Growth impairment in children with extrahepatic portal vein obstruction is improved by mesenterico-left portal vein bypass

Lautz, Timothy B.; Sundaram, Shikha S.; Whitington, Peter F.; Keys, Lisa; Superina, Riccardo

doi:10.1016/j.jpedsurg.2009.05.016

Cited by 51 publications

(33 citation statements)

References 21 publications

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“…46 as it preserves portal blood flow to the liver and may improve growth in them. [77][78][79][80] The third phase involves management of cases who continue to have biliary obstruction and remain symptomatic despite shunt procedure either due to blockage of shunt or due to persistent obstruction despite patent shunt. Second-stage biliary drainage surgery like hepaticojejunostomy or choledochoduodenostomy surgery on the bile ducts is performed in most of these cases with good resolution of symptoms as well as liver function tests.…”

Section: Managementmentioning

confidence: 99%

Portal Cavernoma Cholangiopathy: Consensus Statement of a Working Party of the Indian National Association for Study of the Liver

Dhiman

Saraswat

Valla

et al. 2014

Journal of Clinical and Experimental Hepatology

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Portal cavernoma cholangiopathy (PCC) is defined as abnormalities in the extrahepatic biliary system including the cystic duct and gallbladder with or without abnormalities in the 1st and 2nd generation biliary ducts in a patient with portal cavernoma. Presence of a portal cavernoma, typical cholangiographic changes on endoscopic or magnetic resonance cholangiography and the absence of other causes of these biliary changes like bile duct injury, primary sclerosing cholangitis, cholangiocarcinoma etc are mandatory to arrive a diagnosis. Compression by porto-portal collateral veins involving the paracholedochal and epicholedochal venous plexuses and cholecystic veins and ischemic insult due to deficient portal blood supply or prolonged compression by collaterals bring about biliary changes. While the former are reversible after porto-systemic shunt surgery, the latter are not. Majority of the patients with PCC are asymptomatic and approximately 21% are symptomatic. Symptoms in PCC could be in the form of long standing jaundice due to chronic cholestasis, or biliary pain with or without cholangitis due to biliary stones. Endoscopic retrograde cholangiography has no diagnostic role because it is invasive and is associated with risk of complications, hence it is reserved for therapeutic procedures. Magnetic resonance cholangiography and portovenography is a noninvasive and comprehensive imaging technique, and is the modality of choice for mapping of the biliary and vascular abnormalities in these patients. PCC is a progressive condition and symptoms develop late in the course of portal hypertension only in patients with severe or advanced changes of cholangiopathy. Asymptomatic patients with PCC do not require any treatment. Treatment of symptomatic PCC can be approached in a phased manner, coping first with biliary clearance by nasobiliary or biliary stent placement for acute cholangitis and endoscopic biliary sphincterotomy for biliary stone removal; second, with portal decompression by creating portosystemic shunt; and third, with persistent biliary obstruction by performing second-stage biliary drainage surgery such as hepaticojejunostomy or choledochoduodenostomy. Patients with symptomatic PCC have good prognosis after successful endoscopic biliary drainage and after successful shunt surgery. ( J CLIN EXP HEPATOL 2014;4:S2-S14)

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Section: Managementmentioning

confidence: 99%

Portal Cavernoma Cholangiopathy: Consensus Statement of a Working Party of the Indian National Association for Study of the Liver

Dhiman

Saraswat

Valla

et al. 2014

Journal of Clinical and Experimental Hepatology

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“…It is proposed that reduced portal blood flow results in decreased insulin reaching the liver and thereby decreased production of IGF-I and IGFBP-3. Shunt surgery, mesenterico-left portal vein bypass (MLPVB) with improvement in growth parameters in children with EHPVO supports this hypothesis (Lautz, 2009;Stringer, 2007). -Frequency of bleeding has been linked to the growth in these children.…”

Section: Specific Issues In Children 61 Growth Retardationmentioning

confidence: 83%

“…Although these shunts are effective in controlling variceal bleeding, encephalopathy is a concern in patients with poor liver functions. Follow up studies have shown shunt patency in 85-98% of children with long term survival in >95% cases (Lautz, 2009;Bismuth, 1980;Gauthier, 1989;Mitra, 1993;Prasad, 1994;Orloff, 1994;Superina, 2006;Shariff, 2010). Risk of variceal recurrence and rebleed is there in case of shunt blockage.…”

Section: Surgical Management Of Ehpvomentioning

confidence: 99%

“…The mesoportal bypass (MPB) is a recent advancement in shunt restoring mesenteric blood flow to the liver through the Rex venous recessus (interposition of a jugular venous allograft between the superior mesenteric vein and the intrahepatic left portal vein). This "Rex shunt" restores the physiological hepatopetal flow and thereby corrects the liver dysfunction, coagulation parameters and also improves the growth potential (Superina, 2006;Mack, 2003;Lautz, 2009). It appears that doing the Rex shunt early after diagnosis rather than waiting till complications will be beneficial.…”

Section: Surgical Management Of Ehpvomentioning

confidence: 99%

See 1 more Smart Citation

Extra Hepatic Portal Venous Obstruction in Children

Arora¹,

Das²

2012

Portal Hypertension - Causes and Complications

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“…Do sada objavljeni, ne tako brojni literaturni podaci ukazuju da restauracija portnog protoka krvi kod dece kod koje je uradjen portosistemski šant, u postoperativnom periodu dovodi do ubrzanog rasta i razvoja, naročito tokom prve godine nakon operacije. 132,133 Takođe, kao posledica EHPH mogu se razviti i izvesni neuro-kognitivni poremećaji indikativni za portosistemsku encefalopatiju uzrokovanu prirodno nastalim porto-sistemskim šantovima. Simtomi i znaci encefalopatije mogu se povući nakon ponovnog uspostavljanja adekvatnog portnog protoka.…”

Section: Zaključakunclassified

Assessing the effectiveness of surgical treatment in children with extrahepatic portal hypertension

Sretenović¹

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Growth impairment in children with extrahepatic portal vein obstruction is improved by mesenterico-left portal vein bypass

Cited by 51 publications

References 21 publications

Portal Cavernoma Cholangiopathy: Consensus Statement of a Working Party of the Indian National Association for Study of the Liver

Portal Cavernoma Cholangiopathy: Consensus Statement of a Working Party of the Indian National Association for Study of the Liver

Extra Hepatic Portal Venous Obstruction in Children

Assessing the effectiveness of surgical treatment in children with extrahepatic portal hypertension

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