ROP a lifelong disease that needs to be monitored

January 1, 2005

New Orleans-Findings of a retrospective study of ocular sequelae in baby-boomers born prematurely and who developed retinopathy of prematurity (ROP) reinforces the concept that ROP is a lifetime disease requiring ongoing, careful surveillance, said William S. Tasman, MD, at the American Academy of Ophthalmology retina subspecialty day meeting.

Dr. Tasman, professor and chairman of ophthalmology, Jefferson Medical College, Thomas Jefferson University, Philadelphia, in collaboration with Bradley T. Smith, MD, an ophthalmology resident, reviewed the charts of infants born prematurely between 1946 and 1959 and identified 47 patients with ROP. Eight eyes had been enucleated during infancy, and so the study included 86 eyes.

As expected, the group showed a variety of retinal problems, but the majority retained at least some useful vision. Based on data from 84 eyes, 42% had visual acuity of 20/60 or better, and another 21% had vision between 20/60 and 20/400.

"For obvious reasons, the major focus for ROP has been on treatment of the active stage of the disorder, but there has not been much opportunity to look at these patients during adulthood to identify the long-term sequelae," said Dr. Tasman, who is also ophthalmologist-in-chief, Wills Eye Hospital, Philadelphia. "In this group of baby-boomers for whom there was no treatment available during infancy, we found that similar to X-linked juvenile retinoschisis, incontinentia pigmenti, and familial exudative vitreoretinopathy (FEVR), ROP is associated with numerous late-onset problems that underscore the importance of continued follow-up."

Long-term sequelae The 47 patients consisted of 33 (70%) females and 14 (30%) males. Their current ages ranged between 45 and 55 years, with a mean of 50 years. Birth weight was known for 36 patients and averaged 1,251 g in that group, and mean gestational age, based on data from 32 patients, was 30 weeks.

The posterior segment findings showed that dragging of the retina was present in one-third of the eyes and retinal folds had developed in six of those eyes. In 80% of cases, the retinal dragging was temporal, while it was on the nasal side in the rest.

Disseminated pigmentation of the posterior pole was noted in five (5.8%) eyes, and while that finding limited vision, affected patients were still able to get around reasonably well. Dr. Tasman noted there is no apparent explanation for the pigmentation.

"If I were to float a theory, however, I would postulate that the retina was elevated for some time during the active stage and then spontaneously reattached," he said.

Rhegmatogenous retinal detachment had occurred in 20 (23%) eyes, often before the patients reached middle age, and the majority (70%) stayed reattached after surgery. In a few eyes, there were retinal breaks that were very posterior and even required silicone oil for retinal reattachment.

Seven (8.1%) eyes experienced retinal tears. Nine eyes (10.5%) developed exudative detachment, which Dr. Tasman explained is due to traction on peripheral vessels leading to exudation.

"We were not doing vitrectomy when many of these eyes were seen, and in two eyes treated with a scleral buckle to relieve the traction on the vessels, the detachment recurred," he added.

Cataract development The analysis of cataract development showed that 57 (66%) eyes had been operated on for cataracts, and among the 47 patients in the study, 39 (83%) had undergone cataract surgery in at least one eye. Nuclear sclerosis was the most common type of lens change.

"Any extrapolation of the cataract prevalence data must be done carefully considering this is a selected population, but in a control group of 50 patients from our practice who are of the same age and not born prematurely, we found only five (10%) had cataract surgery. That latter group included two patients with FEVR and Stickler syndrome, and if they were excluded, the percentage with cataracts would be even lower," Dr. Tasman said.