Results of a Stepwise Approach to Extrahepatic Portal Vein Obstruction in Children

Alberti, Daniele; Colusso, M.; Cheli, Maurizio; Ravelli, P.; Indriolo, A.; Signorelli, Sergio; Fagiuoli, Stefano; D’Antiga, Lorenzo

doi:10.1097/mpg.0b013e31829fad46

Cited by 31 publications

(30 citation statements)

References 40 publications

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“…Because 2 of our patients with permanent shunt occlusion were converted to portosystemic shunts, our overall success rate was 91% (20/22) with a follow-up period of 12–48 months (mean=25.6 months). This result matched those of some series [ 9 , 10 ], but showed a slightly higher rate than other series [ 15 , 27 , 28 ]. The use of a non-absorbable suture with a relatively narrow vein might have resulted in the stricture of anastomosis observed in 2 patients in our series over 2 years.…”

Section: Discussionsupporting

confidence: 88%

“…We believe that small LPVs result in poor visualization of blood flow and that direct portography through the umbilical vein is the criterion standard for assessing the size and patency of the Rex recessus and LPV. Furthermore, the thrombosed Rex recessus and the LPV of 3 patients with a suspected history of neonatal umbilical catheterization were unsuitable for use after direct inspection [ 27 ] and thus underwent a portosystemic shunting procedure.…”

Section: Discussionmentioning

confidence: 99%

“…Previous studies have described the long-term follow-up results of mesoportal bypass operations [ 9 , 10 , 15 , 27 , 28 ]. In most studies, MRB was performed using IJV grafts.…”

Section: Discussionmentioning

confidence: 99%

“…Sharif et al showed an overall mesoportal patency rate of 92% in a series of 30 children after a median follow-up period of 8 years [ 10 ], and Chin et al showed an overall mesoportal patency rate of 84% in a series of 62 children [ 28 ]. Alberti et al found that 53 of 57 (93%) patients were alive at the final follow-up assessment with controlled bleeding, and 27 of 33 (82%) had a patent conduit [ 27 ]. Recently, Luoto et al showed an overall mesoportal patency rate of 81% in a series of 21 children who received saphenous vein grafting [ 15 ].…”

Section: Discussionmentioning

confidence: 99%

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Partial Splenectomy and Use of Splenic Vein as an Autograft for Meso-Rex Bypass: A Clinical Observational Study

Wei

2014

Med Sci Monit

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BackgroundMeso-Rex bypass (MRB) surgery is being increasingly used to treat chronic prehepatic portal hypertension secondary to extrahepatic portal vein thrombosis (EPVT) and cavernous transformation (EPVCT) in children. Rather than using the internal jugular vein (IJV, the traditional venous graft), we used an autogenous splenic vein segment graft for MRB.Material/MethodsWe examined 25 children with extrahepatic portal hypertension and a history of recurrent upper gastrointestinal (GI) variceal bleeding despite previous endoscopic sclerotherapy. All patients had melena, splenomegaly, hypersplenism, or some combination thereof. Left portal vein (LPV) patency was verified in 22 patients using intraoperative direct portography through the umbilical vein. Partial splenectomy was performed to enable the harvest of the splenic vein trunk, which was anastomosed between the superior mesenteric vein (SMV) and the left portal vein (LPV). All patients were followed for 12–48 months (mean=25.6 months) and no patients were lost to follow-up.ResultsPreoperative Doppler ultrasound (US) imaging indicated that 18/25 patients had adequate intrahepatic portal veins for shunting, with no blood flow in the LPVs of 7 patients. LPV patency in 22/25 patients was verified using intraoperative direct portography, with successful MRB. Shunting was converted into a portosystemic shunt in the remaining 3/25 patients with thrombosed LPVs. A Doppler US evaluation of the vein conduit revealed excellent postoperative flow. The patients’ mean hemoglobin, platelet, and white blood cell counts increased significantly, and in all cases the endoscopic status obviously improved after shunting. Occlusion or narrowing occurred in 2/22 patients after discharge. At 12 months (for 1 patient) and 24 months (for 1 patient), the shunt was converted into a portosystemic shunt. The cumulative graft patency rate was 91% (20/22).ConclusionsPartial splenectomy and splenic vein autografting in MRB surgery can successfully resolve prehepatic portal hypertension and hypersplenism in children.

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Section: Discussionsupporting

confidence: 88%

Section: Discussionmentioning

confidence: 99%

“…Previous studies have described the long-term follow-up results of mesoportal bypass operations [ 9 , 10 , 15 , 27 , 28 ]. In most studies, MRB was performed using IJV grafts.…”

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Partial Splenectomy and Use of Splenic Vein as an Autograft for Meso-Rex Bypass: A Clinical Observational Study

Wei

2014

Med Sci Monit

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“…Patients of EHPVO presenting with features of PHT in the form of variceal bleed either massive or recurrent inspite of pharmacological and/or endoscopic therapy require measures for reduction of portal pressure either in the form of surgical PSS or transjugular intra-hepatic portosystemic shunt (TIPS) [30,31] . Although technically challenging, TIPS is feasible in these patients but it can preclude a future Rex Shunt [31] .…”

Section: Transjugular Intrahepatic Pssmentioning

confidence: 99%

Imaging and radiological interventions in extra-hepatic portal vein obstruction

Pargewar¹,

Desai²,

Rajesh³

et al. 2016

WJR

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Extrahepatic portal vein obstruction (EHPVO) is a primary vascular condition characterized by chronic long standing blockage and cavernous transformation of portal vein with or without additional involvement of intrahepatic branches, splenic or superior mesenteric vein. Patients generally present in childhood with multiple episodes of variceal bleed and EHPVO is the predominant cause of paediatric portal hypertension (PHT) in developing countries. It is a pre-hepatic type of PHT in which liver functions and morphology are preserved till late. Characteristic imaging findings include multiple parabiliary venous collaterals which form to bypass the obstructed portal vein with resultant changes in biliary tree termed portal biliopathy or portal cavernoma cholangiopathy. Ultrasound with Doppler, computed tomography, magnetic resonance cholangiography and magnetic resonance portovenography are non-invasive techniques which can provide a comprehensive analysis of degree and extent of EHPVO, collaterals and bile duct abnormalities. These can also be used to assess in surgical planning as well screening for shunt patency in post-operative patients. The multitude of changes and complications seen in EHPVO can be addressed by various radiological interventional procedures. The myriad of symptoms arising secondary to vascular, biliary, visceral and neurocognitive changes in EHPVO can be managed by various radiological interventions like transjugular intra-hepatic portosystemic shunt, percutaneous transhepatic biliary drainage, partial splenic embolization, balloon occluded retrograde obliteration of portosystemic shunt (PSS) and revision of PSS. Core tip: The present review describes the etiopathogenesis and role of imaging modalities in Extrahepatic portal vein obstruction and role of various radiological interventional procedures in its management. Pargewar SS et al . Imaging and interventions in EHPVOThe advantages and potential limitations of each imaging modality namely endoscopic retrograde cholangiography, ultrasonography and Doppler, multidetector computed tomography, magnetic resonance cholangiography and portovenography, endoscopic ultrasound and ultrasound transient elastography are discussed. Also, the concepts behind the use of interventional procedures like transjugular intra-hepatic portosystemic shunt, percutaneous transhepatic biliary drainage, partial splenic embolization, splenic artery aneurysmal embolization, balloon occluded retrograde obliteration of portosystemic shunt, etc. , are described.

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Wedged hepatic vein portovenography for assessment of Rex vein patency in children with extrahepatic portal venous obstruction

Kaur,

Khanna,

Sood

et al. 2024

J. pediatr. gastroenterol. nutr.

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BackgroundMeso‐Rex bypass is the surgical intervention of choice for children with extrahepatic portal vein obstruction (EHPVO). Patency of Rex vein, umbilical recessus of the portal vein, is a prerequisite for this surgery. Conventional diagnostic modalities poorly detect patency, while transjugular wedged hepatic vein portography (WHVP) accurately detects patency in 90%.ObjectivesWe aimed to assess Rex vein patency and portal vein branching pattern in children with EHPVO using transjugular WHVP and to identify factors associated with Rex vein patency.MethodsTransjugular WHVP was performed in 31 children with EHPVO by selective cannulation of left and right hepatic veins. Rex vein patency, type of intrahepatic portal venous anatomy (Types A–E), and factors associated with patency of Rex vein were studied.ResultsThe patency of Rex recess on transjugular WHVP was 29%. Complete obliteration of intrahepatic portal venous radicles was the commonest pattern (Type E, 38.7%) while Type A, the favorable anatomy for meso‐Rex bypass, was seen in only 12.9%. Patency of the Rex vein, but not the anatomical pattern, was associated with younger age at evaluation (patent Rex: 6.6 ± 4.9 years vs. nonpatent Rex: 12.7 ± 3.9 years, p = 0.001). Under‐5‐year children had a 12 times greater chance of having a patent Rex vein (odds ratio: 12.22, 95% confidence interval: 1.65–90.40, p = 0.004). Patency or pattern was unrelated to local factors like umbilical vein catheterization, systemic thrombophilia, or disease severity.ConclusionLess than one‐third of our pediatric EHPVO patients have a patent Rex vein. Younger age at evaluation is significantly associated with Rex vein patency.

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Results of a Stepwise Approach to Extrahepatic Portal Vein Obstruction in Children

Abstract: Children with EHPVO have a high rate of bleeding episodes early in life. A stepwise approach comprising of medical, endoscopic, and surgical options provided excellent survival and bleeding control in this population.

Cited by 31 publications

References 40 publications

Partial Splenectomy and Use of Splenic Vein as an Autograft for Meso-Rex Bypass: A Clinical Observational Study

Partial Splenectomy and Use of Splenic Vein as an Autograft for Meso-Rex Bypass: A Clinical Observational Study

Imaging and radiological interventions in extra-hepatic portal vein obstruction

Wedged hepatic vein portovenography for assessment of Rex vein patency in children with extrahepatic portal venous obstruction

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