Keywords

Overview

  • Definition

    • An acute, contagious, exanthematous disease caused by Rubrivirus

    • Spread only by humans, via respiratory route or transplacentally

    • Essentially extinct in the United States after the introduction of the measles, mumps, and rubella (MMR) vaccines in 1969; about 20,000 were born with congenital disease in mid-1960s

    • In the United States, acquired cases are typically contracted from foreign visitors or travel

  • Symptoms

    • Redness

    • Purulent discharge

    • Photophobia

    • Sudden vision loss

  • Laterality

    • Unilateral or bilateral

  • Course

    • Acute and self-limited

  • Age of onset

    • All age groups affected

  • Gender/race

    • No gender or racial predilection

  • Systemic association

    • Congenital rubella syndrome (CRS)

      • Cardiac, hearing, and ocular defects

      • Severity depends on time of maternal disease contraction: most severe during first trimester (miscarriage may result); defects unlikely after 20 weeks gestation

    • Acquired rubella (German measles)

      • Spread by respiratory route; common in spring and winter

      • Incubation period of 2–3 weeks; hosts are contagious 1 week prior to rash onset and throughout active symptoms

Exam: Ocular

Congenital Rubella

Ocular findings can be present at birth, shortly after birth, or later in life

  • Salt-and-pepper retinopathy (inactive chorioretinitis, usually confined to RPE)

    • Unilateral or bilateral

    • Typically stable but may progress later in life

    • Vision ranges from normal to 20/200

    • Rarely complicated by CNV. May have focal necrosis of ciliary epithelium, pars plicata, or pars plana

  • Nuclear cataract

    • Significant AC inflammation after cataract extraction due to liberation of live virus from lens. Some cataracts have been known to resorb, leaving KP’s behind

  • Glaucoma

    • Corneal clouding and buphthalmos

  • Microphthalmia

Acquired Rubella

  • Conjunctivitis

    • Most common ocular finding (70%)

  • Epithelial keratitis

    • Infrequent and resolves without sequelae within 1 week

  • Retinitis

    • Rare

    • May present as multifocal exudative RD and RPE detachment

    • No retinal hemorrhage

  • Chronic rubella has been implicated as a cause of Fuchs’ heterochromic iridocyclitis (Chap. 11)

Exam: Systemic

Congenital Rubella

  • Unilateral or bilateral deafness (>80%)

  • Cardiac malformations: patent ductus arteriosus, peripheral pulmonary artery stenosis, and interventricular septal defects

Acquired Rubella

  • Rubella exanthem

    • Erythematous and maculopapular

    • Spreads from face down to hands and feet over 24 hours

    • Resolves by day 3 (thus second nickname “3-day measles”)

    • Not always present

  • Fever

    • Variable

    • After rash in children; before rash in adolescents and adults

  • Postauricular and suboccipital lymphadenopathy

  • Other complications: arthritis, encephalitis, thrombocytopenic purpura

Imaging

  • FA

    • Abnormal hyperfluorescence and hypofluorescence in salt-and-pepper retinopathy

    • Early hyperfluorescence with late leakage in CNV

    • Hyperfluorescence associated with area of retinitis without vascular leakage in acquired rubella

  • ERG

    • Normal in salt-and-pepper retinopathy

Laboratory and Radiographic Testing

  • Maternal serum rubella titers

  • Viral culture and/or reverse-transcriptase PCR of amniotic fluid, nose, throat, urine, blood, or cerebrospinal fluid (CSF)

  • Serum IgM titer is useful in children with anomalies from uneventful pregnancies

  • Hearing test

  • Echocardiogram

Differential Diagnosis

Congenital Anomalies

  • TORCH infections

    • Toxoplasmosis

    • Other infections (syphilis, parvovirus, varicella zoster, Zika)

    • Rubella

    • Cytomegalovirus

    • Herpes simplex

Salt-and-pepper Retinopathy

  • Congenital syphilis

  • Congenital measles

  • Leber’s congenital amaurosis

  • Retinitis pigmentosa carrier

  • Choroideremia carrier

  • Albinism carrier

  • Cystinosis

  • Drug toxicity (e.g., phenothiazine)

  • Kearns–Sayre syndrome

Salt-and-pepper Retinopathy and Hearing Loss

  • Congenital syphilis

  • Usher’s syndrome

Treatment

Congenital Rubella

  • Mothers infected during first trimester should be counseled about possible birth defects

  • Immune globulin within 72 hours of exposure during pregnancy

  • Rubella vaccination is contraindicated during pregnancy

  • CRS infants are contagious at birth and need isolation

Acquired Rubella

  • Treatment is supportive, as there is no known antiviral therapy for rubella

  • Topical NSAIDs and artificial tears to reduce conjunctival hyperemia

  • Retinitis can benefit from corticosteroids

Referral/Co-management

  • Audiology

  • Cardiology

  • Infectious Disease