meso-Rex Bypass Research Articles

Portomesenteric and portosystemic venous reconstructions in children using balloon-expandable endoprostheses.

Portomesenteric and portosystemic venous occlusive disease may lead to portomesenteric hypertension, variceal bleeding, ascites and hypersplenism. Data regarding endovascular reconstructive strategies in children, however, are limited. To report technical success, outcome and patency of portomesenteric and portosystemic venous reconstruction using VIABAHN VBX balloon-expandable endoprostheses in pediatric patients. Five pediatric patients (median age: 15years, range: 4-18years), including 3 (60%) boys and 2 (40%) girls, with portomesenteric or portosystemic venous occlusion or recurrent stenosis, underwent balloon-expandable stent graft reconstruction. Presenting symptoms included acute variceal bleeding, without (n = 2, 40%) or with (n = 1, 20%) splenomegaly, and transfusion-dependent chronic melena (n = 1, 20%). One patient was asymptomatic (n = 1, 20%). Preprocedural imaging included Doppler ultrasound and contrast-enhanced computed tomography (CT) in all patients. Initial imaging showed 4 (80%) occlusions and 1 (20%) recurrent stenosis greater than 50%. Technical aspects of the reconstructions, technical successes, clinical outcomes and adverse events were recorded. Technical success was defined as completion of stent graft reconstruction. Adverse events were categorized according to Society of Interventional Radiology criteria. Clinical success was defined as resolution of the presenting symptoms and/or prevention of portal hypertensive sequela. Venous reconstruction was technically successful in all five patients. Stent graft locations included the main portal vein in 2 (40%), the superior mesenteric vein in 1 (20%), autologous Meso-Rex shunt in 1 (20%) and splenocaval shunt in 1 (20%). Six stent grafts were placed (two stent grafts placed in a single patient). Stent grafts had a median diameter of 7mm (range: 6-10mm) and a median length of 59mm (range: 19-79mm). Median fluoroscopy time was 36.6min (range: 13.4-95.8min) and median air kerma was 301.0mGy (range: 218.0-1,148.2mGy). No adverse events occurred. Median clinical follow-up was 18months (range: 6-29months). Median imaging follow-up was 17months (range: 2-29months). Clinical success was achieved in all patients and maintained during the follow-up period. One patient required follow-up intervention with superior mesenteric vein side extension with a self-expanding bare metal stent due to perigraft stenosis detected on CT 3months after stent placement. There were no stent graft occlusions. Portomesenteric and portosystemic venous reconstruction using balloon-expandable stent grafts in pediatric patients was feasible and clinically successful in this preliminary experience. Additional studies are warranted.

Percutaneous transhepatic interventional therapy of portal vein stenosis in paediatric liver transplantation: A systematic review of efficacy and safety.

To determine the efficacy and safety of percutaneous trans-hepatic balloon and/or stent angioplasty (PTA) in the management of portal vein (PV) stenosis following paediatric liver transplantation. Articles were included from a systematic search of Medline, Embase, Cochrane CENTRAL, ClinicalTrials.gov and International Clinical Trials Registry from inception to the 29th of August 2020. There were 213 paediatric liver recipients who underwent PTA for PV stenosis in 19 included studies published between 1991 and 2019. Balloon angioplasty was the initial treatment in the majority (n=153). Primary stent placement (n=34) was performed for elastic recoil, intimal tears and PV kinks and rescue stent placement (n=14) for recurrent PV stenosis following primary balloon angioplasty. The technical success was 97.6%-100% overall, 97.6%-100% for balloon-angioplasty-only and 100% for primary stenting. The clinical success was 50%-100% overall, 50%-100% for balloon-angioplasty-only and 100% for primary stenting. Long-term PV patency was 50%-100% overall, 37.5%-100% for balloon-angioplasty-only and 100% for primary stenting. Primary balloon angioplasty was successful in 78% of the cases. Of the recurrent PV stenoses, 9% resolved with stent placement and one required a meso-Rex shunt. There was one re-transplantation without stenting. The complication rate was 2.6% for balloon-angioplasty-only (bleeding, liver abscess, 2 PV thromboses) and 5.9% for primary stenting (bleeding, stent-fracture). There was no procedure-related mortality. Percutaneous transhepatic balloon angioplasty may be the initial management of portal vein stenosis in paediatric liver recipients. Stent placement may be a primary option in selected cases and a reliable rescue option for recurrent portal vein stenosis following balloon-angioplasty-only.

Meso-Rex bypass versus portosystemic shunt for the management of extrahepatic portal vein obstruction in children: systematic review and meta-analysis.

Extrahepatic portal vein obstruction (EHPVO) is a major cause of non-cirrhotic portal hypertension in children. Surgical procedures for EHPVO include portosystemic shunts (PSS) and meso-Rex bypass (MRB). We conducted a systematic review and meta-analysis to compare the effectiveness of MRB versus PSS in EHPVO patients. A systematic literature search was performed using four databases. Articles reporting EHPVO and comparing patients who received MRB and PSS were included in the analysis. We retrieved 851 papers, of which five observational studies met the inclusion criteria. There was no difference in shunt complications, mortality, or gastrointestinal bleeding after surgery between MRB and PSS in the meta-analysis. MRB had increased shunt complications compared with PSS in the non-comparative studies. MRB had a potential advantage over PSS in long-term prognosis in one comparative study. Overall, the quality of the evidence was low. Based on available data, our meta-analysis indicates that MRB does not increase shunt complications, mortality, or gastrointestinal bleeding after surgery. The present study did not reveal superiority for either MRB or PSS. The paucity of well conducted trials in this area justifies future multicenter studies and studies that examine long-term outcomes.

Pediatric non-cirrhotic portal hypertension: Endoscopic outcome and perspectives from developing nations.

Non-cirrhotic portal hypertension (NCPH) forms an important subset of portal hypertension in children. Variceal bleed and splenomegaly are their predominant presentation. Laboratory features show cytopenias (hypersplenism) and preserved hepatic synthetic functions. Repeated sessions of endoscopic variceal ligation or endoscopic sclerotherapy eradicate esophageal varices in almost all cases. After variceal eradication, there is an increased risk of other complications like secondary gastric varices, cholangiopathy, colopathy, growth failure, especially in extra-hepatic portal vein obstruction (EHPVO). Massive splenomegaly-related pain and early satiety cause poor quality of life (QoL). Meso-Rex bypass is the definitive therapy when the procedure is anatomically feasible in EHPVO. Other portosystemic shunt surgeries with splenectomy are indicated when patients present late and spleen-related issues predominate. Shunt surgeries prevent rebleed, improve growth and QoL. Non-cirrhotic portal fibrosis (NCPF) is a less common cause of portal hypertension in children in developing nations. Presentation in the second decade, massive splenomegaly and patent portal vein are discriminating features of NCPF. Shunt surgery is required in severe cases when endotherapy is insufficient for the varices. Congenital hepatic fibrosis (CHF) presents with firm palpable liver and splenomegaly. Ductal plate malformation forms the histological hallmark of CHF. CHF is commonly associated with Caroli’s disease, renal cysts, and syndromes associated with neurological defects. Isolated CHF has a favourable prognosis requiring endotherapy. Liver transplantation is required when there is decompensation or recurrent cholangitis, especially in Caroli’s syndrome. Combined liver-kidney transplantation is indicated when both liver and renal issues are present.

P-125 REX SHUNT IN A DEVELOPING COUNTRY – IS IT POSSIBLE?

Extrahepatic portal vein obstruction (EHPVO) is a frequent cause of noncirrhotic portal hypertension in children. Describe the experience in the surgical treatment of EHPVO in children, in a developing country. Retrospective case series study, with medical records review of patients with EHPVO, who underwent surgical treatment, by an experienced surgeon, between July 2016 and May 2019. Patient profile, laboratory test, images, liver histology, surgery performed, postoperative complications and shunt patency were analysed. 12 patients, median age of 4 years, umbilical catheterization was present in 8 patients (66,6%). Ten patients performed portography, and 60% had type A by Baveno VI criteria. Despite normal liver tests, liver biopsy revealed ductular proliferation in 83,3% of patients and mild portal fibrosis in 66,7%. Splenomegaly was present in 91,7% and thrombocytopenia in 83,3%. All patients had oesophageal varices and gastrointestinal bleeding occurred in 83,3%. Among the coagulation tests, the deficiency of C and S proteins is noteworthy in most patients, with 72,3% and 63,6% respectively. It was possible to perform meso-Rex bypass in 10 patients (83,3%); in the other 2 distal splenorenal shunt was performed. Early post-operative complications occurred in 58,3% of patients, the most common was ascites in 4 (33,3%), which resolved in less than 1 month. One patient developed shunt thrombosis in the first 7 days after surgery, not resolved with thrombectomy. In outpatient follow-up one patient developed thrombosis in the Rex shunt and another 4 had stenosis. All of them underwent to interventional radiology. Currently 8 of 10 meso-Rex patients (80%) have patent shunt. Rex shunt is possible in developing countries with an experienced surgeon and multidisciplinary team.

Revascularization of occluded meso-Rex shunt.

Revascularization of occluded meso-Rex shunt.

Modified Meso-Rex bypass with umbilical vein recanalization and intra-operative stenting.

The aim of the study was to evaluate the usefulness of a novel modified Meso-Rex bypass surgical technique with umbilical vein recanalization and intra-operative stenting to treat portal vein cavernous transformation. In total, 13 portal vein cavernous transformation patients underwent Meso-Rex bypass surgery, consisting of bypassgrafts between the superior mesenteric vein (SMV) and the recess of Rex as well as through the ligamentum teres hepatis without stent implantation (Group A, n = 9) and umbilical vein recanalization with intra-operative stent implantation (Group B, n = 4). In Group A, the bypass diameter was 0-6mm (median 3mm) and blood flow velocity 25-115cm/s (median 72cm/s) 1month after Meso-Rex bypass surgery, with open bypass times of 0-67months (median 6months); 6 patients in this group developed postoperative Meso-Rex bypass occlusions. A patient in Group A treated with ligamentum teres hepatis recanalization needed a thrombectomy and stent implantation during a second surgery 2days after the Meso-Rex bypass, because of bypass thrombosis and umbilical vein stenosis. In Group B, the average modified Meso-Rex bypass diameter was 5.5-6.5mm (median 6mm), and the bypass vessels remained open in all patients, with blood flow rates of 45-100cm/s (median 76.5cm/s) 1month after the modified Meso-Rex bypass, up to the endpoint (15-33months, median 24months). The rate of bypass occlusions in Group A and Group B were 22.2% and 0%, 30.0% and 0%, and 55.6% and 0% at 1month, 3months, and 1year, respectively, after bypass surgery. Our novel modified Meso-Rex bypass approach for portal vein cavernous transformation treatment was effective with excellent long-term bypass patency.

Meso-Rex bypass for the management of extrahepatic portal vein obstruction in adults (with video)

BackgroundExtrahepatic portal vein obstruction (EHPVO) results in severe portal hypertension (PHT) leading to severely compromised quality of life. Often, pharmacological and endoscopic management is unable to solve this problem. Restoring hepatic portal flow using meso-Rex bypass (MRB) may solve it. This procedure, uncommon in adult patients, is considered the treatment of choice for EHPVO in children. MethodsFrom 1997 to 2018, 8 male and 6 female adults, with a median age of 51 years (range 22–66) underwent MRB procedure for EHPVO at the University Hospitals Saint-Luc in Brussels, Belgium. Symptoms of PHT were life altering in all but one patient and consisted of repetitive gastro-intestinal bleedings, sepsis due to portal biliopathy, and/or severe abdominal discomfort. The surgical technique consisted in interposition of a free venous graft or of a prosthetic graft between the superior mesenteric vein and the Rex recess of the left portal vein. ResultsMedian operative time was 500 min (range 300–730). Median follow-up duration was 22 months (range 2–169). One patient died due to hemorrhagic shock following percutaneous transluminal intervention for early graft thrombosis. Major morbidity, defined as Clavien-Dindo score ≥ III, was 35.7% (5/14). Shunt patency at last follow-up was 64.3% (9/14): 85.7% (6/7) of pure venous grafts and only 42.9% (3/7) of prosthetic graft. Symptom relief was achieved in 85.7% (12/14) who became asymptomatic after MRB. ConclusionsAdult EHPVO represents a difficult clinical condition that leads to severely compromised quality of life and possible life-threatening complications. In such patients, MRB represents the only and last resort to restore physiological portal vein flow. Although successful in a majority of patients, this procedure is associated with major morbidity and mortality and should be done in tertiary centers experienced with vascular liver surgery to get the best results.

Value of preoperative computed tomography for meso-Rex bypass in children with extrahepatic portal vein obstruction

BackgroundExtrahepatic portal vein obstruction (EHPVO) is the most important cause of hematemesis in children. Intrahepatic left portal vein and superior mesenteric vein anastomosis, also known as meso-Rex bypass (MRB), is becoming the gold standard treatment for EHPVO. We analyzed the value of preoperative computed tomography (CT) in determining whether MRB is feasible in children with EHPVO.ResultsWe retrieved data on 76 children with EHPVO (50 male, 26 female; median age, 5.9 years) who underwent MRB (n = 68) or the Warren procedure (n = 8) from 2013 to 2019 and retrospectively analyzed their clinical and CT characteristics. The Rex recess was categorized into four subtypes (types 1–4) depending on its diameter in CT images. Of all 76 children, 7.9% had a history of umbilical catheterization and 1.3% had leukemia. Sixteen patients (20 lesions) had associated malformations. A total of 72.4% of Rex recesses could be measured by CT, and their mean diameter was 3.5 ± 1.8 mm (range 0.6–10.5 mm). A type 1, 2, 3, and 4 Rex recess was present in 9.2%, 53.9%, 11.8%, and 25.0% of patients, respectively. MRB could be performed in patients with types 1, 2, and 3, but those with type 4 required further evaluation. The sensitivity, specificity, positive predictive value, negative predictive value, and diagnostic accuracy of CT were 100%, 83.8%, 42.1%, 100%, and 85.5%, respectively.ConclusionsAmong the four types of Rex recesses on CT angiography, types 1–3 allow for the performance of MRB.

Increased Prevalence of Left Lobe Atrophy in Long-standing Extrahepatic Portal Vein Obstruction.

The aim of this study was to describe long-term changes in standard blood tests and ultrasound (US) findings in pediatric patients with Extrahepatic Portal Vein Obstruction (EHPVO) who have not undergone Meso-Rex Bypass (MRB) surgery. US, laboratory, and endoscopic data of 77 patients were analyzed and compared at 2 different points in time: at initial workup and at most recent follow-up. Differences were assessed using McNemar and Wilcoxon tests, while correlations were evaluated using generalized estimating equations. Upper gastrointestinal bleeding was the most frequent initial manifestation of EHPVO, in 45.4% of subjects. The mean age at diagnosis was 4.3 years. Seventy-four percentage had a previous history of umbilical catheterization and/or neonatal sepsis. Over time, there was a significant increase in the prevalence of leukopenia, thrombocytopenia, and, interestingly, of left lobe atrophy (LLA), even though the number of esophageal varices and the need for endoscopic interventions reduced. A significant correlation was found between history of umbilical catheterization and LLA, splenomegaly and LLA, and gallbladder wall thickening and LLA. Overall, the number of patients with cholelithiasis at initial workup was low (and therefore, not analyzed with inferential statistics); however, we observed a relative increase over time. In this cohort of patients with EHPVO followed over more than 8 years without treatment with MRB, we report the novel finding of a significant increase in the prevalence of LLA over time. LLA correlated with past medical history of umbilical catheterization, and findings of splenomegaly and gallbladder wall thickening. We propose that that LLA should be further explored as a marker of portal hypertension, particularly in the context of a history of umbilical catheterization.

Extrahepatic Portal Vein Thrombosis, an Important Cause of Portal Hypertension in Children.

One of the most important causes of portal hypertension among children is extrahepatic portal vein thrombosis (EHPVT). The most common risk factors for EHPVT are neonatal umbilical vein catheterization, transfusions, bacterial infections, dehydration, and thrombophilia. Our study aimed to describe the clinical manifestations, treatment, evolution, and risk factors of children with EHPVT. Methods: We analyzed retrospectively all children admitted and followed in our hospital with EHPVT between January 2011–December 2020. The diagnosis was made by ultrasound or contrast magnetic resonance imaging. We evaluated the onset symptoms, complications, therapeutic methods, and risk factors. Results: A total of 63 children, mean age 5.14 ± 4.90 (33 boys, 52.38%), were evaluated for EHPVT during the study period. The first symptoms were upper gastrointestinal bleeding (31 children, 49.21%) and splenomegaly (22 children, 34.92%). Thrombocytopenia was present in 44 children (69.84%). The most frequent risk factors were umbilical vein catheterization (46 children, 73.02%) and bacterial infections during the neonatal period (30 children, 47.62%). Protein C, protein S, antithrombin III levels were decreased in 44 of the 48 patients tested. In 42 of these cases, mutations for thrombophilia were tested, and 37 were positive. Upper digestive endoscopy was performed in all cases, revealing esophageal varices in 56 children (88.89%). All children with gastrointestinal bleeding received an octreotide infusion. In 26 children (41.27%), variceal ligation was performed, and in 5 children (7.94%), sclerotherapy. Porto-systemic shunt was performed in 11 children (17.46%), and Meso-Rex shunt was done in 4 children (6.35%). The evolution was favorable in 62 cases (98.41%). Only one child died secondary to severe sepsis. Conclusions: EHPVT is frequently diagnosed in the last period in our region due to the increased use of umbilical vein catheterization. Furthermore, genetic predisposition, neonatal bacterial infections, and prematurity certainly play an important role in this condition. A proactive ultrasound assessment of children with risk factors for EHPVT should be encouraged for early diagnosis and treatment.

Trombosis venosa portal extrahepática, manejo quirúrgico con derivación meso-Rex. Serie de 3 casos

La vena porta es un conducto que drena el flujo esplácnico al hígado y se puede ocluir por diferentes patologías, variando su presentación clínica de acuerdo con la causa de la obstrucción. Es muy importante diferenciar la trombosis portal asociada o no a la cirrosis, ya que su tratamiento y pronóstico es diferente. La trombosis venosa portal extrahepática es una condición netamente de origen vascular, y es la principal causa de trombosis portal en niños y adultos. Presentamos tres casos tratados con derivación meso-Rex, con seguimiento a 6 meses.

Meso - Rex shunt using the inferior mesenteric vein in a paediatric patient

Abstract Extrahepatic portal vein obstruction primarily results from congenital vascular malformations or acquired portal venous thrombosis. Portal hypertension can lead to various complications such as recurrent upper gastrointestinal hemorrhage secondary to varices, ascites, hypersplenism, or encephalopathy. Meso-rex shunt is a curative surgical procedure that uses autologous vein graft as a bypass to redirect blood from the superior mesenteric vein (SMV) to the patent intrahepatic left portal vein in patients. The most common vein graft used is internal jugular vein (IJV) harvested from the patient's neck. Meso-Rex shunt using the inferior mesenteric vein (IMV) has been rarely described in the literature. It avoids neck dissection and its associated complications. It also reduces the number of anastomoses down to one. We report a 14-year-old girl who underwent a successful meso-Rex shunt using the IMV and summarize the literature.

Sonographic Evaluation of a Unique Meso-Rex Shunt: A Case Study

Portal hypertension is a result of an increase in intrahepatic resistance in the main portal vein. The Meso-Rex shunt is used to bypass the obstructed portal vein and restore the venous flow into the liver. This procedure alleviates the need for a hepatic transplant. The Meso-Rex shunt has proven to be an effective treatment for extrahepatic portal vein obstruction, thus saving children from a complete transplant. There are variants to this bypass surgery, and sonography is commonly used to assess the condition pre- and postoperatively. In this case, the shunt was uniquely different from the typical Meso-Rex bypass surgery. Particular vasculature made it imperative for the sonographer to review the prior sonograms and review the chart information before preforming the examination. It should also be noted that sonographers must adapt the protocols to give the utmost treatment.

Pediatric living donor liver transplantation with non-anatomical portal vein reconstruction in idiopathic extrahepatic portal vein thrombosis

A 13-year-old boy had a history of recurrent hematemesis and melena since the age of 2 years. Owing to recurrent bleeding, he had undergone several endoscopic ligation procedures and had received regular blood transfusions over the last 7 years. Doppler ultrasonography showed extrahepatic portal vein thrombosis, while the hepatic vein was not visualized. Abdominal computed tomography confirmed cavernous transformation in the liver hilum. Liver biopsy revealed fibrosis. A diagnosis of extrahepatic portal vein obstruction (EHPVO) with portal hypertension was established. Living donor liver transplantation (LDLT) was performed owing to recurrent gastrointestinal bleeding and liver fibrosis. The liver graft was obtained from the patient's father, utilizing segments 2, 3, and 4 (graft-to-recipient weight ratio: 0.84%). Portal vein reconstruction using native internal jugular vein and external iliac vein was achieved with non-anatomical anastomosis due to the lack of availability of sizable superior mesenteric vein. The postoperative course was uneventful. We successfully performed LDLT with unusual portal vein reconstruction in a patient with EHPVO. Multiple interposition grafts were used to obtain adequate inflow from the splenorenal shunt. LDLT remains the last resort in such cases as other surgical procedures (such as Meso-Rex bypass) are not feasible.

Emergent Meso-Rex shunt to treat intraoperative unrepairable portal vein rupture: a case report

报道首例术中紧急Meso-Rex分流术治疗不可修复的门静脉破裂。患者男性，68岁，因脂肪肉瘤切除术后复发入院。诊断为复发性脂肪肉瘤拟行手术切除。肿瘤位于门静脉主干后方，被下腔静脉、腹腔干、肠系膜上动脉等重要血管包绕。术中分离肿瘤时，门静脉后壁破裂，破口直达脾静脉汇合至肠系膜上静脉处，无法修复，被迫封闭门静脉主干。为纠正急性肝前型门静脉高压，以人工血管行Meso-Rex分流术，成功恢复门静脉血流。术后患者恢复顺利，至今随访1年余生存良好。

Outcome of portosystemic shunt surgery on pre-existing cholangiopathy in children with extrahepatic portal vein obstruction.

This study was performed to assess the effect of portosystemic shunt surgery (PSS) on portal cavernoma cholangiopathy (PCC) in children with extrahepatic portal veinobstruction (EHPVO). Children with EHPVO and PCC (unfit for Meso-Rex shunt) underwent magnetic resonance cholangiogram (MRC) and magnetic resonance portovenogram (MRPV) before non-selective PSS. PCC was graded by modified Llop classification. Those with patent shunt were re-evaluated at least 6months after surgery with MRC, MRPV and compared with pre-shunt images. Twenty-five children underwent PSS (central end to side splenorenal shunt with splenectomy [n=24], mesocaval shunt [n=1]). Pre-surgery MRC showed PCC grade I in 11, grade II in 1 and grade III in 13. MRPV showed superior mesenteric vein (SMV)block in 20. Re-assessment for PCC 18 (6 to 54) months after surgery showed grade I in 6 and grade III in 19. Thus, PCC was progressive in 6 and static in 19. Density of peribiliary collaterals decreased in 5 (SMV patent, static PCC), increased in 3 and remained unchanged in 17. Splenomegaly-related problems, gastroesophageal varices and other intra-abdominal (esophageal, perisplenic and perigastric) collaterals ameliorated in all. Non-selective PSS decompresses esophago-gastro-splenic venous circuit effectively but fails to ameliorate cholangiopathy and peribiliary collaterals. Persistence of cholangiopathy is attributable to SMV block.

Technique and outcome of domino liver transplantation from patients with maple syrup urine disease: Expanding the donor pool for live donor liver transplantation.

Domino liver transplantation (DLT) using liver allografts from patients with metabolic disorders enhances organ utilization. Short- and long-term course and outcome of these patients can impact the decision to offer this procedure to patients, especially those with diseases that can potentially be cured with liver transplant. We reviewed the outcomes of DLT from maple syrup urine disease (MSUD) patients in our large academic pediatric and adult transplant program. All patients receiving DLT were analyzed retrospectively with a minimum of one-year follow-up period for patient and donor characteristics, early and late postoperative complications and patient and graft survival with their MSUD donors in terms of age, weight, MELD/PELD scores, cold ischemia time, postoperative leucine levels, and peak ALT (alanine aminotransferase) levels during the first 48 postoperative hours. Between 2006 and May 2019, 21 patients underwent domino liver transplantation with live donor allografts from MSUD patients. Four patients transplanted for different metabolic diseases are focus of a separate report. Seventeen patients with minimum one-year follow-up period are reported herein. The indications were primary sclerosing cholangitis (PSC, n=4), congenital hepatic fibrosis (CHF, n=2), alpha-1 antitrypsin deficiency (A-1 ATD, n=2), progressive familial intrahepatic cholestasis (PFIC, n=2), cystic fibrosis (n=1), primary biliary cirrhosis (PBC, n=1), neonatal hepatitis (n=1), embryonal sarcoma (n=1), Caroli disease (n=1), hepatocellular carcinoma (HCC, n=1), and chronic rejection after liver transplantations for PSC (n=1). All patients and grafts survived at median follow-up of 6.4years (range 1.2-12.9years). Median domino recipient age was 16.2years (range 0.6-64.6years) and median MSUD recipient age was 17.6years (range 4.8-32.1years). There were no vascular complications during the early postoperative period, one patient had portal vein thrombosis 3years after DLT and a meso-Rex bypass was successfully performed. Small for size syndrome (SFSS) occurred in reduced left lobe DLT recipient and was managed successfully with conservative management. Biliary stricture developed in 2 patients and was resolved by stenting. Comparison between DLT and MSUD recipients' peak postoperative ALT results and PELD/MELD scores showed lower levels in DLT group (P-value <.05). Patient and graft survival in DLT from MSUD donors was excellent at short- and long-term follow-up. Metabolic functions have been normal in all recipients on a normal unrestricted protein diet. Ischemia preservation injury based on peak ALT was significantly decreased in DLT recipients. Domino transplantation from pediatric and adult recipients with selected metabolic diseases should be increasingly considered as an excellent option and alternative to deceased donor transplantation, thereby expanding the living donor pool. This, to date, is the largest world experience in DLT utilizing livers from patients with MSUD.

The role of surgical shunts in the treatment of pediatric portal hypertension

BackgroundPortal diversion by surgical shunt plays a major role in the treatment of medically refractory portal hypertension. We evaluate our center’s experience with surgical shunts for the treatment of pediatric portal hypertension. MethodsAll patients who underwent surgical shunt at a single institution from 2008 to 2017 were reviewed. The primary outcome was intervention-free shunt patency. ResultsIn this study, 34 pediatric patients underwent portal shunt creation. The median age was 7.7 years (interquartile range 4.3–12.0). Twenty-nine patients (85%) had prehepatic portal hypertension and 5 patients (15%) had intrahepatic portal hypertension. The primary manifestations of portal hypertension were esophageal varices (97%) and gastrointestinal bleeding (77%). Eighteen patients (53%) underwent meso-Rex bypass, 10 patients (29%) underwent splenorenal shunt, and 6 patients (18%) underwent mesocaval shunt. Outcomes were notable for minimal wound complications (9%), rebleeding events (12%), and mortality (3%). In the postoperative setting, 10 patients (29%) experienced a shunt complication (occlusion or stenosis), 4 of which occurred in the early postoperative period and required urgent intervention. The 1-year and 5-year “primary patency” patency rates were 71% and 66%, respectively. ConclusionChildren suffer significant morbidity from the sequelae of portal hypertension. Our experience reinforces the feasibility of surgical shunts as an effective treatment option associated with low rates of morbidity and mortality.

Recanalization of Chronic Extrahepatic Portal Vein Obstruction in Pediatric Patients Using a Minilaparotomy Approach.

Extrahepatic portal vein obstruction (EHPVO) is the most frequent cause of portal hypertension in children. Some patients are not amenable to meso-Rex bypass and alternative surgeries do not restore physiologic flow. We aim to demonstrate the feasibility and safety of minilaparotomy for recanalization of chronic EHPVO. This 2013-2015 single-center, retrospective review included pediatric patients with chronic EHPVO who underwent minilaparotomy, mesenteric vein access, and attempted recanalization of the occluded portal vein. Outcomes included portal patency, resolution of variceal bleeding, size and number of varices, spleen size, and platelet count. There were 6 EHPVO patients. The median age was 9.9 years and median duration of EHPVO was 7 years (3-16 years). EHPVO etiologies were liver transplantation (50%), idiopathic (33%), and umbilical vein catheterization (17%). Four patients (67%) had successful portal vein recanalization and stenting. At last follow-up [median 3.1 years (2.2-4.3 years)] all successfully recanalized patients had patent portal vein stents and resolution of varices and variceal bleeding. The median reduction in spleen size was 26%, with improvement in platelet counts (50-310/μL). The 2 patients with an idiopathic etiology may have never had a main extrahepatic portal vein based on imaging, and both were unable to be recanalized. Recanalization and stenting of a prolonged occlusion of the portal vein via a minilaparotomy approach is feasible, safe, and may provide an alternative to shunt surgery or endoscopic therapy in selected patients.