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How We Nailed the Diagnosis of an Atypical Case
George Graham* was a healthy, 19-year-old, black male who had never experienced any significant eye problems—but this was about to change. As part of his electrician’s apprenticeship he had been helping to rewire an old textile mill. After a long day at work, he was relaxing at his girlfriend’s house when she accidentally poked him in the eye with an acrylic fingernail. This caused him mild redness and irritation, but, to save her blushes, he shrugged it off as no big deal.
Three Weeks of SufferingTwo days later, however, his vision blurred, his discomfort intensified and he decided to visit the local emergency room. He was diagnosed with a corneal abrasion and given gentamicin drops to apply every four hours. Another two days later, Mr. Graham was examined by a local optometrist and told to continue the gentamicin drops. At this point, the follow-up plan was unclear to Mr. Graham, who continued the medication unreliably without significant improvement in his vision or symptoms. In fact, both worsened significantly.
Mr. Graham attempted to “tough it out” with hope that things would spontaneously improve. Finally, three weeks following the initial injury, he visited an ophthalmologist, at which time he was referred to our emergency room for evaluation and further management.
We Get a Look
The exam. Mr. Graham arrived in our emergency room at approximately 10 p.m. His visual acuity measured count fingers at three feet in the right eye and 20/20 in the left; his intraocular pressure was 12 and 17 mmHg, respectively. Upon further examination of the right eye, we noted mild periorbital soft- tissue swelling, significant upper eyelid edema, mild purulent discharge, 3+ conjunctival injection, severe chemosis and a 6-mm x 2-mm corneal ulcer adjacent to the superior limbus. Furthermore, a superior limbal corneal perforation was evident with the iris prolapsing through the wound for 3 clock hours. We found no evidence of hypopyon, and echography showed no evidence of endophthalmitis. Examination of the left eye was unremarkable.
The differential diagnosis. At this time, our differential included infectious keratitis, gonococcal keratoconjunctivitis, traumatic corneal laceration with secondary infectious keratitis, Mooren’s ulcer and peripheral ulcerative keratitis. Given the young age of the patient and his clinical presentation, more history was obtained. The patient denied contact lens wear, recent drug use or trauma, other than that noted in the initial history. He was sexually active with his girlfriend—who was present as we took his history—but denied any previous or recent STDs, urethritis, rashes, pharyngitis or arthritis.
The workup. We cultured the cornea and conjunctiva (chocolate agar, 5 percent blood agar, Sabouraud’s agar and thioglycollate broth) and prepared a smear for gram-stain and Gomori’s methenamine silver stain. Mr. Graham was admitted to our hospital as an inpatient and was started on fortified topical vancomycin and tobramycin every hour. Because of the history of trauma and clinical findings, a CT scan was obtained to evaluate for intraocular foreign bodies and orbital cellulitis, which was normal. We scheduled the patient for surgery in the morning and instructed him not to take any oral foods or fluids. Despite the negative sexual history and denial of other concerning symptoms or risk factors, a clinical suspicion for gonococcal keratoconjunctivitis prompted us to treat him with 1 g of intramuscular ceftriaxone.
What's Your Diagnosis ?
The following morning, the gram stain showed only acute inflammatory cells without evidence of any organisms. The patient was continued on topical fortified antibiotics and was scheduled for therapeutic penetrating keratoplasty in the morning.
The morning of surgery, the microbiology lab reported growth of 3+ gram- negative diplococci, prompting a directed discussion with the patient. He now disclosed a recent history of several STDs including Chlamydia, herpes simplex virus and a recent history of purulent penile discharge treated with an incomplete course of medication, which the patient could only describe as “blue pills.”
The cultures were subsequently confirmatory for Neisseria gonorrhoeae resistant to penicillin and tetracycline and sensitive to ciprofloxacin and ceftriaxone.
The patient underwent successful penetrating keratoplasty. On histopathologic examination, acute and chronic inflammatory cells were noted in the corneal stroma near the area of perforation. Bacterial stains and cultures of the corneal specimen were negative. Postoperatively, the patient did well on moxifloxacin and prednisone 1 percent without any recurrence of the gonococcal infection.
Eight months after initial presentation, the visual acuity was 20/40 with a clear corneal graft. Subsequent to the initial presentation the patient underwent a test for HIV, which was negative. The patient was given extensive counseling—both about STD transmission and the implications of his disease—and his sexual contacts within the previous 60 days were informed of the diagnosis. We also reported the case to the CDC.
Background and Findings
Ocular infections with N. gonorrhoeae have potentially devastating visual consequences. When left untreated, or treated inappropriately, the condition can progress rapidly, leading to corneal ulceration, corneal perforation and, ultimately, severe visual compromise. The timeliness of an accurate diagnosis and subsequent initiation of appropriate parenteral antibiotics is critical.
The hallmark of the disease is a hyperacute (less than 24 hours) purulent conjunctivitis, classically with upper lid edema and severe chemosis. It typically occurs in young, sexually active people. Approximately 75 percent of cases occur in black Americans and 85 percent occur in people who are between the ages of 15 and 29.
In a review of 68 cases of gonococcal conjunctivitis, including 11 bilateral cases, keratoconjunctivitis occurred in approximately 30 percent of patients and limbal corneal perforations in 10 percent of patients.1 The degree of corneal involvement was closely related to the duration of symptoms prior to presentation and inappropriate treatment with topical antibiotics.
In the past, the virulence of N. gonorrhoeae has been attributed to its ability to penetrate an intact corneal epithelium. However, the majority of pathologic specimens reveal little bacterial invasion. It is known that N. gonorrhoeae can cause marked conjunctival chemosis and massive infiltration with polymorphonuclear neutrophils (PMNs). It has been suggested that this edematous PMN-infiltrated conjunctiva can then drape over the peripheral cornea, exposing the underlying area to the neutrophilic lytic enzymes capable of producing epithelial erosion and ulceration.2 This inflammation may lead to rapid and severe stromal thinning and, in some cases, perforation.
Management DiscussionAct swiftly. This case exemplifies the need for prompt evaluation and appropriate treatment in all patients presenting with a limbal corneal perforation with or without a hyperacute purulent conjunctivitis. A presumptive diagnosis can be made in classic cases on the clinical appearance and gram stain, if available. However, parenteral treatment should not be delayed while waiting for confirmatory testing because the outcome of gonococcal conjunctivitis is oftentimes closely related to the severity of disease at the start of adequate therapy.
Follow closely. All patients with suspected gonococcal conjunctivitis should be monitored closely. Topical management includes normal saline lavage to remove the inflammatory and infectious debris. Adjunctive topical antibiotics may also be used but have not been shown to significantly affect visual outcomes and may be unnecessary. Parenteral antibiotics are necessary in all cases. Last April 12, the CDC issued a statement recommending ceftriaxone and cefixime as first-line agents against gonococcal infections as a result of an increase in fluoroquinolone-resistant strains.3 Consequently, all patients suspected of having gonococcal conjunctivitis should be treated with 1 g of intramuscular or intravenous ceftriaxone as a single dose.4 Furthermore, patients should be treated concurrently for Chlamydia and referred to their primary care physician for evaluation of other STDs.
Contact the CDC. Gonococcal conjunctivitis is a reportable disease and all cases should be reported to the CDC.
This atypical case illustrates several learning points.
First, not all patients with gonococcal conjunctivitis will present in the typical hyperacute and hyperpurulent fashion, particularly in delayed presentations and partially treated cases.
Second, gonococcal keratoconjunctivitis should be in the differential diagnosis of any patient who presents with acute inflammation and a peripheral corneal perforation.
Third, patients may present without a known history of gonococcal urethritis or may deny related symptoms, sexual and STD history. Therefore, it is important to maintain a high suspicion for gonococcal conjunctivitis and a low treatment threshold in young otherwise healthy patients who present with signs suggestive of gonococcal infection.
* Patient name is fictitious.
1 Ullman, S. et al. Ophthalmology 1987;94:525–531.
Dr. Feilmeier is a resident, Dr. Karp is an associate clinical professor of ophthalmology and Ms. Valins is a medical student. All are at the University of Miami.