Wilmer-Wide Collaboration Challenges Decades-Old FDA Ruling

Recently, Eric Singman, an associate professor of ophthalmology at Wilmer Eye Institute, Johns Hopkins Medicine, collaborated on a study on the incidence of postsurgical complications in patients with Ehlers-Danlos syndromes (EDS) who underwent ocular surgery. EDS comprises a group of 13 subtypes of connective tissue disorders generally characterized by joint hypermobility (joints that stretch further than normal), skin hyperextensibility (skin that can stretch further than normal) and tissue fragility. It affects approximately 1 in 5,000 people worldwide.

Study co-investigators included Clair Francomano, a world-renowned geneticist who studies EDS; Ehlers-Danlos Society President Lara Bloom; and Singman’s colleagues from every surgical subspecialty at Wilmer, who analyzed ocular surgery complications to determine their incidence in EDS patients versus patients without EDS. 

What they found potentially challenges a 1992 ruling by the FDA advising patients with EDS against undergoing laser vision correction surgery.

Q&A with the Expert

To learn more about this collaborative research and the novel findings, we spoke with Singman, chief of the Patient Access Center for the Eye (PACE) at Wilmer.

What prompted you to examine this issue? 

Many of my patients with EDS have mentioned that they had eye surgery, including LASIK, for which EDS is a contraindication. They only learned of their EDS diagnosis after the surgery, and many seemed to be doing okay. Because the FDA ruling dates back to the time when LASIK first became available, we were never able do a randomized controlled study to determine whether EDS patients, in fact, fare more poorly after LASIK than do patients without EDS. But since the experiment occurred naturally, we were able to evaluate the results.

What did the research entail?

The Department of Ophthalmology at Wilmer partnered with Dr. Francomano and the Ehlers-Danlos Society to explore the ophthalmic surgery experiences of patients with EDS. Social media allowed us to reach a large population of EDS patients, and hundreds responded to the survey link posted on the EDS website. The data has been analyzed, and the findings have now been published online in the journal Molecular Genetics and Genomic Medicine

What did you learn as a result of the survey responses?

A number of key findings came out of the study. First, we learned that many EDS patients had, in fact, undergone ocular surgery before they knew of their diagnosis. This was particularly germane for patients who underwent laser vision correction, since the FDA considers EDS an absolute contraindication for LASIK. We also learned that, although the complication rate may be higher in the EDS population, because the sample size is relatively small, and patients self-selected to be in the study, it’s not fair to say with certainty that the complication rate is higher. Finally, we recognized the need for patients with EDS who have undergone LASIK to be examined closely. 

Is it possible that these findings might change the FDA’s stance on this? 

The FDA ruling was based upon an assumption, but that still has prevented any randomized controlled trial of patients with EDS to determine whether LASIK might be acceptably safe. Now that we know that a “natural experiment” occurred, where many patients with EDS had LASIK anyway, we are obligated to determine whether the FDA ruling is supported by real clinical data. We intend to evaluate patients with EDS in clinic who have had LASIK and cataract surgery to see if we detect any findings to suggest that the complication rate is higher. We may find that for certain types of EDS, LASIK might not be unsafe, and that  the contraindication may be too strong or broad for all patients. Perhaps for some classes of EDS — particularly the hypermobile type, the most common — that will allow for a randomized prospective study to truly determine whether EDS is fairly considered an absolute contraindication.

Your study found that many of the patients underwent surgery before receiving their EDS diagnosis, possibly because many of these patients don’t learn about their diagnosis until later in life. What can be done to safeguard them against the potential risk of complications if they don’t even know they have the disease?

Considering that EDS may put patients at higher risk of surgical complications, it’s reasonable for physicians to preoperatively explore whether patients might have EDS. Simple screenings, such as the Beighton Score and symptom questionnaires, are available online and may help surgeons provide more thorough informed consent, as well as help these patients have more realistic expectations of outcomes.