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.

Gillespie syndrome: An atypical form and review of the literature.

INTRODUCTION: Gillespie syndrome (GS) is a rare genetic disorder that combines ocular and cerebral defects.It was first described in 1965, by Frederick D. Gillespie. He reported a triad of congenital aniridia, cerebellar ataxia and mental retardation in a 22-year-old woman and her 19-year-old brother. Its etiology is still unknown.To date, less than 30 patients have been reported in the literature. OBSERVATION: We report the case of a 2 years old child, born of a consanguineous marriage. At the age of 8 months, the parents consulted for a delay in psychomotor acquisition for which the MRI performed showed a vermian hypoplasia. It was only at the age of 2 years, following a contusive trauma of the left eye that a partial aniridia was objectified on both eyes associated with a lens coloboma on the left eye. In view of these clinico-radiological data, the diagnosis of Gillespie syndrome was retained. DISCUSSION: Gillespie syndrome is a genetic disease. It combines ocular and neurological abnormalities. It was first described in 1965 by Gillespie. The ocular manifestations of Gillespie syndrome mainly concern the iris. Aniridia is always present with, in most cases, a scalloped appearance of the pupillary margin. It can be accompanied with additional ocular findings such as foveal, optic nerve hypoplasia, retinal hypopigmentation, and/or pigmentary macular changes leading to reduced visual acuity.In addition to ocular abnormalities, the Gillespie syndrome. (GS) includes neurological deficiencies, particularly axial hypotonia, lack of coordination, dysarthria and static and kinetic ataxia. CONCLUSION: The diagnosis of Gillespie Syndrome should be evoked in any hypotonic child presenting with bilateral but partial aniridia. Prognosis depends on the proper management and anticipation of ocular and mental symptoms and disabilities.

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.

[Möbius syndrome]. / Le syndrome de Möbius.

Möbius syndrome is a rare congenital disease characterized by facial and abducens nerve palsy. Children are unable to smile, frown, suck, grimace, blink their eyes, and to move their eyes laterally. The aim of this study was to analyze the diagnosis and treatment of this disease. Maxillofacial examination reveals: facial diplegia, retrognathism, palatine and dental malformations. Möbius syndrome is usually associated with multiple cranial nerve involvement, limb or orofacial malformation, and Poland syndrome. Although the pathogenesis of the syndrome is unclear, a number of mechanisms have been suggested including vascular and genetic hypotheses. Symptomatic treatment is used to manage this syndrome. The diagnosis of Möbius syndrome may be difficult in some patients with atypical signs of facial diplegia and other cranial nerve palsies, it requires a multidisciplinary approach.

[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.