December 10, 2014
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Prosthetic device enhances visual function in patients with skin disease

Prosthetic replacement of the ocular surface ecosystem provides sustained improvement in patients with a history of Stevens-Johnson syndrome and toxic epidermal necrolysis.

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A gas permeable large-diameter plastic lens that fits under the eyelids and rests on the sclera, bathing the corneal surface with oxygenated artificial tears, improved visual function and acuity in patients with ocular surface disease related to Stevens-Johnson syndrome/toxic epidermal necrolysis, according to a study.

Perspective from J. Martin Heur, MD, PhD

Treatment for ocular surface disease as a sequela of Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) ranges from punctal occlusion to keratoprosthesis, according to the study authors, with no consensus in the literature on how best to manage these cases.

In a retrospective cohort study, Deborah S. Jacobs, MD, and colleagues reviewed treatment of 86 patients with a history of SJS/TEN with prosthetic replacement of the ocular surface ecosystem (PROSE), formerly known as the Boston scleral lens prosthetic device, to foster healing and maintain the corneal epithelium.

“PROSE is life-changing for patients with SJS/TEN,” Jacobs, medical director of the Boston Foundation for Sight, said. “It eliminates the foreign body sensation and photophobia that arises from the abnormal eyelashes and lid keratinization that are the chronic consequences of this disease. SJS/TEN patients will tell you they cannot live without their devices.”

Improved visual function

Jacobs said PROSE is an attractive interventional option that offers a mean of 23 points of improvement in visual function at 6 months on the National Eye Institute 25-item Visual Function Questionnaire.

In the study of 35 men and 51 women with a median age of 36 years, mean acuity at baseline was 20/60, which improved to 20/25.

“This change was a highly significant P value, less than .001,” Jacobs told Ocular Surgery News. The level of improvement was maintained for the duration of follow-up, a mean of 16 months.

None of the study results were a surprise, Jacobs said, and there were no adverse events.

“[PROSE] replaces the function of a faulty ocular surface system overall, creating an improved environment for those components such as limbal stem cells, corneal epithelial cells, aqueous tears, mucins and lipids that are barely adequate from a physiologic perspective,” Jacobs said.

Jacobs also believes that for symptomatic relief and support of marginal epithelial function, PROSE is more effective than other currently available treatment options, as well as more cost-effective when compared with interventions such as penetrating keratoplasty and limbal stem cell transplantation. Those therapies are at high risk for failure within a few years in cases of SJS/TEN, she said.

Jacobs and colleagues plan to continue collecting data over the long term on patients receiving PROSE for the ocular sequelae of SJS/TEN.

“We hope to analyze and publish our 5-year data for this diagnosis in 2 to 3 years,” Jacobs said.

Younger patients

The study group is also compiling its experience with PROSE for SJS/TEN in the pediatric population.

“Many children in the first decade of their life are excluded from treatment at the time of consultation because of their inability to cooperate, despite all the tricks in the book known to our experienced clinicians, who have the biggest of hearts and infinite patience,” Jacobs said. “It is heartbreaking when we fail because it is precisely in the youngest age group that amblyopia can arise, and so the stakes are the highest. We hope we can learn from our successes and failures with very young children, so that we can increase the ratio of successes to failures in kids with SJS/TEN.” – by Bob Kronemyer 

Reference:
Papakostas TD, et al. Ophthalmology. 2014;doi:10.1016/j.ophtha.2014.08.015.

For more information:
Deborah S. Jacobs, MD, can be reached at Boston Foundation for Sight, 464 Hillside Ave., Suite 205, Needham, MA 02494; 781-726-7337; email: djacobs@bostonsight.org.
Disclosure: Jacobs has no relevant financial disclosures.