disk neovascularization is reported in a 30-year-old female heroin addict with suspected fungal endophthalmitis treated with an intravitreal injection of amphotericin B and fluconazole (200mg/day). Membrane healing was characterized by densification and contraction leading to tractional retinal detachment; excision by vitrectomy was required. Electron microscopy analysis of the membrane removed the tenth month revealed a poorly vascularized fibrous tissue infiltrated by chronic inflammatory cells. Visual improvement was poor owing to a postoperative macular disorder and a full-thickness retinal scar.

[Vitreous changes in Stickler syndrome]. / Modifications vitréennes au cours d'un syndrome de Stickler.

We report the ultrastructural feature of a vitreous membrane with multiple fenestrations in a case of a 40-year-old woman with a Stickler syndrome. The left eye showed a type 2 vitreous phenotype. Vitreous abnormalities of the right eye may correspond to phenotypic conversion to a type 1 appearance of a type 2 vitreous phenotype. Fenestrated membrane may correspond to posterior hyaloid membrane in complete posterior vitreous detachment in this phenotypic conversion. Fenestrated membrane consisted of avascular fibrocellular tissue with cells arranged in a cohesive pattern around the fenestration. Ultrastructural findings of cells were characteristic of proliferative Müller cells. Ultrastructural examination of collagen fibrils showed them to be similar to normal vitreous. This finding suggests that collagen molecules are not functionally altered and are probably quantitatively insufficient during vitreous development.

Longterm survival in acute rhinocerebral mucormycosis with giant cell arteritis and foreign body granulomas.

A case of rhinocerebral mucormycosis occurring in a 41-year-old man with insulin-treated diabetes mellitus is reported. Microscopically, biopsy samples obtained from the left ethmoid and middle turbinate sinuses contained fungi that formed mycotic granulomas associated with multinucleate giant cell arteritis. The multinucleate giant cells contained broad, infrequently septate hyphase consistent with mucormycosis. The patient received surgical debridement with extenteration of the left orbit, and intravenous liposome-encapsulated amphotericin B. After 12 months, examination of the patient revealed complete healing. Multinucleate giant cell granulomas and arteritis are only exceptionally associated with rhinocerebral mucormycosis, but these histologic findings may be correlated with a progressive disease with better prognosis.

[Ultrastructural features of peripheral choroidal neovascular membranes in age-related macular degeneration]. / Caractéristiques ultrastructurales de la périphérie des membranes néovasculaires choroïdiennes dans la dégénérescence maculaire liée à l'âge.

PURPOSE: Some neovascular membranes comprise two parts: a fibro - vascular central body and an avascular marginal component. Certain authors have suggested that this avascular part could be involved in post laser treatment recurrence of subfoveal membranes. The purpose of this study was to determine the ultrastructural features of the marginal avascular component. METHODS: Four well-defined subfoveal neovascular membranes associated with age-related macular degeneration were surgically excised. The peripheral portion of each membrane was evaluated by transmission electron microscopy for cellular and extracellular constituents. RESULTS: Each subretinal neovascular membrane was composed of two regions demonstrated by fluorescein angiography: a central area of hyperfluorescent leakage and an annular rim of hypofluorescence. The edge of the central body corresponded to a richly neovascularized tissue and was surfaced by a hyperplasic retinal pigmented epithelium. The rim was composed of a layer of retinal pigmented epithelium and a layer of photoreceptor outer segments associated with macrophages. The stroma was composed of fibrin and was avascular and acellular. DISCUSSION: Neovascular removal of membrane was surgically removed as well as native retinal pigmented epithelium beyond the surface of fibrovascular central body which could explain why post operative scar surfaces were larger than to the initial preoperative membrane defined by fluorescein angiography. CONCLUSIONS: The peripheral rim of neovascular membrane is an exsudative complication of the outer epithelium made up fibrin, native retinal pigmented epithelium, and photoreceptor outer segments. The front of the active neovascularization is localized in the periphery of the central fibrovascular body.

[Ultrastructural findings of epimacular membrane and detached posterior hyaloid in vitreomacular traction syndrome]. / Aspect ultrastructural des membranes épi-maculaires et de la hyaloïde postérieure décollée dans le syndrome de traction vitréo-maculaire.

PURPOSE: Vitreomacular traction syndrome is characterized by an incomplete posterior vitreous detachment and by the cortical vitreous remaining attached to the macula. In certain case epimacular membrane is clinically well defined. We studied the electron microscopic features of epiretinal tissue and posterior hyaloid removed from the posterior pole. MATERIAL AND METHODS: [corrected] Six epimacular membranes were removed during a pars plana vitrectomy, placed in 2.5% glutaraldehyde solution, embedded in Epoxy resin, and examined in a transmission electron microscope. The detached posterior hyaloid was removed and analyzed separately in two cases. RESULTS: The analysis disclosed five morphologically cell types. Fibrous astrocytes were predominant in five cases. Other cell types were less frequent and were identified as fibroblasts, macrophages and myofibroblasts. In one case epithelial-like cell type was predominant. The stroma was mainly composed of a thin layer of 15-nm diameter collagen evoking a vitreous origin. Internal limiting membrane was present in one case only. Detached posterior hyaloid was constituted by the same cellular constituents on thin layer of vitreous collagen. DISCUSSION: The cellular proliferation is principally constituted by fibrous astrocyte and occurs on the inner surface of cortical vitreous. Some glial cells seem to present an epithelial differentiation. CONCLUSION: The attached and detached posterior hyaloid in vitreomacular traction syndrome is the place of glial cells migration and proliferation.

[Pigmentosum retinis and tubulo-interstitial nephronophtisis in Sensenbrenner syndrome: a case report]. / Rétinopathie pigmentaire et néphropathie tubulo-interstitielle lors du syndrome de Sensenbrenner.

PURPOSE: Sensenbrenner syndrome or cranio-ectodermal dysplasia is an extremely rare autosomal recessive condition (12 cases reported in literature). Our observation shows the possibility of both ocular and renal involvement associated with cranio-ectodermal abnormalities. PATIENTS: and method:We report the case of a girl who presented a typical cranio-ectodermal syndrome with dolicocephaly, short thorax, short limbs, short fingers and teeth abnormalities. At five years, she was found to have pigmentosum retinitis with amblyopy and moderate hyperopia. A chronic renal failure with uncontrollable hypertension underwent a cadaveric-donor transplantation at the age of six years. RESULTS: Two years later, the pigmentosum retinitis was stable. The kidney histology revealed a tubulo-interstitial nephronophtisis. The molecular analysis of the NPH 1 locus, which was associated with nephronophtisis, was negative. DISCUSSION: Our observation and two recent publications have in common ocular and renal abnormalities associated with cranio-ectodermal dysplasia. The underlying genetic defect would involve not only morphogenesis but also development and maturation of organs as eye and kidney. Sensenbrenner syndrome would thus be similar to certain disorders affecting the eye, kidney, skeleton and ectodermal structures such as the EEM, Senior-Loken, Mainzer-Saldino, and Jeune syndromes. CONCLUSION: The retinal dystrophy falls within the spectrum of clinical and genetic forms of pigmentosum retinitis. Our observation would confirm possible links between Sensenbrenner syndrome and oculorenal syndromes.

[Diathermic scleral retraction]. / Le raccourcissement scléral par diathermie.

Diathermic scleral retraction appears to be an efficient technique, with little aggressivity, allowing both scleral indentation and radial and circumferential shortening of the ocular wall. We compared experimental results of a diathermic technique with those obtained by other authors using infrared laser scleral photocoagulation. The large number of variables, especially IOP, complicates standardization, but the results of the two methods on cadaver eyes were found to be quite similar. Diathermic scleral retraction creates a buckle effect and a 10 to 30% scleral shortening. This retractile effect decreases with rising IOP. This method would appear to have therapeutic interest for the management of retinal detachments with anterior proliferation and for the treatment of residual detachments after prolonged tamponade with silicon oil. These techniques of scleral shortening necessitate other experimental studies to evaluate their effects in the long-term, but the absence of induced chorioepithelial lesions would indicate therapeutic applications.

[Retinitis pigmentosa and vitreo-macular traction syndrome. An anatomo-clinical and histopathological study apropos of a case]. / Rétinopathie pigmentaire et syndrome de traction vitréo-maculaire. Etude anatomo-clinique et histopathologique à propos d'un cas.

PURPOSE: A case of congenital epiretinal membrane associated with retinitis pigmentosa is reported. PATIENT: A 30 year old man with retinitis pigmentosa was operated for a vitreomacular traction syndrome. The epiretinal membrane removed during surgical procedure was analyzed in electronic microscopy. RESULTS: Visual acuity, 6 months after the intervention: improve at 20/400, because of amblyopia. The study in electronic microscopy reveals many fibroblasts in an abundant collagenic tissue without glial cells. DISCUSSION: The observation of such an epiretinal membrane is unusual during retinitis pigmentosa. Its origin is probably congenital and the vitreous may play some role in its evolution. The systematic examination of the vitreoretinal interface represents an important element of the surveillance of retinitis pigmentosa.

[Course of epithelial scars after surgical removal of small-size subfoveal neovessels]. / Evolution des cicatrices épithéliales après ablation chirurgicale des néovaisseaux rétrofovéolaires de petite taille.

PURPOSE: We analyzed outcomes of 18 subfoveal neovascular membrane scars after surgery removal in age related macular degeneration. Surface and aspect of pigment epithelial defects were evaluated on angiographic data. RESULTS: We differentiated glial scars and atrophic scars, the most numerous. Scar surfaces were larger compared to the initial preoperative lesions. Central epithelial pigments in scars disappeared in the three months following surgery. After on year, scar surfaces moderately enlarged. DISCUSSION: Ideal approach for retinotomy as well as clinical aspects have to be well evaluated in subfoveal neovascular surgical removal. The angiographic mask effect of the avascular ring surrounding the neovessel is sometime detectable; the approximative scar surface is by this fact more predictable. CONCLUSION: Iatrogenic damages of surgery and postoperative inflammatory lesions must be considered before when a surgical removal of neovascular membranes is suggested.