Unilateral Chronic Papillary Conjunctivitis
A small number of conditions cause chronic papillary conjunctivitis (CPC) that is more typically unilateral than bilateral. These include lacrimal drainage infections (eg, chronic dacryocystitis and canaliculitis); the giant fornix syndrome; masquerade syndrome due to a tumor, most commonly sebaceous carcinoma; and factitious conjunctivitis.
Lacrimal Drainage Infections
Patients who present with a unilateral chronic or recurrent conjunctivitis, limited to one eye, should first have a thorough exploration of their lacrimal drainage system. Symptoms of chronic epiphora, a larger-than-normal tear meniscus, and prolonged retention of fluorescein dye strongly suggest lacrimal drainage obstruction. Swelling of the canalicular region of the eyelid along with the appearance of a "pouting punctum" suggest canaliculitis. In nasolacrimal duct obstruction and chronic dacryocystitis, digital massage over the lacrimal sac, or lacrimal irrigation, will usually produce reflux of fluid along with purulent discharge (Figure 1).
This condition presents as a chronic or recurrent bacterial conjunctivitis, usually limited to one eye, although bilateral cases do occur. Patients typically complain of epiphora and a chronic mucopurulent discharge, redness of the eye, and, as in most cases of bacterial conjunctivitis, sticking together of the lashes in the morning. Physical findings are those of a bacterial conjunctivitis, with a diffuse papillary response and mucopurulent discharge. There may be swelling of the medial canthal region overlying the lacrimal sac, although the area is usually not tender or acutely inflamed. As this disease occurs as a result of obstruction of the nasolacrimal duct, some patients will give a history of chronic sinus disease or facial trauma, while others, especially elderly women, have progressive essential dacryostenosis. In some cases, intermittent obstruction is caused by a dacryolith. Chronic infection in the lacrimal sac occurs as the result of stagnation of tears that cannot progress past the obstruction. Retrograde drainage of purulent material into the eye causes the conjunctivitis. A definitive diagnosis is made when purulent material refluxes into the eye with pressure over the lacrimal sac, or with reflux of saline and pus on attempted nasolacrimal irrigation.
Gram-positive organisms including Staphylococcus aureus, coagulase-negative staphylococci, and Streptococcus pneumoniae are found in approximately two-thirds of cases. Gram-negative bacteria, most commonly Pseudomonas aeruginosa, are found in approximately 25%. While topical or systemic antibiotic treatment may provide temporary relief of symptoms, without relief of the obstruction, the infection always recurs. A dacryocystorhinostomy is curative.
This condition occurs because of a diverticulum of the canaliculus, with stasis of fluid within the diverticulum leading to secondary infection. Because the distal lacrimal drainage system is not obstructed, patients do not have obstructive symptoms, such as epiphora, nor is there a delay of fluorescein drainage or elevated tear meniscus. Patients complain of symptoms similar to other forms of bacterial conjunctivitis and demonstrate papillary conjunctivitis with a catarrhal discharge. The disease should be suspected when there is inflammation and swelling along the lid margin medial to the punctum, with the usually normal opposite side used for comparison (Figure 2 and Figure 3). The diagnosis is confirmed by expression of the canaliculus. After the eye has been anesthetized with topical proparacaine, the area of swelling is squeezed between a cotton-tipped applicator and the end of a finger, or between two applicators, and both are rolled toward the punctum. Delivery of a granular, cheesy material from the punctum establishes the diagnosis. Canalicular concretions are found in more than 70% of patients.
Canaliculitis can be caused by a variety of organisms, most commonly streptococci, staphylococci, and the anaerobic filamentous bacteria Actinomyces species (Figure 4). The latter are found in more than 90% of patients with concretions ("sulfur granules"). In some cases, complete expression followed by irrigation with penicillin or another antibiotic solution can be curative. In recalcitrant cases, the diverticulum must be obliterated to achieve a cure. The diverticulum can be located by inserting a lacrimal probe into it, with the probe hitting a dead end within the canaliculus. The walls of the diverticulum are then scraped with a small curette, which can reach the diverticulum either through a small trans-conjunctival incision cutting down to the probe, or through the punctum, after a "one-snip" enlargement of the punctal opening. In most cases, the diverticulum can be eliminated in this way, leaving normal canalicular drainage intact.
Giant Fornix Syndrome
Giant fornix syndrome (GFS) is a chronic or recurrent mucopurulent conjunctivitis that occurs in elderly individuals with deeper-than-normal superior conjunctival fornices related to upper lid ptosis from dehiscence of the levator aponeurosis. Affected individuals are in their eighth to tenth decade, and the majority are women. The vast majority of cases are unilateral. A consistent finding is a coagulum of mucopurulent material in the recesses of a large upper fornix. Cultures are nearly always positive for S aureus. Many patients also have concomitant nasolacrimal duct obstruction and chronic dacryocystitis. Diagnosis of GFS is often delayed, with an average duration of symptoms of 2 years. When the disease is longstanding, corneal complications, including punctate epitheliopathy, vascularization, scarring, persistent epithelial defect, chronic corneal ulceration, and perforation, are common. Treatment with antibiotic regimens used for routine cases of conjunctivitis generally results in only temporary improvement. Recommended treatment strategies include the prolonged use of systemic anti-staphylococcal antibiotics, and intensive topical antibiotics and corticosteroids. More recently, supratarsal injections of antibiotics and steroids, along with irrigation and sweeping of the fornix with povidone-iodine solution, have been advocated. Given the increasing frequency of methicillin-resistant S aureus (MRSA) in the general population, conjunctival cultures before starting treatment can help guide the appropriate choice of antibiotic. In addition, surgical correction of the ptosis may play a role in management.
Chronic unilateral conjunctivitis can sometimes be caused by a malignant tumor involving the conjunctiva. The most common cause of this syndrome is sebaceous carcinoma of the eyelid. Sebaceous carcinoma usually arises in the meibomian gland, although it can also arise in the glands of Zeis or from sebaceous tissue in the caruncle. The disease occurs more commonly in women, and the upper lid is involved more frequently than the lower lid. The peak age is the fifth to eighth decade, although younger individuals are sometimes affected, particularly those with a history of periocular radiation. The primary lid tumor can be occult. The tumor has a predilection for intraepithelial spread through the conjunctiva, so-called "Pagetoid spread," in which case it can mimic inflamed conjunctival tissue or cause secondary inflammation. In cases of unexplained chronic unilateral conjunctivitis, any focal area of conjunctival thickening or nodularity should be biopsied (Figure 5), using appropriate fixation techniques (ie, not embedded in paraffin), and stained with oil red O dye as well as standard histologic stains. If the lesion is found to be malignant, appropriate treatment, whether surgical, radiation, or chemotherapy, is necessary to cure the conjunctivitis.
Chronic conjunctivitis can sometimes be the result of self-inflicted disease, often termed factitious conjunctivitis. This occurs when individuals gain some psychological benefit by occupying "the sick role," or, in some cases, there is a more tangible gain, in which case it would be termed malingering. A reported example of the latter involved soldiers causing conjunctivitis by placing scraped dental plaque in their eye in an effort to avoid arduous or dangerous duty. While factitious disease can be bilateral, as much secondary gain can usually be derived from unilateral disease, so there is usually little reason for the patient to involve the second eye.
Making a diagnosis of factitious conjunctivitis requires a very high index of suspicion, as affected individuals tend to be very good at denying their role in the disease and hiding their method of producing conjunctival inflammation. "Red flags" that might indicate the possibility of factitious conjunctivitis include an unrealistic history; noncompliance with medication regimens; a seeming indifference to the severity of the disease; failure to respond to what should be effective treatment; focal, rather than diffuse, disease; and unusual patterns of conjunctival staining. In the latter case, the inferonasal quadrant is most commonly involved, presumably because this is the easiest location for the patient to traumatize. Ultimately, treatment is psychiatric, but requires identifying the nature of the problem and confronting the patient with the diagnosis.
A variant of factitious conjunctivitis has been termed the mucus fishing syndrome. These patients have an underlying cause for chronic ocular surface inflammation, most commonly keratoconjunctivitis sicca, chronic blepharitis, or ocular allergy, causing a chronic ocular discharge. Affected patients try to mechanically remove the discharge, either with their fingers or a cotton applicator, causing conjunctival trauma. The resulting traumatic conjunctivitis further increases the amount of discharge, creating a vicious cycle. An almost universal finding is conjunctival staining in the inferonasal quadrant of the bulbar conjunctiva, presumably because that is the easiest site to try to remove the discharge from the eye. In contrast to patients with factitious conjunctivitis seeking secondary gain, these patients will readily admit the problem when they are advised of the likely pathogenesis. Treatment is directed at the underlying condition, with the admonition to avoid manipulation of the eye.