681/1916. Laser treatment of eye disorders in children. The increasing range of indications for laser treatment and the development of new modalities of lasering have led to a growing tendency to extend this type of treatment to the pediatric population. Problems of compliance in this age group often necessitate the use of general anesthesia. This report describes the operative technique used in 11 children (13 eyes) under the age of 13 years, all of whom underwent laser treatment under general anesthesia for a variety of ocular conditions. argon or krypton laser photocoagulation was performed in eight children (10 eyes), seven of whom were treated for various retinal pathologies and one for an iris cyst. The other three children underwent Nd:YAG posterior capsulotomy for secondary cataract. ( info) |
682/1916. Electroretinographic responses in patients with pulseless disease vary with head and body positions. We examined bright-flash electroretinograms obtained in two Japanese women with pulseless disease. One 48-year-old woman (case 1) who has had the disease for 17 years showed no oscillatory potentials. When she sat and tilted her head to the right, a- and b-wave amplitudes diminished in the left eye. A second 48-year-old woman (case 2) who has had the disease for 10 years also demonstrated absent oscillatory potentials. When she sat and raised her chin, a- and b-wave amplitudes diminished in both eyes. These amplitudes were normal when the patients were in a supine position. ( info) |
683/1916. Niemann-Pick disease type B with ocular involvement: report of a case. Niemann-Pick disease (NPD) type B was diagnosed clinically and enzymatically in a 5-year-old girl presenting with failure to thrive, hepatosplenomegaly, diffuse interstitial infiltration of both lungs on chest roentgenograms, cherry red spot, and foam cells in the bone marrow aspirate. intelligence and neurological examination were normal, sphingomyelinase activity was severely deficient in cultured skin fibroblasts. We present the first case of NPD type B in taiwan and relevant literatures were reviewed. ( info) |
684/1916. Ultrastructural study of Norrie's disease. We studied the clinicopathologic and ultrastructural features of a full-term infant with Norrie's disease. The infant had bilateral retrolental fibrous vascular masses and retinal detachment with no other apparent physical abnormalities and no family history of ocular defects. A vitrectomy and a membrane peeling were attempted, and specimens of the retina, the retrolental membrane, and a vascularized epiretinal peripheral mass were examined by light and electron microscopy. The retrolental membrane was composed of layered collagenous tissue and contained structures resembling blood vessels. Inner and outer neuroblastic layers were identified in the retinal tissue, but no vessels were present. In the epiretinal mass, portions of retina and cortical vitreous were seen along with primitive vascular structures. The histologic appearance of these specimens suggests that the major pathologic event of Norrie's disease occurs in the retina in the third to fourth gestational month. We believe the subsequent ocular abnormalities found in this patient were secondary to this early retinal malformation and did not represent a progressive ocular disorder. ( info) |
685/1916. adult-form galactosialidosis: ocular findings in three cases. We reported ocular findings of 3 patients with adult-form galactosialidosis. Diffuse fine opacities in the deep layer of the corneal stroma, an obscure cherry-red spot of the macula and optic nerve atrophy were noted in all cases. Punctate lenticular opacities were observed in 2 cases. Slowly progressive visual disturbance was seen in all cases, and it might be due to secondary optic nerve atrophy caused by retinal ganglion cell death. ( info) |
| Eales' disease is a noninflammatory occlusive disorder of the retinal vasculature that causes recurrent hemorrhages into the retina and vitreous and ischemic changes in the eye. Extraocular manifestations of Eales' disease are rare and limited to the central nervous system. We report the case of a patient with Eales' disease and ischemic stroke, and we review the neurologic manifestations of this primarily ophthalmologic vasculopathy. ( info) |
687/1916. Microangiopathy with retinopathy, encephalopathy, and deafness (RED-M) and systemic features. The case of a young woman with a rare syndrome of acute encephalopathy followed by deafness and retinopathy developing over 1 year is reported. Unlike previously described similar cases, she had considerable systemic symptoms and signs including polyarthralgia-arthritis, diffuse myalgia, malar rash, livedo reticularis, night sweats, and fatigue suggestive of systemic lupus erythematosus. However, results of most immunological investigations were repeatedly normal, including antinuclear antibodies. Anticardiolipin antibodies were elevated on one occasion. cyclophosphamide has been the most effective treatment for exacerbations of the disease, which have continued to occur over 6 years. This microangiopathic syndrome more likely relates to an immunologically mediated vasculitis of small blood vessels than to a thromboembolic etiology. ( info) |
688/1916. Hemorrhagic choroidal detachment with anterior vitreoretinal adhesions. We present three cases of large intraoperative or postoperative hemorrhagic choroidal detachment with subsequent adherence of the retina to anterior segment structures. Surgical management involved bimanual vitrectomy through anterior sclerotomy sites and dissection within the anterior segment. Successful retinal reattachment was achieved in two of these cases (18-months' follow up). In the third case, the retina remained attached for 4 months but then redetached. ( info) |
689/1916. Choroidal vasculitis in acute posterior multifocal placoid pigment epitheliopathy. A 24-year-old Caucasian female presented with acute posterior multifocal placoid pigment epitheliopathy (APMPPE) and associated infiltration round some of the larger choroidal blood vessels. This infiltration dissipated as the patient's clinical condition improved and did not induce any permanent alteration of the overlying retinal pigment epithelium. We suggest that the infiltration round the choroidal vessels was due to a choroidal vasculitis. The finding of choroidal inflammation in this case lends support to the hypothesis that choroidal vasculitis is an underlying pathological process in APMPPE. ( info) |
690/1916. aicardi syndrome. A clinicopathologic case report including electron microscopic observations. aicardi syndrome is characterized by infantile spasms, agenesis of the corpus callosum, severe mental retardation, and a characteristic chorioretinopathy with lacunar defects. The authors report on a 2-year-old girl with congenital hydrocephaly who was found unresponsive by the baby-sitter and died shortly thereafter. At autopsy, the histopathologic findings, which were confined to the brain and eyes, were found highly characteristic of AIC. The main abnormalities included agenesis of the corpus callosum, micropolygyria, bilateral papillomas of choroid plexi, bilateral microphthalmia, bilateral hypoplasia of the optic nerves, bilateral colobomas of the juxtapapillaris choroid and optic disc, bilateral total retinal detachment with dysplastic rosettes and chorioretinal lacunae with focal thinning, and atrophy of the retinal pigment epithelium and choroid. A detailed histopathologic study of the ocular findings and the brain anomalies is presented. The results of scanning electron microscopy of the chorioretinal lacunae demonstrated peculiar papillary proliferations of the retinal pigment epithelium in both eyes. ( info) |