Infectious and Inflammatory Retinal Diseases
Brittany E. Powell
Victoria M. Hammond
THE CLINICAL CHALLENGE
Infectious and inflammatory retinal diseases are typically progressive and have significant ocular morbidity. Early and accurate diagnosis is critical to initiating immediate therapy and hinges on a high index of suspicion. The clinical challenge for the retinal pathology described in what follows is that the described disease entities are rare and might be encountered only occasionally during the career of any physician. However, a high index of suspicion with initiation of treatment and engagement with an ophthalmologist will result in dramatically improved outcomes.
PATHOPHYSIOLOGY
Infectious Retinal Inflammatory Syndromes
Exogenous Endophthalmitis
Endophthalmitis is an infection inside the eye. It most commonly occurs following surgery. Cataract surgery is the most frequent etiology, but it can also occur following glaucoma surgery, intravitreal injections, or rarely retina surgery. The incidence of endophthalmitis following cataract surgery has been reported to be from 0.012% to 1.3%.1 By definition, it occurs within 6 weeks of anterior segment surgery, but usually it presents within the first week after surgery. The most common organisms are coagulase-negative Staphylococcus species, Streptococcus species, and gram-negative organisms, specifically Pseudomonas species.1
Posttraumatic endophthalmitis is associated with an open globe injury and has been reported to have an incidence as high as 15% to 30%.2,3 The clinical presentation is variable and can be both rapidly progressive, as in posttraumatic Bacillus endophthalmitis, or more indolent, as in posttraumatic fungal endophthalmitis. In contrast to endophthalmitis following surgery, Streptococcus pneumoniae and gram-negative bacilli are much more prominent in these cases, although Staphylococcal infections also occur.3 Bacillus cereus needs to be assumed in those cases with a soil-contaminated intraocular foreign body. The prognosis of posttraumatic endophthalmitis is much worse and can result in loss of the eye within hours after the injury.
Endogenous Endophthalmitis
Endogenous endophthalmitis via hematogenous spread of infectious organisms is rare, with an incidence of 0.04% to 0.4% but requires an extensive systemic workup and rapid treatment.4 Endogenous endophthalmitis has been associated with intravenous drug use, endocarditis, diabetes mellitus, immune compromise, malignancy, prolonged hospital admission, and intravenous antibiotic administration.5 Worldwide, common causes included liver abscesses, closely followed by pneumonia, endocarditis, sustained intravenous catheters, and meningitis. In cases of suspected
endophthalmitis, while actively addressing the infection of the eye, it is important to simultaneously investigate for the underlying source of hematogenous spread.
endophthalmitis, while actively addressing the infection of the eye, it is important to simultaneously investigate for the underlying source of hematogenous spread.
Other Infectious Retinal Inflammatory Syndromes
Patients with retinal inflammatory syndromes from infection are typically immunocompromised and present with gradually decreasing vision and floaters. These infectious triggers lead to a severe immune-mediated inflammatory cascade on the retina and an obliterative vasculitis. Infections are most commonly caused by members of the herpes virus family (varicella zoster, herpes simplex viruses, cytomegalovirus, and Epstein-Barr virus). Other pathogens can result in retinal inflammation and should be considered in patients with known disease and eye symptoms, including syphilis, tuberculosis, toxoplasmosis, toxocariasis, Lyme disease, and West Nile virus. Viral meningoencephalitis has also been reported in association with viral necrotizing retinitis.
Noninfectious Retinal Inflammatory Syndromes
There are a variety of systemic inflammatory disorders that can affect the retina, including systemic lupus erythematosus-associated vasculitis, multiple sclerosis with pars planitis, sarcoidosis, and Bechet disease. The presence of a hypopyon in a patient who has not had intraocular surgery and no evidence infectious etiologies such a corneal ulcer has been reported in patients with Bechet disease and HLA-B27-associated acute anterior uveitis. Referral to an ophthalmologist should be considered if patients with known inflammatory disease are experiencing visual symptoms.
APPROACH/THE FOCUSED EXAM
The most important part of the exam is the medical history and timeline of symptoms in order to develop an adequate differential diagnosis. Endophthalmitis is rare but must be suspected in patients experiencing vision loss, pain, and photophobia following intraocular surgery or trauma. Endogenous endophthalmitis must be considered in those who are systemically ill or immunocompromised with gradual deterioration of vision or worsening of visual symptoms. Inflammatory retinal diseases typically present with gradually worsening photophobia, photopsias, floaters, and decreased vision. In contrast to iritis, vision loss is usually severe. Consideration for an undiagnosed systemic inflammatory condition in these patients is critical.
The history should include recent trauma, recent intraocular surgery, current medications, and the potential for immune suppression caused by underlying diseases or medication use. Is the patient experiencing vision loss, pain, eye discharge, redness, flashes, or floaters? When did the symptoms start? Have the symptoms progressed over hours, days, or weeks? Patients who have had glaucoma surgery are at risk for endophthalmitis even months or years after surgery, owing to the presence of a drainage bleb or implant. After cataract surgery or intraocular injection, the risk of endophthalmitis is significant only in the acute postoperative period.
The examination should consist of vital signs, visual acuity, intraocular pressure, and an external exam of the eyelids to assess for swelling, redness, and discharge. A slit lamp exam can assess for a red reflex, conjunctival injection, evidence of an open globe such as a corneal laceration, or a hypopyon (Figures 43.1 and 43.2). An ophthalmic ultrasound can also be helpful in showing vitritis but is not mandatory.