Corrective eye surgery may be an option for some types of Ehlers-Danlos syndrome (EDS), according to a recent case report.
Eye surgery is generally not recommended for EDS patients because of the high risk of scarring and other complications. The disorder is often caused by mutations that affect the structure of various types of collagen, a molecule used for structural support and wound healing.
However, the new case report details the story of one patient who underwent laser-assisted in situ keratomileusis (LASIK) surgery prior to her EDS diagnosis.
The report, “Myopic regression and recurrent Salzmann nodule degeneration after laser in situ keratomileusis in Ehlers Danlos Syndrome,” was published in the American Journal of Ophthalmology Case Reports.
In 2001, doctors performed LASIK to correct myopia, or nearsightedness, in a 21-year-old Caucasian woman.
She had a history of hypermobile joints and intermittent arthralgias (joint pain), but denied any history of connective tissue disease, diabetes, or abnormal scar formation. Her medications included loratidine for seasonal allergies and citalopram for depression and anxiety. As the patient was adopted, physicians could not obtain her family history.
Despite complaining of dry eyes post-surgery, the patient achieved normal visual acuity (20/20 vision) in each eye. A physical examination of her eyes revealed mild superficial punctate keratitis — an inflammation of the outer eye — but no other signs of irregularities.
At a follow-up two years later, the patient complained of increased dryness in her eyes and itchiness. An examination revealed two Salzmann nodules in each eye. Salzmann nodules are a sign of slowly progressive degeneration of the surface of the eye.
Doctors treated the patient’s dry eyes with cyclosporin ophthalmic emulsion (Restasis, from Allergan) and preservative-free artificial tears. Despite this, the patient continued to experience inflammation in her eyes.
At five years post-surgery, the patient’s primary care physician diagnosed her with vascular EDS after she had experienced multiple miscarriages while on various fertility treatments. This type of EDS is characterized by defects in type III collagen and, more rarely, in type I collagen, both of which associate strongly with the eyes. Type I collagen comprises 75% of the vertebrate eye, while type III collagen is present in smaller amounts but is increased during wound healing and inflammation.
Although the patient’s Salzmann nodules appeared unchanged, doctors noted a decline in vision and moderate Meibomian gland dysfunction, which aggravates dry eye by altering the composition of the tear film, a thin layer protecting the cornea and conjunctiva. Treatment consisted of olopatadine drops, cyclosporin, and artificial tears.
Seven years after her eye surgery, the patient required punctal cautery in her right lower eyelid. This procedure permanently seals the tear ducts to keep the eyes moist by preventing tears from draining too quickly. This operation followed several failed attempts at punctal plugs, which seek to accomplish the same goal as cautery, but are not permanent.
Over the next 10 years, the patient experienced abdominal hernias and a uterine tear, and underwent multiple reparative surgeries. She was diagnosed with two disorders related to EDS: postural orthostatic tachycardia syndrome and mast cell activation syndrome.
She successfully gave birth to a son after several years of in vitro fertilization. The child also showed symptoms of EDS, including hypermobile joints and severe myopia.
Throughout this time, the patient continued to experience poor night vision and severely dry eyes. This led to punctual cautery of her left eyelid.
At 11 years after her LASIK surgery, the patient’s doctors saw neovascularization — the growth of new blood vessels into deoxygenated regions — of her right cornea, extending into the Salzmann nodules. This growth continued to develop over the next six years, during which the patient’s vision declined further, progressing to 20/150 in her right eye and 20/300 in her left eye.
Her dry eye symptoms continued to be managed conservatively, with no further surgical interventions.
“In summary, we describe the first patient with Ehlers Danlos Syndrome to have undergone LASIK surgery and be published in the academic literature,” the researchers wrote. “Our patient experienced postoperative complications, such as myopic regression, Salzmann nodular degeneration, and dry eye syndrome, possibly influenced by her underlying disorder of collagen synthesis.”
Currently, LASIK surgery is not recommended for EDS patients because of the high risk of scarring on the eye. The team argues, however, that because different types of EDS alter specific types of collagen, some EDS types may be more amenable to the surgery.
More research is needed to evaluate the risk of laser eye surgery in different EDS subtypes, the team noted.
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