A 12 year old boy presents with hemetemesis, malena and mild splenomegaly. There is no obvious jaundice or ascitis. The most likely diagnosis is:
Answer is A (EHPVO): Presence of hemetemesis, malena and splenomegaly suggests a diagnosis of poal hypeension. The most common cause of poal hypeension in a male child (12 year old boy) is Extra Hepatic Poal Vein Obstruction (EHPVO). EHPVO is therefore the single best answer by exclusion Non Cirrhotic Poal Fibrosis presents in a similar fashion clinically, however it is more common in females and in an adult population (third or fouh decade). NCPF is usually associated with a moderate/large (massive) splenomegaly Cirrhosis is an uncommon cause of poal hypeension in children and the absence of jaundice and ascitis makes this diagnosis more unlikely. 'An infant or child presenting with hemetemesis and mild/moderate splenomegaly in the absence of features of chronic - liver disease is likely to be suffering from EHPVO. In adults, one needs to exclude diseases such as NCPF and compensated cirrhosis - 'Poal Hypeension': by Robeo De Franchis (Wiley - Blackwell) 2006 /314 Ate of presentation ors a diagnosis of EHPVO over NCPF Age at presentation is an impoant factor in distinguishing EHPVO from NCPF - Indian Journal of Gastroenterology; 1984: Vol 3; No 4; 201-202 'More than three quaers of Indian Children with poal Inpeension were found to have extra hepatic poal vein obstruction (EHPVO) and Indian studies have shown extra hepatic poal vein obstruction (EHPVO) to be the commonest non cirrhotic cause for poal hypeension' - 'Diseases of the Liver and Biliary System in Children' by Kelly 3rd /559 'In a prospective study 'Yaccha etal provided suppo for the hypothesis that the etiology of zipper gastro intestinal bleeding in children may differ between developed and developing countries. They showed that 95% of children had upper gastrointestinal variceal bleeding, 92% of whom had bleeding due to EHPVO, a .figure significantly higher than that seen in the west' - Disease of the Liver & Bihar/ system in Children by Kelly 3rd /560 *Occasional impairment may be observed NCPF, non-cirrhotic poal fibrosis: EHPVO, extrahepatic poal vein obstruction: US, ultrasound
EHPVO
NCPF
Cirrhosis
Malaria with DIC