Abstract: Langerhan's cell Histiocytosis of the orbit. Langerhan's cell Histiocytosis is a rare condition that affects children and less frequently young adults. It is characterized by a proliferation of histiocytes derived from Langerhan's cells. Orbital involvement is described in 20% of cases where orbital eosinophilic granuloma located in the frontal bone is the most frequent. The malignant nature of this disease is not established. Its evolution is unpredictable and spontaneous regression after simple biopsy were described. The authors report four cases of Langerhan's cell Histiocytosis and will discuss on the clinical and radiological aspects as well as on the evolution of orbital histiocytoses X.
Abstract: The authors report a case of eosinophilic granuloma involving the roof of the orbit in a 3-year-old girl. The clinical presentation and the imaging features suggested a malignant tumor and the final diagnosis was obtained by biopsy with histopathologic examination. Despite its alarming radiologic appearance, there was spontaneous healing of the eosinophilic granuloma.
Abstract: Ocular amyloidosis is infrequent, and the palpebral location is uncommon. It usually has a primary localized form, but can occur in systemic or familial amyloidosis. The diagnosis is mainly made by histopathology. Its treatment is surgical and the prognosis depends on clinical presentation and recurrences. We report the case of a 54-year-old woman with no medical history of the disease, who had bilateral isolated palpebral amyloidosis presenting as bilateral upper lid swelling with ptosis and corneal dystrophy. Diagnosis was confirmed by palpebral biopsy and the patient underwent excision of the involved lid tissue with reconstruction of the affected lid with cartilage autograft. Clinical progression showed improvement of the ptosis and the patient's comfort. Although rare, palpebral amyloidosis poses management problems, particularly in advanced cases, because of corneal complications and recurrence.
Abstract: Primitive neuroectodermal tumor (PNET) of the orbit is rare. We present a case of a young patient with orbital PNET extending to the endo-crane and sinuses, which had been treated 10 years before with the diagnosis of rhabdomyosarcoma. The diagnosis was corrected with immunohistochemistry. Through this observation, we discuss the clinical aspects, the diagnosis difficulties, and the therapeutic options of this rare affection.
Abstract: Retinal arterial macroaneurysms are ectatic modifications of the arterial wall. They are a relatively uncommon condition wich can threaten visual function by pre- or subretinal hemorrhage in case of rupture or by massive macular exudation.
Abstract: Cutis laxa is a heterogeneous group of connective tissue disorders,characterized by loose skin and variable systemic involvement. The characteristic symptomatological pattern is resulting from paucity of elastic fibers.
Abstract: Aniridia is a rare familial or sporadic disorder affecting not only the iris but also the cornea, angle structures, lens, optic nerve, and fovea. This disorder may be associated with many other systemic abnormalities such as urogenital malignancies.
Abstract: A 26 year-old woman, whose parents were consanguineously married, was admitted to our center because of bilateral juvenile cataract. The patient exhibited short stature, sclerodermalike appearance of the skin with a typical bird-like facies, thinning and graying of hair, high pitched voice and hypogonadism. Werner's syndrome, was diagnosed. History, pathogeny, clinical features, diagnosis and cataract surgery are discussed.
