[Benign but not harmless intracranial hypertension: a case report]. / Hypertension intracrânienne bénigne, mais pas anodine. À propos d'un cas.

Benign intracranial hypertension (BIH) is characterized as an intracranial pressure increase occurring in the absence of brain tumour, sinus thrombosis or hydrocephaly. But contrary to what its designation might suggest, it threatens the visual prognosis. We report the case of a 15-year-old girl with lymphocytic meningitis, developing secondary a BIH. Cerebrospinal fluid pressure was 70cm water, without enlargement of the cerebral ventricles. Along with the progression, bilateral 6th nerve palsy, impairment of visual acuity and bilateral papilledema appeared. No cause was found after a complete assessment. Treatment consisted in oral acetazolamide and 9 depletive spinal taps. Clinical examination, fundus examination and Goldmann visual field normalized after 8 weeks. No relapse occurred after a 1-year follow-up. This case shows that BIH, which is not a well-known disorder, is incorrectly referred to as benign: both prompt diagnosis and proper management are of major importance.

[Cerebral visual impairment in brain-damaged children - four case studies]. / Les pathologies neuro-visuelles chez l'enfant cérébrolésé - à propos de quatre cas.

Cerebral visual impairment is one of the main causes of childhood visual impairment in developed countries. These disorders are often linked with pre- or perinatal hypoxic brain injuries. The patterns of brain injuries depend on the severity and duration of hypoxia and the child's age. In premature children, periventricular leukomalacia affects the optic radiations and the subcortical visual brain. In full-term newborn babies, chronic hypoxia leads to the damage of the visual cortex and acute hypoxia damages the basal ganglia. They recover from cortical blindness in variable ways. Visual dysfunction is characterized by fixation troubles, subnormal acuity (crowding), difficulty with perceiving visual fields, movements, depth, cognitive defects (agnosia of images, objects or faces, visuospatial disorders), ocular motility disorders (tonic gaze deviation, strabismus, nystagmus). Accompanying these cerebral injuries, there are accommodation defects and optic disk abnormalities that vary according to the gestational age at the time of hypoxia.

[Implantation of preserved human amniotic membrane for the treatment of shield ulcers and persistent corneal epithelial defects in chronic allergic keratoconjunctivitis]. / Greffe de membrane amniotique et traitement des ulcères de cornée lors des kératoconjonctivites chroniques allergiques.

PURPOSE: To determine whether amniotic membrane implantation is a safe and effective alternative treatment for shield ulcers and persistent corneal epithelial defects associated with ulcers in chronic allergic keratoconjunctivitis (vernal or atopic keratoconjunctivitis). METHODS: Amniotic membrane implantation was performed in four consecutive patients with persistent corneal epithelial defects or vernal plaques unresponsive to conventional medical treatment lasting an average of 18 weeks. Surgery was done under general anesthesia using amniotic membrane as a therapeutic contact lens. RESULTS: A significant decrease in symptoms and complete reepithelialization of the corneal ulcers were observed in all cases within the first 7 days. These remained stable during a mean follow-up of 12 weeks, with no intraoperative or postoperative complications. Early detachment occurred in all cases with no negative consequences on ulcer healing. CONCLUSION: Patients with severe chronic allergic keratoconjunctivitis derive benefits from amniotic membrane implantation used as a therapeutic contact lens in the treatment of persistent corneal epithelial defects and vernal plaques unresponsive to conventional medical treatment.

[Graves'ophthalmopathy: usefulness of T2 weighted muscle signal intensity]. / IRM et ophtalmopathies associées aux maladies thyroïdiennes: intérêt de l'analyse du signal des muscles oculomoteurs en T2.

OBJECTIVE: Immunosuppressive treatments may be usefull when Graves' ophthalmopathy is active. We evaluated the interest of hypertrophied muscle T2 weighted signal intensity as an argument of disease activity, to help for the therapeutic decision. PATIENTS AND METHODS: 46 MRI in 37 patients with Graves' ophthalmopathy were retrospectively studied. RESULTS: During the active phase of the disease, centro-muscular hypersignal was always observed (6 cases); signal intensity was not so bright or even disappeared with immunosuppressive treatment (7 cases). We never observed hypersignal when the disease was inactive (4 cases). When there was no hypersignal on T2 weighted images, the disease was inactive in 70% cases, and of low activity in the other cases. CONCLUSION: T2 weighted centro-muscular signal intensity analyse helps for the therapeutic decision in Graves' ophthalmopathy.

[A familial case of chronic progressive external ophthalmoplegia associated with mitochondrial disease]. / Une observation familiale d'ophtalmoplégie externe chronique progressive liée à une mitochondriopathie.

Mitochondrial myopathies are rare hereditary diseases that affect the energy functions of the mitochondria. Clinical manifestations are variable and sometimes multisystemic. Progressive external ophthalmoplegia constitutes the most frequent clinical form. Unfortunately, the diagnosis and the treatment of these mitochondrial abnormalities stay, today, even difficult. We report ophthalmic findings and the course of the disease in members of a family with chronic progressive external ophthalmoplegia presenting with severe acquired blepharoptosis. From study at the family background, the inheritance seemed autosomal dominant. In one case, a comprehensive workup, including muscular biopsy and molecular genetics disclosed a mitochondrial myopathy. During the 30-year follow-up, the patients were operated on for their ptosis several times, because of recurrences and uneven results.

[Ophthalmologic manifestations of systemic vasculitis: report of six cases and review of the literature]. / Manifestations ophtalmologiques des vascularites systémiques: analyse de six observations et revue de la littérature.

PURPOSE: Study of characteristics of ocular involvement in systemic vasculitis. METHODS: We describe six cases of systemic vasculitis with ocular involvement observed between 1992 and 2000. These cases are compared with those reported in the literature. RESULTS: Our patients suffered from Wegener's granulomatosis (four cases), periarteritis nodosa and Churg-Strauss syndrome. Ocular manifestations were conjunctivitis, scleritis, orbital pseudotumor, optic neuritis and extraocular muscle palsy. These manifestations are similar to those reported in the literature. Their treatment requires steroids and immunosuppressive drugs. In one of our cases, intravenous immunoglobulins were effective in controlling an optic neuritis. CONCLUSION: Ocular involvement in systemic vasculitis may concern any orbital structure. It usually occurs during the course of vasculitis but may be one of its first manifestations. It requires an appropriate treatment to prevent ophthalmic complications and especially blindness.

[Vernal keratoconjunctivitis in the child. Clinical and complementary study apropos of 22 cases]. / Kérato-conjonctivites vernales de l'enfant. Etude clinique et complémentaire à propos de 22 cas.

PURPOSE: Vernal kératoconjunctivitis was studied in a population of 22 children aged 3 to 14 years and followed up in an allergy and ophthalmology outpatient clinic. The role of allergy and the severity of inflammation where assessed by a systematic exploration, which combined a detailed allergy evaluation and blood and lacrimal sampling. MATERIALS AND METHODS: Allergy criteria chosen and recorded in 9 cases are: an increase of total IgE over the higher limit for the age, a positive skin prick test to one allergen, a positive serum specific IgE dosage (> 0.35 IU/mL) of specific IgE. Conjunctival allergy was present in 6 of the 9 children with a positive allergenic provocation test, or with a high local production of total IgE and a lacrimal/serum eosinophilic cationic protein ratio greater than one. RESULTS: Criteria used for supporting the IgE mediated hypersensitivity diagnosis are discussed: they have to be very strict to eliminate false positive results. Allergen involvement can only be evidenced by a specific provocation test. When evidenced as described, limbic or palpebral conjunctivitis had the same frequency. Lacrimal ICAM 1 levels seemed to be higher (p < 0.05) in the severe limbal forms (24.7 +/- 3 pg/mL) than in the palpebral ones (8.1 +/- 6.5 pg/mL). Interpretation of biological parameters evidencing conjunctival inflammation is more difficult. CONCLUSION: Allergic involvement in child vernal keratoconjunctivitis can only be assessed through a detailed evaluation, leading to a specialised ophthalmic and allergic management. A specific treatment can then be established, based on allergen eviction and possibly on specific immunotherapy (5 cases). H1 antihistamin treatments are dedicated only to children with a positive allergic evaluation.

[Long-term results of cobalt 60 curietherapy for uveal melanoma]. / Résultats à long terme de la curiethérapie par le cobalt 60 des mélanomes de l'uvée.

PURPOSE: To analyze 65 patients with uveal melanomas treated with cobalt plaque therapy with regards to mortality, visual results and complications. PATIENTS AND METHODS: Most of the melanomas were large (T3: 52.5%), with a mean largest dimension of the base of 11 mm, and a mean thickness of 6 mm. Most of the tumors were located in the choroid (95%), with an anterior margin behind the equator (65%), and a posterior margin at less than 3 mm of the disc and/or of the macula (69%). The plaque radiotherapy delivered a mean dose of 95 Gy to the tumor apex, either with a cobalt plaque alone (51 cases), or in association with a ruthenium plaque (14 cases). The mean follow up period was over 8 years. RESULTS: The local control was achieved initially in 86% of the eyes. The estimated melanoma specific survival rate was 83% after 5 years and 74% after 10 years. The main parameter associated with the metastases was the largest dimension of the base (p < 0.01). The eye was retained in 83% of the cases. The probability of keeping a vision better than or equal to 0,1 was 39% after 5 years and 27% after 10 years. The main parameter associated with the visual loss was the tumor size (p < 0.01). The complications included cataract (39%), radiation retinopathy (34%), with maculopathy (19%) and/or papillopathy (13.5%), vitreous hemorrhages (22%), neovascular glaucoma (15%) and retinal detachment (12%). CONCLUSION: These results supported the value of cobalt plaque radiotherapy in the management of uveal melanomas.

Study of the inferior oblique muscle of the eye by MRI.

The utility of the inferior oblique m. radioanatomical study by a FLASH 3D sequence in magnetic resonance imaging (MRI). Twenty eyes (ten healthy volunteers aged 21-32 years, without any history of significant ocular pathology) were explored by MRI (1 Tesla; cranial coil) and comparisons were made between spin echo (SE) T1 sequence (through the neuro-ocular and coronal planes; thickness of slices = 3 mm) and a gradient echo FLASH 3D sequence (thickness of slices = 1 mm). This enabled a mm by mm reformation of the inferior oblique m through the frontal-oblique plane, made possible by new SE T1 sequences through the same plane. Position, height, and signal of the m. were estimated. The mean frontal angle formed by the muscle and the sagittal axis measured 29 degrees for the right eye and 27 degrees for the left eye. The muscle was always identifiable in the reformation despite its thin dimensions: 1.9 mm (1.5 - 2.8) on the right and 2 mm (1.7 - 2.5) on the left, in low signal silhouetted by the high signal of orbital fat. Thus, thanks to mm by mm reconstructions using FLASH 3D sequence, a good radioanatomic study of the inferior oblique m. by MRI is possible. This could be useful particularly for the strabismus of children.

[Malignant melanoma of the choroid associated with neurofibromatosis]. / Mélanome malin de la choroïde associé à une neurofibromatose.

A sixteen-year-old white girl with peripheral neurofibromatosis (NF1), who had been treated for a glioma of the optic nerves and chiasma developed a choroidal mass in her only functional eye. After a transchoroïdal biopsy, the pathologic examination disclosed a choroïdal melanoma of epithelioid cell type, using morphological criteria as well as an immunohistochemical study. After treatment with a ruthenium plaque, the tumor completely regressed over 4 months. According to the data of the literature, neurofibromatosis seems to predispose to the development of uveal melanomas.

[Giant peripheral tear of the retinal pigmented epithelium]. / Déchirure géante et périphérique de l'épithélium pigmentaire rétinien.

The authors present a case of gigantic tear of the retinal pigmented epithelium. This tear was situated in the middle periphery and seems to have appeared spontaneously together with a carotid-cavernous fistula. The functional outcome was favorable. The authors deal with the various possible patho-physiological mechanisms.

[Oncologic and functional results of 57 malignant melanoma of the uvea treated by curietherapy]. / Résultats carcinologiques et fonctionnels de 57 mélanomes malins de l'uvée traités par curiethérapie.

Fifty-seven uveal malignant melanomas (T2: 36.8%; T3: 49.1%) were treated between 1983 and 1989 with Cobalt 60 or/and Ruthenium 106 Rhodium 106 plaques. The mean follow-up was 32 months (from 6 to 69 months). A diminution of tumor size was observed in 88% of the cases, either rapid and marked (57%), or slower and more moderate (31%). A multivariate study showed the radiation dose administered to the tumor edge to be the most significant parameter associated with the tumor control. Seven patients developed metastases, and the probability of survival at 60 months was 83.3%. This probability was identical to that of 59 comparable patients treated, some years before, by enucleation. The most frequent complications were radiation retinopathy (28.1%) and retinal detachment (15.8%). The treated eye was retained in 86% of cases. In 57% of treated eyes, vision remained better or equal to 0.1. The predictive parameters of vision impairment after treatment seemed to be tumor size, tumor site near the macula and the optic nerve, and the radiation dose to the tumor base. The use of Ruthenium 106 Rhodium 106 appeared to be advantageous in controlling the disease as well as in reducing the incidence of complications.

[Treatment of various malignant melanomas of the choroid using a combination of ruthenium 106 and cobalt 60]. / Traitement de certains mélanomes malins de la choroïde par l'association ruthénium 106 et cobalt 60.

The association 106Ru/106Rh and 60Co was used to treat choroidal malignant melanomas with a height of 7 mm above the scleral surface (4 cases) and tumors unsuccessfully treated with a first 60Co plaque therapy (3 cases). The association of both radionuclides allowed a dose of 85 Gy at the apex of the tumor and of 700 Gy at least on the base in every case. All the patients were alive without evidence of metastases with a minimum follow up of one year and the tumor regression was constant and sometimes spectacular.

[Intraorbital abscess of dental origin in a child]. / Abcès intra-orbitaire de l'enfant d'origine dentaire.

A two years old girl developed orbital infection secondary to dental trauma. Computed tomographic scanning of the orbit shows subperiosteal abcess in the medial orbital wall and proptosis of left globe. Teeth removal, appropriate antibiotics and surgical drainage are essential for preservation of vision.