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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Extrahepatic portal vein obstruction.
The low prevalence in pediatric studies clearly suggests that the duration of portal hypertension plays an important role in the development of rectal 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.
The effect of increased portal pressure in EHPVO is not localized to the esophagus and stomach; it affects the entire gastrointestinal tract. Patients with choledocholithiasis and stricture will require multiple sessions of endoscopic therapy with balloon dilatation and stent placement.
Nevertheless, EST cannot be totally ruled out as a therapeutic modality, especially in children. This suggests that children with EHPVO have growth failure and decreased growth velocity despite adequate nutrition. After hemodynamic resuscitation all such patients should undergo endotherapy [either sclerotherapy EST or band ligation EVL ].
Idiopathic portal hypertension and extrahepatic portal venous obstruction.
Growth failure, portal biliopathy and minimal hepatic encephalopathy are additional concerns in EHPVO. The exact mechanism of the growth failure is not known. Irreversible changes in the portal vein may decrease the compliance of the portal vein for adaptation if the shunt is done late. Unfortunately, the experience of EVL in children is limited.
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. Bile duct obstruction due to portal biliopathy in extrahepatic portal hypertension: As of now, we do not have sufficient data to say what proportions of EHPVO cases are suitable for this new shunt. Etiological spectrum of esphageal varices due to portal hypertension in Indian children: In patients with endoscopic failure, a staged procedure portosystemic shunt followed by biliary surgery should be preferred.
The portal vein is transformed into a cavernoma, resulting in portal hypertension and oesophagogastic varices. The latter mechanism has been further substantiated by the fact that after conventional shunt surgery the levels of anticoagulant proteins tend to further go down. Incidence of haemorrhoids and anorectal varices in children with portal hypertension. However, controversy exists regarding prevention of further bleed. Portal hypertensive gastropathy in children with extrahepatic portal venous obstruction: 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.
Management of portal biliopathy.
Natural history is defined by episodes of variceal bleed and symptoms related to enlarged spleen. In a study of five cases, Dhiman et al [49 ] showed that repeat ERCP done after weeks of shunt surgery demonstrated total disappearance of cholangiographic changes in one, partial disappearance in two and no change in remaining two cases. 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.
Improvement of growth after restoration of hepatic blood flow with mesenteric-left-portal bypass or Rex shunt, has been documented. This observation again supports the compression theory in most cases of portal biliopathy.
Pseudosclerosing cholangitis in extrahepatic portal venous obstruction. Endoscopic outcome beyond esophageal variceal eradication in children with extrahepatic portal venous obstruction.
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.
Extrahepatic portal venous obstruction. Bleeding in extrahepatic portal vein obstruction. This indicates while in some patients compression plays an important role, in others ischemia alone or in combination with compression causes biliary changes. Eur J Pediatr Surg. Symptomatic biliary obstruction can be managed endoscopically but shunt surgery followed by biliary bypass if necessary seems to be the best management option.
Regarding endotherapy for variceal bleeding, there is no doubt that band ligation is superior to sclerotherapy. Relation of insulin-like growth factor-1 and insulin-like growth factor binding protein-3 levels to growth retardation in extrahepatic portal vein obstruction.
EHPVO is the commonest cause of portal hypertension and variceal bleeding in children. The etiology and clinical presentation are different in children and adults. The natural history of portal hypertensive gastropathy: Proximal splenorenal shunts for extrahepatic portal venous obstruction in children. EST remains the only therapeutic option in children younger than 2 years of age due to insertion difficulty with a comparatively larger banding cylinder.
The definitive method of diagnosis of portal biliopathy is ERCP. Management of gastric varices and portal hypertensive gastropathy Gastric varices: 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.
Factor V Leiden and prothrombin gene GA mutations are uncommon in portal vein thrombosis in India. Most of these bleeding episodes occurred within the first 4 years of variceal eradication.
In adults, overt or occult primary myeloproliferative disorders MPD are the commonest cause of portal vein thrombosis. Endoscopic band ligation followed by sclerotherapy. 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.
Growth impairment in children with extrahepatic portal vein obstruction is improved by mesenterico-left portal vein bypass. Liver function tests took 2 to 2.