Congenital Varicella Syndrome: cause for CNS and sight abnormalities ?

I had chickenpox (varicella) during my fourth month of pregnancy. I was not immune to VZV as evidence of VZV antibodies were negative at the time of my pregnancy. Six months after my son's birth, he was diagnosed as suffering from: Sight: cortical dysplasia or pathway defect ;
Brain: MRI showed two large middle cranial fossa arachnoid cysts and parenchymal changes in the occipital lobes (heterotopias or cell migration errors), microcephaly but his head circumference at birth was normal (35cms). EEG was frankly epileptic. I was told by his Consultant Paediatric Neurologist that he will be left with Severe Learning Difficulties. He does not speak and cannot feed himself and still wears nappies as he is now three years old.

I would love to know if this is the result of me having chicken pox during pregnancy, as blood samples taken recently from my son showed the presence of VZV IgG, but he  had no chicken pox since birth except a heat-look-alike rash 4 to 5 months after his birth which disappeared within a day.

Looking forward to hearing from you soon,

Yours sincerely,

Safia

[CCF Neuro[P] MD, RPS]

Dear Safia:

Sorry to hear about the problems your son is having.  Certainly the neuronal migration problems seen in your son MIGHT be related to the VZV infection during the pregnancy.  But, there are several things that go against that thought. Prenatal infection with VZV is usually more of wide spread disorder with expecially the lung and adrenal gland involved.  The immunoglobulin seen in your newborn was likely the result of your passing your IgG antibody across the placenta.  The lesion in the CNS would be more of cystic lesions within the brain matter itself, rather than simple migrational problems.  The arachnoid cysts seen in your son are not usual type of process seen in VZV.  So, although anything is possible, I would favor that the neuronal migration problems seen in your son are not the result of in utero infection.

Hang in there with your son.  Early intervention is absolutely necessary, PT, OT, Speech, and interaction to produce as much as possible to reach his potential.  Nothing is written in stone about his future, so keep hopeful.

Sincerely,

CCF Neuro MD