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Symptoms ⬇

Decreased vision, floaters, and pain that is often bilateral. Patients typically have a history of recent hospitalization, recent abdominal surgery, being immunocompromised, possessing a long-term indwelling line or catheter (e.g., for hyperalimentation, hemodialysis, or antibiotics), or using intravenous drugs.

Signs ⬆ ⬇

(See Figure 12.17.1.)

Critical

Discrete, multifocal, yellow-white, choroidal to chorioretinal fluffy lesions from one to several disc diameters in size. With time, the lesions increase in size, break into the vitreous, and appear as “cotton balls” or a “string of pearls”.

Other

Vitreous cell and haze, vitreous abscesses, retinal hemorrhages with or without pale centers, anterior chamber cells, and hypopyon. A retinal detachment may develop.

12-17.1 Candida chorioretinitis with vitreous involvement.

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Differential Diagnosis ⬆ ⬇

The following should be considered in immunocompromised patients.

  • CMV retinitis: Multifocal areas of granular retinal whitening with a minimal to mild vitreous reaction, more likely to be associated with retinal hemorrhage. See 12.9, CYTOMEGALOVIRUS RETINITIS.
  • Toxoplasmosis: Yellow-white retinal lesions often with an adjacent chorioretinal scar. See 12.5, TOXOPLASMOSIS.
  • Pneumocystis choroidopathy: Rare manifestation of widely disseminated Pneumocystis carinii infection. Usually in AIDS patients. Often asymptomatic. History of P. carinii and treatment with aerosolized pentamidine. Multifocal, yellow, round, deep choroidal lesions approximately one-half to two-disc diameters in size, located in the posterior pole. No vitritis. Patients are often cachectic. Treatment is with i.v. trimethoprim/sulfamethoxazole or i.v. pentamidine in conjunction with an infectious disease specialist.
  • Others: Herpes viral retinitis; Nocardia, Aspergillus, and Cryptococcus species; Atypical Mycobacterium, coccidioidomycosis, and others.

Work Up ⬆ ⬇

Workup
  1. History: History of bacteremia or fungemia? Underlying medical conditions? Medications? Indwelling catheter? Intravenous drug use? Other risk factors for immunocompromised state?
  2. Skin examination for signs of intravenous drug injection.
  3. Most clinicians recommend that all patients with candidemia have a complete, dilated fundoscopic examination (ideally within 72 hours), as ocular involvement may be asymptomatic. A repeat fundoscopic examination is recommended 2 weeks after the initial negative examination.
  4. Blood, urine, and catheter tip fungal cultures; these often need to be repeated several times and may be negative despite ocular candidiasis. Blood cultures may need to be held a full 7 days and may take 3 to 4 days to become positive for Candida species.
  5. Consider vitrectomy to obtain a specimen and remove opacified vitreous. Cultures and smears can confirm the diagnosis. Amphotericin B 5 to 10 µg in 0.1 mL or voriconazole 50 to 100 µg in 0.1 mL is injected into the vitreous cavity after the procedure.
  6. Baseline CBC, renal function tests, and liver function tests.

Treatment ⬆ ⬇

  1. Suspected fungal endophthalmitis without a clear source should be considered evidence of a disseminated infection and requires further systemic evaluation and workup.
  2. Hospitalize all unreliable patients, systemically ill patients, or those with moderate to severe vitreous involvement.
  3. An infectious disease specialist should be consulted for systemic workup to evaluate for a source and other sites of involvement.
  4. Typically, chorioretinitis without vitreous involvement can be successfully treated with systemic therapy alone with one of the following regimens: Fluconazole 800 mg p.o. loading dose followed by 400 to 800 mg p.o. daily. Alternatively, voriconazole 400 mg i.v. b.i.d. daily for 2 doses followed by 300 mg i.v. or p.o. b.i.d. may also be considered in fluconazole-resistant species. For fluconazole- and voriconazole-resistant species, liposomal amphotericin B (3 to 5 mg/kg i.v. daily) is recommended. Therapy should be guided by cultures and sensitivities. Other agents that may be used include caspofungin, itraconazole, and micafungin.
  5. Intravitreal injection of antifungal agents as above (voriconazole or amphotericin B) if there is vitreous involvement. Depending on the response and location of retinal involvement (anterior versus posterior), injections may be repeated.
  6. Topical cycloplegic agent (e.g., atropine 1% b.i.d. to t.i.d.).
  7. See 9.7, INFLAMMATORY OPEN ANGLE GLAUCOMA, for IOP control.

Follow Up ⬆

  1. Patients are seen daily early on. Visual acuity, IOP, and the degree of anterior chamber and vitreous inflammation are assessed.
  2. Patients receiving azole antifungals require liver function tests every 1 to 2 weeks and as clinically indicated. Patients receiving amphotericin require monitoring of electrolytes, kidney function, and CBC as directed by an infectious disease specialist.