Cornea editor's corner of the world
Overlooked conjunctival disease

Conjunctivochalasis and superior limbic keratoconjunctivitis are two conditions that are often unnoticed by clinicians. Patients with these slowly progressive anatomical disorders of the conjunctiva have non-specific symptoms and are often treated for more common ocular surface diseases such as aqueous tear deficiency dry eye, meibomian gland dysfunction, and allergic eye disease. Since conjunctivochalasis and superior limbic keratoconjunctivitis are not nearly as common as other ocular surface conditions, they do not make it into the differential diagnosis. Patients are frequently treated for months—or even years—with a variety of topical therapies before the correct diagnosis is made. Clinicians must do two things in order not to miss these conditions. 1) Have these two diagnoses in your differential for chronic ocular surface disease. Although conjunctivochalasis and superior limbic keratoconjunctivitis don't occur frequently, they are certainly not rare. Cornea specialists make these diagnoses regularly, and these patients have typically seen numerous clinicians without an accurate diagnosis. 2) Utilize the proper diagnostic steps to assist in the diagnosis. For conjunctivochalasis, careful examination of the inferior conjunctiva with respect to the orientation to the lower lid is important. The amount of excess conjunctival can fluctuate so patients may need several examinations to confirm that the conjunctival redundancy is the cause of the patient's symptoms. Lissamine green stain is a useful tool for all conjunctival disease, and in conjunctivochalasis the staining will occur on the conjunctiva adjacent to the lower lid. For the diagnosis of superior limbic keratoconjunctivitis, the clinician must incorporate the technique of lifting the upper lid in all patients in order to examine the superior conjunctiva. Movement of the upper lid over the superior conjunctiva will elicit the redundancy of the tissue over the superior cornea. Once again lissamine green is extremely beneficial in the diagnosis. The superior cornea will light up with lissamine green, whereas fluorescein dye will often show no staining.
The importance of diagnosing conjunctivochalasis and superior limbic keratoconjunctivitis is that these conditions typically do not respond to medical management but are cured with surgical treatment. These patients can be some of the most appreciative you will manage as a relatively straightforward resection of the redundant conjunctiva can cure these chronically uncomfortable patients. If you don't look for conjunctivochalasis and superior limbic keratoconjunctivitis, you will never diagnose it. In this month's Cornea corner of the world, John A. Hovanesian, M.D., and Rick Palmon, M.D., discuss their experiences with these disorders.

Edward J. Holland, M.D., cornea editor

Conjunctivochalasis and superior limbic keratoconjunctivitis often go unnoticed when presenting with other surface diseases

An example of chemosis Source: John A. Hovanesian, M.D.
The arrows in this photo indicate where the conjunctivochalasis is occurring Source: John A. Hovanesian, M.D.

Common symptoms of dry eye can sometimes mask other conjunctival anatomic disorders such as conjunctivochalasis (CCh) or superior limbic keratoconjunctivitis (SLK). The former, a condition where redundant conjunctival tissue overlies the lower eyelid margin or covers the lower punctum, disrupts the normal tear flow. SLK, by contrast, presents as an inflammation of the superior bulbar conjunctiva and typically involves the superior limbus as well.
"These both mimic so many other diseases we routinely see that they're severely underdiagnosed," said Rick Palmon, M.D., partner, Southwest Florida Eye Care, Fort Myers, Fla. "Patients come in with burning, blepharitis, and/or dry eye with a secondary irritation, and it's common to lump the people with CCh and SLK into those two categories and not specifically look for either disorder."
Confounding the issue is that CCh and SLK can co-exist with dry eye, but in the case of CCh, patients will complain about a distinct foreign body sensation and pain, said John A. Hovanesian, M.D., clinical instructor, Jules Stein Eye Institute, University of California, Los Angeles.

"The good news is it's curable," he said. "I'd start with conservative medical therapy, such as a once-daily nonsteroidal anti-inflammatory, but if the patient does not improve, then I'd treat it surgically."
With SLK, Dr. Palmon said the key to correctly diagnosing the issue is "to have the patient look down while you're holding the lid up to see the pathology." If patients only look straight ahead, it's likely the lid pathology will be overlooked altogether. Although both CCh and SLK are often overlooked or lumped with dry eye, the two are differentiated primarily by geography, Dr. Palmon said, with CCh affecting the lower conjunctiva and SLK the superior conjunctiva.

Treating conjunctivochalasis

Risk factors for CCh include age (older than 50 years), a history of dry eye, and prior ocular surgery, particularly in cases where a peribulbar or retrobulbar anesthetic was used, Dr. Hovanesian said. Listening to the patient's overall complaints before presuming a diagnosis of dry eye is necessary to differentiate dry eye alone from dry eye concurrent with CCh, Dr. Hovanesian said. Because CCh is where the surface conjunctiva loses its attachment to the globe because of the loss of Tenon's fascia that would normally secure the conjunctiva, friction with the eyelids ensues and the situation can become overly irritating to the patient. Typically, the disorder is found in the lower lid margin temporally, he said, although 360-degrees can occur as well. A useful test to localize where the pain originates is to press on the eyelid and have the patient look up and down. If the pain can be replicated, it's likely CCh and not just severe dry eye, Dr. Hovanesian said. "Surgeons need to identify the loose conjunctiva and excise it," he said. "They need to take just a few millimeters to allow relaxation of the loose tissue away from the cornea. It's important to leave about 1 mm of healthy conjunctiva to preserve the limbal stem cells." He warned about being careful not to place the incision too posteriorly or the conjunctiva will likely swell and lead to additional tissue loosening.
After excision, he recommended cutting dehydrated amniotic membrane to the shape of the defect, using fibrin glue for adhesion, and placing the membrane over the wound. Post-op, his treatment regimen includes prednisolone acetate 1%, fluoroquinolone, and topical NSAIDs.

Treating SLK

SLK can be associated with thyroid disease and is more predominant in the superior limbus, Dr. Hovanesian said. Obvious filaments can be associated with dry eye, leading to an underdiagnosis of SLK, Dr. Palmon said. "Patients can also experience times of flares, where they're very symptomatic, but then the SLK can calm down and be more comfortable," Dr. Palmon said. If the flare occurs when patients are first beginning a dry eye treatment and then regresses, it's likely the physician and patients will attribute the relief to the new drops, he said. "They may end up OK for weeks or months before another flare-up occurs," he said. "That's usually when I start seeing patients—after they've had several flare-ups and the dry eye treatment has not been successful."
Dr. Palmon advised treating SLK medically first, usually aggressively treating the dry eye component with topical cyclosporine 0.05%, artificial tears, and/or punctal plugs. "If there are filaments, I'll put them on Mucomyst (acetylcysteine, Bristol-Myers Squibb, New York) 10% twice a day," he said, because the drug dissolves the mucus attachments common with SLK. He'll also prescribe topical mild steroids, such as Lotemax (loteprednol etabonate ophthalmic suspension, Bausch + Lomb, B+L, Rochester, N.Y.) until symptoms quiet down. "If the conjunctiva is not too thickened and stretched out, patients can be comfortable with aggressively treating the dry eye component," he said. If bandage contact lenses also fail to provide comfort, surgeons can remove the thickened conjunctiva superiorly and use amniotic membrane as well as fibrin glue to help prevent scarring, "but I think that should be a last resort," Dr. Palmon said. "Most patients, when medically treated aggressively, will quiet down and most of the flare-ups can be managed without performing resection." If patients are being treated surgically, however, "you have to remind them surgical treatment is not the only management—if the Schirmer's scores are still low, they will continue to need aggressive dry eye treatment," Dr. Palmon said. Drs. Palmon and Hovanesian advised treating either condition medically before surgically.

Editors' note: Dr. Hovanesian has financial interests with Allergan (Irvine, Calif.), B+L, and IOP Ophthalmics (Costa Mesa, Calif.). Dr. Palmon has no financial interests related to this article.

Contact information

Hovanesian: 949-951-2020, johnhova@gmail.com
Palmon: 239-768-0006, rpalmon@swfleye.com