We report a case of extensive thrombosis in a 12 year old boy, who had
varicella 15 days earlier. The child presented with headache and
generalized tonic clonic seizures for 2 hours. On examination, he had
healed scars of varicella. Neurological examination revealed a Glasgow
Coma Score of 9/15. CSF analysis, complete blood counts, RFT, LFT, blood
sugar, serum electro-lytes, PT and APTT were normal. CT Brain revealed
multiple areas of hemorrhage with perilesional edema involving bilateral
parietal and left frontal region. The child was started on
anticonvulsants. Magnetic resonance venography of brain revealed superior
sagittal and bilateral transverse sinus thrombosis. Prothrombotic screen
(Protein S, Protein C levels, Factor V Leiden Mutation, Anti-thrombin III
levels and anti-cardiolipin antibodies) was planned, but could not be done
due to financial constraints.
The child was started on LMW heparin along with oral
anticoagulants. The child’s sensorium improved gradually over 5 days. On
day five of admission, he developed left leg pain with swelling. Doppler
study revealed extensive thrombosis of left external iliac, femoral and
popliteal veins. He improved with limb elevation and analgesics. He was
discharged on anticonvulsants and oral anticoagulants maintaining an INR
of 2-3. On follow up there are no neurologic sequelae or subsequent
episodes of thrombosis. Fresh frozen plasma was not used in child as the
response to the above treatment was satisfactory.
The incidence of serious complications after varicella
infection is 8.5/1 lakh population(1). Thrombotic complications are known
especially involving the cerebral vasculature(2). Eidelberg, et al.(3)
suggested that virus mediated endothelial injury promotes local
thrombosis but transient deficiency of protein S activity (due to
induction of anti-protein S auto antibodies) is also a causal factor(4,5).
These antibodies persist for only a few months. The frequency with which
antibodies to proteins S are induced in children during varicella
infection is unknown. Thrombosis is more common in individuals with Factor
V Leiden, which is a factor V variant resulting from a single point
mutation. It increases the risk for thrombosis as it confers resistance to
activated protein C [5]. Prognosis in post varicella thrombosis is good. A
prothrombotic screen after recovery, to diagnose hereditary
prothrombotic states that need life long anticoagulants, is advisable.
References
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Schmitt. Severe complications of varicella in previously healthy children
in Germany: a 1-year survey. Pediatrics 2001; 108: e79.
2. Bodensteiner JB, Hille MR, Riggs JE. Clinical
features of vascular thrombosis following varicella. Am J Dis Child 1992;
146: 100-102.
3. Eidelberg D, Sotrel A, Horoupian DS, Neumann PE,
Pumarola-Sune T, Price RW. Thrombotic cerebral vasculopathy associated
with herpes zoster. Ann Neurol 1986 ;19 :7-14 .
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stroke after chickenpox. Arch Dis Child 1997; 76: 522-525.
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fulminans associated with heterozygosity for factor V leiden and transient
protein S deficiency. Pediatrics 1998; 102: 1208- 1210.