Aicardi syndrome in one dizygotic twin.

The Aicardi syndrome consists of infantile spasms, defects of the corpus callosum, dorsal vertebral anomalies, and chorioretinal lacunar defects. The etiology is, as yet, unknown. The most likely cause, however, is an X-linked mutational event that is lethal in males. The first case of the Aicardi syndrome known to occur in one twin is reported. The patient was female and her unaffected sibling was male. This provides strong evidence to support the theory of an X-linked mutational event as the cause of this condition. The typical chorioretinal defects, often difficult to document because these children die at an early age, are clearly illustrated in this report.

Tomographic measurements of in vivo cataracts by slit-lamp photography.

A practical method for detecting in vivo cataracts has been developed using a commercially available slit-lamp camera and by utilizing a measuring grid photographic technique. The basis of the method is to photograph a series of slit-beam sections through a lens at varying angles to the optic axis. The areas of opacity show up as localized regions of light back-scatter where the opacities intersect the path of the beam. A set of measuring grids compensated for angle distortion have been prepared. These are superimposed on the appropriate photograph. By taking a sufficient number of sections through a cataractous lens, a tomographic representation of the opacities can be constructed using a lens map. An example of the procedure of three-dimensional mapping is presented using actual lens pictures. The photographic parameters used in this method are explained in detail. The method achieves the goals of ease of use, reproducibility, and applicability to research and clinical studies.

Use of an angiotensin converting enzyme inhibitor in ocular hypertension and primary open-angle glaucoma.

We studied the effects of a new topical angiotensin converting enzyme inhibitor, SCH 33861, in lowering intraocular pressure in 20 patients with ocular hypertension or primary open-angle glaucoma. In a double-masked, four-way crossover study with placebo and timolol, SCH 33861 was well tolerated and effective in lowering intraocular pressure. The magnitude of the drug's effect in lowering intraocular pressure was less than that of timolol 0.5%.

Control of intraocular pressure in glaucomatous eyes after extracapsular cataract extraction with intraocular lens implantation.

Two hundred sixty eyes of 195 patients having extracapsular cataract extraction (ECCE) with insertion of a posterior chamber intraocular lens were studied retrospectively. Of these cases, 160 eyes had preexisting glaucoma while 100 had no ocular pathology except for cataract. Intraocular pressure decreased significantly after ECCE in both groups and slowly returned to baseline within two years. The average postoperative visual acuity was better in the control group than in the glaucoma group. Patients with glaucoma were controlled with less medication after surgery. We believe that ECCE with insertion of a posterior chamber intraocular lens can be safely performed in glaucoma patients and has a beneficial effect on the control of glaucoma.

Superficial epithelial keratectomy in the treatment of epithelial basement membrane dystrophy.

Thirty-three eyes with symptomatic epithelial basement dystrophy, unresponsive to current standard modalities of therapy, were treated with a total superficial epithelial keratectomy. Follow-up was from 2 months to 7 years 5 months with an average of 47 months. All patients were relieved of their preoperative symptoms and only 3% have shown any evidence of recurrence of their dystrophic process.

Corneal topography and photokeratoscopy.

A keratometer measures the radius of curvature of the two principal meridians that correspond to the steepest and flattest meridians, by projecting an illuminated circle onto the corneal surface from a fixed distance. A photokeratoscope allows a permanent record to be made and further evaluated. It takes a picture primarily of the intermediate zone of the cornea. By measuring the distance between reflected rings and comparing them to the actual distance between the rings on the instrument, the curvature of the cornea can be ascertained at any given point.

Superficial epithelial keratectomy in the treatment of epithelial basement membrane dystrophy.

Thirty eyes with symptomatic epithelial basement membrane dystrophy that were unresponsive to current standard modalities of therapy were treated with a total superficial epithelial keratectomy. Follow-up ranged from two months to 89 months (average, 37 mo). One hundred percent of patients were relieved of their preoperative symptoms, and only 3% have shown any evidence of recurrence of their dystrophic process.

A modified extracapsular cataract extraction for pediatric cataracts.

We present a modified procedure of extracapsular cataract extraction (ECCE) with a small central posterior capsulectomy for pediatric cataracts that is designed to eliminate posterior capsule opacification and to keep open the option of later secondary implantation of a posterior chamber intraocular lens. Of 76 study eyes in which the procedure was performed only three developed posterior capsule opacification. All seven control eyes that had standard ECCE with the posterior capsule left intact developed secondary membranes shortly after surgery.