Sandbox: Reddy

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Aravind Kuchkuntla, M.B.B.S[2]

Historical Perspective

• In 1947, the first case of congenital varicella syndrome was reported.

Pathophysiology

Pathogenesis

• Once a pregnant women has a primary varicella infection, transplacental transmission of the virus can take place affecting the fetus in utero.
• The risk of abortion is high when the infection in the fetus occurs before 20 weeks of gestation.

Natural History, Prognosis and Complications

Prognosis

Prognosis of infants with congenital varicella syndrome is poor, early death is due to gastroesophageal reflux and recurrent aspiration pneumonia and respiratory failure.

Complications

• Fetal demise
• Intrauterine growth restriction
• Hydrops
• Limb deformities
• Microcephaly

Diagnosis

Physical Examination

Clinical manifestations suggestive of Congenital varicella syndrome include:

	Clinical Manifestations in congenital varicella syndrome
Skin	Cicatricial lesions Cutaneous defects Hypopigmentation
Eye	Chorioretinitis Cataracts Micropthalmia Anisocoria
Central Nervous System	Intrauterine encephalitis Cortical atrophy/porencephaly Seizures Mental retardation Autonomic instability
Musculoskeletal system	Limb hypoplasia Muscle hypoplasia
Gastrointestinal	Gastrointestinal reflux
Systemic Manifestations	Intrauterine growth retardation Developmental delay
Urinary Tract	Hydroureter Hydronephrosis