At 13+6 weeks gestation, a 37 year old para 1+2 woman presented after her daughter developed chickenpox. As booking bloods were IgG negative for varicella, varicella zoster immunoglobulin was administered. At 16 weeks, she developed chickenpox lasting 14 days. No other maternal complications. 20 week detailed anomaly scan normal. Repeat scanning at 28+6 weeks revealed dispersed liver calcification and borderline ventriculomegaly (10.8-11.4mm). Fetal MRI at 31 weeks, showed asymmetric mild ventriculomegaly with no associated brain abnormality and no structural abnormality in the abdomen.
Baby born at term by SVD. Clinical examination normal other than 3 skin lesions:1)6x3cm aplasia cutis congenita, right groin 2)0.8x0.5cm scar, left axilla 3)0.6x0.5cm scar, philtrum.
Postnatal cranial ultrasound showed tiny calcific foci within deep grey matter structures bilaterally. Abdominal ultrasound demonstrated multiple calcific foci within liver, spleen, adrenal glands. Mildly elevated AST on LFTs. Initial urine/nasal/skin swabs positive for VZV at very low levels. Repeat day 4 swabs negative. As baby was well, acyclovir was not immediately given. Following discussion with Virology, oral acyclovir commenced on day 5 with intention of reducing risk of re-emergence of active infection. However at 2 weeks, baby developed herpes zoster and admitted for intravenous acyclovir. This healed well, he is making good progress and oral acyclovir discontinued.
This illustrates a rare case of congenital varicella syndrome which has a recognised spectrum of anomalies. There is little information available however to guide the clinician on the likely impact of infection when anomalies such as hepatic calcification or ventriculomegaly are seen antenatally.
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