Unilateral retinoblastoma in an adult: report of a case and review of the literature.

PURPOSE: The authors report the clinical, cytologic, and histopathologic findings of a unilateral retinoblastoma occurring in a 26-year-old woman. This tumor usually affects young children; the mean age at the time of diagnosis usually ranges from 10 to 25 months. METHODS: Histopathologic examination of the enucleated right eye included using standard techniques, as well as immunohistochemical stains of formalin-fixed, paraffin-embedded tissues. RESULTS: Histologic examination of sections of the eye showed a retinal tumor that was centered in the equatorial region and had the typical features of a poorly differentiated retinoblastoma. Focal choroidal invasion and seeding of the anterior and posterior chambers were observed. Immunoreactivity of the tumor cells for neuron-specific enolase confirmed that the tumor is a neuronal neoplasm consistent with retinoblastoma. CONCLUSION: Retinoblastoma occurring in adults is a rare finding. In most large series of retinoblastomas, no adults are included. Only eight patients 20 years of age or older with retinoblastomas have been documented previously. In the current case, the patient had no evidence of orbital recurrence or metastasis 6 years after enucleation of the eye. It may be important for clinicians to consider this diagnosis when confronted with a retinal mass of unknown etiology in adults.

Visual field defects after macular hole surgery. A new finding.

PURPOSE: The purpose of the study is to report the problem of a temporal visual field defect occurring after macular hole surgery. METHODS: The authors reviewed the records of 13 patients found to have visual field defects after vitrectomy for macular holes. Fluorescein angiograms (13 patients), optic nerve photographs (13 patients), focal electroretinograms (3 patients), and nerve fiber analyses (8 patients) were performed in patients with visual field defects. RESULTS: An absolute, temporal, usually inferior field defect was noted in 13 patients. In eight patients, the defect was detected because of specific reports or retrospective field examination results. Five patients examined in a prospective manner were found to have field defects. No history of abnormal intraocular pressure or direct trauma to the optic nerve or retinal vessels was identified. Four patients showed optic nerve pallor and three had an anomalous-appearing disc. Focal electroretinograms were of similar amplitude in the involved retina compared to corresponding areas in the healthy fellow eye. Nerve fiber analysis showed a reduction in nerve fiber layer thickness correlating to the visual field defect in those eight patients in which this test was used. CONCLUSION: A significant temporal field defect may occur in patients after otherwise uncomplicated surgery for macular holes. The cause is unclear; however, reductions in nerve fiber layer thickness from the superior and nasal peripapillary area suggest that acute surgical release of the posterior hyaloid and the use of long-acting intraocular gas may in certain patients result in visual field defects.

The incidence of corneal abnormalities in the Silicone Study. Silicone Study Report 7.

OBJECTIVE: To determine factors that were prognostic of corneal abnormalities in eyes following surgery for severe proliferative vitreoretinopathy. DESIGN: Subgroup analysis of the Silicone Study. SETTINGS: Community and university-based ophthalmology clinics. MATERIALS: Eyes with attached maculae at the 24-month follow-up examination that did not have a pre-existing corneal abnormality. INTERVENTIONS: Vitrectomy surgery with long-acting gas or silicone oil. OUTCOME MEASURES: Epithelial and/or stromal edema, corneal opacity and/or previous corneal transplant. RESULTS: The incidence of corneal abnormalities at 24 months was 27% and did not differ significantly between treatment groups. Prognostic factors were preoperative aphakia or pseudophakia (P = .003), preoperative iris neovascularization (P = .006), reoperation (P = .001), the absence of a fluid/gas exchange (P = .03), corneal touch by silicone oil (P = .02), and the presence of aqueous cells (P = .009) or aqueous flare (P = .08). In a multivariate analysis, independent prognostic factors were iris neovascularization (relative risk [RR] = 13.1), aphakia or pseudophakia (RR = 3.0), postoperative aqueous flare (RR = 5.4), and reoperations (RR = 3.4). Corneal abnormalities were correlated with poor visual acuity and hypotony (P < .001). CONCLUSIONS: To our knowledge, this is the first study to document that the incidence rates of corneal abnormalities are equivalent between oil and gas. The incidence of corneal abnormalities in gas-filled eyes was higher than expected, and remained high in oil-filled eyes, despite the use of an inferior iridectomy. Successful surgical repair of the retinal detachment with a single operation, and prevention and early management of corneal touch by silicone oil should help to prevent corneal abnormalities. If rubeosis iridis or severe aqueous flare is present, preoperative treatment with intense topical and possibly periocular steroids might reduce inflammation, which might mediate corneal damage.

The effects of silicone oil removal. Silicone Study Report 6.

OBJECTIVE: To evaluate the advisability of removing silicone oil from eyes after surgery for severe (with a classification of at least C-3) proliferative vitreoretinopathy. DESIGN: Subgroup analysis of the Silicone Study, a randomized, multicentered, surgical trial. SETTING: Community- and university-based clinics. PATIENTS: Two hundred twenty-two eyes with severe proliferative vitreoretinopathy followed up in the Silicone Study. INTERVENTIONS: Vitrectomy for proliferative vitreoretinopathy with silicone oil as the intraocular tamponade. OUTCOME MEASURES: Changes in visual acuity, recurrent retinal detachment, and incidence of complications. RESULTS: Ninety-nine (45%) of 222 eyes had surgery for silicone oil removal (oil-removed eyes). Compared with the eyes that did not undergo silicone oil removal (oil-retained eyes) evaluated at a comparable time after oil injection, oil-removed eyes at the examination prior to oil removal were more likely to be attached (85% vs 40%; P < .0001), have a visual acuity of 5/200 or greater (63% vs 35%; P < .0001), and not be hypotonous (5% vs 22%; P < .001). There was no association between the length of oil retention and incidence of recurrent retinal detachment after oil removal. Eyes with attached retinas at the time of oil removal generally improved in visual acuity at the last follow-up examination (P < .0001), which was not evident in eyes with detached retinas at the time of oil removal. In a matched-pair cohort analysis comparing both sets of eyes, there was an increased risk for recurrent retinal detachment at the last follow-up examination in the oil-removed eyes (odds ratio [OR], 2.1; P = .09). However, overall visual acuity improved for oil-removed eyes in 19 (29%) of 66 pairs and for oil-retained eyes in one (2%) of 66 pairs (OR, 19.0; P < .0001). Although nonsignificant, incidence rates of keratopathy (OR, 0.5) and hypotony (OR, 0.5) were lower in oil-removed eyes. CONCLUSION: Removal of silicone oil in anatomically successful eyes significantly increases the likelihood of improved visual acuity with a slight increase in the likelihood of recurrent retinal redetachment. There was a trend for a reduction in the incidence of complications in the oil-removed eyes.

Limiting applications of cryotherapy for severe retinopathy of prematurity.

In an effort to minimize surgical and visual morbidity of cryotherapy for retinopathy of prematurity (ROP), 18 eyes of 13 patients with 3 to 7 clock hours of stage 3 ROP with "plus" disease were treated by cryotherapy applications limited to the avascular retina adjacent to the areas of stage 3 disease. In 17 of 18 eyes, this limited use of cryotherapy was sufficient to cause regression of ROP without further treatments. After at least 3 months follow-up, ROP outcome showed a normal macular appearance in 16 eyes; two eyes developed macular dragging; no retinal detachments occurred.

Retained intravitreal lens fragments after cataract surgery.

PURPOSE: The purpose of this study is to review the possible benefits and complications of vitrectomy for retained lens fragments after cataract surgery. METHODS: The authors reviewed the charts of 65 patients referred over a 12-year period for retained lens fragments after cataract surgery. Of these, 56 underwent vitrectomy and 9 were followed. Of these 56 eyes, 29 (52%) had received an intraocular lens (IOL) at the time of cataract surgery. RESULTS: Resulting complications from retained lens material included glaucoma (52%), corneal edema (46%), uveitis (56%), and decreased vision (100%). These sequelae responded equally to vitrectomy in eyes with or without an IOL and irrespective of type of cataract surgery (phacoemulsification or extracapsular cataract extraction). The timing of surgery did not statistically influence the final vision or the incidence of glaucoma. CONCLUSION: Removal of retained lens fragments allows rapid visual restoration, enhances resolution of uveitis, and improves control of glaucoma. Insertion of an IOL at the time of cataract surgery in the face of dislocated lens fragments is not contraindicated provided that it could be performed safely.

Vitrectomy in eyes at risk for macular hole formation.

Fifteen eyes believed to be at increased risk for macular hole formation underwent vitrectomy in an attempt to prevent macular hole formation. Full-thickness macular holes have not developed in 10 of 11 eyes with stage 1 macular holes. Four eyes were noted to have small full-thickness foveal defects (stage 2 macular holes) at the time of vitrectomy. Two of the four eyes have not progressed to macular hole formation and have 20/25 visual acuity. All patients have been followed for a minimum of 13 months (median, 18 months). The 12 eyes that have not experienced macular hole formation have had a significant (P less than 0.001) improvement in vision with seven (58%) attaining visual acuity of 20/25 or better. The postoperative foveal electroretinogram (ERG) amplitude was higher than the preoperative amplitude in five of the six eyes tested.

Prediction of postoperative vision in eyes with severe trauma.

The single most important factor determining the potential for return of useful vision in severely injured eyes is the magnitude of damage incurred by the macula or optic nerve at the time of injury. Most gravely injured eyes have media opacities that prevent funduscopic examination. In such eyes, the flash visually evoked potential is the single best predictor of postoperative vision. The second most reliable predictor is the bright-flash electroretinogram. Ultrasonography is an important part of the preoperative assessment of injured eyes with opaque media, but is of less value than electrophysiological testing in predicting postoperative vision in eyes with major trauma.

Procoagulant effects of intraocular sodium hyaluronate (Healon) after phakic diabetic vitrectomy. A prospective, randomized study.

A procoagulant effect was reported previously after injection of 0.50 to 0.75 ml of sodium hyaluronate (Healon) into the vitreous cavity at the end of phakic diabetic vitrectomies. A multicenter, randomized, prospective trial was done to study this effect. In the treatment group the media was clear in 50% of eyes in the immediate postoperative period compared with only 8% in the control group. At 2 to 6 weeks, there was no statistically significant difference between the two groups. The authors conclude that such sodium hyaluronate injection provides clear media in a significant number of eyes in the immediate postoperative period, potentially allowing thorough postoperative evaluation and early retinal photocoagulation if needed.

Temporary keratoprosthesis for combined penetrating keratoplasty, pars plana vitrectomy, and repair of retinal detachment.

The Landers-Foulks temporary keratoprosthesis was used to combine penetrating keratoplasty, pars plana vitrectomy, and scleral buckling in the management of 13 eyes with opaque cornea and posterior segment abnormalities. In seven cases, trauma precipitated the ocular disease. Complications of cataract surgery resulted in anterior and posterior segment pathology in six cases. The corneal graft was initially clear in all cases. However, corneal edema complicated phthisis bulbi in four cases and followed homograft reaction in two cases. Eight eyes with retinal detachment (RD) preoperatively were successfully reattached. In five eyes, the retina redetached as these eyes became phthisical. Visual function improved in six cases. In general, eyes with a history of trauma had a much poorer outcome than did eyes with anterior and posterior segment problems related to previous cataract surgery.

Cataract extraction in the diabetic eye after vitrectomy.

We examined 289 phakic, diabetic eyes for evidence of cataract formation after a successful vitrectomy. Of 289 eyes, 107 (37%) developed or had significant progression of a cataract over a follow-up period of 16 to 108 months. Postvitrectomy hemorrhage and vitreous lavage were associated with an increased rate of cataract formation. Of the 107 eyes, 33 (31%) subsequently had cataract extraction, with 24 eyes (73%) treated by extracapsular cataract extraction and nine eyes (27%) with intracapsular surgery. A posterior chamber lens was used in 11 of the 24 eyes that underwent extracapsular surgery. Of the 33 eyes, 31 (94%) had improved visual acuity after cataract extraction. Intraoperative and postoperative complications were minimal and did not result in permanent reduction of vision. Intraocular lenses were well tolerated in the 11 patients in whom they were used.

Factors influencing final visual results in severely injured eyes.

We reviewed 194 consecutive eyes treated by pars plana vitrectomy for severe injury with posterior segment involvement. By using multivariate analysis, we determined which of a large number of clinical, ultrasonographic, and electrophysiologic factors influenced or predicted postoperative visual acuity. Additionally, the effects of the timing of vitrectomy on final vision and the usefulness of prophylactic scleral buckling were analyzed. We found that although clinical data have predictive value, it is electrophysiological information, specifically the visual-evoked potential, which most accurately predicts the postoperative vision of an injured eye. We could not demonstrate a significant effect of the timing of surgery on the final visual result but could determine that prophylactic scleral buckling clearly reduces the rate of delayed retinal detachments.

Diabetic traction retinal detachment. Factors influencing final visual acuity.

A group of eyes with clear media and diabetic traction retinal detachments treated with vitreous surgery were statistically analyzed to identify those parameters that would allow us to improve case selection. The configuration of the retinal detachment proved to be important, with hammock and flat diffuse central detachments having a good prognosis, and highly elevated, atrophic detachments such as table-top and tent-shaped demonstrating a poor prognosis. Eyes with florid diabetic retinopathy did less well than those with more inactive forms of retinopathy. Preretinal membranectomy, although accompanied by an increased rate of iatrogenic retinal tears, has a definite role in the treatment of more complex traction detachments and increases the number of eyes that may benefit from surgery. Eyes with preoperative vision better than 5/200 did significantly better than eyes with poorer preoperative vision.

Anterior chamber suture in pseudophakic retinal detachments requiring intraocular gas.

We have devised a simple technique to facilitate the use of vitreous cavity gas in complicated cases of pseudophakic retinal detachment with pupillary support intraocular lenses. A 10-0 monofilament suture is placed across the anterior chamber just prior to instilling gas in the vitreous cavity. This retaining suture prevents the pseudophakos from being displaced forward by the gas bubble, possibly damaging the corneal endothelium. The anterior chamber suture is removed easily at the slit-lamp examination several days postoperatively when intraocular gas is no longer present.

Ultrasonographic features of choroidal malignant melanomas.

In a 12-month period 26 eyes were enucleated after an ultrasonographic diagnosis was made of malignant melanoma of the choroid. Histologic features of the tumors were reviewed. The ultrasonographic diagnosis was correct in 100% (26/26) of enucleated eyes. Spontaneous vascular movement (SVM) was identified by standardized A-scan echography in 69% (18/26) of the tumors. Contact B-scan ultrasonography demonstrated choroidal excavation in 65% (17/26) of the melanomas. During the 12-month study period, choroidal excavation was also demonstrated in cases of metastatic choroidal tumors, hemangiomas of the choroid, and long-standing choroidal nevi. The standardized A-scan finding of SVM is a compelling sign in the diagnosis of choroidal malignant melanoma. In contrast, the B-scan finding of choroidal excavation is a less useful sign since it may also be seen in various other ocular lesions.

Retinal detachment and pseudophakia.

A consecutive series of pseudophakic retinal detachments was statistically compared with a matched group of aphakic retinal detachments. Distribution of breaks, anatomic results, and final visual acuities were tabulated. Success in reattachment was equal in the two groups, but final visual acuities were better in the aphakic group. Emphasis was placed on the intraoperative ophthalmoscopic examination of pseudophakic eyes with compromised peripheral fundus visibility.