Paradoxic pupillary constriction in a patient with congenital stationary night blindness.

Paradoxic pupillary constriction to an "off-step" of light was studied in a 35-year-old woman with congenital stationary night blindness. ERG was of the Schubert-Bornschein type (scotopic B-wave absent); fundi were normal for a high myope. Paradoxic constrictions were larger in response to full field and peripheral off-steps from low photopic levels than from higher photopic levels. The patient's "on response" was smaller in magnitude and slower in both latency and dynamics than the on-response of normal subjects. Pupillary hippus was larger in magnitude and more peaked in the patient than in normal subjects. Steady-state (tonic) pupil size increased paradoxically with increased light level over the range 1-2 log fL.

Paradoxical pupillary responses in congenital stationary night blindness.

Three unrelated boys, ages 2, 6, and 10 years, who have congenital stationary night blindness with myopia and a Schubert-Bornschein-type electroretinogram finding, were found to show a "paradoxical" pupillary constriction in darkness. When examining room lights are turned out, the patient's pupils briskly constrict and slowly dilate. Older night blind male relatives of these boys did not show this abnormal constriction to darkness.

The value of routine dilated pupil screening examination.

We evaluated the cost-effectiveness of routine dilated fundus examination in improving visual outcomes. The cost of routine dilated fundus examination was related to the number of preventable cases of vision-threatening peripheral retinal disease. Patients with these diseases who had no risk factors were ascertained in a population of 1.75 million adults for a period of 6 months. Those whose last examination had been undilated were identified because only for them could routine dilated examination (RDE) have been substituted for undilated examination. The number of preventable cases was calculated for multiples of a 10% probability of prevention. The cost of RDE was determined from the number of undilated examinations in the same population and period and the cost of a single RDE. The number of patients who underwent undilated examination was estimated by random medical record review. The additional cost of a single RDE was determined from estimated examination times and payroll costs. Among patients without risk factors, 38 were identified for whom undilated examination rather than RDE had been performed. If prevention had been 10% effective, the substitution of 50,000 RDEs for undilated examinations costing the provider $433,000 would have been required per prevented case. These results suggest that most peripheral retinal diseases cannot be prevented by RDE. Routine dilated examination is an expensive test per prevented case. Published clinical guidelines lack evidence to recommend its use.

Vitelliform lesions developing in normal fundi.

Two patients with Best's macular dystrophy, photographically documented, developed vitelliform lesions in previously normal or nearly normal fundi. The patients were studied prospectively because their brother had Best's disease. The first patient, a 6-year-old boy, had normal-appearing fundi in February 1974, but in October 1974 he had developed a vitelliform lesion in the left macula. His electro-oculogram (EOG) was severely abnormal in both eyes. His 12-year-old syster had an essentially normal fundus appearance and an abnormal EOG in both eyes in November 1971. In October 1975, she had an extramacular vitelliruptive lesion in the right eye and a typical Best's lesion in the left macula.

Bare sclera closure in retinal detachment surgery.

Our modification of the limbal peritomy technique in retinal detachment surgery involved recession of the confunctiva 3 mm posterior to the corneoscleral limbus as the conjunctiva was closed. This recession offered some advantages over the standard nonrecessed method: fewer sutures were required; postoperative ophthalmoscopy was not impaired by conjunctiva swelling over the cornea; the eye was comfortable and quiet in appearance because there was no suture material or inflamed conjunctiva adjacent to the corneoscleral limbus. We have not encountered any disadvantages in several hundred cases.

A technique for improving corneal clarity during retinal surgery.

To maintain corneal clarity during scleral buckling operations, the surgeon firmly rolls a dry cotton applicator across the edematous corneal surface, and the epithelial edema fluid is pressed out and absorbed by the applicator. This technique, which may be repeated a number of times, reduces the number of cases that require removal of the epithelium.

Degenerative retinoschisis with giant outer layer breaks and retinal detachment.

Five unusual cases of retinal detachment were caused by giant (more than 90 degrees) posterior breaks in the outer layer of degenerative retinoschisis. All retinas were successfully reattached by a variety of surgical techniques. The two cases managed with scleral buckling required subsequent buckle removal because of macular distortion. Treatment with cryotherapy or laser, together with intraocular air and postoperative positioning, gave better anatomic results.

Synergism between diabetic and radiation retinopathy: case report and review.

It is suspected that radiation retinopathy is more likely to develop in an eye with preexisting diabetic retinopathy than in a normal eye. However, there is only one report of this occurring, at a radiation dose of 4500 rads. We present a woman with minimal diabetic retinopathy who had breast carcinoma which was treated with chemotherapy but metastasised to the choroid. Within nine months of external beam radiation (3000 rads in fractions of 200 rads) a fulminant retinopathy evolved in that eye, while the non-radiated eye showed no change. The histopathology of radiation and diabetic retinopathy and causes for possible synergism are discussed. As this case report shows, radiation oncologists and ophthalmologists need to be aware of the risk that patients with minimal diabetic retinopathy who have undergone chemotherapy may suffer a dramatic visual loss from radiation therapy despite a radiation dose which is considered adequate, safe, and properly fractionated.

Extended retinal tamponade in the treatment of retinal detachment with proliferative vitreoretinopathy.

We have used intraocular sulphur hexafluoride or liquid silicone as an adjunct to vitreous surgery in the treatment of a non-randomised sequential series of 19 eyes with retinal detachment complicated by proliferative vitreoretinopathy. We have studied the surgical results and complications of these two tamponades and drawn preliminary conclusions on their use in retinal reattachment surgery. After a seven-month minimum follow-up 13 (68%) of the eyes have reattached retinas. Six (60%) of 10 eyes treated only with silicone have reattached retinas, and four (67%) of six eyes treated only with sulphur hexafluoride gas tamponade have reattached retinas. An additional three eyes treated initially with silicone oil subsequently developed retinal detachments; all were successfully reattached with sulphur hexafluoride tamponade after silicone oil removal. Intraoperative pneumatic retinal reattachment to assess relief of retinal traction combined with the production of widespread chorioretinal adhesions to wall off persistent anterior traction and detachment, as well as extended postoperative gas tamponade of the retina, appears to enhance the surgical results in retinal detachment complicated by proliferative vitreoretinopathy. Silicone oil tamponade of the retina appears to be useful in cases where retinal traction cannot be entirely relieved and in patients who are unable to tolerate the head positioning required for effective gas tamponade of the retina. A controlled clinical study recently begun will be required to define further the precise role of these methods of retinal tamponade.