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2003-08-12-09 Answer to case of the week #106 © Duchatel

Answer to case of the week #106

October 31-November 13, 2003

François Duchatel MD

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 1) and bright bowel (Figure 2) less echogenic than adjacent bones. The biometry is normal, between the 30th and 40th 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 25th and 30th percentile. No limb anomalies are noted.


At 29 weeks, the biometry is between the 10th and 40th percentile. There is a slight bilateral clubfoot, with symmetric ankle masses (Figures below). 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 30th percentile for the age). The head perimeter is 302mm, which is between the 50th and 90th 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 congenital  Varicella .








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