A 12-year-old girl presenting with hematemesis , melena and gradual abdominal distension

Dr. Md. Benzamin (MD Resident): A 12-year-old girl, 2nd issue of non-consanguineous parents, immunized as per EPI schedule, presented with the history of hematemesis and melena 3 months back and abdominal distension for 1 year. After the episodes of hematemesis and melena, she received 1 unit of blood transfusion. She had no history of fever, jaundice, abdominal trauma, umbilical catheterization, umbilical sepsis, severe dehydration, offending drug intake and abdominal trauma or any surgery.


Provisional Diagnosis
Portal hypertension (extra-hepatic cause)

Differential Diagnosis
Dr. Mukesh Khadga (Resident): As the patient presented with a history of gastrointestinal bleeding (hematemesis, melena) with gradual abdominal distension and on examination she had anemia, moderate underweight and stunting, huge splenomegaly, ultrasonography of the whole abdomen showed coarse hepatic echotexture and huge splenomegaly. So, we differentially thought of chronic liver disease (compensated) with portal hypertension.

Chronic liver disease (compensated) with portal hypertension
Dr. Mukesh: The term chronic liver disease implies a longstanding irreversible change in the hepatic structure that may end in complications like cirrhosis, portal hypertension leading to premature death. 1 The main etiologies of chronic liver disease are chronic viral hepatitis (Hepatitis-B), Wilson's Disease and autoimmune liver disease. Patients of chronic liver disease with PHTN commonly present with edema, ascites, jaundice, pallor, variceal bleeding, splenomegaly, stigmata of chronic liver disease. 2 Compensated cirrhosis refers to the condition where hepatic synthetic functions are preserved despite the cirrhosis, in contrast to decompensated cirrhosis, where patients suffer from progressive complications of liver disease (fatigue, ascites, variceal bleeding, hepatic encephalopathy) with associated hepatic dysfunction. 3, 4 Dr. Benzamin: Serum alanine aminotransferase (20 U/L), prothrombin time and albumin levels (56 g/L) were normal. The HBsAg was negative. The serum ceruloplasmin level was normal. The eye evaluation for the KF ring and sunflower cataract were negative. The total IgG level was normal and ANA was negative. So, chronic liver disease was excluded.
Dr. Mukesh: Portal hypertension is a disease which occurs due to the formation of portalsystemic collaterals, that shunts a portion of the portal blood flow to the systemic circulation, bypassing the liver. Nasreen Sultana (Professor): Extrahepatic portal vein obstruction is a primary vascular condition characterized by chronic long-standing blockage and the cavernous transformation of the portal vein with or without additional involvement of intrahepatic branches, splenic or superior mesenteric vein. 9 It is the predominant cause of pediatric portal hypertension in developing countries. It is a prehepatic type of portal hypertension in which liver functions and morphology are preserved to date.
Doppler ultrasound is an accurate non-invasive method for evaluation of portal hemodynamics that provides valuable information on morphological, qualitative and quantitative changes. 10 In the normal portal vein, spectral doppler ultrasound shows antegrade or hepatopetal flow and a waveform above the baseline. Abnormally slow portal venous flow is diagnostic when peak velocity is less than 16 cm/sec. In some cases, the flow is still hepatopetal but spectral analysis may demonstrate loss of respiratory phasicity and more pronounced cardiac periodicity which can progress to an absence of end-diastolic flow, arterialized flow or bidirectional to-and-fro flow. Slow or reverse (hepatofugal) flow are two of the most specific findings. Absent flow in the portal vein may be due to stagnant flow in portal hypertension. Recanalization occurs in some cases of thrombosis but more frequently if portal vein thrombosis persists, portal flow is reestablished via cavernous transformation (portal vein undergoes fibrosis and a tangle of tortuous collateral veins are seen along the usual course of the portal vein). The dilated superior mesenteric vein and splenic vein may be seen. In The use of the congestive index has been recommended in helping to diagnose. It is the ratio of the portal vein cross-sectional area divided by the mean portal flow velocity. In the normal subject, this ratio is less than 0.7. 12

Dr. Benzamin's Diagnosis
Portal hypertension due to extrahepatic portal vein obstruction The child was treated with endoscopic variceal ligation, blood transfusion followed by Tab. propranolol 1 mg/kg/day in three divided doses.

Dr. Alam (Assistant Professor):
Portal hypertension is defined as portal vein pressure >5 mmHg or a portal vein to hepatic vein gradient of >10 mmHg. 13 Physical examination is helpful in diagnosis. Splenomegaly is common and sometimes massively enlarged. In extrahepatic portal hypertension no hepatomegaly. In cirrhosis patient, the liver is usually small and shrunken, but children with biliary atresia, the liver may be moderately enlarged. In Budd-Chiari syndrome or congenital hepatic fibrosis, the liver may be massively enlarged. Ascites is generally only present when portal hypertension is at the sinusoidal level. 14

Dr. Sharmin Akter (Resident): What is primary prophylaxis and what is secondary prophylaxis?
Dr. Benzamin: Prophylactic therapy given before the 1 st episode of hematemesis and melena is known as primary prophylaxis and after the episode of hematemesis and melena is known as secondary prophylaxis. 6

Dr. Ferdous Ara Begum (Resident): How propranolol acts?
Dr. Rukunuzzaman: Propranolol is a non-selective β blocker. It primarily blocks β2-adrenoceptors of the splanchnic bed, leaving unopposed α-adrenoceptor stimulation and thus decreased splanchnic and portal perfusion. It also decreases heart rate by β1adrenoreceptor blockade, thus lowering cardiac output and portal perfusion. It also decreases collateral circulation (e.g. azygous vein blood flow

Final Diagnosis
Portal hypertension due to extrahepatic portal vein obstruction