This 11 yr old boy came to me for evaluation of bloody vomiting, one episode one week back. Physical examination revealed distended abdomen and enlarged liver and spleen Ultrasound exam revealed enlarged liver and spleen, with changes in the texture of liver as happens in cirrhosis.
On careful further examination, we found clots in veins draining liver and many bypass channels draining blood from the liver.
Thus the diagnosis of Budd Chiari malformation leading to cirrhosis like changes was made.
How & Why is such mindful reporting important : Management is completely different from regular cirrhoisis as the pathology is completely different, the cause of liver dysfunction is not in the liver cells per se, but much beyond that, because of venous occlusion and resultant congestion in liver bed. Diverting procedures such as TIPSS & Angioplasty are very beneficial in modifying course of disease process
Further evaluation so as to cause of clotting such as myeloproliferative disorders, sickle cell anaemia etc is advocated
To conclude: mindful timely reporting of cases of cirrhosis especially with hepatomegaly by going a step further and evaluating the venous drainage of liver by colour doppler examination will help in disease modulation and early management before significant morbidity has occurred.
occluded veins of liver just before opening into main draining vein of body: inferior venacava
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