January 29, 2020
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Woman experiences 4 months of unilateral irritation, decreased vision

Eversion of the left upper lid revealed a mixed follicular and papillary reaction of the tarsal conjunctiva without obvious discharge or membrane formation.

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Alison J. Lauter
Alison J. Lauter
Sarah E. Thornton
Sarah E. Thornton

A 35-year-old woman was referred to the New England Eye Center for a second opinion regarding 4 months of foreign body sensation, discharge and mild blurred vision in the left eye. She had been evaluated by several outside ophthalmologists who prescribed topical antibiotics for presumed bacterial conjunctivitis, but the treatment did not improve her symptoms. The patient’s referring ophthalmologist diagnosed her with giant papillary conjunctivitis and trialed 2 weeks of prednisolone and ketotifen, which was ineffective.

Before this episode, the patient had no ocular history and had never worn contact lenses. A detailed review of systems, including autoimmune, GI/GU, musculoskeletal, skin and constitutional symptoms, was negative. The patient’s medical history included mild asthma and chlamydial cervicitis, which had been fully treated years earlier. Her family and surgical history was unremarkable, and she was on no medications. She was a smoker with a 20 pack-year history. She was monogamous with her husband. She had recently been exposed to farm animals and exotic birds. Her travel history included a trip through Saudi Arabia, Egypt, Turkey and Jordan within the previous 6 months.

Trace swelling
Figure 1. Trace swelling of left upper eyelid.

Source:Gavin Gorrell, MD, and Helen Wu, MD

mixed follicular reaction
Figure 2. Everted left upper eyelid demonstrates mixed follicular reaction with scattered papilla. Large follicles can be seen nasally (a), and smaller, more numerous follicles are observed temporally (b).

Examination

The patient’s best corrected visual acuity was 20/20 in the right eye and 20/25 in the left eye. Pupils were symmetric with no afferent pupillary defect. IOP was within normal limits in each eye. Motility was full.

On external examination, the left upper eyelid was mildly swollen with no skin lesions (Figure 1). On slit lamp examination, the anterior segment was unremarkable on the right and notable for superficial punctate keratopathy concentrated superiorly in the left eye. Eversion of the left upper lid revealed a mixed follicular and papillary reaction of the tarsal conjunctiva without obvious discharge or membrane formation (Figures 2a and 2b). Fundus examination was unremarkable in both eyes.

What is your diagnosis?

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Irritation, decreased vision

Follicular conjunctivitis may be classified as chronic when the duration of symptoms is longer than 4 weeks. The differential diagnosis of chronic follicular conjunctivitis includes multiple infectious, inflammatory and toxic etiologies, so a detailed history is necessary to determine the appropriate management. The most common causes of chronic follicular conjunctivitis include toxic conjunctivitis from topical medication usage, molluscum contagiosum and chlamydial infection. Less common potential etiologies include sarcoidosis and tuberculosis. It is important to differentiate chronic follicular conjunctivitis, which has associated injection and irritation, from benign lymphoid folliculosis, which can be observed in children and young adults.

Workup and management

In our patient with a history of chlamydial cervicitis, exposure to exotic birds including parrots and travel to endemic areas of Chlamydia trachomatis, chronic follicular conjunctivitis due to chlamydial infection was felt to be the most likely diagnosis. With the possibility of a urogenital source, urine studies were obtained, and co-testing for other sexually transmitted diseases, including gonorrhea and syphilis, was performed. A chest radiograph and blood work were ordered to rule out a diagnosis of sarcoidosis or tuberculosis.

Superior tarsal follicular reaction
Figure 3a. Superior tarsal follicular reaction with mucoid discharge.

Source: American Academy of Ophthalmology, copyright 2019

Given the strong clinical suspicion for a chlamydial infection, the patient was empirically started on doxycycline 100 mg twice daily.

Limbal depressions
Figure 3b. Limbal depressions (Herbert’s pits) due to apposition of superior limbus with necrotic tarsal follicles.

Source: American Academy of Ophthalmology, copyright 2019

One week later, the patient reported moderate improvement in her symptoms, and she was informed that her conjunctival swab was positive for chlamydial conjunctivitis.

linear scarring of the superior tarsal conjunctiva
Figure 3c. Healing of necrotic tarsal follicles leads to linear scarring of the superior tarsal conjunctiva (Arlt’s line) with multiple cycles of infection.

Source: American Academy of Ophthalmology, copyright 2019

Discussion

The two major types of chlamydial conjunctivitis are inclusion body conjunctivitis (serotypes D-K) from contact with infected genital secretions and trachoma (serotypes A-C) from contact with infected ocular or nasal secretions. A rare form of chlamydial conjunctivitis has been documented from Chlamydophila psittaci (formerly Chlamydia psittaci), which is more commonly associated with pneumonia after exposure to parrots.

Given the history of recent travel to endemic trachoma regions, exposure to exotic birds including parrots and history of chlamydial urethritis, it was possible that the patient’s chlamydial conjunctivitis could have been related to trachoma, inclusion body conjunctivitis or C. psittaci. Her normal urine studies and lack of genitourinary symptoms made inclusion body conjunctivitis unlikely, and her normal chest X-ray and lack of anti-C. psittaci antibodies made C. psittaci conjunctivitis unlikely as well. Given her travel to endemic areas around the time of symptom onset, it was felt that trachoma was the most likely etiology of her culture-positive chronic follicular chlamydial conjunctivitis.

Chlamydia trachomatis affects approximately 150 million people worldwide. It is endemic in the Middle East and parts of Africa but is rare in the United States and other developed countries. Trachoma presents like viral conjunctivitis with mucus discharge and a red, irritated eye. On exam, a follicular reaction can be seen primarily in the upper tarsal conjunctiva (Figure 3a), which may be accompanied by epithelial keratitis and a superficial fibrovascular pannus at areas of apposition with inflamed conjunctiva. As follicles regress over several weeks, they can become necrotic and cause limbal depressions (Herbert’s pits) as well as subconjunctival scarring (Arlt’s line) and fibrosis (Figures 3b and 3c). Repeated episodes of trachoma conjunctivitis cause successive cicatrization, leading to entropion, trichiasis and corneal blindness from mechanical and superimposed infectious corneal insults.

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Transmission of the obligate intracellular bacteria C. trachomatis occurs when ocular or nasal secretions from a carrier are inoculated into a healthy eye through direct contact or through fomites such as bedding or shared towels. Flies are thought to be a significant vector as some species feed on ocular secretions and can rapidly transmit infection. For these reasons, trachoma primarily affects areas with poor sanitation and limited access to clean water.

Acute treatment of trachoma is accomplished with oral antibiotics or topical antibiotics, most commonly a single dose of 1 g azithromycin or through 100 mg doxycycline given twice daily for 2 weeks. Topical therapy can be used but requires 2 months of twice daily erythromycin or tetracycline, leading to a greater chance of missed doses and incompletely treated infections. The WHO developed a protocol for the treatment and eradication of trachoma in endemic areas. This SAFE protocol employs surgery for trichiasis, antibiotics given en masse to the community, facial cleanliness education, and environmental improvements to remove breeding areas for flies.