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 is no controversy about the management of acute variceal bleeding.
Consensus on extra-hepatic portal vein obstruction.
With darin 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. This indicates while in some patients compression plays an important role, in others ischemia alone or in combination with compression causes biliary changes.
IPH and EHPVO are both characterized by normal hepatic venous pressure gradient, moderate to massive splenomegaly with preserved liver synthetic functions.
With the availability of effective endotherapy, the mortality due to variceal bleeding has become a rarity but the morbidity due to late sequelae like growth failure, portal bilipathy, gastric varices, rectal varices etc. However, controversy exists regarding prevention of further bleed. However, in EHPVO unlike in cirrhosis where cyanoacrylate injection, beta-blockers or TIPS are used in econdary prophylaxis preventing further bleed from gastric varices ,37 large fundal varices are considered as an indication for shunt surgery as it provides a onetime treatment for the disease.
Following surgery, serum aminotransferases and gamma glutamyl transpeptidase GGT activities returned to normal levels within 1 to 6 weeks in 5 children and remained normal on follow-up from 5 to 15 years. The effect of increased portal pressure in EHPVO is not localized to the esophagus and stomach; it affects the entire gastrointestinal tract.
Of the two modalities of endotherapy, EST is an established modality to tackle varices in children Table 1. Variceal bleeding in EHPVO can be successfully managed by endoscopic obliteration of varices, which has low morbidity but requires repeated visits, or by portosystemic shunt surgery, which provides good control of bleeding, possibly helps growth retardation, hypersplenism, and protects against future development of portal biliopathy but is associated with surgical mortality and is sometimes not feasible due to nonavailability of a satisfactory vessel.
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 Extrahepatic portal vein obstruction EHPVO is an important cause of noncirrhotic portal hypertension, especially in Third World countries.
A longer interval up to 1 year and documentation of a patent shunt with decompressed collaterals on color Doppler or MR angiography may help in determining the optimum time for surgery. Hence the management of EHPVO is no longer restricted to the management of variceal bleeding alone; it encompasses the management of growth failure, portal biliopathy, colorectal varices, massive splenomegaly with its associated complications like infarction, hypersplenism, physical disability due to pain, early satiety etc.
Side-to-side lienorenal shunt without splenectomy in noncirrhotic portal hypertension in children. Management of colorectal varices. In another study in 20 children with EHPVO, Dubuisson et al  have shown that almost half of the patients had protein- C, protein-S and anti-thrombin III deficiencies but their levels were normal in all parents, suggesting that these deficiencies are not genetic in origin.
A significant proportion of cases in adults are due to procoagulant state but the same has not been documented in children. Natural history is defined by episodes of variceal bleed and symptoms related to enlarged spleen.
Endoscopic outcome sxrin esophageal variceal eradication in children with extrahepatic portal venous obstruction. Relation of insulin-like growth factor-1 and insulin-like growth factor binding protein-3 levels to growth retardation in extrahepatic portal vein obstruction.
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 sarkn, thereby reducing the risk of recurrence.
Non-cirrhotic portal hypertension – diagnosis and management.
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. Prevalence, classification and natural history of gastric varices: 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.
The low prevalence in pediatric studies clearly suggests that the duration of portal hypertension plays an important role in the development of rectal varices. Improvement of growth after restoration of hepatic blood flow with mesenteric-left-portal bypass or Rex shunt, has been documented.
Surgical shunts revert the complications secondary to PHT. Band ligation plus sclerotherapy versus sclerotherapy alone in children with extrahepatic portal vein obstruction.
Consensus on extra-hepatic portal vein obstruction.
Eur J Pediatr Surg. Management of portal biliopathy. Etiological spectrum of esphageal varices due to portal hypertension in Indian children: Management of gastric varices and portal hypertensive gastropathy Gastric varices: Symptomatic biliary obstruction can be managed endoscopically but shunt surgery followed by biliary sarinn if necessary seems to be the best management option.
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 asrin to thrombosis at the junction of splenic and superior mesenteric veins.