[A case of a retinitis pigmentosa and asteroid hyalosis]. / Rétinopathie pigmentaire et hyalopathie astéroïde: à propos d'un cas.

Asteroid hyalosis is a rare degenerative condition of the vitreous. No causal relationship between retinitis pigmentosa and asteroid hyalosis has been established. We report a case of a 65-year-old male admitted for progressive hemeralopia. Visual acuities were 2/10e in the right eye and 4/10e in the left eye. Ophthalmic examination revealed a large number of asteroid and refringent bodies, a typical retinitis pigmentosa with a cystoid macular oedema confirmed by fluorescein angiography. Electrodiagnostic testing revealed an altered electroretinogram. Visual fields showed a typical generalized constriction. We discuss the clinical aspects and physiopathogenic mechanisms of this rare association through this case and other cases found in the literature.

[Macular damage in Behçet's disease]. / La maculopathie dans la maladie de Behçet.

INTRODUCTION: The macular damage in Behçet's disease requires particular attention because of its poor prognosis. PURPOSE: The purpose of our study was to specify the frequency of macular alterations in Behçet's disease and discuss the clinical, physiopathological and therapeutic aspects. MATERIALS AND METHODS: Our report is based on 244 patients with Behçet's disease examined between January 1995 and December 1997. The authors studied macular alterations in detail. RESULTS: Sixty-nine patients had a maculopathy affecting 108 out of 432 eyes (24%). The mean progression of Behçet's disease in these cases was 4 years. Visual acuity was

[Ocular and systemic complications of homocystinuria: a report of five cases]. / Complications oculaires et systémiques de l'homocystinurie: à propos de cinq cas.

Homocystinuria is an autosomal recessive disorder of methionine metabolism due to cystathionine B-synthetase deficiency. It is the second most common inborn error of amino acid metabolism after phenylketonuria. In addition to the eyes, the skeletal, central nervous and vascular systems are usually affected by homocystinuria. We report two family cases of two sisters and two brothers with homocystinuria revealed by lenticular dislocation into the anterior chamber, associated with mental and growth retardation, and an isolated case of homocystinuria revealed by poor vision with Marfan syndrome and mental retardation. They all underwent surgical lensectomy with anterior vitrectomy under general anesthesia with anaesthesic precautions to prevent vascular thrombosis. Ocular complications are common in patients with homocystinuria. Treatment must include dietary changes to reduce the incidence of ectopia lentis and mental retardation. Surgical treatment with modern microsurgical techniques should be considered in advanced ocular manifestations of homocystinuria.

[Ocular impairment during type II membranoproliferative glomerulonephritis]. / Atteinte oculaire au cours de la glomérulonéphrite membrano-proliférative type II.

Membranoproliferative glomerulonephritis type II (MPGN) is characterized by dense deposits within glomerular basal membrane and Bruch's membrane which result in retinal lesions similar to drusens. We observed a 50-year-old patient with chronic renal deficiency who developed central bilateral serous retinopathy with diffuse punctiforme yellow subretinal lesions. Ophthalmoscopic and angiographic aspects led to an MPGN type II diagnosis. Specific posterior segment lesions are described during MPGN type II. Dense deposits concerned both lamina densa of glomerular basal membrane and Bruch's membrane with choriocapillaris. The main ocular complications were central serous chorioretinopathy and choroidal neovascularization. We review the clinical and evolutive aspects of this disease.

[Weill Marchesani syndrome. Report of a case]. / Le syndrome de Weill Marchesani. A propos d'une observation.

Weill Marchesani syndrome is a congenital disease that combines microspherophakia and skeletal abnormalities. The authors report a 19-year-old male, born of a consanguineous marriage, with a progressive decrease in visual acuity. The general examination showed a squat look, dwarfism, muscle hypertrophy, short hands and feet, and joint stiffness. The ophthalmological examination showed that visual acuity was limited to hand motion in the right eye despite correction and no perception of light in the left eye. Intraocular pressure was 36 mmHg in the right eye and 40 mmHg in the left eye. The anterior chamber was irregular with iridophakodonesis and microspherophakia of both lenses. The zonula was partially ruptured in the right eye. The iridocorneal angle was narrow. Fundus eye examination showed a pale optic disc with an excavation of 9/10 on the right. In the left eye, the optic disc was totally excavated. Cardiovascular check-up revealed rheumatic aortic valvular cardiopathy. The therapy consisted of combined surgery: phakophagia with anterior vitrectomy plus trabeculectomy operated on the right eye. Weill Marchesani syndrome is a rare congenital affection with a recessive autosomal transmission. The visual prognosis is dominated by secondary glaucoma due to pupillary blockage by the mobile eye lens. This observation illustrates the seriousness of spontaneous progression in Weill Marchesani syndrome, justifying the necessity of lens extraction before the onset of complications.

[Gyrate atrophy of the choroid and retina: a case report]. / Atrophie gyrée choriorétinienne: a propos dún cas.

BACKGROUND: Gyrate atrophy of the retina and choroid is a rare disease, with recessive autosomal transmission, characterized by progressive chorioretinal atrophy causing blindness. It results from a congenital deficit in aminotransferase ornithine. CASE REPORT: The authors present the case of a young patient aged 15 years old consulting for a progressive fall of visual acuity with hemeralopia. Eye funduscopy showed regions of confluent rounded chorioretinal atrophy. The visual field, the electroretinogram and the retinal angiography were all alterated. Gyrate atrophy of the retina and choroid was evocated. DISCUSSION: It is a systemic and rare metabolic disease where ocular features are dominating. Differencial diagnosis are pigmentary retinopathies. Cataract and/or myopia are often joined to the retinal lesions. General signs could consist in muscular weakness, thin and rare hairs and mental retardation. More than; visual fields, electroretinogram, retinal angiography that are alterated; the plasmatic dosage of the ornithine is often high. The treatment is based on the dietetics with uncertain results. The genic therapy would be the treatment of future.

[Juvenile xanthogranuloma with intraocular involvement. A case report]. / Xanthogranulome juvénile avec localisation intra-oculaire. A propos dún cas.

Juvenile xanthogranuloma (JXG) is a rare and usually benign disease occurring in early childhood. It causes skin and deep seated lesions, notably in the eye. We report a case of JXG in the iris of a 9-month-old infant. Examination under general anesthesia revealed megalocornea, iris xanthogranuloma occupying the entire anterior chamber and high intraocular pressure. Skin lesions on the left lid and on the back were also found. Ocular hypertension resisted medical and surgical treatment. Cyclodestruction was necessary. The incidence of JXG is low (0.4%). The iris is the most frequently affected ocular tissue. Early diagnosis is necessary to avoid complications. Moreover, JXG can be associated in rare cases with neurofibromatosis or leukemia which must be systematically searched for.

[The oculomotor effects of the fractures of the floor of the orbit]. / Les séquelles oculomotrices des fractures du plancher de l'orbite.

PURPOSE: Fractures of the orbit floor have very disabling oculomotor after effects. The objective of this study was to report that close collaboration between the maxillo-facial specialist and ophthalmologist is necessary to avoid oculomotor after effects. METHODS AND PATIENTS: Our study included 10 cases of the fracture of the orbit floor. These cases were recorded between 1992 and 1996. RESULTS: This pathology affected young subjects aged between 20 and 40 years in 70 % of the cases. 80 % of the subjects were males. 90 % of the patients presented a vertical diplopia as a result of a transitory oculomotor deficit. 20 % of the subjects presented a permanent oculomotor deficit at the stage of after effects. All these deficits were of a mechanical origin and, contrary to other authors, we found no deficit with nervous sensory origin. CONCLUSION: Therapeutic success is based on early diagnosis and treatment that requires close collaboration between the maxillo-facial and ophthalmology teams.

[Acquired oculomotor paralysis: a new therapeutic approach. Apropos of 10 cases]. / Les paralysies oculomotrices acquises : nouvelle approche thérapeutique. A propos de 10 cas.

PURPOSE: The aim of this work is to report our rehabilitation scheme and outcome in patients with acquired oculomotor palsy. PATIENTS AND METHODS: We cared for 10 patients with oculomotor palsy between January 1996 and March 1998 at the Casablanca University Hospital. Our orthoptic rehabilitation scheme was based on reinforcing the patient's sensorial potential using a prism dioper and motor capacities by soliciting vergency and version movements. RESULTS: The 10 patients (7 males, 3 females, mean age 39.5 years) had unilateral IV palsy (n =3), bilateral IV palsy (n= 1), unilateral VI palsy (n= 2), bilateral VI palsy (n= 1), partial unilateral III palsy (n= 1) and dissociated bilateral III palsy (n= 2). Mean delay to initiation of rehabilitation was 49 days and mean duration for treatment was 3 months. We achieved total recovery in 50% of the cases and partial recovery in 40%. Intermittent diplopia persisted in 10 per thousand of the cases. These results differ slightly from those in the literature where total regression is reported in about 50% of the patients after therapeutic abstention. CONCLUSION: This small series is insufficient to validate our method. The results obtained do however show that rehabilitation is safe and should be applied more widely in patients with oculomotor palsy.

[Cockayne's syndrome with unusual retinal involvement (report of one family)]. / Syndrome de Cockayne avec atteinte rétinienne inhabituelle (à propos j'une famille).

Cockayne's syndrome is a very rare autosomal recessive affection. Ocular involvement is an essential element for positive diagnosis; the retina shows a typical salt and pepper retinitis with optical atrophy. We report a family with four brothers who had Cockayne's syndrome with unusual retinal involvement. The patients' parents were first cousins. Ophthalmologic examination of the mother showed unilateral left pigmentary retinopathy localized in the peripapillary region. The father's ophthalmological examination was normal. The four brothers presented disharmonious dwarfism, cutaneous hyperpigmentation of skin areas exposed to sun with old-appearance of the skin, sensorineural deafness, kyphoscoliosis, a cerebellar syndrome and mental retardation. The ophthalmological examination showed hypermetropia in all four brothers and bilateral maculopathy with no papillary or vascular abnormalities. The electroretinogram was in favor of cone dystrophy. Computed tomography showed one case of calcifications of the basal ganglia and cerebral atrophy. The karyotypes of the four brothers and the mother were normal. We discuss the ocular symptoms and the etiopathogenesis of this syndrome.

[Pseudotumorous choroid granuloma in miliary tuberculosis]. / Granulome choroïdien pseudotumoral au cours de la tuberculose miliaire.

Recently, tuberculosis re-emerged as a serious public health problem, in industrialized countries, while remaining it remains a major cause of mortality and morbidity in third world countries. Intraocular localization is rare. The most common manifestation of ocular tuberculosis in patients with miliary is Bouchut tubercules. An atypical feature of ocular tuberculosis is discussed: a pseudotumoral choroid granuloma. The behavior of the lesion and its response to antituberculous therapy was documented clinically by fundus photography on fluorescein angiography.

[Isolated and primary palpebro-conjunctival amylosis. Apropos of 2 cases]. / Amylose palpébro-conjonctivale primitive et isolée. A propos de 2 cas.

Ocular amyloidosis a an uncommon condition and conjunctival involvement rarely occurs, representing 15% of all ocular localizations. Clinical manifestations show a polymorphism requiring a histological diagnosis. Based on two cases of primary isolated conjunctivo-palpebral amyloidosis, we discuss the different ocular sites. The first case involved false ptosis, a conjunctival infiltration and a lower eyelid tumefaction; all located in the left eye. The second case had ptosis, a right lower eyelid tumefaction with ectropion and esthetic damage. Amyloidosis was confirmed at histologic examination of the conjunctival mucous after a special congo red coloration. In order to affirm the isolated conjunctival localization, it is necessary to eliminate another amyloidis site. The treatment was surgical with excision and cure of ptosis. Ocular amyloidosis is a rare condition with polymorphism manifestations. Confirmation is histological. The greatest difficulty is management due to disease recurrence.

[Bilateral Sturge-Weber-Krabbe syndrome. Apropos of a case]. / Syndrome de Sturge-Weber-Krabbe bilatéral. A propos d'un cas.

We report a case of bilateral Sturge-Weber-Krabbe syndrome. The patient was a child aged 2 years. He presented facial and jugal angioma, bilateral glaucoma and epilepsy. The cerebral scan showed calcifications characterised by their localisations and their aspects. Cure and prognosis of this disease are discussed.

[Ophthalmological manifestations of Von Recklinghausen disease]. / Manifestations ophtalmologiques de la maladie de Von Recklinghausen.

PURPOSE: To evaluate the frequency of Von Recklinghausen neurofibromatosis type 1 (NF1) ocular abnormalities. METHODS: A retrospective study was conducted in 11 patients during two years. RESULTS: We found Lisch nodules in 4 cases associated to conjunctival lesion in one case and to retinal hamartoma in another case. In two cases, we found an eyelid neurofibroma. One patient had orbital fibrosis dysplasia. Ocular disease revealed the NF1 in 4 cases, the tumoral exophtalmia in two cases. CONCLUSION: The ocular manifestations of Von Recklinghausen disease are frequent and can reveal the disease. Ophthalmologic examination must always be performed if Von Recklinghausen is suspected.

[Ophthalmological involvement in Behçet disease. Apropos of 520 cases]. / L'atteinte ophtalmologique dans la maladie de Behçet. A propos de 520 cas.

PURPOSE: The aim of this paper is to compare our results with the literature and to discuss some therapeutical aspects of the disease. METHODS: This retrospective study concerned 520 cases of Behçet's disease followed by internal diseases department and ophthalmology service of U.H.C. Averroes (Casablanca, Morocco) during 10 years. RESULTS: There were 432 men (83%) and 88 women (17%). The mean age of the patients was 20 years. Ophthalmological involvements are found in 80%, bilateral in 60%. The disease occurred at the rate of 2 or 3 episodes a year in 5% of cases. Irreversible blindness was noted in 24.4% of cases, predominately panuveitis in 37% of cases, followed by anterior uveitis in 36.3% and retinal vasculitis in 37% of cases. Good results were obtained by a medical care with corticoids and chloraminophen in case of threatening blindness. CONCLUSION: Behçet's disease remains frequent in our country. It requires early diagnosis and steady ophthalmological surveillance in order to delay onset of blindness.