11/453. Optic neuropathy following amiodarone therapy. Ocular changes during treatment with amiodarone are almost universal but are rarely serious. In this article we describe three patients from a single electrophysiology practice in whom optic neuropathy developed during treatment with amiodarone. All three patients were more than 65 years of age. The doses of amiodarone ranged from 100 to 400 mg/day, and the time intervals between the initiation of the amiodarone therapy and the appearance of first symptoms of optic neuropathy were 5 to 19 months. Two patients had bilateral involvement, and one had only unilateral involvement. Whether this result was due solely to amiodarone therapy, to the underlying poor health of these patients, or to the combination of these two factors is uncertain. These findings prompt us to recommend that all patients who receive amiodarone undergo complete ophthalmologic examinations, including careful evaluation of the ocular fundus regularly during such therapy. No randomized study had been undertaken to determine the true incidence of complications associated with this medication. ( info) |
12/453. microcephaly, microphthalmia, congenital cataract, optic atrophy, short stature, hypotonia, severe psychomotor retardation, and cerebral malformations: a second family with micro syndrome or a new syndrome? We report on four children of both sexes from a highly inbred family with hypotonia, spastic diplegia, microcephaly, microphthalmia, congenital cataract, optic atrophy, ptosis, kyphoscoliosis, short stature, severe mental retardation, and cerebral malformations. Six other children may also have been affected. The differential diagnosis and the possibility of a second family with the micro syndrome are discussed. ( info) |
13/453. Surgical chorioretinal venous anastomosis for ischemic central retinal vein occlusion. BACKGROUND AND PURPOSE: To report results of a pilot study to create chorioretinal venous anastomosis (CRVA) in eyes with ischemic central retinal vein occlusion (CRVO) via a pars plana approach. patients AND methods: Five eyes of 5 patients with ischemic CRVO underwent surgical CRVA. Following pars plana vitrectomy, the posterior hyaloid face was removed, and slit-like incisions were made with a microvitreoretinal blade adjacent to a major retinal vein in each quadrant. Small pieces of 50 Mersilene sutures (Ethicon, Somerville, NJ) were positioned over the vein and inserted into these incisions to promote vascularization. Panretinal photocoagulation was applied. RESULTS: A functional CRVA site was noted at 10 of 16 attempted sites (4 sites in 1 patient could not be evaluated because of cataract). Minor fibrous proliferation was noted at CRVA sites in all eyes. optic atrophy developed in 3 eyes. visual acuity improved in 3 eyes, remained unchanged in 1, and deteriorated in 1 eye after a mean follow up of 13.4 months (range 8-20 months). CONCLUSION: Surgically induced CRVA may improve the prognosis in some eyes with ischemic CRVO. ( info) |
14/453. Conradi-Hunermann syndrome with ocular anomalies. We report a Japanese girl with the Conradi-Hunermann form of chondrodysplasia punctata and anterior segment malformations characteristic of Axenfeld-Rieger syndrome. The patient also had cataracts and unilateral optic atrophy. A possible role for homeobox-containing genes in the etiology of this type of chondrodysplasia punctata is suggested as an explanation for the coincidence of these two syndromes. ( info) |
15/453. Retinal red-free light photographs in two congenital conditions: a case of optic hypoplasia and a case of congenital hemianopia. Two patients with congenital anomalies involving the optic pathways are described. The first case presented a unilateral hypoplastic optic nerve as well as an ipsilateral inferior conus and an elevated disc. The second case showed the features of homonymous hemianopia with sparing of the macula and decreased visual acuity on the side of the affected cerebral hemisphere. Red-free photographs were obtained in both cases. The importance of this old-new investigative tool in completing a neuro-ophthalmological study is stressed. ( info) |
16/453. optic atrophy following prophylactic chemotherapy and cranial radiation for acute lymphocytic leukemia. Two patients with acute lymphocytic leukemia developed progressive optic nerve and chiasmal lesions eight to nine months after the initiation of identical chemotherapy protocols that included intrathecal medication and prophylactic radiation of only 2,400 rads to the central nervous system. Both patients eventually lost all vision despite additional radiotherapy, and there was no evidence of leukemia involving the central nervous system after acute lymphocytic leukemia was diagnosed. optic nerve biopsy in one case showed changes consistent with radiation necrosis. ( info) |
17/453. Infantile cerebral aneurysms with visual pathway compression. Intracranial aneurysms are rare in infancy. The commonest presentation is intracranial hemorrhage, but signs of mass effect are more frequent than in adults. We report 2 infants with cerebral aneurysms, one presenting with macrocephaly and another with strabismus. Both had visual loss and optic disc pallor; MRI revealed a suprasellar mass and anterior visual pathway compression. In both cases, the preoperative diagnosis was craniopharyngioma. It is essential to recognize that, although exceedingly uncommon, cerebral aneurysms do occur in infants and have features that differ from those in adults. ( info) |
18/453. Optic glioma with characteristic bilateral optic atrophy in a 3-year-old girl. We report a case of optic glioma with bilateral optic atrophy. A 3-year-old girl presented with vomiting and left hemiparesis. She had hypothalamic dysfunction, right ptosis, right monocular nystagmus, left facial palsy, left hemiparesis, and left pes adductus. neuroimaging studies showed obstructive hydrocephalus with a large suprasellar calcified tumor with a ring-like enhancement mimicking craniopharyngioma. Visual-evoked potentials showed delayed latency of N75 in the right occipital lead. The tumor, a pilocytic astrocytoma in the right optic tract and chiasma, was partially removed via a right frontotemporal craniotomy. The right optic nerve had shrunk to half the normal diameter and became twisted downwardly. intracranial pressure (ICP) increased to 40 cm H2O. The fundus had bilateral optic atrophy without disc swelling. To our knowledge, this is the first report of a lamina/dot sign of the optic disc in a small child with a brain tumor and a normal neuroretinal fiber layer. These ocular findings may result from possible interruption of the axonal flow caused by the tumor and not increased ICP. ( info) |
19/453. GAPO syndrome: a new case of this rare syndrome and a review of the relative importance of different phenotypic features in diagnosis. The case of GAPO syndrome reported here is the 24th recorded case, 23 cases having been published previously. The 29-year-old male under discussion presents all the typical features of the syndrome, having short stature, dysmorphic craniofacial features, total alopecia and pseudoanodontia. Orally, the erupted primary dentition was extremely worn and on radiographic examination, the second mandibular molars were found to be unerupted, together with the entire permanent dentition. cephalometry revealed the absence of facial pneumatisation, a deficient cranial base with diminished upper face height and maxillary and mandibular hypoplasia with a prognathic skeletal pattern. Histological examination of an extracted primary incisor and its surrounding root bone revealed extensive ankylosis. This paper describes in detail the clinical findings and reviews, and discusses previously published cases in relation to the present one. As with prior cases, parental consanguinity was present in the pedigree. ( info) |
20/453. DeSanctis-Cacchione syndrome. We report a case of DeSanctis-Cacchione syndrome presenting with unusual features like early onset of cutaneous lesions and optic atrophy. ( info) |