EHPVO SARIN PDF

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). Nonā€Cirrhotic Shiv Kumar Sarin MD, DM. Director Treatment of chronic EHPVO in children.

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Extrahepatic portal vein obstruction EHPVO is an important cause of noncirrhotic portal hypertension, especially in Third World ehlvo. Of the two modalities of endotherapy, EST is an established modality to tackle varices in children Table 1.

EHPVO is the commonest cause of portal hypertension and variceal bleeding in children. Longterm studies after endotherapy have shown almost no mortality.

Studies in children have shown that hereditary or acquired coagulation disorders do not play a role in the pathogenesis of EHPVO in children. Infections, autoimmunity, drugs, immunodeficiency and prothrombotic states are possible etiological agents in IPH. Though the role of anticoagulation therapy remains controversial in adults,12 there is no role of anticoagulation therapy in children with EHPVO.

Idiopathic portal hypertension and extrahepatic portal venous obstruction.

Other indications of shunt surgery include symptomatic hypersplenism, growth retardation, portal biliopathy, massive splenomegaly affecting the quality of life, rare blood group, and remote area of residence. Improved body mass index after mesenterico-portal bypass.

Pant Hospital, New Delhi, India. Besides variceal bleeding, which is the commonest presentation, patients may have symptomatic portal biliopathy, hypersplenism, and growth retardation. This has been substantiated in other studies in children. Whether these patients should have shunt surgery after control of acute bleeding by endotherapy EST or EVL or should undergo further endotherapy for variceal eradication and surveillance is a long-standing debate. 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.

Conventional shunts proximal or distal splenorenal are not possible in almost one-third of cases due to blockage of splenic vein SV or small SV. Fifteen-year follow up of endoscopic injection sclerotherapy in children with extrahepatic portal venous obstruction. PHG classically described in cirrhosis. In a follow up study from our institute we have shown that factor V Leiden and prothrombin gene mutations were uncommon in children with EHPVO.

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Non-cirrhotic portal hypertension – diagnosis and management.

Portal biliopathy is the term used to describe cholangiographic abnormalities of the extrahepatic and intrahepatic bile ducts in patients with EHPVO. Diminished portal blood flow results in decreased insulin delivery to the liver and thereby decreased production of insulin-like growth factor-1 IGF-I and insulin-like growth factor binding protein-3 IGFBP Unfortunately, the experience of EVL in children is limited.

Portal hypertensive gastropathy in children with extrahepatic portal venous obstruction: Frequency of gastropathy and gastric varices in children with extrahepatic portal venous obstruction treated with sclerotherapy.

It has also been observed that while all symptomatic cases are adults but cholangiographic changes are equally evident in both children and adults,25 thereby implying that bile duct disease portal biliopathy in EHPVO may be progressive in nature and would manifest clinically in adulthood. The etiology and clinical presentation are different in children and adults.

The effect of lienorenal shunts. Regarding endotherapy for variceal bleeding, there is no doubt that band ligation is superior to 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.

Sarin SK, Gupta R. In adults, overt or occult primary myeloproliferative disorders MPD are the commonest cause of portal vein thrombosis. Incidence of haemorrhoids and anorectal varices in children with portal hypertension.

Idiopathic portal hypertension and extrahepatic portal venous obstruction.

Portal hypertensive gastropathy PHG: Magnetic resonance MR imaging with intravenous gadolinium injection delineate the cavernoma and biliary changes simultaneously and may be extremely useful in children. Shunt surgery should be considered for asymptomatic portal biliopathy in presence of another indication like growth failure, symptomatic hypersplenism or ectopic varices. Endoscopic sclerotherapy in children.

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.

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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. It is expected that the incidence of rectal varices with and without rectal bleeding will rise in children as they approach adolescence and adulthood. The only pediatric study by Gauthier-Villars et al [53] in 8 children with symptomatic biliopathy in EHPVO showed regression of cholestasis in all cases after shunt surgery mesocaval shunt in 6 and Rex in 2.

A significant proportion of cases in adults are due to procoagulant state but the same has not been documented in children.

Gastrointestinal bleeding in children. Endoscopic outcome beyond esophageal variceal eradication in children with extrahepatic portal venous obstruction.

Extrahepatic portal venous obstruction EHPVO is the commonest cause of portal hypertension and variceal bleeding in children. Endoscopic sclerotherapy for esophageal varices in children with extrahepatic portal vein obstruction; a follow -up study.

It may present with occult bleed or as an unusual cause of overt gastrointestinal bleeding.

Thapa BR, Mehta S. H-type shunt with an autologous venous graft for treatment of portal hypertension in children. Eur J Pediatr Surg. Management of portal biliopathy Portal biliopathy is the term used to describe cholangiographic abnormalities of the extrahepatic and intrahepatic bile ducts in patients with EHPVO. Though mortality related to variceal bleeding is uncommon, morbidity due to massive splenomegaly with hypersplenism, growth failure, ectopic varices like rectal varices and portal biliopathy is significant.

Natural history is defined by episodes of variceal bleed and symptoms related to enlarged spleen. After a follow up of 4. As of now, we do not have sufficient data to say what proportions of EHPVO cases are suitable for this new shunt. Management ehovo gastric varices and portal hypertensive gastropathy Gastric varices: