The hallmark of congenital varicella syndrome (chikenpox) in the neonatal period is the presence of cutaneous scars (Figure 306.1). Neonates with congenital varicella may present with brachial plexus palsy, limb hypoplasia, Horner syndrome, or seizures. These presentations result from invasion of the nervous system by the varicella virus. Congenital varicella syndrome occurs in infants exposed to maternal varicella between 8 and 20 weeks gestation. The incidence of congenital varicella syndrome in a mother infected with varicella prior to 20 weeks of pregnancy is 1% to 2%.

Figure 306.1.— Typical cutaneous scars in the shoulder area due to congenital chickenpox infection.

This entity is characterized by macrocephaly, cutis marmorata telangiectatica, asymmetrical head, face or body, overgrowth and hemangioma of the lip and philtrum. The most frequent MRI findings are hemimegalencepahly, hydrocephalus, hypoplasia of the corpus callosum, and white matter abnormalities. These patients may develop different types of neoplasm. The cause is unknown. Increase paternal age was noted in some studies.

A		B

Figure 299.1.—— A. cutis marmorata telangiectatica. B. White matter abnormalities.