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Case Reports
, 168 (5), 561-3

Severe Hydrocephalus Associated With Congenital Varicella Syndrome

Case Reports

Severe Hydrocephalus Associated With Congenital Varicella Syndrome

Massimo Mazzella et al. CMAJ.


Congenital varicella syndrome refers to the spectrum of fetal anomalies associated with maternal varicella zoster virus (VZV) infection during the first trimester of pregnancy. The syndrome is rare and the risk to the fetus uncertain. We describe an unusual case of congenital varicella syndrome in which hydrocephalus was the main consequence and likely represented VZV reactivation in utero.


Fig. 1: Gross appearance of patient showing hydrocephalus, left-sided microphthalmia and cutaneous ulceration involving the head, neck and right lower limb.
Fig. 2: Panel A: Necrotizing encephalitis with varying degrees of gliosis and mononuclear infiltration by lymphocytes and plasma cells (hematoxylin–eosin, original magnification х10). Panel B: Focal discontinuities of the ependymal lining with subependymal infiltration by lymphocytes and gliosis (hematoxylin–eosin, original magnification х10). Panel C: Perivascular cuffing of the subependyma vessels by lymphocytes (hematoxylin–eosin, original magnification х25).

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