Jessica Ciralsky

Current ophthalmologic treatment strategies for acute and chronic Stevens-Johnson syndrome and toxic epidermal necrolysis.

Purpose of review To review the newer, effective ophthalmologic treatments for acute Stevens-Johnson syndrome (SJS) as well as the emerging treatment options for patients with chronic, severe ocular surface damage from the disease. Recent findings Amniotic membrane transplantation (AMT) applied to the eyes and eyelids in the acute phase of SJS can prevent the devastating scarring and visual problems that characterize the chronic phase of the disease. The severity of ocular inflammation in the acute phase does not always correlate to the severity of skin and systemic involvement. Thus, it is crucial that all patients with SJS be evaluated by an ophthalmologist familiar with the current management of the disease, the potential urgency of the situation and the option of AMT. Although challenging, the severe, chronic ocular problems of SJS can be at least partially alleviated with autologous serum drops, mucous membrane grafting to replace scarred tarsal conjunctiva, specialized contact lenses (PROSE), conjunctival replacement surgery (COMET), limbal stem cell transplantation and kerotoprostheses. Summary Early AMT is an effective treatment of acute SJS. Emerging treatments offer increased hope for those who have already suffered damage from SJS, but emphasis on the prevention of damage in the acute phase is most crucial.

Prompt versus delayed amniotic membrane application in a patient with acute Stevens-Johnson syndrome

Background Stevens-Johnson syndrome is often associated with blinding ocular surface cicatricial sequelae. Recent reports have described markedly improved clinical outcomes with the application of amniotic membrane to the ocular surface during the acute phase. Here we describe the clinical outcome of a patient with acute Stevens-Johnson syndrome and severe ocular surface involvement in whom the evolving medical condition and family consent resulted in amniotic membrane application to each eye at differing intervals from disease onset. Methods We undertook a retrospective chart review of a woman with Stevens-Johnson syndrome who presented within hours of disease onset. She underwent application of amniotic membrane to the ocular surface of the left eye during the hyperacute phase (<72 hours after disease onset) and to the right eye at a later time point during the acute phase (six days after disease onset). The clinical outcomes of the two eyes, as well as associated ocular symptoms, were compared over a one-year postoperative period. Results The right eye, treated later in the course of the disease, required additional surgical procedures and ultimately exhibited significantly more advanced ocular surface pathology than the left. Further, the patient reported more pronounced issues of chronic eye pain and visual difficulties in the right eye. Conclusion Earlier intervention with application of amniotic membrane to the ocular surface in this patient with severe ocular involvement secondary to Stevens-Johnson syndrome proved superior. Application of amniotic membrane as soon as possible after disease onset, preferably in the hyperacute phase, appears to result in a significantly better clinical outcome than application later in the disease course.

Treatment of Refractory Persistent Corneal Epithelial Defects: A Standardized Approach Using Continuous Wear PROSE Therapy

Abstract Purpose: To evaluate continuous wear of a fluid-ventilated, gas-permeable scleral PROSE device using a standardized protocol as treatment for refractory persistent corneal epithelial defects in patients with severe ocular surface disease. Methods: Retrospective review of eight eyes of seven consecutive patients with persistent epithelial defects refractory to traditional therapies. The standardized treatment regimen consisted of: (1) 24-hour-a-day PROSE wear until re-epithelialization was achieved, (2) brief daily device removal, cleaning, disinfection, and reservoir fluid replacement, (3) addition of a benzalkonium chloride (BAK)-free fourth-generation fluoroquinolone antibiotic drop to the reservoir, and (4) transition to long-term, daytime PROSE wear upon re-epithelialization. Results: All eight eyes exhibited resolution of the persistent epithelial defect. No eyes developed microbial keratitis. Four eyes exhibited recurrences; all recurrences promptly responded to reinstitution of continuous wear. Conclusions: Continuous wear of a PROSE device, using a strictly standardized regimen, constitutes an effective, safe treatment option for refractory persistent epithelial defects.

Corneal Nerve Structure and Function After Long-Term Wear of Fluid-Filled Scleral Lens

Purpose: The aim of this study was to determine whether long-term wear of a fluid-filled scleral lens alters basal tear production, corneal sensation, corneal nerve density, and corneal nerve morphology in 2 disease categories. Methods: Patients recruited from the Prosthetic Replacement of the Ocular Surface Ecosystem (PROSE) treatment program at the Weill Cornell Medical College were categorized into 2 groups: distorted corneas (DC) or ocular surface disease (OSD). We measured tear production, central corneal sensation, subbasal nerve density and tortuosity, and stromal nerve thickness before and after long-term wear of the prosthetic device used in PROSE treatment, defined as at least 60 days of wear for a minimum of 8 hours a day. Results: Twenty patients were included in the study. After long-term wear of the prosthetic device, tear production decreased in patients with DC (21.2 ± 8.5 to 10.4 ± 4.6 mm; P < 0.0001) but did not change in patients with OSD (7.5 ± 5.2 to 8.7 ± 7.2 mm; P = 0.71). Corneal sensation increased in the DC group (45.6 ± 9.2 to 55.0 ± 5.6 mm; P < 0.05). There was no significant change in sensation in patients with OSD (45.0 ± 8.7 to 49.1 ± 14.8 mm; P = 0.37). Subbasal nerve density, subbasal nerve tortuosity, and stromal nerve thickness remained unchanged in both DC and OSD groups after long-term wear (P > 0.05). Conclusions: Patients with DC had significantly reduced basal tear production and increased corneal sensation after long-term wear of the scleral lens, but patients with OSD did not show any changes in tear production or corneal sensation.

Tear film assessments for the diagnosis of dry eye.

Purpose of reviewDry eye disease (DED) is a complex, multifactorial condition that is challenging to diagnose and monitor clinically. To date, diagnosis has consisted largely of self-reported symptom questionnaires and a collection of clinical tests including vital dye staining, estimation of tear breakup time and Schirmers testing, as no gold standard exists. As the dry eye field has made progress in understanding disease pathogenesis, new methods for assessment of this condition have been developed. Recent findingsDED is now known to be characterized by tear hyperosmolarity and ocular surface inflammation, and there are now commercially available devices that accurately and reliably measure tear osmolarity and matrix metalloproteinase 9, a marker of inflammation and tissue breakdown. In addition, there are a variety of imaging modalities that have shown promise in their ability to identify patients with DED by assessing tear film dimensions and tear film instability. SummaryThere is a significant need for the development of tear film assessments for accurate diagnosis and monitoring of dry eye. There are a number of new devices and techniques that have shown promise in their ability help clinicians manage patients with DED.

Ultraviolet-blocking intraocular lenses: fact or fiction.

Purpose of review Ultraviolet-blocking intraocular lenses (IOLs) are used routinely in cataract surgery and are widely accepted. Blue-blocking IOLs, however, have been much debated since their inception. In this article, we will review the advantages and disadvantages of blue-blocking IOLs. Recent findings In experimental and animal studies, acute blue light exposure induces retinal damage and the use of blue-blocking IOLs lessens this damage. Many large epidemiologic studies have further investigated this relationship between blue light exposure and the development of age-related macular degeneration, and have shown conflicting results. Visual performance and circadian rhythm disturbances have also been explored in patients with blue-blocking IOLs; no significant negative effects have been shown. Summary The current literature on blue-blocking IOLs is contradictory. Studies have failed to conclusively prove that blue-blocking lenses provide photoprotection against age-related macular degeneration or cause any significant detrimental effects on visual function or circadian rhythms.

Dry Eye Disease After Refractive Surgery.

Laser in situ keratomileusis (LASIK) and photorefractive keratectomy (PRK) are the 2 most commonly performed corneal surgeries used to correct refractive errors. In the United States, an estimated 800,000 cases are completed each year. Since FDA approval was granted in 1995 (PRK) and 2000 (LASIK), significant advancements have been achieved, both in instrumentation and technique, rendering these procedures highly accessible, safe, and effective. LASIK and PRK are 2 of the most successful elective surgeries, with an estimated 95% patient satisfaction rate. Despite high patient satisfaction and overall success rates following LASIK and PRK, complications do occur, with dry eye disease being one of the most common. Symptoms of dry eye disease can significantly impact a patient’s quality of life, and even interfere with activities of daily living. Chronic dry eye has also been associated with refractive regression. After an elective procedure, such as LASIK or PRK, these effects can be especially problematic.

Boston Keratoprosthesis Type 1 in the Pediatric Population

Purpose of ReviewPediatric corneal blindness is a worldwide problem that is very difficult to address. Traditional penetrating keratoplasty is considered a high-risk procedure in this population due to the host of complications that are often encountered. Even when anatomic success is achieved with penetrating keratoplasty, visual recovery is not guaranteed.Recent FindingsPediatric keratoprosthesis is an alternative procedure that offers rapid visual recovery in a quiet eye without the risk of allograft rejection. Visually significant complications, including glaucoma, retinal detachments, retroprosthetic membranes, and endophthalmitis, can occur. Extreme care should be taken in selecting appropriate candidates for pediatric keratoprosthesis surgery.SummarySuccess with pediatric keratoprosthesis surgery can be achieved with a dedicated team of ophthalmic specialists and a motivated family.

Cicatrizing Conjunctivitis in a Patient Diagnosed With Drug Reaction With Eosinophilia and Systemic Symptoms/Drug-Induced Hypersensitivity Syndrome but With Features of Stevens-Johnson Syndrome.

Purpose: Severe cutaneous adverse reactions to drugs (SCARs) such as Stevens–Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) and drug reaction with eosinophilia and systemic symptoms/drug-induced hypersensitivity syndrome (DRESS/DIHS) serve as one of the main reasons for inpatient ophthalmic consultation. Although it is well-recognized that SJS/TEN is associated with severe ocular mucosal inflammation and cicatrizing, potentially blinding, sequelae, this association has not been described in relation to other SCARs. We present a patient fulfilling the diagnostic criteria for probable DRESS/DIHS but not for SJS/TEN, yet exhibiting the severe ocular surface involvement characteristic of SJS/TEN. Methods: Case report. Results: A 64-year-old man presented with bilateral pseudomembranous conjunctivitis and conjunctival denudation (sloughing) in the setting of a maculopapular rash, fever, liver dysfunction, and hematologic abnormalities 1 month after initiating several medications. A skin biopsy was not consistent with SJS/TEN. The patient was diagnosed with probable DRESS/DIHS and treated with high-dose systemic corticosteroids. The ocular surface inflammation was addressed with intensive topical corticosteroid ointment. The pseudomembranes resolved over a 6-week period, but the patient exhibited residual conjunctival scarring of all palpebral surfaces. Conclusions: The development of severe ocular surface mucosal inflammation and denudation with cicatrizing sequelae in a patient carrying a diagnosis of DRESS/DIHS has diagnostic and therapeutic implications for the ophthalmologist. Careful ophthalmic assessment is indicated in any SCAR patient with ophthalmic symptoms, regardless of formal diagnosis. Furthermore, the early therapeutic interventions recently recommended in SJS/TEN to limit the ophthalmic cicatricial sequelae, such as systemic or topical corticosteroids, may be indicated.

Evaluation and Management of Acute Stevens-Johnson Syndrome

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are eruptive mucocutaneous blistering diseases that can yield severe scarring of the eyelids and ocular surface. This scarring frequently leads to significant consequences such as severe dry eye, photophobia, and decreased vision. The severe sequelae are difficult, if not impossible, to reverse and can lead to lifelong disability for the patients. When applied in the first week of the illness, amniotic membrane transplantation (AMT) has been shown to effectively and consistently prevent the more serious sequelae of SJS and TEN [1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15]. The cases presented will explore various aspects of the ophthalmologic evaluation of acute SJS/TEN and decisions regarding the use of AMT in the acute phase of the disease, generally defined as the initial blistering portion of the disease that lasts anywhere from 2 to 4 weeks in most cases.