Congenital Cytomegalovirus

Aka: Congenital Cytomegalovirus, Congenital CMV

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II. Pathophysiology

  1. CMV is a TORCH Virus acquired in utero

III. Findings

  1. Asymptomic or Minimally Symptomatic (most cases)
    1. Isolated Hearing Loss occurs in 10% for early onset (another 10% late onset)
    2. Progressive Hearing Loss occurs in 40 to 65% with Hearing Loss
    3. Hearing Loss risk increases with first trimester CMV infection
  2. Symptomatic (10 to 15% of cases)
    1. Microcephaly
    2. Intrauterine Growth Retardation
    3. Hepatitis
    4. CMV Retinitis
    5. Cerebritis
    6. Seizures
    7. Long term Intellectual Disability (most common viral cause)
    8. Petechiae (due to Thrombocytopenia), or other rash
    9. Hearing Loss (33% of cases)

IV. Labs: Congenital CMV Screening

  1. Perform in first 3 weeks of life (prevents False Positives from postnatal CMV infection)
  2. Dry Blood Spot PCR
    1. Initial Screening Test as part of Universal Screening panel
  3. Saliva PCR
    1. Typical first-line screening in symptomatic CMV or failed Hearing screening
    2. False Positive from Saliva contaminated by maternal Breast Milk
  4. Urine PCR
    1. Indicated as confirmatory testing for positive Dry Blood PCR or Saliva PCR
    2. Also consider as first-line study after failed Hearing sceen or suspected symptomatic CMV

V. Evaluation: Confirmed Congenital CMV

  1. Exam
    1. Growth and Development
    2. Hepatomegaly
    3. Neonatal Jaundice
    4. Rash
  2. Labs
    1. Complete Blood Count with differential
    2. Liver Function Test
  3. Imaging: Head Ultrasound
    1. Periventricular Calcifications
    2. Lenticulostriate Vasculopathy
  4. Specialty Consultation
    1. Audiology evaluation
    2. Ophthalmology (evaluate for CMV Retinitis)

VI. Management: Antiviral

  1. Indications: Mixed Efficacy
    1. Symptomatic CMV
  2. Efficacy
    1. Treatment efficacy studies have been limited to symptomatic Congenital CMV
    2. May reduce Intellectual Disability for symptomtic Congenital CMV
    3. No strong evidence of Hearing protection in asymptomatic Congenital CMV
      1. However may have Hearing protection benefit at 12 to 24 months
    4. References
      1. Kimberlin (2015) N Engl J Med 372(10):933-43 +PMID: 25738669 [PubMed]
  3. Dosing
    1. Valganciclovir 16 mg/kg/dose orally twice daily for 6 months
  4. Monitoring
    1. Complete Blood Count with differential
      1. More frequently first 6 to 8 weeks (then monthly)
    2. Liver Function Tests monthly

VII. References

  1. Gaensbauer (2024) Mayo Clinic Pediatric Days, lecture accessed 1/17/2024

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