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Biomedical subjects

E G Buckley

Publications and source records attributed to E G Buckley.

At least 19 recordsLinked to original sources

Long-term visual results of children after initially successful vitrectomy for stage V retinopathy of prematurity.

PURPOSE: To assess the effectiveness of vitrectomy for stage V retinopathy of prematurity (ROP) at the authors' institution, 33 patients with ROP who had initially successful total or partial anatomic retinal attachments (51 eyes) were evaluated for visual function and long-term anatomic stability. In addition, this study was an attempt to differentiate levels of visual function in children with very low vision and attendant developmental delays. METHODS: Visual function was assessed before retinal examination by an experienced pediatric vision specialist. The behavioral visual assessment was divided into seven segments with two to five tasks in each segment designed to establish a level of functional vision, ranging from light perception to form identification (and ambulation). The status of the retina was classified by the patient's retinal surgeon. RESULTS: The median follow-up was 61 months, and median age at the time of examination was 68 months. Of 51 eyes treated, form identification was achieved in 5, and all five patients had ambulatory vision. Of 51 eyes, 15 had no light perception, 11 had light perception, 6 could localize light, 10 could follow lights, and 4 were able to detect form. Only one eye in ten children with comparable ROP in each eye underwent surgery, and vision improved in six of ten of the surgical eyes. Redetachment was high, with 35 retinas totally or partially detached and only 13 retinas fully attached 61 months after surgery. CONCLUSION: The visual results are very low and disappointing. Initially successfully attached retinas can detach. There is some evidence that vitrectomized eyes function better than nonvitrectomized eyes. There is also evidence that visual function lower than form identification is useful to these children and that they are able to use their limited vision better than previously though.

Child

A randomized, controlled trial of corticosteroids in the treatment of acute optic neuritis. The Optic Neuritis Study Group.

BACKGROUND AND METHODS: The use of corticosteroids to treat optic neuritis is controversial. At 15 clinical centers, we randomly assigned 457 patients with acute optic neuritis to receive oral prednisone (1 mg per kilogram of body weight per day) for 14 days; intravenous methylprednisolone (1 g per day) for 3 days, followed by oral prednisone (1 mg per kilogram per day) for 11 days; or oral placebo for 14 days. Visual function was assessed over a six-month follow-up period. RESULTS: Visual function recovered faster in the group receiving intravenous methylprednisolone than in the placebo group; this was particularly true for the reversal of visual-field defects (P = 0.0001). Although the differences between the groups decreased with time, at six months the group that received intravenous methylprednisolone still had slightly better visual fields (P = 0.054), contrast sensitivity (P = 0.026), and color vision (P = 0.033) but not better visual acuity (P = 0.66). The outcome in the oral-prednisone group did not differ from that in the placebo group. In addition, the rate of new episodes of optic neuritis in either eye was higher in the group receiving oral prednisone, but not the group receiving intravenous methylprednisolone, than in the placebo group (relative risk for oral prednisone vs. placebo, 1.79; 95 percent confidence interval, 1.08 to 2.95). CONCLUSIONS: Intravenous methylprednisolone followed by oral prednisone speeds the recovery of visual loss due to optic neuritis and results in slightly better vision at six months. Oral prednisone alone, as prescribed in this study, is an ineffective treatment and increases the risk of new episodes of optic neuritis.

Acute Disease

Leptomeningeal dissemination of optic pathway gliomas in three children.

We treated three children with optic pathway gliomas who had progressive disease associated with metastatic spread to the leptomeninges. One patient had radiographic resolution of leptomeningeal disease after treatment with intravenous carmustine and oral mercaptopurine but died of progressive pulmonary fibrosis. The second patient was treated with intravenous thiotepa, and the leptomeningeal disease remained stable. The third patient was treated with intravenous vincristine sulfate, cyclophosphamide, cisplatin, and etoposide and had a significant size reduction of the leptomeningeal lesion. Although leptomeningeal dissemination is a seemingly rare event, it is important that all children with optic pathway gliomas be considered for this possibility, particularly after the onset of new, atypical neurologic symptoms.

Antineoplastic Agents

A simple transposition procedure for complicated strabismus.

We combined a recession or resection of recti muscles with a vertical or horizontal transposition to correct a complicated paralytic ocular deviation in eight patients. The transposed muscles were reattached to the globe parallel to the spiral of Tillaux and adjacent to the paralyzed muscle. Postoperatively, seven patients demonstrated fusion in the primary position or required a slight head turn to fuse. There were no surgical complications, and no patient developed symptomatic cyclotropia, diplopia, or anterior segment ischemia.

Adolescent

Use of botulinum toxin in strabismus after retinal detachment surgery.

Botulinum toxin was used to treat 20 patients with strabismus after retinal detachment surgery. Preinjection motility deviations ranged from 10 to 60 prism diopters (D). Postinjection deviations ranged from 0 to 20 prism D, with 75% being 10 D or less. Eighty-five percent achieved fusion that persisted, with 73% requiring only one or two injections. Only muscles in the eye that had undergone retinal reattachment surgery were injected. The average period of follow-up was 12 months. Complications were rare and all resolved spontaneously. Botulinum toxin appears to be useful as a primary treatment modality for persistent strabismus following retinal detachment surgery, possibly obviating the need for complicated strabismus surgery.

Adolescent

Vertical rectus muscle transposition with intraoperative botulinum injection for treatment of chronic sixth nerve palsy.

Six adult patients with acquired, chronic, complete sixth nerve palsy had vertical rectus muscle transposition to the lateral rectus muscle insertion with intraoperative injection of botulinum toxin into the ipsilateral medial rectus muscle. Five of six patients were orthophoric in primary gaze, and the remaining patient achieved single binocular vision with a small head turn. This method compares favorably with previously described transposition procedures for sixth nerve palsy in terms of amount of correction and size of the window of single binocular vision with a lower risk of anterior segment ischemia.

Abducens Nerve

Oncogenic point mutations in the human retinoblastoma gene: their application to genetic counseling.

Mutations of the retinoblastoma gene, most of which cannot be detected by conventional Southern blotting, are known to cause both the nonhereditary and hereditary forms of retinoblastoma and have been implicated in the development of other cancers. Nonhereditary retinoblastoma is caused by a somatic mutation. Hereditary retinoblastoma is caused by a germ-cell mutation, most often a new one, and thus there is usually no family history of the disease. Unlike patients with the nonhereditary disease, those with the hereditary form are at risk for additional retinoblastomas, and their progeny are at risk for the tumors. We used a sensitive technique of primer-directed enzymatic amplification, followed by DNA sequence analysis, to identify mutations as small as a single nucleotide change in tumors from seven patients with simplex retinoblastoma (with no family history of the disease). In four patients the mutation involved only the tumor cells, and in three it involved normal somatic cells as well as tumor cells but was not found in either parent; thus, these mutations appeared to be new, germ-cell mutations. In addition, we found point mutations in cells from a bladder carcinoma, a small-cell carcinoma of the lung, and another retinoblastoma. We conclude that the technique that we have described can distinguish hereditary from nonhereditary retinoblastoma and that it is useful in risk estimation and genetic counseling.

Base Sequence

Staying the distance.

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Education, Medical, Continuing