[Marcus Gunn Syndrome. Study of 3 rare cases]. / Syndrome de Marcus Gunn. Etude de 3 cas rares.

PURPOSE: reporting three rare cases of Marcus Gunn Syndrome and analyzing the clinical features of this entity. METHODS: Case reports of three patients with a Marcus Gunn Syndrome, treated at the Ophthalmology B CHU service Rabat - Morocco. RESULTS: Our patients are two boys aged 1 and 2 years old, and a 31 year old woman with a Marcus Gunn Syndrome. As special features, in the boys' cases this syndrome is bilateral, as for the other case it is associated with congenital fibrosis clinically predominant of the Ipsilateral inferior rectus muscle in an adult. Surgical treatment was not offered for children because of their young age. The adult patient has benefited from a recession of the inferior rectus muscle and a pleating in the superior rectus muscle. The forced duction test objectified a fibrosis of the inferior rectus muscle. Surgical correction of Ptosis and Synkinesis has not been indicated in this patient, given the residual oculomotor disorder and the risk of exposure keratitis. CONCLUSION: Marcus Gunn Syndrome is exceptional in adults. This can be explained by the tendency of the lid retraction to fade with age. Bilateral involvement in Marcus Gunn Syndrome is rare. Its association with congenital fibrosis of extraocular muscles has allowed a better etiopathogenic approach highlighting a supra nuclear origin of this Syndrome.

[Macular serous detachment revealing acute lymphoblastic leukemia]. / Décollement séreux maculaire révélateur d'une leucémie aiguë lymphoblastique.

BACKGROUND: Leukemias are a group of malignant diseases caused by immature hematopoietic cells proliferating in the blood marrow. Some manifestations result from ocular-orbital involvement, which usually occurs through the central nervous system. Other manifestations stem from vasculopathy and/or hemorheologic disorders (anemia, thrombocytopenia, hyperviscosity). OBSERVATION: We report a case of a 42-year-old women presenting with loss of vision caused by serous macular detachment. The investigations showed the diagnosis of acute lymphoblastic leukemia. Steroids and chemotherapy led to complete remission with normal visual acuity during a follow-up of 29 months. DISCUSSION: Ocular involvement is seen in 28%-80% of leukemia cases. The most obvious findings are the presence of retinal hemorrhages, which are most commonly located in the posterior pole. The intraretinal hemorrhage may contain a white component that usually is a white dot in the center of the hemorrhage, but other clinical features are described. Serous detachment of the neuroepithelium is seldom reported, and can be the first symptom of the disease. Other ocular findings include infiltrative involvement of the chorioretina, anterior segment involvement, and orbital location. Ocular location is estimated to be an equivalent of central nervous system involvement, and subsequently requires adequate treatment (steroids, chemotherapy and radiotherapy of the central nervous system). CONCLUSION: Ocular manifestations of leukemia are frequent but rarely reveal the disease. Serous detachment is rarer. However, the diagnosis of leukemia should be considered in case of pigmentary epithelium involvement.

[Value of ultrasonography in persistent hyperplastic primary vitreous body. Apropos of 4 cases]. / Intérêt de l'échographie dans la persistance et l'hyperplasie du vitré primitif. A propos de 4 cas.

The authors report 4 patients with complicated persistent hyperplastic primary vitreous. The complications observed are cataract (2 eyes) and retinal detachment (2 eyes). The affection is bilateral in two cases and unilateral in the two other cases. After a short embryologic vitreous recall, the authors present clinical and paraclinical aspects of the condition. The authors focus on the importance of echographic data.

[Visual prognosis factors in congenital cataract]. / Les facteurs de pronostic visuel dans la cataracte congénitale.

PURPOSE: Study of different factors which can change the visual prognosis in congenital cataract. METHODS: One hundred seventy eyes of 100 children with congenital cataract are operated by extracapsular cataract extraction with limbic incision. Posterior rhexis and anterior vitrectomy were associated in children under 2 years. Implantation was used in 42%, contact lens in 8%. Other cases were corrected by glasses. The prognostic factors studied were: uni- or bilaterality, partial or complete aspect of cataract, age of apparition and management, and association with other malformations. RESULTS: Only 9 eyes out of 30 with unilateral congenital cataracts had visual acuity > or = 2/10; 44.4% of bilateral cataracts had visual acuity > or = 2/10; The best visual acuity was noted in progressive and partial cataract without associated malformations.

[Orbital schwannoma: clinical, imaging and surgical features]. / Schwannome orbitaire : aspects cliniques, radiologiques et chirurgicaux.

INTRODUCTION: Schwannoma, or peripheral glioma, is a rare tumor of the orbit (1% of orbital tumors). Its diagnosis remains difficult and can be established only by histological analysis. We discuss through this case report the clinical, radiological and surgical features of this tumor. OBSERVATION: We report the case of a young 38-year-old woman with high myopia and no specific medical history, presenting with a rapidly progressive decrease in visual acuity of the right eye, associated with swelling of the supero-lateral orbit, and eccentric, non-pulsatile, ipsilateral proptosis. Clinical examination of the right eye revealed corrected visual acuity of 20/40, mobile swelling near the lacrimal gland and limitation of elevation and abduction. Slit lamp exam revealed secondary central corneal degeneration. Fundus exam was remarkable for stage I optic nerve head edema. Examination of the left eye was unremarkable. Orbital CT revealed a mass of 21.3mm×14.8mm, hypodense and heterogeneous, attached to the lacrimal gland, displacing the globe downward and inward, enhancing with contrast and without bone erosion. Excision of the tumor and the remaining stump of the gland was performed through a subciliairy skin incision followed by histological analysis confirming the diagnosis of schwannoma. No recurrence was noted after a follow-up of a year and a half. CONCLUSION: Schwannoma is a rare tumor of the orbit. Its clinical and radiological diagnosis is always difficult. Only histological study allows confirmation of the diagnosis. The absence of recurrence is dependent upon total surgical excision.

[Devic's neuro-optic myelitis and pregnancy]. / Neuromyélite optique de Devic survenue au cours d'une grossesse.

INTRODUCTION: Devic's neuromyelitis optica associates optical neuritis that is either unilateral or bilateral and acute transversal myelopathy. This disease seldom occurs and is rarely associated with pregnancy. OBSERVATION: We report the case of a young 20-year-old woman who was 8 weeks pregnant. She was admitted to our ophthalmological department for a profound and bilateral drop in visual acuity. The ophthalmological examination showed bilateral optic disc swelling. Three days later, the patient presented a spastic paraplegia with sphincter disorders. Devic's neuromyelitis optica was suspected and then confirmed by examination of cerebrospinal fluid and cerebrospinal MRI. Corticoid treatment in bolus of prednisolone methyl relayed by prednisolone per bone was established. The neurological and visual defects were clearly improved and the case was stabilized at 1 year. DISCUSSION: Devic's neuromyelitis optica is related to nosologic problems with multiple sclerosis. Clinical, immunological, and pathological elements provide arguments in favor of a separate entity.