RESUMEN
Authors presented two cases of typical Groenblad-Strandberg's syndrome and reviewed concerning pathogenesis, histology, heredity and its related conditions. Followings are to be considered in these cases. 1. Pseudoxanthoma elasticum, generally considered; mainly appears from 25 to 50 years of life, but we met in case 2 that he was eleven years old. 2. In ocular complications Case 1 showed disciform macular degeneration of the left eye and macular hemorrhages in superficial layers of retina of the right eye. The other case was several retinal exudates of the left eye without any amcular complications. 3. Case 2 had suggestive signs of Paget's disease in radiograms of the skull and pelvic bone. Routine laboratery studies including serum calcium, phosphorus and alkaline phosphatase were normal. 4. Suspicious heroditary occurrence was noted in case 2. 5. There were no significant abnormalities in blood and cardiovascular system.
Asunto(s)
Fosfatasa Alcalina , Calcio , Sistema Cardiovascular , Exudados y Transudados , Hemorragia , Herencia , Degeneración Macular , Huesos Pélvicos , Fósforo , Seudoxantoma Elástico , Retina , Retinaldehído , CráneoRESUMEN
A case of optic nerve glioma is presented. This 11 years old Korean boy was seen because of proto nasion of the risht eye ball of 2 months duration. Vision of the right eye was reduced to 20/30 and optic disc showed blurring of the margin with some venous engorgement. Visual field showed enlarged blind spot of Mariotte and contraction of temporal field of vision in the right eye. Radiograpic study of the optic foramina was normal. There was no cafe-au-lait spot. On April 19. 1967. the orbit was exposed through anterior approach because of the tumor possibly invading the intraocular pertion. On operating procedure it was easy to remove the tumor and optic nerve by aspiration of 3 cc. of vitreous using 19 gauge needle. We confirmed a diagnosis of astrocytoma of the optic nerve in pathologic study. A year later the removal of the tumor, the patient was in good health and there had been no recurrence. A review of the literature of recent years related to glioma of the optic nerve, particularly its signs, symptoms, diagnosis and treatment was added.