Marian S. Macsai

Ocular surface squamous neoplasia: a review.

Even though ocular surface squamous neoplasia (OSSN) has been recognized for well over a century, the past decade has witnessed advances that have helped rewrite many of the paradigms for the diagnosis and management of these lesions. OSSN occurs predominantly in the elderly for whom they are the third most common oculoorbital tumors after melanoma and lymphoma. In addition to advanced age and male sex, other major risk factors linked to its pathogenesis are ultraviolet light, cigarette smoking, and the human papilloma virus. Although the latter has been linked to OSSN for nearly 4 decades, its identification and role in the pathogenesis of these tumors has been elucidated recently and is addressed in detail in this review. Newer techniques of impression cytology represent a noninvasive and reliable method of diagnosing OSSN and monitoring treated cases. The efficacy of chemotherapeutic agents such as mitomycin C and 5-fluorouracil have been proven in the recent past, making them a clear alternative to the time-tested treatment of surgical excision and cryotherapy. Early reports on the efficacy of topical Iterferon alpha 2b indicate significant promise in providing another alternative for the treatment of some of these neoplasms. These advances thus represent a minimally invasive and highly successful approach to the diagnosis and treatment of OSSN.

Eye bank survey of surgeons using precut donor tissue for descemet stripping automated endothelial keratoplasty.

PURPOSE To assess surgeon satisfaction with precut corneal tissue from 1 eye bank for Descemet stripping automated endothelial keratoplasty (DSAEK). Surgical techniques and predictors of procedural success were also examined. METHODS A 19-question survey was completed by 53 surgeons around the United States for 197 DSAEK cases using prepared corneal allograft tissue from the Iowa Lions Eye Bank. Surgeries were performed between April 1 and December 31, 2006; surveys were completed retrospectively within a few weeks of surgery. RESULTS Tissue was found to be acceptable in 98% of DSAEK cases reported. Difficulties with precut tissue (eg, lack of anterior cap adherence to the posterior lamella, not visible or decentered central dot, anterior edge undermining) were reported in approximately 10% of cases. A rebubbling procedure was performed in 23% of cases for donor dislocations. The donor lenticule adhered, with resulting corneal deturgescence, in 86% of cases. Surgeons declared a successful procedure in 92% of cases. Of the 14 unsuccessful cases, donor tissue quality was the underlying etiology in only 1 case. Procedural success rates were related to surgeon experience (P = 0.002), lenticule adherence after only 1 anterior chamber air bubble (P = 0.005), no small perforations to release fluid (P = 0.005), and the presence of corneal deturgescence (P = 0.002). CONCLUSIONS The use of precut tissue for DSAEK is not associated with increased risk of complications related to tissue preparation. With standardization of precutting donor tissue, safety of DSAEK surgery may be improved while increasing surgeon efficiency.Background:Syphilis outbreaks among men who have sex with men (MSM) in the United States have raised concerns about increased HIV transmission in this population. We sought to estimate HIV incidence among men diagnosed with primary or secondary (P&S) syphilis in sexually transmitted disease (STD) clinics in Atlanta, San Francisco, and Los Angeles. Methods:We analyzed deidentified sociodemographic information from routine syphilis surveillance databases and matching remnant sera from consecutive male patients with P&S syphilis who were tested for syphilis at 3 public health laboratories during January 2004 through January 2006. Deidentified sera positive for Treponema pallidum by particle agglutination were screened for HIV-1 antibodies by enzyme immunoassay (EIA). Specimens that were confirmed HIV-positive by Western blot analysis were then tested for recent HIV infection using the less sensitive (LS) HIV-1 Vironostika EIA and BED HIV-specific IgG/total IgG assay. Results:Of 357 men with P&S syphilis (98 in Atlanta, 151 in San Francisco, and 108 in Los Angeles), 32% had primary syphilis and 85% were MSM (12% no MSM risk and 3% no information). The median age was 36 years; 40% were white, 31% black, 20% Hispanic, and 8% other. Among men with P&S syphilis, 160 (45%) were HIV-positive, of whom 8 were classified as having acquired recent HIV infection by the LS-Vironostika EIA (all confirmed by BED) and had no history of antiretroviral use or HIV-positive results >6 months earlier. Seven of the 8 men with recent HIV infection were MSM. The estimated HIV incidence was 9.5% per year (95% confidence interval [CI]: 2.9 to 16.0) among all men and 10.5% per year (95% CI: 2.7 to 18.3) among MSM. Conclusions:We found high HIV incidence among a high-risk population of US men diagnosed with P&S syphilis in STD clinics in Atlanta, San Francisco, and Los Angeles. Intensive integrated HIV/STD prevention programs are needed for this population.

Tyrosinemia type II: nine cases of ocular signs and symptoms.

PURPOSE To report nine cases of tyrosinemia type II, with ocular signs and symptoms. METHODS Participants included nine patients (18 eyes) who were followed for a mean follow-up period of 6.5 years (range, 2 to 8 years). Intervention included dietary restriction of tyrosine and phenylalanine, which led to resolution of ocular and cutaneous lesions, improved behavior in one patient, and may have prevented developmental delay in others. The main outcome measures were visual acuity and serum tyrosine levels to determine the response to dietary therapy. Intelligence testing and developmental screening were performed when appropriate. RESULTS All patients presented with ocular signs and symptoms as the primary manifestation of disease and serve to illustrate the typical ocular findings of this syndrome. Three patients presented with cutaneous manifestations, and one patient had mild mental impairment at the time of diagnosis. CONCLUSIONS Recalcitrant pseudodendritic keratitis may be the presenting sign in tyrosinemia type II. Awareness of the presenting signs and symptoms may accelerate the diagnosis and dietary intervention. Initiation of a tyrosine-restricted and phenylalanine-restricted diet in infancy is most effective in preventing cognitive impairment.

What causes keratoconus

Purpose: The etiology of keratoconus has been the subject of numerous studies. Here, we review the body of literature that attempts to determine what causes keratoconus. Methods: A review of the published literature was performed to evaluate the possible causes of keratoconus and how the different etiologies may play a role in the development of this disease. Results: Peer-reviewed published papers and recent abstracts concerning the different etiologies of keratoconus were reviewed and discussed. Biochemical, genetic, and environmental factors are reviewed. The role of eye rubbing and atopic disease are explored. Conclusions: We agree with the hypothesis that there is a genetic predisposition that requires a “second hit” or environmental event to elicit progressive disease in keratoconus. Eye rubbing may serve as the “second hit” in some predisposed individuals. Inflammatory mediator studies question if keratoconus is really a noninflammatory thinning disorder of the cornea.

REPAIR OF DESCEMET'S MEMBRANE DETACHMENT WITH PERFLUOROPROPANE (C3F8)

PURPOSE To report the use of perfluoropropane (C3F8) gas in the repair of Descemets membrane detachments. METHODS Descemets membrane detachments after cataract surgery in three eyes and pars plana vitrectomy in a fourth underwent anterior-chamber gas-exchange descemetopexy with an isoexpansile 14% mixture of C3F8 to facilitate reattachment of Descemets membrane. RESULTS Descemets membrane detachment was successfully reattached after anterior-chamber gas exchange with 14% C3F8 in three of the four eyes treated. The fourth eye treated with 14% C3F8 probably failed Descemets membrane reattachment because of an unrecognized viscoelastic bleb situated anterior to Descemets membrane. No corneal decompensation or fluctuations in intraocular pressure were believed to be attributable to isoexpansile C3F8 gas exchange. CONCLUSION Early recognition and repair of Descemets membrane detachments may prevent complications, such as corneal decompensation, corneal opacities and edema, and an overall decline in visual acuity. Isoexpansile C3F8 is demonstrated as a safe and efficacious alternative for the repair of Descemets membrane detachment.

Suture technique for Descemet stripping and endothelial keratoplasty.

Purpose: To describe a suture technique for Descemet stripping and endothelial keratoplasty (DSEK). Methods: The technique was performed in 2 eyes of 2 patients with Fuchs endothelial corneal dystrophy. A double-armed 10-0 prolene suture on a CIF-4 needle was used to pull the donor corneal button into the recipients anterior chamber without the use of forceps to hold the button. The suture was also used to unfold the lenticule in the eye without marking the stroma with gentian violet, to center the lenticule and to secure that in place. Results: No intraoperative problems occurred in either case. The suture technique was successfully used to pull the donor button into the hosts eye and to unfold and center the button. Both lenticules were well centered and had minimal corneal edema. At 6 months, the central endothelial loss was 8% in the first case and 10% in the second. Conclusion: This suture technique for DSEK avoids the use of forceps and allows less traumatic donor button manipulation. It may be an effective alternative with low endothelial damage; further follow-up will determine the long-term effect on the central endothelial cell count.

Keratectasia After Laser In Situ Keratomileusis: A Histopathologic and Immunohistochemical Study

OBJECTIVE To examine histopathologic and immunohistochemical features of human corneal buttons from patients who developed keratectasia after laser in situ keratomileusis (LASIK). METHODS Five corneal buttons were obtained during penetrating keratoplasty from patients who developed keratectasia after LASIK. Histologic features were examined by hematoxylin-eosin staining using paraffin-embedded sections and by transmission electron microscopy. Immunostaining for alpha(1)-proteinase inhibitor, Sp1, and matrix metalloproteinases 1, 2, and 3 was performed with 2 healthy corneas and 2 corneas with keratoconus as controls. RESULTS Central stromal thinning was observed after hematoxylin-eosin staining in all corneas with keratectasia. No histologic features specific to keratoconus, including Bowman layer disruption, were identified in the corneas with keratectasia. By transmission electron microscopy, collagen fibril thinning and decreased interfibril distance were observed in the stromal bed. Immunostaining intensity and/or pattern for alpha(1)-proteinase inhibitor and Sp1 in the corneas with keratectasia was comparable to that of healthy corneas and differed from that in the corneas with keratoconus. No significant staining with anti-matrix metalloproteinases 1, 2, and 3 antibodies was observed in either the corneas with keratectasia or the healthy corneas. CONCLUSIONS Histologic findings suggest that post-LASIK keratectasia results in collagen fibril thinning and decreased interfibril distance within the residual stromal bed. Discrepant results between keratectasia and keratoconus suggest that the pathogenesis of the 2 conditions differ.

Epidermal growth factor and insulin use in corneal preservation : results of a multi-center trial

Purpose: The ability of DexSol medium, supplemented with two growth factors, human epidermal growth factor (hEGF) and human insulin, to improve long-term endothelial survival after penetrating keratoplasty was evaluated in a multi-center, randomized, prospective, double-masked clinical trial. Methods: Donor cornea pairs, one stored in DexSol and the other in DexSol with hEGF (10 μg/ml) and human insulin (10 pug/ml) (ProCell), were transplanted into 105 pairs of recipients matched by diagnosis and procedure and followed postoperatively for graft and endothelial survival. Results: No primary donor failures occurred in either group. Graft clarity did not differ between the ProCell and DexSol groups at all postoperative periods: 3 months (98% versus 99%), 6 months (94% versus 98%), and 1 year (95% versus 97%), respectively. Postoperative complications (e.g., glaucoma, rejection) occurred with comparable frequencies in both groups. Mean endothelial cell loss did not significantly differ between the ProCell and DexSol groups at 3 months (5.7% versus 5.1%), 6 months (8.1 % versus 10.1 %), and 1 year (12.3% versus 15.6%), respectively. Similarly, there were no clinically and statistically significant differences in other endothelial morphometric parameters. Conclusions: The use of corneas stored in DexSol medium with added hEGF and insulin in corneal transplantation resulted in a safety and efficacy profile comparable with that observed in patients receiving DexSol-stored corneas; however, there were no clinically and statistically significant differences in postoperative endothelial morphometric parameters.

Management of oculus fragilis in Ehlers-Danlos type VI.

Purpose. To describe an alternative, novel surgical approach to the repair of a ruptured globe in the second reported patient with keratoglobus, Ehlers–Danlos type VI, and normal lysyl hydroxylase. Methods. Under general anesthesia, a 360° conjunctival peritomy was performed and the epithelium was removed from the keratoglobus cornea. Descemets membrane and endothelium were removed from a fresh donor corneoscleral ring. An onlay epikeratoplasty was then performed and the entire donor corneoscleral button was secured to the sclera with 9-0 nylon suture. The conjunctiva was brought into position and tacked down over the edge of the donor graft. After 4 months, a full-thickness penetrating keratoplasty was then performed. Results. The patient ultimately had a ruptured globe in the fellow eye and thus required the identical procedure OU. Both eyes were successfully repaired with a final visual acuity OU of 20/100. Conclusion. The described surgical approach allowed successful surgical repair of bilateral ruptured globes in a patient with keratoglobus, Ehlers–Danlos type VI, and normal lysyl hydroxylase levels. In the previous report of such a patient by Judisch et al. (1), the attempt at surgical repair was unsuccessful and ended in enucleation. The described surgical technique may be used to treat advanced keratoglobus or oculus fragilis.

Keratoconus and Turner's syndrome.

Purpose To report the association of keratoconus and Turners syndrome in three patients and to review the ophthalmic manifestations of Turners syndrome. Methods Three patients with keratoconus and Turners syndrome were identified and reported in a retrospective review. Results These three cases represent the first series of patients with keratoconus and Turners syndrome. All three patients underwent penetrating keratoplasty with good visual rehabilitation. None of the patients had other ocular features associated with Turners syndrome. Conclusion Turners syndrome is commonly associated with ocular problems. In this series we identify an association of keratoconus with Turners syndrome. Clearly, a careful ocular examination in this condition with attention to ocular features of Turners syndrome is important.