Orbital metastases from prostate adenocarcinoma: Case report and review of the literature.

Introduction: Prostate carcinoma metastasizes usually to lymph nodes and bone. Its metastases to the orbital cavity remain very rare. Observation: We report here the case of an 80-year-old man diagnosed with a non-metastatic prostate adenocarcinoma 9 months earlier, who was found to have an orbital metastasis revealed by a proptosis of his left eye. He received hormonal therapy, chemotherapy and radiotherapy. Discussion: Orbital metastases from prostate carcinoma have many similarities to other orbital metastases in their presentation. Their diagnosis is easily done when there is a history of a primary tumor. Presenting symptoms include proptosis, limitation of eye movements, diplopia and decreased vision. Conclusion: Through this case report and a review of literature, we discuss the incidence, the clinical presentation and the management of these tumors.

Leber's idiopathic stellate neuroretinitis: A clinical case.

Introduction: In 1916, Leber's idiopathic stellate neuroretinitis (LISN) was described by Theodore Leber as a rare disease characterized by optic disc swelling associated with a macular star. This fundus appearance can have multiple causes but the etiology of Leber's idiopathic stellate neuroretinitis remains unknown. Case report: A 40 year-old man consulted for a progressive decline in visual acuity and a blurred vision in his left eye. Corrected Visual acuity of the left eye was hand motion, Funduscopy of the left eye revealed a stellate maculopathy with loss of foveolar depression and a normal optic disc. The angiography confirmed an optic disc oedema. Laboratory investigations were normal. No infectious nor inflammatory etiology was found. Brain imaging was normal. Patient received 3 days of intravenous methylprednisolone at 10mg/kg/D for 3 days in a row and an oral relay was started with a progressive degression over 2 weeks. The evolution after treatment was satisfactory, the visual acuity 3 weeks after the intravenous injection of corticoids increased to 2/10. Discussion: Leber's idiopathic stellate neuroretinitis (LISN) is a disorder characterized by disc oedema, peripapillary and macular hard exudates and, often, the presence of vitreous cells. The changes in the optic nerve are the primary cause of reduced vision in this condition. The more common treatable causes must be excluded wich are cat scratch disease (CSD) and vascular disease. 50% of cases have no identifiable cause and are labeled idiopathic neuroretinitis. There is no consensus regarding optimal treatment. The prognosis of Leber's idiopathic stellate neuroretinitis is good in most cases. Conclusion: The cause of neuroretinitis must be aggressively pursued before a diagnosis of lebre's idiopathic neuroretinis can be retained in order to formulate an appropriate treatment strategy.

Sclerochoroidal calcification associated with chondrocalcinosis: A clinical case.

INTRODUCTION: Sclerochoroidal calcifications is a rare condition corresponding to senile plaques due to the deposition of calcium and phosphate in the sclera and choroid. It is an elderly patient's pathology. In most cases, it is idiopathic. However, some patients have systemic disorders leading to disturbances in phosphocalcic metabolism, which are at the origin of this disorder. A check-up is therefore necessary to detect them. OBSERVATION: We report the case of a 65 year old patient admitted to the ophthalmology department for a renewal of optical correction. The patient is known to have articular chondrocalcinosis on anti-inflammatory medication. Fundus examination revealed multiple white supra macular choroidal lesions with tumour-like appearance in both eyes. The ultrasound perfomed showed the calcic nature of the lesions. Fluorescein and indocyanine green angiograms showed no sign of activity or presence of neovessels.A complete metabolic work-up, mainly phosphocalcic, was ordered again and the systemic diagnosis of articular chondrocalcinosis was retained. DISCUSSION: Shields et al. first described, in 1997, a case of sclerochoroidal calcification in a patient with chondrocalcinosis and a normal metabolic profile. Few more have been added to the literature. It is generally considered to be predominantly bilateral and is most often seen as yellowish lesions. Two types of calcifications have been described to date, the plaque type and the pseudotumor type. Given the asymptomatic presentation, a fundus examination should be performed in patients with chondrocalcinosis. Generally, no treatment is necessary. CONCLUSION: Sclerochoroidal calcifications usually manifests as multiple discrete yellow placoid lesions in elderly asymptomatic patients. Visual prognosis for sclerochoroidal classification is good since the lesions tend to be away from the macula. They should not be confused with choroidal metastasis or achromic melanoma which require more extensive treatment.

Retinal angioma of Von hippel-lindau disease: A case report.

INTRODUCTION: Von Hippel-Lindau disease (VHL), also known as Von Hippel-Lindau syndrome, is a rare genetic disorder with multisystem involvement. It is characterised by the development of multiple vascularised tumours, particularly cerebellar, retinal and/or visceral. The disease can occur at any age and usually starts with retinal hemangioblastomas. CASE REPORT: We report the case of a 45-year-old female patient with no particular pathological history, who. consulted the ophthalmology department for a change of optical correction.The funds examination showed an uncomplicated bilateral hemangioma with no other associated signs. Fluorescein angiography confirmed the diagnosis by showing in the left eye a multiple retinal hemangioma visible in the mid-periphery facing the branches of the superior temporal arches. The brain MRI showed a multifocal hemangioblastoma in the posterior cerebral fossa. A renal ultrasound returned normal. The patient had undergone photocoagulation of the retinal lesions to avoid any complications. DISCUSSION: The German ophthalmologist Eugen von Hippel first described angiomas in the eye. The term Von Hippel-Lindau disease was first used in 1936; however, its use became common only in the 1970s.Tumours called hemangioblastomas are characteristic of von Hippel-Lindau syndrome. These growths are made of newly formed blood vessels and occurs in the periphery of the retina. Spontaneous progression occurs leading to visual impairment as a result of maculopathy or exudative retinal detachment.Early recognition and treatment of specific manifestations of VHL can substantially decrease complications and improve quality of life.Conventional treatment of the retinal hemangioblastomas is laser photocoagulation or cryotherapy depending on the location and size of the lesions. It must be based on the patient's visual symptoms and tumor progression. CONCLUSION: Management of patients with VHL disease often requires a multidisciplinary approach. The role of the ophthalmologist is important in the management of this condition since the ocular involvement may be indicative of the disease.

13q interstitial deletion in a moroccan child with hereditary retinoblastoma and intellectual disability: A case report.

Retinoblastoma is the most common malignant tumor of the eye in children (incidence:1/15,000 to 1/20,000 births), with a sex ratio of 1,5/1. Retinoblastoma, in its inherited form, is a disease caused by a syndrome of genetic predisposition to cancer. The RB1 gene, a tumor suppressor gene, is localized at 13q14. This case report shows the indication of the cytogenetic analysis in the management of patients with retinoblastoma, and the interest of a genetic counseling. We report the medical observation of a five and a half years old patient who was followed in the medical genetic's department for intellectual disability: associated with facial dysmorphia. The cytogenetic study objectified the presence of an interstitial deletion of the long arm of chromosome 13: 46, XX, del (13) (q14q22). A genetic counseling, with study of the karyotype of the parents is planned, specially to search for a balanced insertion: 13q14 insertion and deletion. In addition, the patient has been followed since the age of 9 months at the pediatric ophthalmology department for a bilateral retinoblastoma, in remission. A subject carry in constitutional mutation of the RB1 gene has a greater than 90% risk of developing retinoblastoma, and moreover has a genetic predisposition to secondary tumors. This medical observation shows the benefit of the constitutional cytogenetic study for patients with retinoblastoma, in particular in the event of bilateral retinoblastoma. The monitoring of psychomotor development must supplement the ophthalmological monitoring of these patients, with a systematic genetic counseling.

[Retinoblastoma: preliminary results of national treatment protocol at Casablanca university medical center]. / Rétinoblastome : résultats préliminaires du protocole national de prise en charge au CHU de Casablanca.

Retinoblastoma is the most frequent intraocular cancer, affecting almost exclusively children. We report prospective study results assessing the national protocol for retinoblastoma treatment in Morocco. Treatment included, depending on stage and laterality, primary chemotherapy either to facilitate enucleation or to make conservative treatment possible, postoperative chemotherapy, enucleation and conservative treatments such as transpupillary thermotherapy, thermochemotherapy and cryotherapy. Radiation was used in a few cases. Close supervision was performed until the age of 5. The incidence of retinoblastoma within the study period was 18 new cases per year in our department. Observations of 32 children were included in the study: 18 unilateral retinoblastomas (56%) and 14 bilateral retinoblastomas (44%), for a total of 46 eyes. Leucocoria was the most frequent presenting symptom (69%). Buphthalmia or proptosis were present in 47% of cases. The stage of retinoblastoma was V/D or E (Reese-Elsworth/ABC) in 69.5% of cases. Enucleation was necessary for 28 eyes. Transpupillary thermotherapy or thermochemotherapy were used for 13 eyes (11 children) and cryotherapy for 13 eyes (10 children). After an average follow-up period of 52 months, among 32 children, 4 died and 2 abandoned treatment. Ocular salvage rate was 85.7% (12 eyes out of 14, among which 11 without radiation). Retinoblastoma is a genetic tumor, which occurs in two forms: sporadic, always unilateral, and hereditary, often bilateral. The latter is the most challenging case. Current treatment protocols rely primarily on chemotherapy and local treatments. The future is oriented toward purely local treatments such as intra-arterial chemotherapy and intraocular chemotherapy.

[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.

[Avulsion of the optic nerve: two case studies]. / L'avulsion du nerf optique. A propos de deux cas.

Avulsion of the optic nerve is a rare and serious injury. The authors report two cases of optic nerve avulsion. The first one concerns a 5-year-old boy who presented ocular trauma after falling on the handlebars of a bicycle. His visual acuity was light perception in the right eye, and his right pupil was unresponsive to light. The anterior segment was normal. The ophthalmoscopic examination showed a total separation of the optic nerve head from the sclera with peripapillary hemorrhage. The second case concerns a 30-year-old man who was hit in the right eye with a stick. On admission, he had no light perception in the right eye, a right afferent pupil defect, a small laceration of the right lower eye lid and no abnormalities on the anterior segment. The fundus examination showed a mild vitreous hemorrhage. Ocular ultrasonography showed vitreous hemorrhage coming directly from the optic nerve head in a mushroom pattern. A CT scan of the orbit revealed a thickened optic nerve. Color Doppler ultrasonography documented slowing of blood flow in the central retinal artery. The two patients received 1 mg/kg/day of prednisone for 2 weeks. No improvement was noted. Optic nerve avulsion is often caused by sudden and forceful rotation of the eye with tearing of the optic nerve as it exits the globe. The nerve can be partially or totally avulsed. The prognosis is usually poor.

[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.

[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.

[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.