Perry Rosenthal

Gas-permeable scleral contact lens therapy in ocular surface disease

PURPOSE To describe the therapeutic benefits of nonfenestrated gas-permeable scleral contact lenses in the management of patients with ocular surface disease. METHODS The charts of 49 consecutive patients (76 eyes) with ocular surface disease whose management included the use of gas-permeable scleral contact lenses were reviewed. We also developed a questionnaire to assess the impact of lens wear on subjective aspects of activities of daily living. RESULTS The mean age of the 49 patients was 44.6 years (range, 3 to 87 years); 31 patients were female and 18 were male. The most common indication for fitting of the lenses was Stevens-Johnson syndrome (54 [71%] of the 76 eyes). Other indications included ocular cicatricial pemphigoid, exposure keratitis, toxic epidermal necrolysis, postherpetic keratitis, congenital deficiency of meibomian glands, superior limbal keratoconjunctivitis, Sjögren syndrome, and inflammatory corneal degeneration. The mean follow-up was 33.6 months (range, 2 to 144 months). Improvement in best-corrected visual acuity (defined as a gain of 2 or more Snellen lines) was observed in 40 (53%) of the eyes. In eight (53%) of the 15 eyes with active corneal epithelial defects at the time of lens fitting, the defects healed, whereas in the remaining seven eyes the corneal epithelial defects remained unchanged. Forty-five (92%) of the 49 patients reported improvement in their quality of life as a result of reduction of photophobia and discomfort. The mean wearing time of the gas-permeable scleral contact lenses was 13.7 hours per day (range, 4 to 18 hours). Many patients had preparatory surgical procedures before lens fitting (for example, punctal occlusion or mucous membrane grafting), and some had visual rehabilitation surgical procedures (for example, keratoplasty and/or cataract surgery) after lens fitting. CONCLUSIONS Gas-permeable scleral contact lens wear provides an additional effective strategy in the surface management and visual rehabilitation of patients with severe ocular surface disease.

Boston scleral lens prosthetic device for treatment of severe dry eye in chronic graft-versus-host disease.

Purpose: To determine if the Boston Scleral Lens Prosthetic Device (BSLPD) reduces symptoms and improves quality of life in patients with severe dry eye from chronic graft-versus-host disease (cGvHD). Methods: This is a noncomparative interventional case series reporting 33 consecutive patients with severe dry eye from cGvHD, unresponsive to conventional therapy, who were fitted with the BSLPD. A patient survey was undertaken after lenses were dispensed and worn regarding the effect of scleral lens wear on their symptoms, quality of life, and activities of daily living. The patient population was characterized from a retrospective chart review. Survey data were tabulated. Results: BSLPD wear resulted in improvement in pain, photophobia, and general quality of life in nearly all patients, with more than half reporting the highest improvement level for pain (52%) and photophobia (63%), and more than two thirds (73%) reporting the highest improvement level for quality of life. There was improvement in reading and driving in >90% of those who reported previous compromise, with >60% reporting the highest improvement level for each of these activities. Conclusions: The BSLPD mitigates symptoms and improves quality of life in patients with severe dry eye from cGHvD.

The Boston Scleral Lens in the management of severe ocular surface disease

Fluid-ventilated, gas-permeable scleral lenses are a valuable front-line tool in the management of severe ocular surface disease. In addition to enhancing vision, they have the potential to reduce greatly the disabling ocular pain and photophobia associated with SJS, TEN, and ocular cicatricial pemphigoid. They are also useful in healing some PEDs that are refractory to all other treatment strategies and in reducing PED recurrence in stem cell-deficient and neurotrophic corneas. The therapeutic benefits of these lenses are provided by the oxygenated aqueous environment they create over the corneal epithelium. The oxygenated precorneal fluid compartment that is maintained at neutral pressure protects the epithelial surface from the desiccating effects of exposure to air and the friction generated by blinking and avoids the shearing forces generated during the blink-induced movement of soft lenses.

Corneal Pain without Stain: Is it Real?

Clinicians often encounter patients who report corneal pain suggestive of dry eye disease, yet lack equivalent signs. These patients represent a diagnostic and therapeutic challenge that is more easily dismissed than addressed. We review the physiology of pain and the pathophysiological mechanisms of neuropathic corneal pain and speculate on the mechanisms of certain etiopathogenic triggers, such as LASIK, severe dry eye disease, and Sjogren syndrome. Recognizing corneal neuropathic pain as a disease in its own right is the first step toward developing more effective treatments for these severely disabled and presently inadequately served patients.

Ocular neuropathic pain

As the biological alarm of impending or actual tissue damage, pain is essential for our survival. However, when it is initiated and/or sustained by dysfunctional elements in the nociceptive system, it is itself a disease known as neuropathic pain. While the critical nociceptive system provides a number of protective functions, it is unique in its central role of monitoring, preserving and restoring the optical tear film in the face of evaporative attrition without which our vision would be non-functional. Meeting this existential need resulted in the evolution of the highly complex, powerful and sensitive dry eye alarm system integrated in the peripheral and central trigeminal sensory network. The clinical consequences of corneal damage to these nociceptive pathways are determined by the type and location of its pathological elements and can range from the spectrum known as dry eye disease to the centalised oculofacial neuropathic pain syndrome characterised by a striking disparity between the high intensity of symptoms and paucity of external signs. These changes parallel those observed in somatic neuropathic pain. When seen through the neuroscience lens, diseases responsible for inadequately explained chronic eye pain (including those described as dry eye) can take on new meanings that may clarify long-standing enigmas and point to new approaches for developing preventive, symptomatic and disease-modifying interventions for these currently refractory disorders.

PROSE treatment of corneal ectasia

PURPOSE Prosthetic replacement of the ocular surface ecosystem (PROSE) uses custom designed and fabricated prosthetic devices in a treatment that restores vision, supports healing, reduces symptoms and improves quality of life in patients with complex corneal disease. We report the success rate for PROSE treatment of corneal ectasia. METHODS Records of 59 patients with corneal ectasia seen in consultation over 6 months were reviewed. Candidacy for treatment, topographic indices, change in visual acuity, achievement of satisfactory fit, device wear status and change in visual function at 6 months were recorded. RESULTS Sixteen eyes were non-candidates because conventional correction was adequate. Trial devices were inserted but not dispensed for 13 eyes. No eyes were excluded for severity of ectasia. In the remaining 89 eyes, satisfactory fit was achieved and a device was dispensed. Twenty-one eyes (15 patients) had undergone penetrating keratoplasty. Device wear at 6 months was documented in 78/89 eyes (88%). NEI VFQ-25 score improved 27.6 points (p<0.001) on a 100 point scale in patients wearing a device at 6 months. CONCLUSION All candidate eyes with corneal ectasia could be fitted with a PROSE device. PROSE treatment has a high success rate when measured by ability to achieve satisfactory fit, impact on visual acuity and 6 month data on both rate of continued wear and impact on visual function. PROSE treatment is an alternative to penetrating keratoplasty for patients with corneal ectasia who are contact lens intolerant.

Wavefront-Guided Scleral Lens Prosthetic Device for Keratoconus

Purpose To investigate the feasibility of correcting ocular higher order aberrations (HOAs) in keratoconus (KC) using wavefront-guided optics in a scleral lens prosthetic device (SLPD). Methods Six advanced KC patients (11 eyes) were fitted with an SLPD with conventional spherical optics. A custom-made Shack-Hartmann wavefront sensor was used to measure aberrations through a dilated pupil wearing the SLPD. The position of SLPD, that is, horizontal and vertical decentration relative to the pupil and rotation were measured and incorporated into the design of the wavefront-guided optics for the customized SLPD. A submicron-precision lathe created the designed irregular profile on the front surface of the device. The residual aberrations of the same eyes wearing the SLPD with wavefront-guided optics were subsequently measured. Visual performance with natural mesopic pupil was compared between SLPDs having conventional spherical and wavefront-guided optics by measuring best-corrected high-contrast visual acuity and contrast sensitivity. Results Root mean square of HOA in the 11 eyes wearing conventional SLPD with spherical optics was 1.17 ± 0.57 &mgr;m for a 6-mm pupil. Higher order aberrations were effectively corrected by the customized SLPD with wavefront-guided optics, and root mean square was reduced 3.1 times on average to 0.37 ± 0.19 &mgr;m for the same pupil. This correction resulted in significant improvement of 1.9 lines in mean visual acuity (p < 0.05). Contrast sensitivity was also significantly improved by factors of 2.4, 1.8, and 1.4 on average for 4, 8, and 12 cycles/degree, respectively (p < 0.05 for all frequencies). Although the residual aberration was comparable to that of normal eyes, the average visual acuity in logMAR with the customized SLPD was 0.21, substantially worse than normal acuity. Conclusions The customized SLPD with wavefront-guided optics corrected the HOA of advanced KC patients to normal levels and improved their vision significantly.

The Boston Scleral Lens in the treatment of pediatric patients

PURPOSE To report the use of a custom-designed, fluid-ventilated, gas-permeable scleral lens in the treatment of patients under 13 years of age. METHODS We retrospectively reviewed the medical records of all patients under 13 years of age who were fitted with the Boston Scleral Lens at the Boston Foundation for Sight from January 1996 through June 2006. Age, sex, ophthalmic diagnosis, systemic diagnosis, prior surgical intervention, complications, lens fit and wearing failures, and duration of lens use are reported. RESULTS Boston Scleral Lenses were fitted in 47 eyes of 31 patients referred after failure of conventional therapy. Patients ranged in age from 7 months to 12.92 years (mean, 7.75 years) at time of fitting. There were 16 girls and 15 boys in this group. The mean duration of documented scleral lens use was 24 months (range, 0-85 months). A broad range of refractive and ocular surface disorders was treated with this modality, with the vast majority of patients having ocular surface disease (27/31, 87%) rather than refractive disorders (4/31, 13%). Congenital corneal anesthesia syndromes and Stevens-Johnson syndrome each accounted for over one-third of the patients. CONCLUSIONS The Boston Scleral Lens is a custom-designed, fluid-ventilated, rigid gas-permeable scleral lens that vaults the cornea retaining a pool of oxygenated artificial tears over the corneal surface. The Boston Scleral Lens is a treatment option, after failure of conventional therapy, for a broad range of ocular surface and refractive disorders in the pediatric age group. Pediatric ophthalmologists should be aware of this treatment modality, particularly in the management of severe ocular surface disease.

Superior Limbic Keratoconjunctivitis in Contact Lens Wearers

Forty patients with daily wear, cosmetic contact lenses (CL) presented with symptoms of ocular irritation and a keratoconjunctivitis clinically resembling superior limbic keratoconjunctivitis (SLK). Typically the patients were either successful hard CL wearers who changed to soft lenses and used a chemical aseptisizing solution or preserved saline solution, or successful soft CL wearers using salt tablets who switched to a preservative-containing system. In all cases, exposure to thimerosal-preserved solutions was documented. Upon discontinuation of lens wear, all signs and symptoms slowly resolved without permanent sequelae. Positive skin and ocular sensitivity reactions to thimerosal were present in one-third (5/15) of patients tested. Light and transmission electron microscopic examination of conjunctival specimens disclosed intercellular epithelial edema, pseudoepitheliomatous hyperplasia, acute and chronic inflammation, and decreased numbers of goblet cells. Exposure to thimerosal is implicated in the etiology of contact lens-superior limbic keratoconjunctivitis (CL-SLK).

Bevacizumab for Corneal Neovascularization

ministration approved and Clinical Laboratory Improvement Amendments waived for directly detecting adenovirus from the conjunctiva of patients with conjunctivitis. Because of the reported clinical efficacy of the RPS Adeno Detector in the clinical setting, it was deemed desirable to implement this system in the laboratory setting to provide a rapid diagnostic test for adenovirus. For the convenience of outside testing in a laboratory setting, the office practice may want to avoid the purchase of the kits, the training of personnel, and the expense of discarding outdated tests. This is particularly relevant as the manufacturer suggests confirming negative test results with cell culture. The RPS Adeno Detector laboratory evaluation was accomplished by testing: (1) de-identified conjunctival samples that were collected on swabs and inserted into chlamydia transport medium ([CTM] viral transport medium without antibiotics), and (2) concentrated adenovirus antigen from these same collected samples. The RPS Adeno Detecto was evaluated for swab samples using 11 de-identified true-positive clinical specimens that were cell-culture positive, shell vial positive, and polymerase chain reaction positive for adenovirus, and 16 truenegative samples that included 4 de-identified Herpes simplex virus culture-positive clinical samples or clinical isolates of bacteria (validation bank). The RPS Adeno Detector was evaluated for concentrated samples using 11 de-identified true-positive samples (8 samples were part of the swab experiment; however, 3 new samples were added) and 10 true-negative samples. The concentrated samples were prepared from the swab samples by centrifuging 0.5 ml of the CTM at 12,000g for 15 minutes using a Millipore (Billerica, MA) protein concentrator (UFV5BCC25, USA) (Performed by Uma Mahesh Babu, PhD from RPS). The swab (100 l) and concentrated (20 l) samples were added directly onto the sample transfer window of the test cassette body. Figures 1 and 2 (available at http://aaojournal.org) demonstrate a positive and negative test. Testing was performed in a masked fashion by having one person (coauthor, RPK) prepare the true-positive and true-negative samples, and randomly arrange the samples to prevent any biased interpretation by 5 observers (co-authors: NMS, PPT, EGR, RMQS, YJG). The median values for sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), and efficiency were calculated using the test interpretation of the observers. In the laboratory setting, the RPS Adeno Detector demonstrated: 9% sensitivity, 100% specificity, 100% PPV, 61.5% NPV, and 62.9% efficiency, with swab samples. Using concentrated samples, the sensitivity increased to 27%, the specificity and PPV remained 100%, NPV decreased to 56%, and efficiency was 62%. Table 1 (available at http://aaojournal.org) presents the descriptive statistics of sensitivity, specificity, PPV, NPV, and efficiency as median values of 5 masked observers, and the range of the values for the testing of the swab and concentrated samples. Although the FDA has approved the RPS Adeno Detector as the first true “Point of Care” test for detecting adenovirus antigen from conjunctivitis, it demonstrated to be less reliable for detecting adenovirus antigen in the laboratory from specimens submitted in CTM compared with