A 4 years old girl with splenomgaly, anemia and thrombocytopenia

Dr Ira Shah

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A 4 year old with splenomegaly, anemia and thrombocytopenia.

Dr Ira Shah.

Medical Sciences Department, Pediatric Oncall, Mumbai.

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Medical Sciences Department, Pediatric Oncall, Mumbai

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Clinical Problem
Case :- A 4 year old girl born of second degree consanguineous marriage presented with lump in left side of abdomen since 2 years with pallor and intermittent ecchymotic patches over the body. There was history of jaundice with high coloured urine at 2 years of age. She now presented with hematemesis and malena since 3 days. On examination, she had splenomegaly with pallor. Investigation showed:
 Hemoglobin = 3.4 gm/dl
 WBC count = 6,500/cumm [45% polymorphs, 53% lymphocytes, 2% eosinophils]
 Platelet count = 19,000
 Reticulocyte count = 2.7%
 Hemoglobin electrophoresis = Normal
 S. Bilirubin = 1.1 gm%
 SGOT = 616 IU/L, SGPT = 265 IU/L
 USG Abdomen with colour doppler :- Splenohematomegaly with peripheral infarcts in spleen with normal portal vein and no collaterals.
 Prothrombin Time, Partial Thromboplastin Time = Prolonged

She was treated with plasma, platelet blood transfusion and packed cell transfusion with vasopressin drip. A repeat colour doppler by the same sonologist after 5 days showed presence of portal cavernoma.

Why did the earlier colour doppler not pick up portal cavernoma?

Discussion
Expert’s opinion: Dr Ira Shah
This child has portal hypertension which has lead to hypersplenism and variceal bleed. Usually the colour doppler will pick up the portal collaterals as well as depict the flow of blood in these vessels. However the doppler results are very much operator dependent. In this child the colour doppler on both instances has been done by the same sonologist. Thus it is unlikely that the portal cavernoma was not picked up due to operator variability. This child had an acute episode of hematemesis. This relieves the pressure in the portal circulation leading to collapse of the portal collaterals and non visualization of the vessels. After few days due to refilling of the portal circulation, the vessels are picked up on colour doppler and thus portal cavernoma may be found. Thus in a child with portal hypertension always look for portal vessels after a few days after an acute bleed or the results may be falsely normal.

Compliance with ethical standards

Funding: None

Conflict of Interest: None

Cite this article as:
Shah I. A 4 years old girl with splenomgaly, anemia and thrombocytopenia. Pediatr Oncall J. 2007;4.