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Background: Pediatric living-donor liver transplantation (LDLT) has become one of the most effective therapies for pediatric end-stage liver diseases. We aim to investigate the risk factors for intra-operative portal vein thrombosis (PVT) and the short- and long-term outcomes in children post LDLT., Methods: This was a retrospective analysis from 584 cases of biliary atresia (BA) patients who had undergone LDLT from January 2014 to December 2019 at our hospital. Patients were divided into PVT and non-PVT groups according to the occurrence of PVT during LDLT., Results: The median age of recipients at transplantation was 7.22 (quartiles, 6.03, 9.50) months, the incidence of intra-operative PVT was 5.31% (31/584). The independent risk factors for intra-operative PVT were the diameter of the recipient's PV not greater than 4 mm and a higher ratio of graft-to-recipient PV diameter. The cumulative survival rates of grafts and recipients were 93.5% and 93.5% in the PVT group, and 94.9% and 95.3% in the non-PVT group, respectively, without significant difference. The recovery of graft function was similar in recipients with or without interposed graft vessel (IGV). However, the incidence of PV stenosis was higher in recipients with IGV after LDLT., Conclusion: Intra-operative PVT is a common complication in pediatric LDLT, but an excellent prognosis can be achieved by appropriate and individualized surgical treatment. We noted that intra-operative PVT did not affect the survival rates of grafts and recipients, but there was a higher incidence of PV complications after LDLT., Level of Evidence: Ⅲ., Competing Interests: Declaration of competing interests The authors declare no conflicts of interest. We would like to thank all authors who contributed to this research and all authors are in agreement with the content of the manuscript. We certify that we have participated sufficiently in the work to take public responsibility for the experimental design and method, and the analysis and interpretation of the data., (Copyright © 2022. Published by Elsevier Inc.)

Abstract: Background/Purpose: Infants with biliary atresia undergoing living donor liver transplantation (LDLT) are at increased risk of portal vein (PV) complications because of their smaller vascular caliber and sclerosis because of previous Kasai portoenterostomy and recurrent cholangitis. Method: Of 154 children who underwent transplantation between November 2005 and January 2011, 34 with biliary atresia received a transplant while younger than 1 year. Six patients underwent PV reconstruction with an interposition vein graft, and the others underwent the branch patch technique. The clinical characteristics of those who underwent the interposition reconstruction or the branch patch technique were compared, and the PV complications were assessed. Results: Portal vein complications occurred in 5 patients (14.7%) in the branch patch group. There were 4 patient deaths, and all of them had received branch patch reconstruction. The branch patch reconstruction cases with a sclerotic small caliber (<4 mm) determined by using preoperative ultrasonography showed a significantly high mortality rate (44.4%). All patients with interposition vein graft reconstruction are still alive with excellent graft function without anticoagulation therapy. Conclusion: The interposition vein graft appears to be a feasible option with better graft survival and less PV complications when performing LDLT for biliary atresia in infants younger than 1. [Copyright &y& Elsevier]

Abstract: Background: Portal vein thrombosis (PVT) or stenosis (PVS) often requires challenging techniques for reconstruction in living donor liver transplantation (LDLT). Materials and Methods: A total of 57 LDLTs were performed between October 1996 and December 2010. There were 16 cases (28%) with PVT/PVS that underwent modified portal vein anastomosis (m-PVa). The m-PVa techniques were classified into 3 groups: patch graft (Type-1), interposition graft (Type-2), and using huge shunt vessels (Type-3). The reconstruction patterns were evaluated with regard to age, graft vessels, PV flow, and complication rate. Results: The m-PVas were Type-1 in 10 cases, Type-2 in 3 cases, and Type-3 in 3 cases. The vessel graft in Type-1 was the inferior mesenteric vein (IMV) in 8 and the jugular vein in 2 cases, whereas the vessel graft in Type-2 was IMV in 2 and the saphenous vein in 1 case; in Type-3, the vessel grafts were renoportal, gonadal-portal, and coronary-portal anastomoses, respectively. The postoperative PV flow was sufficient in all types and slightly higher in Type-3. The postoperative complications occurred in 20% of the patients who underwent Type-1, in 33% who underwent Type-2, and in 0% who underwent Type-3. Conclusion: The m-PVa was effective to overcome the surgical difficulty during transplantation. Pretransplant planning for the selection of the type of reconstruction is important for recipients with PVT/PVS. [Copyright &y& Elsevier]

Abstract: Hepatocellular carcinoma (HCC) in children is rare, and the prognosis has been poor because of its advanced stage at diagnosis and unresponsiveness to chemotherapy. We report a 13-year-old boy with ruptured HCC in the left trisegment. When hemostasis of the ruptured surface was achieved in the emergency operation, the left branch of the portal vein and the left hepatic artery were ligated at the same time. The volume of the future liver remnant (FLR), that is, his right posterior sector, increased from 56% on admission to 70% of his standard liver volume on day 2. Blood level of serum protein induced by vitamin K absence or antagonist ІІ started to decrease immediately. Left trisegmentectomy was successfully performed 10 days later, followed by chemotherapy. He has been well with a 2-year survival without recurrence. When the FLR is considered relatively small for a major hepatic resection, the selective ligation of the portal vein and the hepatic artery, which feed HCC, seems to be beneficial. This is because it may induce enlargement of the FLR, increasing the safety of the hepatectomy as preoperative portal vein embolization does before a major hepatectomy in adult patients with HCC, and the latter suppresses the tumor while waiting for the planned hepatectomy. [Copyright &y& Elsevier]

Abstract: A 3-year-old girl with idiopathic extrahepatic portal vein obstruction undergoing mesenterico-left portal vein bypass (MLPVB), or Rex shunt, for recurrent variceal bleeding was intraoperatively discovered to have duplicated round ligaments and Rex recessi. The left and right portal veins connected to their respective round ligaments but had minimal communication within the liver. Intraoperative venography identified a better preserved portal system on the left, and standard MLPVB resulted in excellent shunt flow. The shunt remains patent and the patient asymptomatic 9 months after her operation. Abnormal portal anatomy is not a contraindication to MLPVB. [Copyright &y& Elsevier]

Abstract: Aim: Extrahepatic portal vein obstruction (EPVO) is a common cause of portal hypertension in children and can lead to life-threatening bleeding, thrombocytopenia, and coagulation disorders. Mesoportal bypass (MPB) restores normal physiologic portal flow to the liver and corrects portal hypertension. There is, however, little long-term outcome data after MPB. The aim of our study was to analyze the long-term outcome after MPB in children. Methods: Retrospective single-center review of all MPB with more than 5-year follow-up was performed in children between 1998 and 2003. Results: Thirty children underwent MPB, which was successful in 29. Long-term follow-up is available for 24. Median age at the time of bypass was 8.5 years (range, 0.4-14.2 years). Material used for bypass was as follows: left internal jugular vein (n = 20), recanalized umbilical vein (n = 2), gastric vein, and a large colic vein (n = 2). Median time since MPB is 8 years (range, 5.3-8.8 years). One MPB using recanalized umbilical vein thrombosed at 4 months but was successfully refashioned using Gortex. One MPB with left internal jugular vein was thrombosed at 1 year after cardiac surgery. Overall, 23 of 24 children have a patent bypass and resolution of portal hypertension. All showed an decrease in spleen size. Recurrent blood flow in the cavernoma with secondary increase in spleen size occurred in 5 children (median time, 3.4 years after MPB). Further evaluation of these 5 revealed no recurrence of portal hypertension. Conclusion: The MPB provides long-term correction of portal hypertension owing to EPVO. Where technically feasible, MPB is the preferred surgical procedure for treatment of EPVO. [Copyright &y& Elsevier]

Abstract: Background: Endoscopic sclerotherapy (ES) has been the standard treatment for children with idiopathic extrahepatic portal vein obstruction (EHPVO). Portosystemic shunts are indicated when variceal bleeding cannot be controlled by ES. Recently, mesenteric left portal vein bypass was indicated as a surgical intervention and preventative measure for hepatic dysfunction in children with long-term EHPVO. Nevertheless, there is a lack of published data confirming the extent of hepatic dysfunction, hypersplenism, and physical development in children with long-term follow-up. Method: We retrospectively verified the long-term outcomes in 82 children with EHPVO treated with ES protocol, focusing on mortality, control of bleeding, hypersplenism, and consequent hepatic dysfunction. Results: Of the children, 56% were free from bleeding after the initiation of ES. The most frequent cause of rebleeding was gastric varices (30%). Four patients had recurrent bleeding from esophageal varices (4.6%). Four patients underwent surgery as a consequence of uncontrolled gastric varices. There were no deaths. Most patients showed good physical development. We observed a mild but statistically significant drop in factor V motion, as well as leukocyte and platelet count. Conclusion: Endoscopic sclerotherapy is an efficient treatment for children with EHPVO. The incidence of rebleeding is low, and there was no mortality. Children develop mild liver dysfunction and hypersplenism with long-term follow-up. Only a few patients manifest symptoms of hypersplenism, portal biliopathy, or liver dysfunction before adolescence. [Copyright &y& Elsevier]

Abstract: Background: Liver nodules have been reported after portal systemic shunt surgery (PSSS) in animal experiments or in humans with liver cirrhosis. The aim of our study was to assess the incidence of liver nodules after surgery for extrahepatic portal vein obstruction (EHPVO) in children without associated liver disease. Methods: We retrospectively reviewed the charts of 45 children who had surgery from 1979 to 2005 for EHPVO in our institution, consisting of 38 PSSS and 7 portal reperfusion procedures (PRPs). We assessed the presence of liver nodules on ultrasonography. Results: Of 45 patients, 7 (15%) had liver nodules during a median of 80 months of follow-up. All the nodules occurred after PSSS. Five nodules were subjected to biopsy; we found 2 liver cell adenomas and 3 focal nodular hyperplasias. Conclusions: In this study, liver nodules occurred in 18% of cases after PSSS for EHPVO in children and not after PRP. As many children have undergone PSSS throughout the world, the presence of liver nodules should be considered during the follow-up of those patients. [Copyright &y& Elsevier]

Abstract: Purpose: The prognostic significance of portal venous gas (PVG) in neonatal necrotizing enterocolitis (NEC) for operative intervention (OP), neonatal complications, and mortality remains uncertain. The authors designed a long-term prospective study to describe the natural history of PVG related to these outcomes and to test the hypothesis that PVG does not mandate OP. Methods: All infants admitted to a single center between October 1991 and February 2003 were evaluated weekly to identify all cases of NEC (defined as Bell stage II or higher). Demographic, radiological, surgical, and outcome data were abstracted prospectively. Radiographic studies were performed at the onset of illness and at subsequent 6- to 8-hour intervals or as clinically indicated. A single pediatric radiologist reviewed all radiographs. Values are expressed as mean ± SD. Odds ratios and relative risk ratios are reported with 95% CIs. The level of significance was P ≤ .05. Results: After the exclusion of 24 infants with lethal diseases, major congenital or chromosomal anomalies, or recurrent episodes of NEC, 194 of 5891 infants developed NEC. The overall incidence of NEC was 3.7%. In 194 infants with NEC, the incidence of PVG was 33% (n = 64). Gestational age (30.8 ± 4 vs 29.3 ± 4.2 weeks; P = .02) but not birth weight (1609 ± 761 vs 1434 ± 810 g; P = NS) was greater in infants with PVG compared with infants without PVG (n = 130). Sixty-six (34%) infants with NEC underwent OP. Operative intervention occurred more frequently in infants with PVG compared with infants without PVG (OR, 2.5; CI, 1.37-4.76; P = .003)—only 48% of infants with PVG underwent OP. Among the variables, gestational age, severe NEC (Bell stage III), severe intramural gas (in all 4 abdominal quadrants), and the presence of PVG, severe NEC was most highly associated with OP (OR, 77.47; CI, 10.36-580.16; P < .0001). Bell stage III NEC was present in 98% of infants who underwent OP compared with 40% of infants without OP (P < .0001). Of all infants with NEC, 37 (19%) died. Mortality was higher among infants who underwent OP (33% vs 12%; P < .0003). A multivariate regression model identified Bell stage III (OR, 3.74; CI, 1.20-11.62; P = .02), but neither PVG nor OP, to be significantly associated with mortality. Of interest is that survival in infants with PVG was greater (but not significantly so) than in infants without PVG in both OP (74% vs 59%) and non-OP (91% vs 87%) groups. Furthermore, 30 of 64 (47%) infants with PVG survived without OP, and of all 33 infants with PVG who did not undergo OP, 30 (91%) infants survived. Conclusions: Decision for OP should be based on the severity of NEC and not on the presence of PVG alone because nearly half of infants with PVG survive without OP. Overall, the presence of PVG does not increase the risk of mortality among infants with NEC. Severe NEC, but not OP, is associated with higher mortality. [Copyright &y& Elsevier]

Abstract: Background/Purpose: Blood drainage of the graft into the recipient portal vein reestablishes the physiological venous outflow after small bowel transplantation (SBT). However, although this approach is likely beneficial for the host, it may be technically more demanding making portocaval venous drainage the preferred arrangement during human SBT. The aim of this study was to examine in a syngeneic model of SBT the possible benefits of portoportal anastomosis (PPA) vs portocaval anastomosis (PCA) in terms of body and organ weights and bacterial translocation. Methods: Syngeneic SBT was carried out in 25 Brown-Norway male rats weighing 249 ± 17.5 g using either PPA (n = 13) or PCA (n = 12). Half the animals in each group were killed, respectively, on postoperative day 2 or 7. Liver, spleen, and lungs were weighed and under sterile conditions the regional lymph nodes were excised. The nodes and venous samples from the cava and portal veins were cultured for aerobes and anaerobes. Bacterial components were detected in blood by polymerase chain reaction. The findings in both groups were compared by χ 2 or Mann-Whitney U tests. Results: Mean postoperative body weight change was −3.6% ± 1.5% in PPA and −6.0% ± 1.2% in PCA animals (ns) on day 2 and −6.5% ± 2.6% and −8.0% ± 5.0% (ns) on day 7. Liver, spleen, and lung weights were not significantly different between both groups on either end point. Gram-negative enteric bacteria were found in 3 of 7 PCA animals and 2 of 6 PPA animals at day 2 (ns) and in 1 of 6 and 4 of 6 on day 7 (ns). Aerobic gram-positive bacteria were found in 1 of 7 and 1 of 6 (ns), 3 of 6 and 3 of 6 (ns), respectively, in the 4 groups. Most positive cultures corresponded to portal blood and lymph node samples. There were no anaerobic growths. Conclusions: [-] No body or organ weight change suggesting significant functional advantages of one technical alternative over the other could be demonstrated. [-] Bacterial translocation in the absence of rejection was frequent after SBT independently of the variety of venous outflow used. No difference in bacterial translocation between both anastomosis could be demonstrated. [-] Orthotopic venous drainage did not seem to be advantageous in the present experimental setting. [Copyright &y& Elsevier]

Purpose: To report our initial experience with splenectomy and proximal spleno-left portal shunt as an alternative to the standard Rex shunt, when not applicable, in children with Extrahepatic Portal Vein Obstruction (EHPVO)., Methods: Patients from March 2015 till September 2018, with EHPVO not suitable for Rex shunt or whose caregivers refused to consent for Internal Jugular Vein (IJV) dissection were assessed and prepared for splenectomy with proximal spleno-left portal shunt. The operative technique includes splenectomy, freeing of the splenic vein from the pancreatic bed till its junction with the inferior mesenteric vein, and then anastomosis with the intrahepatic left portal vein at the Rex recess. A distal lieno-renal shunt was performed in one patient and was excluded from the study., Results: A total of 14 patients (mean age: 4.6 years) underwent splenectomy with proximal spleno-left portal shunt during the study period. The mean operative time was 246 min, while the mean postoperative hospital stay was 4.1 days. The patients' follow up period ranged from 6 to 42 months (median: 19.6 months). Only two patients had a single attack of variceal bleeding, 2 and 2.5 months postoperative respectively, and required endoscopic management with no further bleeding episodes. While the rest of patients showed an improvement of their variceal grades after the surgery., Conclusion: Splenectomy with proximal spleno-left portal shunt seems to be a valuable alternative to the standard Rex shunt in treatment of children with EHPVO unsuitable for or following unsuccessful Rex shunt., Level of Evidence: IV., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Objective: Distinguishing retrohepatic end-to-side portocaval shunts (ES-PCS) and side-to-side portocaval shunts (SS-PCS) can be difficult, but it is essential for determining the treatment strategy. Our experience with retrohepatic PCS is analyzed., Methods: Since 2007, 9 children (5/9 ES-PCS and 4/9 SS-PCS) were surgically treated. Radiology studies included Doppler-ultrasound, CT/MRI and angiography/occlusion test (8/9)., Results: CT in 5/5 ES-PCS revealed the portal vein (PV) entering the left side of the vena cava with a uniform shape. 4/4 SS-PCS showed aneurysmal PV containing the origin of the main intrahepatic portal branches (IHPB) entering the cava anterior aspect or slightly to the right with a variable length (from long to short/wide). ES-PCS anatomy showed caudate lobe absence with the fistula entering the left cava aspect free of parenchyma, but anterior through the caudate lobe in SS-PCS. With the angiography/occlusion test, the IHPB was undeveloped in ES-PCS (portal pressure > 38 mmHg) and hypoplasic in SS-PCS (portal pressure < 25 mmHg). ES-PCS treatment included: 1/5 hepatectomy and 4/5 definitive banding (one by laparoscopy); while in SS-PCS: 1/4 liver transplantation, 2/4 1-step closure (one by laparoscopy), and 1/4 definitive banding., Conclusion: Fistula shape, cava relationship, IHPB and portal pressures differ between the two shunt types. A question arises regarding the need for secondary complete closure after banding., Level of Evidence: Level IV., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Purpose: Extrahepatic portal vein thrombosis (EPVT) is one major cause of portal hypertension in children. Surgical reinstallation of portal venous flow can be achieved in patients with patent intrahepatic portal venous system/Rex recess. Our study aimed to compare the ability of magnetic resonance imaging (MRI) and retrograde portography (RP) to assess patency of the intrahepatic portal venous system in children with EPVT., Methods: All pediatric patients with EPVT who were examined with contrast enhanced MRI (1.5 T) and invasive RP between 2013 and 2017 were included in this retrospective study. Medical records were reviewed for demographic, biochemical and clinical data. Patency of the Rex recess as detected by MRI and RP was retrospectively reviewed., Results: Sixteen children (7.6 ± 5.0 years) with EPVT were included. Sensitivity, specificity, positive and negative predictive value for the detection of patent Rex recess by MRI compared to RP were 55%, 57%, 63% and 50%. Diagnostic accuracy was 56%. Diagnostic failure of MRI compared to RP was explained by the following: I. Problems differentiating collaterals from portal venous vessels II. Incapability showing dynamic blood flow in compromised portal venous flow III. Poor spatial resolution, especially in small children., Conclusion: RP is a reliable method for the visualization of the Rex recess and the intrahepatic portal venous system in children with EPVT, whereas MRI has shown to be unsuitable for the assessment of the intrahepatic portal vein in these patients. In the preoperative setup, we recommend both procedures, RP and MRI for the visualization of the intrahepatic portal venous system, and the extrahepatic vessels, respectively., Level of Evidence: Level III., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Purpose: To study the varied presentations and the outcomes in children with Type 2 Abernethy malformation following shunt ligation., Material and Methods: Children with Type 2 Abernethy who had had been operated between 2013 and 2017 were included in the study. The diagnosis had been confirmed on ultrasonography, CECT or angiography. All patients underwent laparotomy. The shunt was identified, clamped and the bowel congestion was noted. The shunt was ligated if the bowel congestion was not significant or had improved. Relevant follow-up investigations were done to document the resolution or amelioration of symptoms and the patency of the shunt., Results: Five patients were included in the study with a median age of 6 years. Hepatopulmonary syndrome was the presentation in 4 patients while one patient presented with liver tumor. Ultrasonography and CECT were able to diagnose Type 2 malformation in 4 patients whereas in 1 patient the distal portal vein was not seen. The postoperative period was complicated in 3 patients. At the median follow up at 14 months, good intrahepatic portal flow in all patients. All patients demonstrated improvement/ resolution of symptoms., Conclusion: Abernethy is rare malformation which can have a varied presentation. Additional investigations may be needed to confirm the diagnosis of Type 2 variety. Most patients have gradual improvement of symptoms., Level of Evidence: Level IV/ Treatment study., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Objective: Rex shunt (mesenteric-to-left portal vein bypass) is considered a more physiologically rational treatment for EHPVO than other portosystemic systemic shunts in children. However, about 13.6% of children with EHPVO do not have usable left portal veins and up to 28.1%. Rex operations in children are not successful. Hence, a Rex shunt in these children was impossible. This study reports a novel approach by portal-to-right portal vein bypass for treatment of children with failed Rex shunts., Material and Methods: Eight children (age 6.1years, range 3.5-8.9years) who underwent Rex shunts developed recurrent gastrointestinal bleeding and hypersplenism 13months (11-30months) postoperatively. After ultrasound confirmation of blocked shunt, they underwent exploration. Three patients were found to have right portal vein agenesis. Five patients (62.5%) were found to have the patent right portal vein, with the diameter of 3-6mm. Four patients underwent bypass between the main portal vein in the hepatoduodenal ligament and the right portal vein by interposing an inferior mesenteric vein autograft, whereas the remaining patient underwent a bypass using ileal mesenteric vein autograft., Results: The operations took 2.3h (1.9-3.5h). The estimated blood loss was 50ml (30-80ml), with no complication. The portal venous pressure dropped from 34.6cmH 2 O (28-45 cmH 2 O) before the bypass to 19.6cmH 2 O (14-24cmH 2 O) after the bypass. The 5 patients were followed up for 10.2months (4-17months) and the post-operative ultrasound and CT angiography confirmed the patency of all the grafts and disappearance of the portal venous cavernova in all five patients., Conclusion: The portal-to-right portal vein bypass technique is feasible and safe for treatment of children with EHPVO who have had failed Rex shunts. Our preliminary result indicates that this technique extends the success of Rex shunt from left portal vein to right portal vein and open a new indication of physiological shunt for some of the children who not only have had failed Rex shunts or but also are not suitable for the Rex shunts., Type of Study: Treatment study., Level of Evidence: Level IV., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Background: Congenital portosystemic shunts (CPSS) with intrahepatic portal vein (IHPV) hypoplasia or absence cause encephalopathy or pulmonary hypertension (PH). Acute shunt closure may result in postoperative portal hypertension. The aim of this study was to propose a surgical strategy according to the anatomical types of CPSS and IHPV., Methods: Twenty-three CPSS patients were diagnosed from1990 to 2015. All patients were evaluated by computed tomography, angiography, and PV pressure monitoring under a shunt occlusion test. CPSS were categorized into 5 types according to the anatomical shunt location., Results: The median age at diagnosis was 34months. Three of 23 total patients, who had an extrahepatic portosystemic shunt with a hypoplastic IHPV, died before treatment initiation because of severe PH. Fourteen cases received surgical or interventional treatment at the median age of 5years. A total of 6 cases received surgical therapy, including liver transplants for 2 absent IHPV cases. The remaining 8 cases received interventional coiling. All shunt ligations were successfully accomplished in 1-stage ligation without any complications. After the treatment, the hypoplastic IHPV gradually enlarged with an efficient portal inflow., Conclusion: A precise pretreatment anatomical evaluation of CPSS and IHPV types is mandatory for the selection of surgical treatment., Level of Evidence: Diagnostic study - level II and treatment study - level III., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Background: Hepatoblastoma (HB) is a highly malignant primary liver tumor in children. Although liver transplantation (LT) is an effective treatment for unresectable HB with good long-term outcomes, post-transplant survival is mainly affected by recurrence, despite adjuvant chemotherapy. Novel strategies are needed to improve the outcomes in patients undergoing LT for unresectable HB., Patients and Methods: Twelve children received LT for unresectable HB. In 9 patients, we applied early exclusion of hepatic inflow (hepatic artery and portal vein) and creation of a temporary portocaval shunt during LT., Result: There were differences in the duration of and the blood loss during operation as compared with previously reports. The estimated glomerular filtration rate was well preserved at 3, 6, and 12months and the latest follow-up after LT, and the recurrence-free survival was 88.9%., Conclusion: Early inflow control during LT for unresectable HB may benefit recurrence-free survival by minimizing blood loss and tumor dissemination, preserving renal function and allowing early adjuvant chemotherapy., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Resection is the only curative treatment option for primary and secondary malignant tumors of the liver. Although curative resection is associated with long-term survival rates, it can only be performed in 10% of patients with primary tumors and 25% of patients with liver metastases. Liver insufficiency is one of the most serious postoperative complications of patients undergoing extensive liver resections. When total liver resection is necessary liver transplant is mandatory, with the burden of long-term immunosuppression and its complications. Among several different strategies to increase the resectability of liver tumors, portal vein occlusion (embolization or ligature), bilateral tumor resection in two stages, and resection combined with loco regional therapy are the most popular. A new strategy for patients with marginally resectable liver tumors previously considered to be unresectable was formally reported by Baumgart et al. in 2011, originally developed by Hans Schlitt in 2007. This technique consists of a two-staged hepatectomy with initial portal vein ligation and in situ splitting of the liver parenchyma, and it is known as ALPPS (associating liver partition with portal vein ligation for staged hepatectomy). The aim of this study is to present the first series of pediatric patients with marginally resectable liver tumors previously considered to be unresectable treated with two-stage hepatectomy with initial portal vein ligation and in situ splitting of the liver parenchyma. Two patients were diagnosed with hepatoblastoma, and one each with rhabdomyosarcoma, hepatocellular carcinoma, and nodular focal hyperplasia. ALPPS technique was considered whenever the future liver remnant (FLR) was 40% or less of the total liver volume (TLV) determined by CT or MRI scans. The ratio of FLR to TLV before the first procedure ranged from 0.15 to 0.38, with a mean±sd of 0.253±0.07. In all patients, a rapid growth of the FLR was observed. Estimates of the FRL volume prior to surgical treatment ranged from 110cc to 750cc, with a mean±sd of 361.6±213.75cc. Just before the second procedure, the volume of the remnant liver ranged from 225cc to 910cc, with a mean±sd of 563.6cc±221.7cc. The FRL volume increase had a mean±sd of 72.56%±29.05%, with a median of 83.8%. The second procedure was performed after 7 to 12days with a median of 11days. The only postoperative complication observed in one patient was an asymptomatic right pleural effusion that was aspirated during the second procedure with no further complications. ALPPS was shown to be effective and a safe procedure to treat large tumors in children., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Purpose: The Rex shunt has been employed successfully to treat patients with extrahepatic portal hypertension. In the conventional Rex shunt, the internal jugular vein is used as a venous graft. Inevitably, such a procedure requires neck exploration and sacrifice of the internal jugular vein. The authors describe a novel adaptation of spleen-preserving spleno-Rex bypass, successfully carried out in children with extrahepatic portal hypertension., Methods: The mean age of the four patients (1 boy, 3 girls) was 46 months at the time of operation. All children had a history of upper gastrointestinal bleeding, and suffered from splenomegaly and hypersplenism. Spleen-preserving proximal splenic-left intrahepatic portal shunt was performed in all patients. The splenic artery and vein were ligated at the splenic hilum, and the splenic vein was completely separated from the bed of the pancreas to its junction with the inferior mesenteric vein. The freed splenic vein was anastomosed to left portal vein. The short gastric and left gastroepiploic vessels were kept intact to supply and drain the spleen. All patients were followed-up for 7-33 months (median: 21.5 months)., Results: The spleen-preserving spleno-Rex bypass was successfully performed in all 4 patients. The median operative time was 225 min (range: 215-260 min). One patient received blood transfusion, and the postoperative length of hospital stay varied from 4 to 6 days (median: 4.5 days). Intraoperative portal venous angiography demonstrated the patency of the shunt in all patients. Postoperatively, the complete blood count normalized and the biochemistry tests were within normal range. Postoperative ultrasound confirmed shunt patency and satisfactory flow in the proximal splenic-portal shunt in each patient. The size of the spleen decreased and there was no recurrence of variceal bleeding., Conclusions: The spleen-preserving spleno-Rex bypass is a viable option to treat EHPVO in children., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Background and Purpose: It is controversial whether small size recipient is associated with adverse outcome in liver transplantation. This study aims to evaluate the outcomes of pediatric liver transplantation according to body weight of recipients., Methods: Liver transplant recipients (age <18years, from 1993 to 2011) were studied retrospectively. They were categorized according to the body size at the time of transplantation (A: <6kg; B: between 6kg to 10kg; C: >10kg)., Results: A total of 113 patients (83 LDLTs and 30 DDLTs) were studied. Thirteen (11.5%) belonged to group A, 56 (49.6%) belonged to group B, and 44 (38.9%) belonged to group C. The best graft and patient survivals were found in group A (Figs. 1 and 2), and none of the patients required re-laparotomy for general surgical complications, while 32 patients (32%) in groups B and C did. Regarding transplant-related complications, although group A patients had the highest incidence of biliary tract complications (38.5%, n=5), the incidence of vascular complications (hepatic artery: 7%, portal vein: 0%, hepatic vein: 0%) in this group was the lowest among the three groups., Conclusion: Outcomes of small-sized recipients are not inferior. Less technical-related vascular complications, which may lead to early graft loss, were observed. This could be patient-related (less advanced cirrhosis) or surgeon-related (additional attention paid)., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Abstract: Preduodenal portal vein (PDPV) is a rare congenital anomaly. In most patients, it is associated with other congenital defects including situs inversus, malrotation, and biliary atresia or occurs as part of the heterotaxia syndrome or polysplenia syndrome. We describe a newborn affected by high jejunal atresia, malrotation, and a complex cardiac anomaly, in whom PDPV was diagnosed at early relaparotomy because of stenosis of the jejunal anastomosis. Occurrence of PDPV with intestinal atresia has not been previously reported in the literature. [Copyright &y& Elsevier]

Background: Children with extrahepatic portal hypertension typically present with cavernomatous transformation of the portal vein and a poorly defined intrahepatic portal vein system on conventional imaging. With the Meso-Rex Bypass becoming the gold-standard intervention for a cure, a precise assessment of the intrahepatic portal vein system provides helpful data for deciding whether a Meso-Rex Bypass is feasible or not., Methods: All children with extrahepatic portal hypertension were prospectively assessed by wedged hepatic venous portography. Venous anatomy was categorized into five subtypes (A to E), depending on the presence of thrombosis in the Rex recessus, or not, and its extension within the intrahepatic portal venous system., Results: Eighty-nine children entered the study. Previous umbilical vein catheterization is usually associated with Rex thrombosis, while the Rex recessus and the intrahepatic portal venous system are patent in idiopathic cases, thus allowing for the performance of a Meso-Rex Bypass with a good outcome., Conclusions: Wedged hepatic venous portography is a very effective tool for detailed preoperative assessment and identification of children being considered for Meso-Rex Bypass surgery. An anatomic-radiological classification is useful in selecting patients for Meso-Rex Bypass with anticipation of a high rate of success., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Introduction: Portal vein aneurysms (PVAs) are usually located at the vein trunk or at its bifurcation, rarely intra-hepatic, or at the umbilical portion. Etiology remains unclear., Methods: Three children with PVA were identified over a 5-year period. PVA anatomy was assessed by Doppler Ultrasound, Angio CT/MRI, and trans-jugular retrograde portography., Results: Three children with intrahepatic PVA (including the umbilical portion) were identified during assessment for pre-hepatic portal hypertension: all had splenomegaly and hypersplenism. One presented with massive variceal bleeding. In two cases, a portal vein cavernoma was found, and in the third a severe stricture at the portal bifurcation was observed. Restoration of portal venous flow was achieved by a meso-Rex bypass in two cases and transposing the PV into the Rex in one. High hepatopetal portal flow was restored immediately, with follow-up confirming long-term patency and resolution of signs of portal hypertension with time., Conclusions: These original observations suggest a common initial malformative pattern consisting of a portal venous stricture/web causing a post-stenotic aneurysmal dilatation of the intrahepatic portal branches complicated by thrombosis and cavernomatous transformation of the portal vein trunk. Importantly, the Meso-Rex bypass allows restoring a normal portal flow and cures the portal hypertension., (© 2014.)

Purpose: Children with portal vein cavernous transformation (PVCT) can develop life-threatening variceal hemorrhage from progressive portal hypertension. While spleno-renal shunt ± splenectomy is the most common portosystemic decompression surgery performed in children, we have adopted a modified spleno-adrenal (SA) shunt for complicated PVCT. We describe our 10 year experience focusing on technique evolution and treatment efficacy., Methods: Between 2001 and 2011, 15 children (9 girls and 6 boys, ages 3-11 years, median: 6 years) with PVCT, portal hypertension, and hypersplenism were treated with SA shunt with splenectomy in Shanghai Children's Medical Center. All children in the study had endoscopy proven active esophageal variceal bleeding requiring multiple transfusions (mean: 4.2 units) with failed sclerotherapy (mean: 2.6 times). Greater omental vein pressure (GVP) approximating portal venous pressure was measured pre- and post-SA shunt. Pre- and post-operative ammonia levels were obtained. Follow-up ranged from 6 months to 10 years (mean: 4.2 ± 2 years)., Results: Intra-operative adrenal vein diameter and length ranged from 0.7 to 1.8 cm and 2 to 3 cm, respectively. Intra-operative GVPs pre-and post-SA shunt were (30 ± 11) and (22 ± 7) mmHg, respectively (p<0.01). On follow-up, there have been no recurrences of GI bleeding. Liver function tests remained normal in all children with the exception of elevated post-operative mean blood ammonia levels [Pre (18 ± 7) mmol/L, post (60 ± 17) mmol/L (p<0.05)] in all children. Ammonia levels normalized in all cases on outpatient follow-up. There have been no cases of hepatic encephalopathy, and all have normal age appropriate neurodevelopment (Bayley's assessment). Barium swallow and/or upper endoscopy showed interval resolution of esophageal varices in all children, and vascular ultrasound showed patent shunt anastomosis without stricture in 14 (93%)., Conclusions: The left adrenal vein is a viable conduit for effective selective portosystemic decompression. Similar to the more traditional spleno-renal shunt, SA appears also to have the advantage of preventing hepatic encephalopathy preserving neurodevelopment, although the rise in post-operative ammonia levels was unexpected. Longer follow-up is needed to look for late signs of encephalopathy assessing neurodevelopment long term., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Extrahepatic portal vein (PV) obstruction (EHPVO) is a rare disorder in early childhood with unknown incidence and mostly unknown etiology. In children with EHPVO, the hepatopedal flow of the mesenteric venous blood is hindered by an obstruction of the PV resulting in prehepatic portal hypertension. The curative treatment with a meso-Rex shunt connects the superior mesenteric vein to the left PV by a venous autograft. Despite good primary patency, reocclusion of a meso-Rex bypass and its treatment can be challenging. We present 2 patients with EHPVO with subtotal functional occlusion of a meso-Rex shunt treated by percutaneous interventions with short- and mid-term follow-up., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Background: The internal jugular vein is routinely used as a graft for the Rex shunt. We analyzed results of mesoportal bypass using an alternative autologous graft., Methods: Twenty-one children with extrahepatic portal vein obstruction were treated with a Rex shunt constructed using both greater saphenous veins. Follow-up included ultrasound and blood count screening at 3, 6, and 12 months and annually thereafter., Results: Median age was 8.7 years (range, 3.6-14 years), and follow-up time, 5.3 years (range, 0.6-7.1 years). Occlusion or narrowing occurred in 6 patients after a median of 20 months (range, 2.6-52 months). In 2 cases, patency was restored, giving an overall success rate of 81%. During follow-up, no variceal bleeding occurred while hemoglobin, platelet count, and leukocyte levels increased (P ≤ .02 for all) and spleen size decreased (P = .001). Patients with occlusive shunt complications weighed less (P = .01), had higher preoperative platelet levels (P = .02), and tended to have a smaller spleen preoperatively (P = .06) than patients without shunt complications. Cumulative graft patency at 6 months, 1 year, 3 years, and 5 years was 100%, 89%, 82%, and 74%, respectively., Conclusions: Rex shunt constructed using the greater saphenous veins is a valuable alternative to the internal jugular vein graft, allowing long-term reversal of portal hypertension, splenomegaly, and hypersplenism. Low patient weight and high platelet count predicted shunt occlusion., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Congenital intrahepatic portosystemic shunts are rare hepatic vascular anomalies that often lead to severe secondary conditions. A 6-year-old boy was referred for assessment of severe hypoxia, and a large liver mass was diagnosed with such a malformation and was managed by direct closure of the venous fistula by interventional radiology. Follow-up assessment shows normalization of the respiratory condition and a progressive reduction of the vascular liver lesion. Review of literature suggests that radiologic interventions are currently the criterion standard for managing these conditions, with surgery reserved for patients who are not eligible for radiologic procedure and those requiring liver transplantation., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Purpose: Extrahepatic portal venous obstruction is the most common cause of portal hypertension in children. The Rex shunt has been used successfully to treat patients with extrahepatic portal hypertension. In the conventional Rex shunt, the internal jugular vein is used as a venous graft. Inevitably, such a procedure requires neck exploration and sacrifice of internal jugular vein. The authors describe a novel adaptation of gastroportal shunt, successfully carried out in 8 children with extrahepatic portal hypertension., Methods: The mean age of the 8 patients (6 boys and 2 girls) was 66.6 months at the time of operation. All children had portal hypertension. Seven had a history of upper gastrointestinal bleeding, and 4 had splenomegaly and hypersplenism. Gastroportal shunt was performed in all patients. The left gastric vein was mobilized and anastomosed to left portal vein. In 1 patient, the left gastric vein was not of adequate length and required a venous graft (the inferior mesenteric vein). All patients were followed up for 3 to 20 months (median, 9 months)., Results: The gastroportal shunt was successfully performed in all patients. The median operative time was 265 minutes (range, 205-360 minutes). Operative blood loss was 21 ± 7.4 mL, and the length of hospital stay varied from 9 to 19 days (median, 15 days). Intraoperative portal venous angiography demonstrated the patency of the shunt in all patients. Postoperatively, the complete blood count normalized, and the biochemistry tests were within reference range. Postoperative ultrasound confirmed shunt patency and satisfactory flow in the gastroportal shunt in each patient. The size of spleen decreased. There was no recurrence of variceal bleeding., Conclusions: The gastroportal shunt is an effective treatment of extrahepatic portal hypertension., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Children with extrahepatic portal vein obstruction can be managed successfully by surgical intervention and should be evaluated for potential meso-Rex bypass. A Rex shunt variation is described to treat portal and superior mesenteric vein thrombosis. This technique uses the internal jugular vein as a conduit between the splenic vein and the left portal vein with splenic preservation., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Background: Congenital portosystemic shunts (PSS) with preserved intrahepatic portal flow (type II) present with a range of clinical signs. The indications for and benefits of repair of PSS remain incompletely understood. A more comprehensive classification may also benefit comparative analyses from different institutions., Methods: All children treated at our institution for type II congenital PSS from 1999 through 2009 were reviewed for presentation, treatment, and outcome., Results: Ten children (7 boys) with type II PSS were identified at a median age of 5.5 years. Hyperammonemia with varying degrees of neurocognitive dysfunction occurred in 80%. The shunt arose from a branch of the portal vein (type IIa; n = 2), from the main portal vein (type IIb; n = 7), or from a splenic or mesenteric vein (type IIc; n = 1). Management included operative ligation (n = 6), endovascular occlusion (n = 3), or a combined approach (n = 1). Shunt occlusion was successful in all cases. Serum ammonia decreased from 130 ± 115 μmol/L preoperatively to 31 ± 15 μmol/L postoperatively (P = .03). Additional benefits included resolution of neurocognitive dysfunction (n = 3), liver nodules (n = 1), and vaginal bleeding (n = 1)., Conclusion: Correction of type II PSS relieves a wide array of symptoms. Surgery is indicated for patients with clinically significant shunting. A refined classification system will permit future comparison of patients with similar physiology., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Background: Extrahepatic portal vein thrombosis (EHPVT) is an important cause of portal hypertension in children. Rex shunt has been used successfully to treat these patients., Methods: We report our experience in 19 infants and children (5 months to 14 years) with HPVT eligible for a mesenteric-portal surgical shunt with left internal jugular vein autograft. Eight children had idiopathic EHPVT, nine had post-umbilical catheterization EHPVT, one had portal vein agenesis, and one had posttransplant EHPVT., Results: It was possible to perform the Rex shunt in all patients except for 8 of 9 cases in the post-umbilical catheterization EHPVT group. A Warren procedure was performed in 4 of those patients and a proximal splenorenal shunt in 1. Current follow-up ranges from 3 to 26 months. Shunt thrombosis occurred in one patient with portal vein agenesis and associated cardiac anomaly. Portal hypertension has significantly improved after surgery. None of our patients have experienced new bleeding episodes until now., Conclusions: The Rex shunt should be considered in the treatment of children with idiopathic EHPVT experiencing repeated gastrointestinal bleeding episodes refractory to endoscopic treatment. Nevertheless, the role of this operation for children with post-umbilical catheterization EHPVT is yet to be clearly evaluated., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Unlabelled: Meso-Rex shunt (MRS) can relieve portal hypertension and restore a physiological portal flow in patients with portal vein thrombosis. We describe a technical variant where the autologous internal jugular vein (IJV) was too short to bridge the superior mesenteric vein (SMV) and the Rex recessus., Patient: A 15-year-old boy with portal cavernoma had several episodes of gastrointestinal bleeding despite repeated sclerotherapy. Preoperative assessment, including retrograde transjugular portography, showed persistent esophageal and gastric varices, severe hypertensive gastropathy, obstructed portal vein, patent SMV and splenomesenteric confluence, patent intrahepatic portal branches, and normal transhepatic pressure gradient. An MRS was planned. The left IJV was retrieved from its infracranial part to its confluence with subclavian vein. After performing the Rex recessus to IJV graft anastomosis, the IJV graft proved to be too short for classical end-to-side anastomosis onto the SMV. After clamp testing showing good tolerance of the small bowel, the proximal jejunal branches of the SMV were tied, the proximal SMV was mobilized and transsected 4 cm below the pancreas, and an end-to-end anastomosis between SMV and IJV was performed. Portal pressure decreased from 23 to 13 mm Hg, and intraoperative Ultra Sound Doppler (US Doppler) showed good flows in the shunt. Postoperative course was uneventful, and 1 year after surgery, the child is clinically well, off medication, with a patent shunt, and no portal hypertension., Conclusion: This modified MRS technique may be useful when the autologous IJV graft is too short, avoiding the need for prosthetic conduits and prolonged postoperative anticoagulation.

Background: Extrahepatic portal vein obstruction (EHPVO) has been associated with growth impairment in children. We hypothesized that growth parameters improve after reversal of portal hypertension and restoration of mesenteric venous blood flow to the liver by the mesenterico-left portal vein bypass (MLPVB)., Methods: A retrospective review of 45 children with idiopathic EHPVO who underwent MLPVB between 1997 and 2007 and had follow-up data for analysis was carried out. Growth was assessed using SD scores (z scores) for height, weight, and body mass index (BMI) at the time of operation and at early (5-12 months) and late (13-24 months) follow-up., Results: The mean height and weight of children with EHPVO was significantly lower than the general population before surgery. Mean BMI was also lower, although statistically insignificant. All parameters increased significantly after MLPVB as follows: height from -0.42 before surgery to -0.12 (P = .027) at 5 to 12 months and -0.14 (P = .026) at 13 to 24 months; weight from -0.49 before surgery to 0.03 (P < .001) at 5 to 12 months and 0.35 (P < .001) at 13 to 24 months; and BMI from -0.22 before surgery to 0.17 (P = .001) at 5 to 12 months and 0.48 (P < .001) at 13 to 24 months., Conclusion: Restoration of portal blood flow to the liver by MLPVB improves growth in children with EHPVO.

Background: In children with extrahepatic portal vein obstruction (EHPVO), formation of a mesentericoportal bypass (Rex shunt) restores hepatopetal flow, relieves portal hypertension, and reduces variceal bleeding and hypersplenism. The Rex shunt is created by inserting a vein graft between the superior mesenteric vein and the umbilical segment (Rex) of the left portal vein within the Rex recess of the liver. The preoperative evaluation of a patient with EHPVO includes an accurate assessment of the venous inflow and outflow. The inflow portal vein is readily assessed by ultrasound and magnetic resonance imaging. The outflow intrahepatic portal vein is harder to assess. We report our experience of patients evaluated with wedged hepatic vein carbon dioxide portography (WHVCP)., Method: All children referred for venography from October 2001 to October 2007 were prospectively identified, and clinical and radiologic data were reviewed retrospectively. The imaging findings were correlated to findings at surgery., Results: Eleven children (range, 3-14 years, median, 6 years) were referred for preoperative wedged hepatic venography. The left portal vein at the Rex recess was clearly identified in 9 patients (82%). In the other 2 patients (18%), the Rex segment was not identified despite opacification of left and right intrahepatic portal veins; this was taken to indicate an occluded segment. Wedged venography was performed with carbon dioxide in 10 patients (91%). Carbon dioxide was contraindicated in the final patient because of the presence of a ventricular septal defect., Conclusion: Our series demonstrates the use of WHVCP as a diagnostic tool in preoperative assessment of the Rex segment of left portal vein in children with extrahepatic portal vein obstruction.

We describe a child who presented with a traumatic hepatic artery pseudoaneurysm and arterioportal fistula, which were subsequently managed with an endovascular stent graft and coil embolization using flow control with balloon remodeling. This case demonstrates a rarely seen condition in the pediatric population and a novel management strategy, which should be considered in the management of this complex injury.

Intrahepatic arterioportal fistula (IAPF) is a rare cause of portal hypertension in young children. We report the case of a 2-year-old girl with severe undernutrition, chronic watery diarrhea, and gastrointestinal bleeding because of a congenital intrahepatic arterioportal fistula. Radiographic embolization and surgical ligation of the left hepatic artery were attempted, with no resolution of the symptoms. So, a left lobectomy was performed, with excellent results and prompt disappearance of the diarrhea. Hepatectomy should be considered as a definitive and reliable therapy for congenital IAPF.

Congenital hepatoportal arteriovenous fistula is a rare cause of portal hypertension in young children. Unlike the acquired form, which is usually isolated and can be cured by hepatic artery (HA) embolization, recurrence of portal hypertension often occurs with congenital hepatoportal arteriovenous fistula after embolization and/or HA ligation because of early, rapid collateralization and the presence of multiple arterioportal fistula. Although long-term outcome after embolization is not known, liver transplantation has been proposed as the only option for this condition. However, portal vein and hepatic arterial anastomoses are made difficult because of the presence of portal vein arterialization and previous HA ligation, with a significantly increased risk of vascular complications. We report a case where resolution of portal hypertension has been achieved by an end-to-side portocaval shunt, to preserve the portal vein and HA for future liver transplantation, should it be required.

This report describes a case of Abernethy malformation associated with hepatopulmonary syndrome, which was resolved after shunt ligation. The clinical course indicated that hepatopulmonary syndrome can develop in Abernethy malformation in which liver function and portal pressure is normal, and liver transplantation is not the exclusive therapy for hepatopulmonary syndrome. The levels of endotoxin and endothelin 1 in the shunt blood were high, whereas those of tumor necrosis factor alpha and interleukin-1beta were within reference range. Although pathogenesis of hepatopulmonary syndrome remains unknown, the findings in this case suggest that bacterial translocation as well as elevated endothelin 1 may play a causal role in development of hepatopulmonary syndrome.

Purpose: The mesenteric to left portal vein bypass (MLPVB) has been successfully used to treat extrahepatic portal vein obstruction (EHPVO) in children. We examined the effect of failed prior surgical or radiological procedures intended to treat complications of portal hypertension on the success rate of subsequent MLPVB surgery., Methods: Sixty-two patients younger than 18 years with EHPVO underwent MLPVB between 1997 and 2006. Children were divided into 3 groups: those with no prior surgery related to portal hypertension, those with prior portosystemic shunts, and those with either splenectomy or mesenteric vascular embolization procedures. The effect of prior procedures on the patency rate of the MLPVB was then examined., Results: Of 62 children, 11 (17.7%) had significant procedures to treat symptoms of portal hypertension: 6 had at least 1 portosystemic shunt attempt, 3 had isolated splenectomy, and 2 had embolization of the splenic artery or coronary and peripancreatic varices. Patients with previous portal hypertension surgery were significantly older and larger than those with no surgery. Patients with no prior interventions had a significantly higher MLPVB patency rate (88.2%, 45/51) than those with no prior interventions (63.6%, 7/11). Prior splenectomy alone was not found to adversely affect MLPVB. Patients with prior embolization procedures or unsuccessful shunts had significantly poorer successful outcomes (0% and 66.7%) than those with no prior interventions (88.2%; P < .005)., Conclusions: The results demonstrate that prior portosystemic shunts or mesenteric embolizations have a deleterious effect on outcome after MLPVB and should be avoided whenever possible. This study suggests that patients with symptomatic EHPVO should undergo MLPVB as a primary intervention rather than as a rescue procedure to optimize MLPVB patency.

A giant paraesophageal hiatal hernia with preduodenal portal vein, nonrotating gut, intrathoracic spleen, and left inferior vena cava has not been reported to date. This set of complex anomalies can have significant clinical implications. Awareness of these anomalies is essential to avoid further complications.

The meso-Rex bypass procedure has been used to treat patients with portal hypertension from extrahepatic portal vein obstruction. This report describes modifications of this procedure in 5 patients. Either the splenic or coronary vein was used as the venous inflow point, and the bypass was performed either directly through transposition of the vein or with the use of a venous conduit.

The authors describe a case of extrahepatic portal vein (EHPV) thrombosis and portal hypertension treated with a variant of mesenterico-left portal vein bypass (MLPVB) or Rex shunt. In this case, a segment of autogenous greater saphenous vein was used to bridge the distance between the left gastric vein inflow and the left portal vein. Use of such nontraditional conduit in similar circumstances may expand the application of portal revascularization/decompression procedures in treating these patients.

An 18-day-old female neonate presented with abdominal distention and bilious vomiting for 5 days. Abdominal examination showed hepatomegaly and a mass in the right hypochondrium. Ultrasound showed an extrahepatic cyst with internal echoes and dilated intrahepatic radicals. A contrast computer tomography showed a large cyst arising from the falciform ligament, extending into the liver parenchyma and a dilated portal venous system. Diagnosis of falciform ligament abscess with portal pyemia was made. Excision of the falciform ligament was done. After a follow up of 1 year, the child remains asymptomatic. To our knowledge, a falciform ligament abscess causing intrahepatic portal pyemia in a newborn has not been previously been described.

Background/purpose: Portosystemic shunt operations are indicated in patients with extrahepatic portal hypertension owing to portal vein thrombosis (EPH-PVT) suffering from recurrent variceal bleeding despite endoscopic sclerotherapy. Mesenterico left portal bypass procedure (MLPB) is an alternative procedure to the portosystemic shunt operations in patients with EPH-PVT. MLPB operation reestablishes hepatopetal portal blood flow. We herein present our experience with MLPB in children with EPH-PVT., Methods: Six patients were treated for EPH-PVT with recurrent bleeding despite endoscopic sclerotherapy (2 boys and 4 girls) in our unit. All patients were evaluated preoperatively with complete blood count, portal duplex system Doppler ultrasonography, magnetic resonance angiography, and upper gastrointestinal (GI) endoscopy. MLPB operation was performed as described by de Ville de Goyet. During the postoperative period, patients were evaluated with complete blood count, portal duplex system Doppler ultrasonography, upper GI endoscopy, and magnetic resonance angiography., Results: Six patients were assessed to be candidates for MLPB procedure and were operated to perform the MLPB procedure. Left portal veins were found to be patent during the operation in 4 patients, and the MLPB procedure was performed. Internal jugular vein was used in 3 patients and enlarged inferior mesenteric vein in 1 patient. Left portal veins of the remaining 2 patients were found to be obliterated; therefore, mesocaval shunt was performed. The postoperative course of the patients was uneventful except for 1 patient. During the following period, the leukocyte and the platelet counts were significantly increased in 3 of the 4 patients after the MLPB procedure. Upper GI bleeding occurred in the early postoperative period in 1 patient with MLPB procedure because of prepyloric ulcer that was successfully treated by endoscopic sclerotherapy. Internal jugular vein graft thrombosis was detected on the 10th postoperative day. This patient underwent a second laparotomy, the distal half of the graft was found to be sclerosed and narrowed that the graft was revised with a synthetic allograft., Conclusions: Based on a review of the literature, the MLPB functions well in patients with portal hypertension caused by portal vein thrombosis and appears to have a physiologic advance over shunts that decompress but do not return blood directly to the liver. Because intra-abdominal veins appear to function well as a conduit in this operation, it may be favored by eliminating additional incision and increased risk in such patients.

Surgically created portosystemic shunts can alleviate the symptoms of bleeding from gastric and esophageal varices and improve the hematologic consequences of hypersplenism in patients with portal hypertension. However, the diversion of mesenteric venous blood away from the liver can result in encephalopathy. In this report, we describe a case in which encephalopathy caused by a proximal splenorenal shunt was reversed by the restoration of portal flow to the liver by a mesenteric-to-left portal vein bypass operation.

A 3-year-old boy had pancreatoblastoma arising in the pancreatic head and body, with metastasis in the mesenteric lymph nodes, tumor thrombus in the portal vein, and a small metastatic lesion in the left lung. After preoperative chemotherapy, aggressive surgical treatment including resection and reconstruction of the portal and superior mesenteric veins was performed. Postoperative chemotherapy was followed by high-dose chemotherapy and rescue with peripheral blood stem cell transplantation, and he has now been well for 6 years. This is the first surviving pediatric case of advanced pancreatoblastoma treated by aggressive surgical therapy including reconstruction of the portal vein and high-dose chemotherapy followed by peripheral blood stem cell transplantation.

Background: Children with portal venous thrombosis often have severe symptoms secondary to portal hypertension including recurrent upper gastrointestinal (UGI) bleeds and hypersplenism. We report results of the use of the mesenterico-left portal bypass (Rex shunt) in 5 consecutive patients., Methods: A retrospective chart review of all patients with portal venous thrombosis who underwent Rex shunt procedure was performed. Children were evaluated preoperatively with magnetic resonance angiogram, Doppler ultrasound, and mesenteric angiogram. Postoperative ultrasounds were performed in follow-up., Results: All patients had evidence of portal hypertension and hypersplenism. The average age of the patients was 13.2 +/- 4.9 (7-19) years. The patient had an average of 2.6 +/- 1.7 UGI bleeds requiring banding and 3.4 +/- 4.2 U of blood transfused before undergoing the shunt. The mean operative time was 383 +/- 46 minutes, and length of stay was 10.4 +/- 7.1 days. In follow-up of 18.8 +/- 5.2 months (11-24 months), all but 1 patient had a patent shunt. One narrowed shunt was successfully dilated by percutaneous angioplasty. Thrombocytopenia improved significantly in patients with functioning shunts but did not correlate with a significant decrease in splenic size., Conclusions: The Rex shunt reestablishes normal hepatopedal portal flow, and in patients with functioning shunts, no recurrent UGI bleeds or transfusions were required or evidence of encephalopathy were noted.

Two cases of living-donor liver transplantation performed in patients with situs inversus are reported. The authors discuss the operative management for a situs inversus recipient to undergo liver transplantation.

Background/purpose: The effect of portal flow deprivation to the liver on bile composition and the biliary system remains undefined in children. This report catalogues the authors' experience with biliary tract problems in children with extrahepatic portal vein thrombosis (EHPVT)., Methods: Twenty-nine children with symptomatic idiopathic EHPVT were evaluated for the Rex shunt procedure (mesenterico-left portal bypass) over a 4-year period. The authors retrospectively reviewed all operative reports and pre- and postoperative abdominal ultrasound findings with regard to associated congenital anomalies and abnormal biliary tract findings., Results: Seven of the 29 patients with EHPVT (24%) had associated nonbiliary congenital abnormalities. Twenty-four of 29 (83%) patients had detectable biliary tract pathology by ultrasound examination. Biliary symptoms developed in 3 of the 9 (33%) patients with either stones or sludge (10.3% of all patients). Two patients were treated by cholecystectomy. There was no statistical correlation between biliary tract pathology and the age of presentation, symptoms of portal hypertension, gender, or underlying medical condition., Conclusions: The authors have noted a high incidence of biliary tract pathology in patients with EHPVT compared with the normal population and a 10% incidence of symptomatic biliary pathology in this series.