[Manifestation of congenital rubella syndrome: clinical and epidemiologic aspects]. / Les manifestations ophtalmologiques de la rubéole congénitale: aspects cliniques et épidémiologiques.

INTRODUCTION: Congenital rubella syndrome is an ensemble of congenital malformations which results from a primary viral infection in non-immunised pregnant women. The main ophthalmologic manifestation is cataract. It involves at the same time visual and vital prognosis and can be responsible for multiple handicaps. METHODS: We did a retrospective study of 16 infants (32 eyes) having undergone congenital cataract surgery compatible with congenital rubella syndrome in the Casablanca paediatric ophthalmology department between January 2001 and December 2005. All the patients underwent a complete ophthalmologic examination, otorhinolaryngologic, cardiovascular and neurological examinations, and paraclinic explorations. RESULTS: The results were compared with those reported in the literature. In our series, 25 eyes (78.12%) had a cataract of which 56% were nuclear, 13 eyes (40.62%) had microphthalmia. One case each of corneal dystrophy and iris coloboma were described. Congenital glaucoma was found in only one case. Pigmentary retinopathy was found in 12 eyes (37.5%). Seven patients (43.75%) had associated cardiac anomalies, 6 (37.5%) deafness, 5 (31.25%) psychomotor delay and 2 (12.5%) facial dysmorphy. CONCLUSION: Considering the permanent disabilities caused by congenital rubella syndrome, care should be taken with the follow-up of the pregnancies and an immunization program should be implemented for good control of the circulation of the virus.

[Bilateral optic neuropathy and non-Hodkin's lymphoma]. / Neuropathie optique bilatérale et lymphome non Hodgkinien.

UNLABELLED: While ocular lesion is commonly known in lymphoma, optic neuropathy is very rare : 1,3% of lymphomas affecting the central nervous systems. OBSERVATION: Authors report the case of a 75 year old patient treated in the haematology department for 8 years, for a large cell B phenotype stage IV lymphoma for which he received 7 chemotherapy courses (CHOP protocol). After a 4 year remission period, he presented a relapse with a rapid progressive bilateral impairment of visual acuity observed for a week before his admission. The ophthalmologic exam revealed no light perception and no afferent reflex on the right eye. There was light perception and weak afferent reflex on the left eye. The anterior segment was normal on both eyes and fundus examination revealed a bilateral stage I papillar oedema. The general exam showed a right facial palsy and an impairment of general condition. The orbital CT scan revealed a significant thickening of both optic nerves caused by lymphomatous infiltration. A chemotherapy with highly dosed IV and intrathecal methotrexate was performed. DISCUSSION: the optic neuropathy is usually associated with a generalized lymphoma with central nervous system involvement, but sometimes can precede the systemic spread of the disease. Apart from infiltration, the optic nerve can be compressed by an intracranial or orbital tumor. The optic neuropathy can also be caused by lymphomatous leptomeningitis.

[Primary palpebral tuberculosis (case report)]. / La tuberculose palpebrale primaire (a propos d'un cas).

Cutaneous tuberculosis is an uncommon disease and tuberculosis affecting the eyelid alone is even more rare. The clinical presentation is polymorphous which explains a diagnosis delay. Its treatment is based on antitubercular drugs chemotherapy. Authors report through an observation the case of a child with eyelid tuberculosis and discuss a difficult diagnosis, a physiopathogeny of eyelid tuberculosis, its diagnosis and treatment.

[Fraser syndrome. A case report]. / Syndrome de Fraser.

Fraser syndrome is a rare autosomal recessive disorder; the most consistent features are cryptophthalmos, syndactyly of fingers and toes, laryngeal stenosis, and urogenital abnormalities. We report a newborn case at day 1 of life who had multiple abnormalities, born from a consanguineous marriage. Clinically, the newborn had an ankyloblepharon on the left side, a cryptophthalmos on the right side, a syndactyly, anorectal abnormalities with ambiguous genitalia, laryngeal stenosis, and ear malformations. TDM of the cranium and orbits and the transfontanel ultrasound were normal. The abdominal ultrasound showed renal abnormalities. Right eye surgery showed a reduced cornea to an opaque thin plate clinging to the iris without an anterior chamber and a nonindividualized eyeball. The authors discuss the morphological abnormalities, the clinical and paraclinical aspects of this syndrome, its multispecialized clinical management, and the importance of prenatal diagnosis.

[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

[Involvement of the optic nerve in the course of Behcet's disease (presentation of 148 cases)]. / Les atteintes du nerf optique au cours de la maladie de Behçet (a propos de 148 cas).

Behçet's disease is a multisystemic vascularitis of still unknown etiopathogeny. Among 400 cases of Behçet disease, 148 cases presented an optic nerve involvement during a period of eight years (1992-1999). The goal of this work is to contribute to the study of optic nerve involvement in Behçet's disease. The involvement is higher in males (64%) with median age of 27 years. The involvement of the optic nerve is noticed in 37% of cases. It's isolated in 7% of cases and occurs on average after 5 years of evolution of the disease. The diagnosis is based on the clinical examination, visual field, visual evoked potentials, retinal angiography and neuro-imaging (TDM and/or MRI). It can be an acute anterior neuropathy, stasis papilledema complicating a benign intracranial hypertension, neuroretinitis or retrobulbar optic neuropathy. The extraocular systemic manifestations were dominated by oral aphthosis (94%), genital aphthosis (70%), joint manifestations (40%) and central nervous system involvement (32.4%). The prognosis is reserved, 44% of patients having vision lower than 1/10 in spite of treatment. The authors insist on the therapeutic emergency that this involvement represents and the interest to consider it in all patients having an unexplained visual loss.

[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.