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The blind and painful left eye of a 88-year-old woman was enucleated and examined by light and transmission electron microscopy. Three years earlier, after unilateral pseudoexfoliation syndrome with advanced open angle glaucoma of the left eye had been diagnosed, argon laser trabeculoplasty and pars plicata diathermy were performed. Light microscopy showed deposits of typical pseudoexfoliative material in the anterior segment and endothelialization of the chamber angle. By transmission electron microscopy we demonstrated pseudoexfoliative fibers not only at the typical intraocular locations and in the bulbar conjunctiva but also ultrastructurally identical material in hitherto not described extraocular locations such as extraocular rectus and oblique muscles, vortex veins and the optic nerve sheaths. Ultrastructurally, the fibers showed all the characteristic features of pseudoexfoliative material. Our findings are another strong indication that pseudoexfoliation syndrome is not a purely intraocular disorder but represents a more widespread disease.

We report on a 17-year-old male with an asymptomatic yellowish-grayish solitary tumor of the right medial upper lid margin. An excisional biopsy was performed and histopathologic examination revealed subepidermal calcinosis. The other eye showed a granulomatous chrorioretinits of unknown etiology. The relative rare occurrence of subepidermal calcification should be kept in mind when making a differential diagnosis of superficial lid tumors. In our histopathology laboratory this is the first diagnosis of subepidermal calcinosis among 1,400 comparable specimens from 1980 to 1990. A possible relationship between the subepidermal calcification and granulomatous chorioretinitis of the other eye is discussed.

Between July 1980 and February 1990 we performed 102 penetrating keratoplasties in 102 eyes of 98 patients with pseudophakic bullous keratopathy. Patients were followed up prospectively with an average follow-up of 14 months. 53 of the implant were iris-supported lenses (ISL), 32 were anterior chamber lenses (ACL), and 17 were posterior chamber lenses (PCL) following extracapsular cataract extraction (ECCE). In 88 eyes the intraocular lens implantation had been performed elsewhere. The interval between lens implantation and keratoplasty ranged from 3 to 451 months (mean 59 months) and was significantly shorter (p less than 0.03) for PCL. Eight intraocular lenses had been removed before keratoplasty. During penetrating keratoplasty, 25 lenses were left in place, 49 were removed, and 20 were exchanged. Since 1988, PCL have been implanted and sutured to the iris after removal of the ACL or ISL in 17 eyes that had undergone intracapsular cataract extraction (ICCE). After keratoplasty, visual acuity improved in 95 of the 102 eyes. However, visual acuity was frequently limited by persisting cystoid macular edema. In eyes that had undergone keratoplasty following ECCE and PCL implantation, visual acuity was significantly better (p less than 0.03) and cystoid macula edema was rarer (p = 0.07) than in eyes with ISL or ACL. the prognosis of penetrating keratoplasty for pseudophakic bullous keratopathy is generally good, although visual acuity is often limited postoperatively by cystoid macular edema. The technique of secondary PCL implantation sutured to the iris appears promising for allowing better optic rehabilitation of patients with pseudophakic bullous keratopathy.

We document a case of unilateral hemiarcus lipoides, which developed de novo 10 months after trabeculectomy in the left eye of a 54-year-old woman. The involved eye showed heterochromia complicata Fuchs and had undergone intracapsular cataract extraction 11 years and trabeculectomy one year earlier. Except for intermittent hypercholesterolemia, the patient did not present any signs of systemic disease. The possible roles of relative ocular hypotony and modified aqueous circulation after trabeculectomy in the development of unilateral hemiarcus lipoides are discussed.

The present paper reports on the clinical findings of a 34-year-old male patient with MELAS syndrome. MELAS syndrome (mitochondrial encephalomyopathy, lactic acidosis and strokelike episodes) belongs to a group of syndromes called mitochondrial encephalomyopathies that are characterized by changes of the mitochondrial respiratory chain and the histological finding of "ragged red fibers" in muscle biopsy. In our case the diagnosis was confirmed by multiple neurologic tests including muscle biopsy and biochemical analysis of the respiratory chain. The ocular findings included reversible, homonymous hemianopic visual field loss documented six years earlier, atypical retinitis pigmentosa with marked attenuation of the scotopic ERG, myopia and nuclear cataract of the right eye. An extracapsular cataract extraction with implantation of a posterior chamber lens was performed on the rigt eye, the course was unremarkable and vision improved. In dealing with patients presenting with ocular or neurologic signs indicating mitochondrial encephalopathy, the ophthalmologist should consider MELAS syndrome or any other of the mitochondrial encephalomyopathy syndromes as a possible etiology and take the necessary steps for further medical and neurologic evaluation of the patient.

Between September 1986 and June 1988 310 penetrating keratoplasties were performed and prospectively analyzed. The purpose of this prospective study was the evaluation of graft rejections and the underlying corneal diseases. The mean follow-up time was 6.9 months. We found early graft rejections in 5.8% of the eyes. The increased rate of graft rejection in eyes with herpetic corneal ulcers was remarkable.

We report the results of direct cyclopexy performed on all 16 eyes of 16 patients between 1980 and 1988. The follow-up was 7 months to 95 months (mean 51.4 months). All eyes had developed persistent ocular hypotony following traumatic (15 eyes) or surgical (1 eye) cyclodialysis. The cyclodialyses extended from 45 degrees-255 degrees (mean 120 degrees). Preoperative intraocular pressure was 0 to 8 mm Hg (mean 2.9 mm Hg); preoperative visual acuity ranged from light perception to 20/50. Direct cyclopexy by suture of the insertion of the ciliary muscle to the scleral spur with 10/0 nylon was performed in 16 eyes according to the technique described earlier. In addition, 7 eyes simultaneously underwent the following operative procedures: tectonic keratoplasty (2 eyes), intracapsular cataract extraction and anterior vitrectomy (3 eyes), extracapsular cataract extraction and implantation of a posterior chamber lens (1 eye), and encircling procedure (1 eye). During and after cyclopexy no severe complications were observed. During the immediate postoperative course four eyes developed temporary increased intraocular pressure (26 to 50 mm Hg), which was controlled by medication and was fully reversible. After cyclopexy the cyclodialysis cleft was closed in 15 of 16 eyes with a small persisting cleft (30 degrees) without ocular hypotony persisting in one eye. No new anterior synechiae developed. Visual acuity improved in 14 of 16 eyes (20/80 to 20/20) being 20/40 or better in 10 eyes. In only two eyes was there no improvement in visual acuity as a result of severe traumatic posterior changes. During further postoperative follow-up, the intraocular pressure was below 20 mm Hg in all eyes.(ABSTRACT TRUNCATED AT 250 WORDS)

A prospective study was undertaken to evaluate the risk factors in extracapsular surgery for eyes with pseudoexfoliation syndrome (PES) with and without open angle glaucoma. The study included 3095 planned extracapsular cataract extractions performed by 18 surgeons between September 1986 and June 1989. Of the 3095 eyes 153 (4.9%) showed clinical signs of PES. We examined three groups of eyes: nonglaucomatous eyes (2476 eyes), glaucomatous eyes controlled by local antiglaucomatous therapy (342 eyes) and glaucomatous eyes that had undergone antiglaucomatous surgery (277 eyes). For each group, the incidence of capsular breaks and vitreous loss was evaluated for eyes with and without PES. In the group of nonglaucomatous eyes, vitreous loss occurred significantly more frequently (p less than 0.05) in eyes with PES. For the group of glaucomatous eyes, complications were more frequent but not significant in the presence of PES (p greater than 0.15). We conclude that PES is a significant risk factor in extracapsular surgery even in the absence of glaucoma. After introducing special precautionary measures for eyes showing signs of PES, the incidence of complications in extracapsular surgery has decreased considerably in our hospital for these eyes.

Between July 1980 and March 1987 the authors performed pseudophakic penetrating keratoplasty in 56 eyes with bullous keratopathy following implantation of an intraocular lens. The postoperative results in 50 eyes (47 patients) are reported in this retrospective study. Average duration of follow-up after keratoplasty was 8.1 months. The intraocular lenses were either iris-supported (31), anterior chamber lenses (12), or posterior chamber lenses (7). The interval between implantation of the lens and performance of the keratoplasty ranged from three to 451 months (average 69.9 months). It varied considerably, depending on the type of lens used. Vision after penetrating keratoplasty improved in 44 of the 50 eyes and was unchanged in three. In ten eyes, improvement in vision was limited by persistent cystoid maculopathy (CMP). Postoperative complications occurred in five eyes and traumatic wound dehiscence in two. However, none of the transplants showed any signs of rejection during the follow-up period. The prognosis for penetrating keratoplasty following intraocular lens implantation is generally good; impaired vision is due to extracorneal factors, in particular CMP.

Patients with myotonic dystrophy show ocular hypotony associated with primary hypogonadism and a secondary increase in the gonadotropins FSH and LH. By examining ten patients (nine men, one woman) with myotonic dystrophy and determining serum FSH and LH concentrations by radioimmunoassay, the authors tried to evaluate whether the decrease in intraocular pressure (IOP) seen in myotonic dystrophy might be related to increase levels of gonadotropins. The IOP of the patients with myotonic dystrophy (mean 10.6 mm Hg, SD 3.9 mm Hg) was significantly lower (P less than 0.0005) than that of a randomly selected control group. In all nine male patients serum FSH concentrations (mean 41.7 mIU/ml, SD 18.9 mIU/ml, normal range 0.9-9.8 mIU/ml) and serum LH concentrations (mean 18.6 mIU/ml. SD 6.8 mIU/ml, normal range 2.2-12.0 mIU/ml) were significantly higher (FSH: P less than 0.0005, LH: P less than 0.05) than those of a control group of four normal men. The female patient's FSH was elevated (11.5 mIU/ml, normal range 1.7-8.5 mIU/ml), her LH concentration was slightly elevated (15.5 mIU/ml, normal range 2.5-15.4 mIU/ml). A statistically significant correlation was found between elevated LH concentrations and decreased IOP (P less than 0.05) but no such correlation between FSH and IOP (P less than = 0.17). Endogenous circulating gonadotropins may play an important role in regulating aqueous inflow via cyclic AMP which is produced by ciliary epithelia and causes a decrease in the rate of aqueous humor production. This theory is corroborated by humor production. This theory is corroborated by the authors' data and by experimental studies by Sears and co-workers.

A prospective study of preoperative risk factors in extracapsular cataract surgery was undertaken. The study included 2042 planned extracapsular cataract extractions in 1772 patients performed by 12 surgeons between February, 1986 and June, 1988. The possible risk factors to be analysed included sex, age, right or left eye, axial length, pseudoexfoliation syndrome, preceding blunt ocular trauma, diabetes mellitus and systemic hypertension. Capsular breaks occurred in 2.9% and vitreous loss in 1.9% of our patients. A significant increase in the frequency of capsular breaks was observed for patients under the age of 41 and for patients with diabetes mellitus, vitreous loss being observed significantly more frequently in eyes with pseudoexfoliation syndrome. A slight but insignificant increase in capsular breaks was observed for male patients, left eyes, eyes smaller than 22 mm, pseudoexfoliation syndrome and systemic hypertension. Advanced age, myopia and previous blunt trauma were not found to be significant risk factors for capsular breaks or vitreous loss. We recommend careful preoperative patient examination to identify preoperative risk factors and possibly avoid intraoperative complications.

A prospective study for evaluation of the pseudoexfoliation syndrome (PES) as a risk factor in extracapsular surgery was undertaken from February 1986 to June 1988. The study included 2042 planned extracapsular cataract extractions performed by 12 surgeons. Seventy-two eyes revealed PES (3.5%). Frequency of PES increased with age (p less than 0.005). The male-female ratio of PES was 1:1.6, and eyes of average axial length showed PES more frequently than myopic or hyperopic eyes, although these differences were not significant statistically. The incidence of vitreous loss was 11.1% in eyes with PES and 1.6% in eyes without PES, a sevenfold increase that was statistically significant (p less than 0.0001). Rupture of the posterior lens capsule was observed in 4.2% of PES eyes compared to 2.8% of eyes without PES (no significance). The pseudoexfoliation syndrome is not a harmless anomaly, but a potentially disastrous disease of the anterior segment of the eye. As precautionary measures we recommend thorough biomicroscopic examination preoperatively for signs of PES, iridotomy or sector iridectomy in cases with poorly dilating pupils, very gentle manipulation intraoperatively, and posterior chamber lens sulcus implantation in eyes with zonular defects or phacodonesis.