Procedure Corrects Kearns-Sayre Syndrome Eyelid Drooping

Chronic progressive external ophthalmoplegia (CPEO) is the most common characteristic of Kearns-Sayre syndrome, which is one member of the family of mitochondrial myopathies. Patients with CPEO may benefit from bilateral fascia lata lid suspension, a procedure explored by researchers in Brazil and Italy to correct blepharoptosis, or severe drooping of the upper eyelids, in seven patients with Kearns-Sayre syndrome.

The study, “Bilateral Lid/Brow Elevation Procedure for Severe Ptosis in Kearns-Sayre Syndrome, A Mitochondrial Cytopathy,” was published in the journal Clinical Opthalmology.

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To conduct the procedure, researchers obtained a small strip of tissue from patients’ own thigh muscles. This tissue was split into four smaller strips and sutured above the eyebrow down to the eye lid, elevating the eyelid. All procedures were conducted by the same surgeon and under general anesthesia with a local anesthetic to reduce pain upon awakening.

Before the procedure, patients were severely limited in their capability to elevate their eyelids. All had less than five millimeters of superior eyelid elevator muscle function.

After the procedure, all patients were satisfied with the look and elevation of their eyelids achieved in the study. Their eyelids remained functional, and they could open and close their eyes at will. Only one patient experienced an adverse event during the procedure. Surgery caused corneal scarring, but since it was an inferior wound, it did not affect her visual acuity. “Brow suspension surgery should be limited because Kearns-Sayre syndrome patients have reduced orbital muscle function as well as a reduced Bell’s phenomenon which renders them susceptible to corneal exposure and ulceration,” noted the researchers. “Surgical correction should therefore be conservative and only performed when the visual axis is compromised.”

Other research groups have also explored similar methods of correcting CPEO in Kearns-Sayre syndrome patients. One study used autogenous fascia lata slings, as did the study at hand, but also used artificial silicone and plastic (“silastic”) slings. These patients had less severely reduced levator muscle function and showed excellent results.

“The surgical technique described to correct the blepharoptosis found in patients with Kearns-Sayre syndrome was found to be efficient and relatively safe,” concluded the authors, indicating that this may be a viable treatment option for patients with the rare mitochondrial myopathy.