[Management of ocular toxoplasmosis in France: Results of a modified Delphi study]. / Prise en charge de la toxoplasmose oculaire en France : résultats d'une étude Delphi modifiée.

OBJECTIVE: To evaluate diagnostic and therapeutic practices and then establish a consensus on the management of ocular toxoplasmosis in France through a Delphi study. MATERIALS AND METHODS: Twenty-three French experts in ocular toxoplasmosis were invited to respond to a modified Delphi study conducted online, in the form of two questionnaires, in an attempt to establish a consensus on the diagnosis and management of this pathology. The threshold for identical responses to reach consensus was set at 70 %. RESULTS: The responses of 19 experts out of the 23 selected were obtained on the first questionnaire and 16 experts on the second. The main elements agreed upon by the experts were to treat patients with a decrease in visual acuity or an infectious focus within the posterior pole, to treat peripheral lesions only in the presence of significant inflammation, the prescription of first-line treatment with pyrimethamine-azithromycin, the use of corticosteroid therapy after a period of 24 to 48hours, the prophylaxis of frequent recurrences (more than 2 episodes per year) with trimethoprim-sulfamethoxazole as well as the implementation of prophylactic treatment of recurrences in immunocompromised patients. On the other hand, no consensus emerged with regard to the examinations to be carried out for the etiological diagnosis (anterior chamber paracentesis, fluorescein angiography, serology, etc.), second-line treatment (in the case of failure of first-line treatment), or treatment of peripheral foci. CONCLUSION: This study lays the foundations for possible randomized scientific studies to be conducted to clarify the management of ocular toxoplasmosis, on the one hand to confirm consensual clinical practices and on the other hand to guide practices for which no formal consensus has been demonstrated.

Prevalence of macular complications associated with high myopia by multimodal imaging.

PURPOSE: To describe the prevalence of macular complications in patients with visual acuity decrease related to high myopia (HM). To establish correlations between these complications and demographic or anatomical characteristics. MATERIALS AND METHODS: Cross-sectional observational study including HM patients undergoing best-corrected visual acuity (BCVA), fundus examination, macular SD-OCT, and fluorescein angiography in the case of suspicion of choroidal neovascularization (CNV). The presence of anatomical criteria (staphyloma, subfoveal choroidal thickness [CT]) and macular complications (CNV, lacquer cracks, central chorioretinal atrophy, dome-shaped macula with serous retinal detachment [SRD], retinal foveoschisis, macular hole and epiretinal membrane) was investigated. RESULTS: A total of 87 eyes of 47 patients were included (39 eyes without macular complication and 48 eyes with macular complications). In the case of macular complications, decrease in BCVA was related to CNV in 33%, macular hole in 25%, chorioretinal atrophy in 19%, foveoschisis in 11%, lacquer crack in 6%, to a dome-shape macula with serous retinal detachment in 4% and epiretinal membrane in 2%. After adjusting for interocular correlation and degree of myopia, staphyloma (P=0.0023), choroidal thinning (P=0.0036), and extrafoveal chorioretinal atrophy (P=0.042) were significantly associated with macular complications. CONCLUSIONS: High myopic patients with staphyloma or choroidal thinning should undergo regular comprehensive retinal screening for retinal complications.

[Dragged disc syndrome in a patient presenting neurofibromatosis type II: a case study]. / Syndrome d'attraction papillaire chez une patiente présentant une neurofibromatose de type II.

We report a case of dragged disc syndrome associated with neurofibromatosis type II (NF2). A 5-year-old girl with amblyopia (right eye, 20/200; left eye, 20/40) presented with dragged disk syndrome and a posterior subcapsular cataract in the left eye associated with an epiretinal membrane in the right eye. Five years later, she developed exophthalmos in the right eye associated with a cervical mass. MRI revealed schwannoma developing in the left optic nerve sheath associated with a cervicothoracic schwannoma. The child was diagnosed with NF2. Association of posterior subcapsular cataract with macular epiretinal membranes is highly suggestive of NF2, although the dragged disk syndrome has not been previously reported in NF2 to our knowledge. It may also be an ocular feature of this disease.

[Childhood Leber hereditary optic neuropathy. A case of a 6-year-old girl with loss of vision]. / Neuropathie optique de Leber de l'enfance. A propos d'un cas de baisse d'acuité visuelle chez une enfant de 6 ans.

Leber hereditary optic neuropathy, also known as hereditary optical atrophy, is passed on by the mother. It involves a papilledema and mostly affects young people. It is a mitochondrial DNA disease due to the mutation of the NADH dehydrogenase enzyme. Mutations are not sufficient to cause visual loss. Indeed, some individuals accommodate with mutations but do not express the disease. The heteroplasmic or homoplasmic character of the mutation among patients explains why they develop the disease or not even though they carry the mutation. Symptomatology starts with a central vision loss: a painless scotoma. The disease can progress either toward progressive vision loss, blindness, or spontaneous improvement. We report the rare case of a 6-year-old girl presenting a decline in central visual acuity. The genetic study revealed the presence of the G11778A primary mutation. The physiopathogenic mechanism, the genetic hypothesis of the female form, diagnostic means, and treatment options will be discussed.

[Atypical Cogan syndrome]. / Syndrome de Cogan atypique.

Atypical Cogan syndrome is a systemic vascular disease close to Cogan syndrome. It is characterized by different ocular manifestations associated with Ménière-like audiovestibulatory dysfunction with symptoms arising more than 2 years before or after the ocular abnormalities. We report the case of a 63-year-old woman whose ocular manifestations involved alternating scleritis, myositis and uveitis associated with audiovestibulatory signs, and the onset of ocular muscle and nerve palsies. In the light of this observation, we discuss the clinical characteristics, diagnostic means, differential diagnoses and therapeutic modalities of this syndrome.

[Granulomatous uveitis and CREST syndrome: a case study]. / Uvéite granulomateuse et Crest syndrome. A propos d'un cas.

PURPOSE: To report a case of recurrent granulomatous panuveitis associated with CREST syndrome. OBSERVATION: A 74-year-old patient with CREST syndrome presented with unilateral granulomatous panuveitis in a pseudophakic eye. She had undergone cataract surgery 6 months before. The patient reported a vision loss that had been evolving for 1 month. Visual acuity was noted at 20/400. The initial clinical examination highlighted retrodescemetic precipitates and granulomatous precipitates on the IOL. A vitreous tyndall was noted. Funduscopic examination revealed papillary edema and cystoid macular edema, confirmed by fluorescein angiography. RESULTS: Topical treatment consisting in corticosteroid eye drops associated with mydriatics controlled uveitis in a few weeks. Visual recovery was 20/30. No granulomatous uveitis etiology could be highlighted. The diagnosis of chronic endophthalmitis was also ruled out. CONCLUSION: The diagnosis retained was uveitis associated with CREST syndrome. To our knowledge, this association has only been reported twice in the literature.

[Orbital extension of sinus plasmacytoma secondarily transforming into multiple myeloma: a case study]. / Extension orbitaire d'un plasmocytome sinusien secondairement transformé en myélome multiple. A propos d'un cas.

We report the case of a 39-year-old man who consulted for severe exophthalmia with diplopia associated with chronic sinusitis evolving over 6 months. A cervicofacial CT scan showed a tumoral mass invading the maxillary and ethmoidal left sinus and orbital cavity. A biopsy of the mass and general evaluation confirmed the diagnosis of extramedullary plasmocytoma. Secondarily it transformed into multiple myeloma. In light of this case of extramedullary plasmocytoma in a young subject, we discuss the clinical characteristics, ophthalmologic manifestations, and the various therapeutic modalities according to the stage of the disease and the ocular repercussions.

[Bilateral anterior acute ischemic optic neuropathy complicating optic nerve head drusen. Apropos of a case]. / Neuropathie optique ischémique antérieure aiguë bilatérale compliquant des druses de la papille. A propos d'un cas.

A case of bilateral Anterior Ischemic Optic Neuropathy (AION) which is related to buried optic nerve head drusen is presented. Such an etiology has rarely been described and is poorly-documented in the literature. This etiologic diagnosis is brought by the imaging techniques: fluorescein angiography, B-scan ultrasonography and computed tomography. The pathogenesis may be related to the compressive effects encountered in a small scleral canal. The other complications of optic nerve head drusen are described and their similar mechanisms discussed.

[Bilateral papilledema as the manifestation of Schwannoma of the cauda equina]. / Oedème papillaire bilatéral révélant un schwannome de la queue-de-cheval. A propos d'un cas.

PURPOSE: A case of schwannoma of the cauda equina in which the initial manifestation was visual loss, due to papilledema, is described. OBSERVATION: A 56-year-old man presented a history of 1 month of progressively blurred vision. On examination, visual acuity was 20/1,000 due to bilateral papilledema without spinal symptoms or neurological signs. Brain computed tomography demonstrated a nonobstructive hydrocephalus. He underwent a ventriculoperitoneal shunt without improvement. One month later, he complained of disturbance while walking associated with lower back pain. A lumbar puncture revealed an increased opening pressure. Analysis of the cerebrospinal fluid demonstrated an elevated protein level (6.8 g/l). Spinal magnetic resonance imaging showed a neurinoma of the cauda equina. We performed complete surgical resection and the histopathology diagnosis was schwannoma. Neurological dysfunctions resolved except visual acuity due to bilateral optic atrophy. DISCUSSION: The association of papilledema and spinal tumor is an unusual but well-known phenomenon. Several theories have been proposed to explain the development of increased intracranial pressure in such cases but the exact physiopathology remains unknown. CONCLUSION: Spinal cord tumor should be considered when acute papilledema associated with normal brain computed tomography and high cerebrospinal fluid protein levels are encountered. Spinal magnetic resonance imaging should then be performed.

Antiphospholipid syndrome in patients with retinal venous occlusion.

INTRODUCTION: We conducted a prospective study to determine the prevalence and the prognosis of antiphospholipid syndrome (APS) in patients with retinal venous occlusion (RVO). PATIENTS: Consecutive patients presenting with retinal vein occlusion were screened for vascular risk factors (diabetes mellitus, hypertension, hyperlipidemia) and for antiphospholipid antibodies (aPL): anticardiolipin (aCL), anti-beta2-glycoprotein I, and lupus anticoagulant. Patients with a serum sample positive for aPL returned at least 6 weeks later for a new screening to determine the prevalence of antiphospholipid syndrome. All patients were followed to determine the outcome. RESULTS: Sixty-eight patients presented with RVO, 16 had vascular risk factors for RVO. After two screenings for aPL, nine cases of antiphospholipid syndrome associated with RVO were diagnosed (13.2%). Eight patients were over age 50 years and none had a previous thrombotic event before RVO. All patients were treated with aspirin (160 mg/day). With a mean follow-up of 26.1+/-8.2 months (range, 16-36 months), there were no recurrences. CONCLUSION: Retinal venous occlusion is multifactorial in origin. In patients aged 50 years and older, without previous thrombotic event, aPL might not be predictive of recurrences and treatment with aspirin might be sufficient. In such patients, the routine screening for aPL does not appear warranted, but a randomized study should be conducted to really ascertain the pathogenic role of aPL and the most appropriate treatment in RVO.