CHU Pontoise, France

A 25-year-old patient, G3, P2, is treated by her physician for a varicella at 9 weeks 2 days of gestational age.

An amniocentesis performed at 19 gestational weeks, shows the presence of a viral DNA in the amniotic fluid, suggesting a fetal infection. The alpha fetoprotein was at 1194mg/1 (n < 3250 mg/1); acetylcholinesterase is normal. The patient was given antiviral treatments (valacyclovir), for 15 days (1g of Zelitrex 3 times per day).

An ultrasound performed at 20 weeks 3 days demonstrates slight bilateral pyelectasis at 4.8mm and 5.0mm (Figure 2) and bright bowel (Figure****) less echogenic than adjacent bones. The biometry is normal, between the 30^th and 40^th percentile. Ten days later, at 21 weeks 4 days, neither the pyelectasis, nor the bright bowels are visible.

An ultrasound performed at 24 weeks 3 days is normal with a biometry between the 25^th and 30^th percentile. No limb anomalies are noted.

At 29 weeks, the biometry is between the 10^th and 40^th percentile. There is a slight bilateral clubfoot, with symmetric ankle masses (Figure****). The masses are relatively heterogeneous and have no vascular flow, measuring about 18 mm by 15 mm. No fetal breathing is noted.

At 31 weeks, a male fetus was born under peridural anesthesia, weighing 1350g (which is in the 30^th percentile for the age). The head perimeter is 302mm, which is between the 50^th and 90^th percentile for the age. The Apgar was 1 at 1 minute. The child was intubated and put on a ventilator immediately, cared for by the neonatal intensive care unit.

The clinical presentation demonstrates signs of congenital varicella:

• IgG = 2.2 et IgM <0.9

• In the cerebrospinal liquid IGG = 0.128 g/l (N = 0.005-0.049) and Ig M = 0.018 g/l, Interferon alpha = 32 UI/ml (N < ou = 2 UI/ml).

• The fibroblastic cultures were negative for varicella zoster virus

• The transfontanel ultrasound (performed at day 1) shows bilateral hyperechogenicity with neither ventricular dilation nor calcification

• Examination of the eyes did not suggest chorioretinitis or retinal necrose

• An MRI performed at 5 days demonstrated bilateral intra-ventricular hemorrhage with enlarged ventricles.

The neurological state of the fetus deteriorated and he died after 6 days.

The macroscopic and microscopic results of the autopsy demonstrated the presence of complete colonic atresia except a small segment of the right ascending colon. Cutaneus scars were also visible, as well as chronic hepatitis, which suggests congenital hepatitis. All of these lesions are associated with congential varicella.