Sarin SK(1), Agarwal SR. Extrahepatic portal vein obstruction (EHPVO) is an important cause of noncirrhotic portal hypertension, especially in Third World. Endoscopic Management. S. K. Sarin, Cyriac Abby Philips, Rajeev Khanna tal vein obstruction (EHPVO), noncirrhotic portal fibrosis. (NCPF; or idiopathic PHT. Extrahepatic Portal Vein Obstruction (EHPVO). Non‐Cirrhotic Shiv Kumar Sarin MD, DM. Director Treatment of chronic EHPVO in children.
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There are many postulated etiologies of EHPVO namely umbilical sepsis, umbilical vein catheterization, abdominal trauma, surgery, intra-abdominal sepsis, dehydration, congenital agenesis or atresia of portal vein. Portal diversion for portal hypertension in children. Some people believe that shunt surgery should be done first in all cases with symptomatic biliopathy and if it fails to resolve biliary obstruction then second stage biliary surgery is recommended.
However, since most of the patients are asymptomatic, this approach is recommended only if a therapeutic intervention is contemplated. In patients with endoscopic failure, a staged procedure portosystemic shunt followed by biliary surgery should be preferred. By using EVL as a primary treatment, one can achieve rapid eradication or down gradation of varices with fewer complications and by using low-dose EST following EVL, one can block perforators and paraesophageal collaterals, thereby reducing the risk of recurrence.
The natural history of portal hypertensive gastropathy: Endoscopic band ligation followed by sclerotherapy Despite the clear cut benefit of EVL when used alone, there is a higher risk of recurrence of varices as it is difficult to ligate smaller varices, and because perforators and paraesophageal collaterals remain patent after EVL. These findings suggest diminished anabolic action of growth hormone on muscle growth affecting the lean muscle mass, and its lipolytic effect resulting in decreased adiposity.
Patients with choledocholithiasis and stricture will require multiple sessions of endoscopic therapy with balloon dilatation and stent placement. Mesoportal bypass for extrahepatic portal vein obstruction in children: Biliary changes in extrahepatic portal venous obstruction: Further the prevalence of rectal varices is more in EHPVO than in cirrhosis, probably due to the duration of portal hypertension or selective redistribution of portal pressure along the inferior mesenteric vein consequent to thrombosis at the junction of splenic and superior mesenteric veins.
Portal biliopathy is universal in adults and common in children but symptomatic cases are mainly in adults; thereby suggesting a progressive nature of the condition.
Management of esophageal varices. In another study Chaudhary et al  documented relief of jaundice in five of seven patients within weeks of shunt surgery and the remaining two patients required second stage hepaticojejunostomy.
Gastrointestinal bleeding in children.
Management of gastric varices and portal hypertensive gastropathy Gastric varices: Portal obstruction in children. Cholestasis in children with portal vein obstruction. The postulated mechanisms of biliary changes in EHPVO are extrinsic compression by portal collaterals,[ 44] ischemic stricture of bile duct due to injury at the time of portal venous thrombosis[ 45 ] or a combination of both.
Non-cirrhotic portal hypertension – diagnosis and management.
Side-to-side lienorenal shunt without splenectomy in noncirrhotic portal hypertension in children. Endoscopic band ligation followed by sclerotherapy. Although the liver may appear normal, functional compromise develops in the long term. Long-term survival is reasonably good with endoscopic surveillance; however, parenchymal extinction leading to decompensation is seen in a minority srin patients in both the disorders.
Rectosigmoid varices and other mucosal changes in patients with portal hypertension. Consequently recurrence of varices has been shown to be higher with EVL. Most of these bleeding episodes occurred within the first 4 years of variceal eradication. Bleeding in extrahepatic portal vein obstruction.
Management of colorectal varices. However, with the availability of new mesenterico-portal mesenterico-left portal-bypass or Rex shunt, the problems of conventional shunts have been largely overcome.
Endoscopic sclerotherapy for esophageal varices in children with extrahepatic portal vein obstruction; a follow -up study. With the availability of the most physiological shunt mesenteric-left-portal bypass or Rex the management paradigm of EHPVO has changed from endotherapy to primary shunt surgery.
A prospective study of endoscopic esophageal variceal ligation using multiband ligator. There is a paradigm shift in EHPVO management towards shunt surgery after the introduction of mesenteric-left-portal vein bypass or Rex shunt as it provides complete cure of the condition.
Acute gastric variceal bleeding can be controlled with tissue glue N-acetyl-2butyl- yanoacrylate injection. Portal hypertensive gastropathy in children with extrahepatic portal venous obstruction: The effect of increased portal pressure in EHPVO is not localized to the esophagus and stomach; it affects the entire gastrointestinal tract.
Proximal splenorenal shunts for extrahepatic portal venous obstruction in children. The low prevalence in pediatric studies clearly suggests that the duration of portal hypertension plays an important role in the development of rectal varices. Nevertheless, EST cannot be totally ruled out as a therapeutic modality, especially in children. Liver function tests took 2 to 2.
Non-cirrhotic portal hypertension – diagnosis and management.
Cholangiopathy associated with portal hypertension: A significant proportion of cases in adults are due to procoagulant state but the same has not been documented in children. Thapa BR, Mehta S. Natural history is defined by episodes of variceal bleed and symptoms related to enlarged spleen.
Conventional shunts proximal or distal splenorenal are not possible in almost one-third sadin cases due to blockage of splenic vein SV or small SV.