APPSPGHAN 2022

UJJAL PODDAR

Professor and Head, Department of Pediatric Gastroenterology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh

India

Dr. Ujjal Poddar is presently the Professor and Head of the Department of Pediatric Gastroenterology at Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India. Dr. Poddar has graduated from North Bengal Medical College, West Bengal and received his postgraduate (MD) degree as well as doctorate in Gastroenterology (DM) from Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India.

He was actively involved in establishing the first academic super-specialty department of Pediatric Gastroenterology in India, in an awareness campaign about celiac disease in North India and neonatal cholestasis across India. He is the author of more than 180 publications, including original papers, reviews, and book chapters. His main research interest has been in the areas of celiac disease, portal hypertension, viral hepatitis B, neonatal cholestasis, and inflammatory bowel disease (IBD). He is the Section Editor (Gastroenterology) of Indian Pediatrics, the official journal of Indian Academy of Pediatrics and Associate Editor of Pediatric Gastroenterology, Hepatology, & Nutrition: Frontiers of Pediatrics.

Dr. Poddar received many National and International Awards, and also served as an active member of several International and National medical organizations, including European Society of Pediatric Gastroenterology, Hepatology, and Nutrition (ESPGHAN).He is the one of the two Pediatric Gastroenterologists from Asia featured in the World’s top 2% scientists in their respective field (Gastroenterology/ Hepatology).

Approach to Portal Hypertension in Children

Liver Symposium

13 October 2022 (1400-1530) @ Sipadan Hall 2

Definitions:
Portal hypertension (PH) is defined as an increase in portal venous pressure of >5mm of Hg. The consequences of PH are the development of ascites, variceal bleeding, and hepatic encephalopathy. The etiology of PH is different in children than in adults. Non-cirrhotic portal hypertension such as extrahepatic portal venous obstruction (EHPVO) is found to be the commonest cause of PH in children in Asia. The most accurate way of assessing PH is hepatic venous pressure gradient (HVPG). Clinically significant portal hypertension (CSPH), is the state of PH when there is an increased risk of developing gastroesophageal varices, ascites, decompensation, and death, and is defined as HVPG>10mm of Hg. The presence of spider nevi, splenomegaly, and dilated tortuous abdominal wall veins are clinical findings that suggest the presence of CSPH. Esophageal varices develop when HVPG is ≥10mm of Hg and variceal bleeding happens when HVPG is >12mm of Hg. Hence, the presence of esophageal varices on endoscopy signifies CSPH. Since HVPG measurement is invasive, non-invasive tests (NIT) such as liver stiffness measurement (LSM) by transient elastography (FibroScan) with or without platelet counts have become popular in assessing the presence or absence of CSPH. As per Baveno VII (2022) guidelines on PH, CSPH is in when the FibroScan value is >25 kPa, and CSPH is out when it is <15 kPa. However, when the value is between 20-25 kPa then we need to add platelet counts (<150,000) to decide whether CSPH is there or not.

Diagnostic approach and primary prophylaxis:
As per Baveno VI (2015) recommendations, endoscopic variceal screening was recommended in all cases of chronic liver disease at the time of diagnosis and the subsequent management plan of PH was based on UGIE findings. However, in the latest Baveno VII guidelines, endoscopic variceal screening is done with, if there is no suspicion of CSPH. In such cases, yearly fibroscan and platelet counts are recommended. Endoscopic variceal screening is recommended when there is CSPH (LSM >20 kPa and platelet <150,000). Primary prophylaxis is recommended if there are large varices (>5mm) on endoscopy, presence of red-color signs (RCS), and the patient is in the Child-Pugh C stage of cirrhosis. Non-selective beta blockers (NSBBs) are the first choice and band ligation (EVL) is recommended if there is a contraindication or the patient is intolerant to NSBBs. Primary prophylaxis with NSBBs is recommended even in cases with low-risk varices if the patient has ascites.

Treatment options (secondary prophylaxis):
Acute variceal bleeding is managed with the combination of endotherapy (EVL or EST) plus vasoactive drugs (injection octreotide for 2-5 days followed with NSBBs). Refractory variceal bleeding in children can be controlled with balloon tamponade (Sengstaken Blakemore tube) followed by a transjugular intrahepatic portosystemic shunt (TIPS) in cirrhosis and portosystemic shunt surgery in EHPVO. Shunt surgery especially Meso-Rex shunt should be considered as a primary modality of treatment in cases with EHPVO with favorable anatomy (patent SMV and left branch of the portal vein).

Conclusions:
The etiology of portal hypertension in children is different than in adults and EHPVO is the commonest cause in Asia. The investigational approach to portal hypertension has been shifted from endoscopy to NIT such as liver stiffness measurement (LSM) with platelet counts. Non-selective beta blockers have become the preferred modality of treatment for primary prophylaxis against first variceal bleeding. Acute variceal bleeding is managed with EVL or EST with vasoactive drugs. Shunt surgery remains a preferred one-time treatment option in children with EHPVO.

Faculty List