Gerald W. Zaidman

A Light and Electron Microscopic Study of Recurrent Granular Dystrophy of The Cornea

Light and electron microscopic studies in three cases of recurrent granular dystrophy with corneal epithelial involvement showed intracellular rod and trapezoidal crystalline granules characteristic of granular dystrophy. Small intraepithelial cytoplasmic granules were rimmed by 15-nm particles, while larger deposits were often membrane-bound. The granules were closely associated with tonofilaments. There were ultrastructural similarities to keratohyaline. Extracellular granules were often intermixed with cytoplasmic organelles of disintegrated squamous epithelial cells. There was no evidence of a subepithelial pannus. These findings suggest an epithelial origin of the granular dystrophic deposits in these recurrent cases.

Microphthalmia with linear skin defects (MLS) syndrome: Clinical, cytogenetic, and molecular characterization of 11 cases

The microphthalmia with linear skin defects (MLS) syndrome (MIM 309801) is a severe and rare developmental disorder, which is inherited as an X‐linked dominant trait with male lethality. In the vast majority of patients, this syndrome is associated with terminal deletion of the Xp22.3 region. Thirty‐five cases have been described to date in the literature since the first description of the syndrome in the early 1990s. We now report on the clinical, cytogenetic, and molecular characterization of 11 patients, 7 of whom have not been described previously. Seven of these patients have chromosomal abnormalities of the short arm of the X‐chromosome, which were characterized and defined by fluorescence in situ hybridization (FISH) analysis. Intriguingly, one of the patients displays an interstitial Xp22.3 deletion, which to the best of our knowledge is the first reported for this condition. Finally we report on the identification and molecular characterization of four cases with clinical features of MLS but apparently normal karyotypes, verified by FISH analysis using genomic clones spanning the MLS minimal critical region, and with genome‐wide analysis using a 1 Mb resolution BAC microarray. These patients made it possible to undertake mutation screening of candidate genes and may prove critical for the identification of the gene responsible for this challenging and intriguing genetic disease.

Incidence of Peters anomaly and congenital corneal opacities interfering with vision in the United States.

Purpose: The aim of this study was to determine the incidence of Peters anomaly (PA) and congenital corneal opacities (CCO) interfering with vision in the United States. Methods: We collected data from the Eye Bank Association of America (EBAA), the Eye-Bank for Sight Restoration (Eye-Bank) in New York City, the New York State Department of Health (NYSDH), and the Pediatric Keratoplasty Association (PKA). Results: The EBAA data for 1996 and 1997 indicate that approximately 44,000 corneal transplants are performed annually. Of them, at least 128 are performed in infants, for various etiologies. A survey by the members of the PKA on 1995 showed that 65% of all grafts in infants are performed for PA. The NYSDH data from 1992 to 1997 indicate that the incidence of CCO interfering with vision was 2.2 infants per 100,000. Of those, PA accounted for 1.5 per 100,000. Eye-Bank data from 1988 to 1997 indicate that 12 children (1.1 per 100,000) received 23 transplants on 19 eyes for PA. Conclusions: Combining the data from all 4 sources indicates that approximately 1 infant corneal transplant is performed for every 24,000 live births and most of all CCO interfering with vision is due to PA. Applying the NYSDH and Eye-Bank data to a national birth rate of 4 million, we would expect approximately 88 children born annually in the United States with CCO interfering with vision, with at least 44 to 60 being due to PA. Many of these children may require more than 1 transplant.

Postkeratoplasty keratoconus in a nonkeratoconus patient

PURPOSE To determine whether postkeratoplasty keratoconus is caused by a recurrence of the host disease or transferred from the donor. METHODS Penetrating keratoplasty was performed on the right eye of a 73-year-old woman with pseudophakic bullous keratopathy; her other eye was normal. After keratoplasty, visual acuity decreased secondary to the appearance of irregular astigmatism, central corneal thinning, and stromal striae in the graft. A repeat keratoplasty was performed. Clinical corneal topographic analysis and histopathologic studies were performed. RESULTS Clinical findings and histopathology of the corneal graft were consistent with keratoconus. The second graft has remained clear for 2 years without signs of keratoconus. CONCLUSION Classic keratoconus developed in a corneal graft in a patient without preexisting keratoconus. This supports the theory that postkeratoplasty keratoconus may be secondary to transfer of the disease from the donor.

Visual and refractive results of combined PTK/PRK in patients with corneal surface disease and refractive errors

PURPOSE: To investigate the changes in symptoms, refraction, and visual acuity (VA) in patients with corneal surface disease and refractive errors who had phototherapeutic keratectomy (PTK) combined with photorefractive keratectomy (PRK). SETTING: University‐based referral practice. METHODS: Patients with myopia or astigmatism and map‐dot‐fingerprint dystrophy or recurrent erosions were treated. The corneal epithelium was removed with a 64 blade, and laser was performed using a Visx Star S3 laser. RESULTS: Nineteen eyes of 14 patients were treated. Preoperatively, mean myopia was −3.76 diopters (D) (range −7.50 to −0.75 D), mean astigmatism was +0.96 D (range 0.00 to +2.25 D), and mean UCVA was 20/400. At 3 months, mean myopia was −0.53 D (range −1.75 to +0.75 D) (P<.001), mean astigmatism was +0.58 D (range 0.00 to +1.25 D) (P = .05), and mean uncorrected VA was 20/23 (P<.001). At 6 months, mean myopia was −0.31 D (range −1.00 to +0.75 D) (P<.001), mean astigmatism was +0.56 D (range 0.00 to +1.25 D) (P = .05), and mean uncorrected VA was 20/23 (P<.001). The change in astigmatism was confirmed by vector analysis. Only 1 patient lost 1 line of acuity, and all patients were asymptomatic. CONCLUSION: Patients with myopia and astigmatism and symptomatic epithelial basement membrane disorders who had PTK/PRK had resolution of their symptoms and nearly achieved emmetropia. Therefore, this procedure is safe and effective for patients with corneal surface disease and myopia.

Optical iridectomy for corneal opacities in Peter's anomaly

Abstract Three children were born with partial corneal opacity and anterior segment anomalies but no cataract (Peter’s anomaly type 1). In each affected eye, the corneal scar was off center and encroached on the visual axis. Glaucoma (if present) was controlled medically or surgically, after which an optical iridectomy was performed in each eye (in lieu of a penetrating keratoplasty). After surgery, in all patients the pupil extended beyond the corneal opacity and the corneal opacity decreased slightly. All could fixate and follow around the opacity. Optical iridectomy should be considered in selected cases of congenital corneal opacities.

Human Immunodeficiency Virus-positive Patients with Posterior Intracorneal Precipitates

PURPOSE To describe an unusual and possibly new keratopathy in human immunodeficiency virus (HIV)-positive patients. DESIGN Retrospective observational case series. PARTICIPANTS : Six HIV-positive patients at four centers. METHODS A complete medical history was obtained and a thorough ophthalmic examination was performed on each of the patients. RESULTS Each patient had bilaterally symmetrical keratopathy consisting of variably pigmented round and reticular posterior intracorneal precipitates at the level of Descemets membrane. The precipitates were diffuse, but larger and more prominent near the limbus and finer toward the central cornea. None of the eyes were inflamed, and all had normal vision. No patient had uveitis on presentation or any history of cytomegalovirus retinitis. CONCLUSION These patients have a unique posterior keratopathy, which requires further investigation.

Listeria monocytogenes Keratitis

We treated a farmer who had Listeria monocytogenes bacterial keratitis. Therapy with topical antibiotics was unsuccessful; it was necessary to treat the patient with topical and systemic penicillin and gentamicin. To elucidate the pathogenesis of this infection, we developed a rabbit model. Using the patients strain of L. monocytogenes, we determined that the severity of the rabbit infection was dose-related. If we used an inoculum of more than 10(7) organisms, many of the features of the human Listeria keratitis were mimicked. We also found that treatment with either penicillin or gentamicin did not control the infection as well as using both antibiotics simultaneously, a combination which resulted in relatively rapid resolution of infection and no corneal scarring. The human and animal data indicate that L. monocytogenes can be a virulent corneal pathogen. Listeria corneal infections must be treated aggressively with both penicillin and gentamicin to prevent permanent visual loss.

Successful surgical rehabilitation of children with traumatic corneal laceration and cataract.

OBJECTIVE To evaluate the visual and refractive outcome of corneal transplant surgery, cataract extraction, and intraocular lens (IOL) implantation in children with traumatic corneal laceration and cataract. DESIGN Retrospective, noncomparative case series. PARTICIPANTS Five patients, 7 years of age or younger, who underwent penetrating keratoplasty, cataract extraction, and IOL implantation for traumatic corneal laceration and cataract were identified. MAIN OUTCOME MEASURES Each case was analyzed retrospectively for the following factors: preoperative findings; surgical technique, including management of the posterior capsule; measurement of axial length and keratometry; calculation of IOL power, style, and type of IOL implanted; graft clarity; final visual acuity; and final refraction. RESULTS The five children ranged from 3 to 7 years of age at the time of trauma. All had primary repair of their injury before referral. Each patient was observed for more than 2 years. Each had a posterior chamber IOL placed in the sulcus. After surgery, no severe complications were observed. The preoperative visual acuity ranged from 20/400 to light perception. After surgery, all five patients had clear grafts and an improved visual acuity ranging from 20/20 to 20/400. The final spherical refraction in each patient was within 3.75 diopters (D) of plano, with 1.50 to 3.25 D of cylinder. CONCLUSIONS Successful surgical rehabilitation was accomplished in these patients. Despite their young age and the difficulty in determining IOL power, combining surgery and aggressive amblyopia therapy resulted in visual rehabilitation with refractions approaching emmetropia.

Efficacy of nonsimultaneous bilateral LASIK after nonsimultaneous bilateral penetrating keratoplasty

Purpose: To show if nonsimultaneous bilateral laser in situ keratomileusis (LASIK) is a safe and effective procedure for patients with bilateral penetrating keratoplasty (PKP). Methods: Five patients (10 eyes), with keratoconus, underwent PKP separately in each eye. After an average PKP follow-up of 45.8 months per eye, (range, 19-92 months), each eye underwent 1-stage LASIK using the Visx Star S3 laser to correct its residual refractive error. We used the cycloplegic refraction as the target for the LASIK surgery. Each patient had his or her eyes done separately 3 weeks apart. Results: Pre-LASIK myopia averaged −3.93 ± 2.9 D, ranging from -10.00 to +0.75 D. Pre-LASIK astigmatism averaged -3.25 ± 0.80 D, ranging from -1.75 to −4.50 D. Mean pre-LASIK keratometry was 45.4 ± 2.6, ranging from 42.2 to 50.5. Uncorrected visual acuity pre-LASIK averaged 20/220, ranging from 20/60 to 20/400. Best-corrected spectacle visual acuity (BCSVA) pre-LASIK averaged 20/22, ranging from 20/20 to 20/30. Nine eyes had no complications. One eye had a flap buttonhole during LASIK, and surgery was aborted. No vision was lost in this eye. Mean follow-up after LASIK in the 9 eyes was 17 ± 15.2 months, (range, 4−56 months). Mean post-LASIK ametropia in these 9 eyes was +0.25 ± 0.45 D, ranging from -0.50 to +0.75 D. Average post-LASIK astigmatism was -0.33 ± 0.38 D, ranging from 0 to −0.75 D. In the 9 treated eyes, uncorrected vision post-LASIK averaged 20/25, ranging from 20/20 to 20/30. BCSVA post-LASIK averaged 20/21, ranging from 20/20 to 20/25. No lines of visual acuity were lost in any of the eyes. Conclusion: Young patients who have had bilateral PKP, with good postoperative vision and low levels of myopia, astigmatism, and minimal wound override, are good candidates for bilateral nonsimultaneous LASIK. Further studies can now be done on the performance of bilateral simultaneous LASIK in patients who have had corneal transplant surgery in both of their eyes.