Cytomegalovirus (CMV) Retinitis

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Definition

  • Most common ocular opportunistic infection in AIDS patients
  • Acquired (with immunosuppression) or congenital

Symptoms

  • Often asymptomatic
  • Floaters, flashes/photopsia, scotomas; usually no pain

Findings

  • Localized necrotizing retinitis:
    • whitish-yellowish retinal lesions with hemorrhages (“pizza pie fundus”), often starting peripherally (central in 10%) and spreading along vessels; peripheral areas more granular with fewer hemorrhages
    • Indolent (more peripheral, less aggressive) and fulminant courses possible
    • perivascular lesions/significant vascular sheathing (“frosted branch angiitis”)
  • Mild vitritis, possibly mild anterior chamber cells/flare
  • Complications: Retinal detachment (in about 1/3 if >25% of the retina is affected)

Treatment

  • HAART (Highly Active Antiretroviral Therapy)!
  • Antiviral therapy: High-dose induction therapy with subsequent maintenance therapy until CD4 cell count rises (therapy until at least CD4 cell count > 100-150 cells/μl)
  • Treatment options:
    • Valganciclovir orally 900 mg twice daily for 3 weeks + maintenance therapy (900 mg orally once daily)
    • Ganciclovir (Cymevene) intravenously 5 mg/kg body weight twice daily for 3 weeks + maintenance therapy (5 mg/kg body weight once daily)
    • Foscarnet intravenously 90 mg/kg body weight twice daily for 2 weeks + maintenance therapy (90-120 mg/kg body weight)
    • Cidofovir intravenously 5 mg/kg body weight once weekly + maintenance therapy (5 mg/kg body weight every 2 weeks)
    • Possible intravenous high-dose therapy (Ganciclovir or Foscarnet) followed by oral maintenance therapy with Valganciclovir
    • For lesions threatening vision (near the macula or optic nerve): Intravitreal Foscarnet or Ganciclovir
    • Infectious disease consultation

Congential CMV Infection

  • Most common congenital viral infection
  • Ophthalmological presentation
    • in 5–30% of confirmed cases
    • chorioretinitis, peripheral retinal scars, macular scars, optic atrophy or hypoplasia, cortical blindness/visual impairment, bilateral anterior polar cataract, stromal corneal scar, coloboma formation, microphthalmia, anophthalmia
  • Screening/Follow-up for confirmed congenital CMV infection
    • Initial examination right after birth
    • Possibly at the age of 6 months
    • Then annually until the age of 5 ¹ (or until the age of 10-12 ²)
      • Chorioretinitis can also develop later in life
  • Therapy for Chorioretinitis
    • For symptomatic chorioretinitis or asymptomatic chorioretinitis involving the posterior pole: Ganciclovir 6 mg/kg body weight intravenously every 12 hours
    • Benefits must be weighed against risks (neutropenia, gonadotoxicity, carcinogenicity)

Sources

  • EyeWiki CMV Retinitis
  • ¹ Consensus Guideline for the diagnosis and management of congenital cytomegalovirus infection (June 2015)
  • ² Ophthalmological Findings in Congenital Cytomegalovirus Infection: When to Screen, When to Treat?; Sofie Ghekiere, MD; Karel Allegaert, MD, PhD; Veerle Cossey, MD; Marc Van Ranst, MD, PhD; Catherine Cassiman, MD; Ingele Casteels, MD, PhD; J Pediatr Ophthalmol Strabismus 2012;49:274-282.
  • The Wills Eye Manual: Office and Emergency Room Diagnosis and Treatment of Eye Disease; Nika Bagheri MD, Brynn Wajda MD, et al; Lippincott Williams&Wilkins; 7th Edition (2016)
  • Kanski’s Clinical Ophthalmology: A Systematic Approach; Jack J. Kanski MD, Brad Bowling MD; Saunders Ltd.; 8th Edition (2015)