Acute annular outer retinopathy.
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OBJECTIVES: To assess the validity and reproducibility of quantitating relative afferent pupillary defects with the swinging flashlight test, using a 3-second pause technique, and to compare this grading system with that using neutral density filters. DESIGN: We prospectively studied patients with relative afferent pupillary defects by using the swinging flashlight test with a standardized illumination time of 3 seconds (pause time) for each eye. Each relative afferent pupillary defect was graded as follows: grade I, a weak initial constriction and greater redilatation; grade II, initial stall and greater redilatation; grade III, immediate pupillary dilatation; grade IV, immediate pupillary dilatation following prolonged illumination of the good eye for 6 seconds; grade V, immediate pupillary dilatation with no secondary constriction. The grades on the individual resident evaluations were compared with those on the group evaluations, and the grading system was compared with the neutral density filter grading system. SETTING: Neuro-ophthalmology service of an urban teaching hospital. STUDY PARTICIPANTS: A total of 119 patients. RESULTS: There was a good correlation in all grades between individual resident and group evaluations, with an overall 78% agreement. The five grades had corresponding values in neutral density filter log units: grade I, 0.4; grade II, 0.7; grade III, 1.1; grade IV, 2.0; and grade V, infinity. CONCLUSIONS: The 3-second pause technique for the swinging flashlight test is a reliable method for the detection and quantitation of relative afferent pupillary defects. The grading system can be compared with the neutral density filter grading system, and each grade has a corresponding value in log units.
OBJECTIVE: To determine and compare the incidence of atonic pupil following modern extracapsular cataract extraction and small-incision phacoemulsification with lens implantation in a large series of consecutive patients. PATIENTS AND DESIGN: A retrospective review was undertaken of 1283 consecutive cases of cataract surgery with posterior chamber intraocular lens implantation (919 extracapsular and 364 small-incision phacoemulsification) performed by two surgeons between October 30, 1991, and June 30, 1993. Postoperative atonic pupil was defined as a pupil that, following cataract surgery, was 6 mm or more in diameter and minimally or nonreactive to light, accommodation, or 1% pilocarpine hydrochloride. RESULTS: Postoperatively, 25 (1.95%) of 1283 eyes developed an atonic pupil following cataract extraction with posterior chamber intraocular lens implantation (21 [2.29%] of 919 extracapsular and four [1.10%] of 364 phacoemulsification procedures). When an atonic pupil was present following surgery, disturbing glare often resulted, despite a well-centered intraocular lens. CONCLUSIONS: To our knowledge, this is the first large, consecutive study of the incidence of atonic pupil following modern cataract surgery and the first study to compare the incidence of atonic pupil following extracapsular surgery vs small-incision phacoemulsification. Although the incidence of atonic pupil may be lower following phacoemulsification, caution is advised in the implantation of ever-smaller optic lenses.
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OBJECTIVE: To assess possible causes of visual field loss following vitreous surgery. DESIGN: Charts of 8 patients prospectively identified, who developed visual field loss following vitreous surgery, were reviewed to characterize this newly recognized syndrome and assess possible causes. RESULTS: Two patients had preexisting chronic open-angle glaucoma and 1 had ocular hypertension. Indications for surgery included 4 eyes with macular holes, 1 eye with epiretinal membrane, 2 eyes with rhegmatogenous retinal detachment, and 1 eye with retinal detachment and giant retinal tear. All patients received retrobulbar anesthesia. Seven of 8 patients had fluid/gas exchange with installation of long-acting bubbles. In 1 patient with a macular hole, a small hemorrhage was noted along a vessel coming off the nerve superotemporally while attempting to engage the posterior cortical vitreous intraoperatively. This patient developed an inferior visual field defect. No intraocular pressure (IOP) measurements greater than 26 mm Hg were recorded in any eye perioperatively. Visual field defects included 4 eyes with inferotemporal defects, 2 eyes with inferior altitudinal defects, 1 eye with a cecocentral scotoma, and 1 eye with a superonasal defect. Only 1 patient had worsened visual acuity. A relative afferent pupillary defect was observed in 4 eyes and disc pallor in 5 eyes. CONCLUSIONS: Central or peripheral visual field loss can now be recognized as a possible complication of vitreous surgery. In some cases, a relative afferent pupillary defect and optic disc pallor are present, suggesting that the optic nerve is the site of injury. Possible mechanisms include ischemia due to elevated IOP or fluctuations in IOP, optic nerve damage from retrobulbar injection, direct intraoperative mechanical trauma to the optic nerve, indirect injury from vigorous suction near the optic nerve leading to shearing of peripapillary axons or vessels, or a combination of these. Certain optic nerves may be more susceptible to injury because of preexisting compromise from glaucoma or vascular disease.
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We observed the occurrence of congenital nephrotic syndrome (CNS) and distinct ocular anomalies in two unrelated families. Eleven children from both families presented with a similar course of renal disease starting with nephrotic syndrome and renal failure prenatally or immediately after birth that resulted in death before the age of 2 months. Kidney histopathology showed diffuse mesangial sclerosis (DMS). Clinically obvious eye abnormalities were recognized in six of the eight patients in whom sufficient clinical data were available. Ocular anomalies included enlarged or large appearing corneae in some cases suggesting buphthalmos, and extremely narrow, nonreactive pupils (microcoria). Pathological examination of the eyes of two aborted fetuses revealed a more complex ocular maldevelopment including posterior lenticonus as well as anomalies of cornea and retina. On the basis of these observations and other cases in the literature, we delineate a previously unrecognized distinct entity characterized by congenital nephrotic syndrome, DMS, and eye abnormalities with microcoria as the leading clinical feature. Pedigrees of affected families with parental consanguinity support autosomal recessive inheritance. We propose that this syndrome should be designated microcoria-congenital nephrosis syndrome or Pierson syndrome. Possible overlap with Galloway-Mowat syndrome and relations to other oculo-renal syndromes are discussed.