Surgical Treatment of Portal Hypertension in Children.

Introduction: Portal hypertension is a syndrome characterized by increased pressure in the portal vein system and can be caused by impaired blood flow in the portal vein, hepatic veins, or inferior vena cava. The main complications of this condition are bleeding from varicose veins of the esophagus (in our study in 100% of patients), splenomegaly with hypersplenism (in our study in 98% of patients), ascites (in our study in 1 patient). The main goal of treating portal hypertension is to prevent bleeding from esophageal varices. However, today the goal of surgical treatment of portal hypertension in children is not only to prevent the development of bleeding but also the possible restoration of intrahepatic blood flow. Materials and Methods: A retrospective analysis of the results of treatment of portal hypertension in 75 children (41 boys, 34 girls) operated in our Center for the period from 2019 to 2022 was carried out. The mean age of the patients was 7 ± 1 years. Sixty-nine patients had an extrahepatic form of portal hypertension, and 6 patients had an intrahepatic form (liver fibrosis). In 14 patients (18.6%), the operation was repeated (a vascular shunt was previously applied in another hospital; 4 children were operated on repeatedly). Results: A good result was obtained in all children, and the risk of bleeding from varicose veins of the esophagus was eliminated. Vascular bypass surgery was performed in all cases: mesoportal bypass in 17 (22.7%) patients, splenorenal bypass in 37 (49.3%) patients, mesocaval bypass in 21 (28%) patients. In 10 (13%) cases, repeated bypass surgery was required due to dysfunction or thrombosis of the previously performed bypass. In 14 (18.6%) patients with mesoportal shunts, blood flow in the liver was completely restored. Conclusions: The main method of surgical treatment of portal hypertension today is portosystemic bypass surgery, which effectively prevents bleeding from varicose veins of the esophagus. Mesoportal shunting is a definitive treatment for extrahepatic portal hypertension that restores portal perfusion of the liver.

The use of autologous peritoneum in surgery of portal hypertension: H-shape splenorenal shunt using simple layer peritoneal tube.

The management of portal hypertension complicated by iterative gastro-intestinal bleeding remains challenging, especially in a low-income environment. Interventional radiology and endoscopic treatments are not always accessible, and a definitive surgical option may prove to be lifesaving. We report a new technique of surgical portosystemic shunt that can be performed in all contexts. We describe the surgical technique of a H-shaped splenorenal shunt using autologous rolled up peritoneum as a vascular graft.

Functional side-to-side splenorenal shunts to treat extrahepatic portal vein thrombosis in children.

BACKGROUND: Surgical shunts are commonly used to manage complications resulting from extrahepatic portal vein thrombosis (EHPVT) in children. We describe a single-center experience utilizing a functional Side-to-Side Splenorenal Shunt (fSRS), created using either an enlarged inferior mesenteric vein (IMV) or left adrenal vein (LAV). METHODS: Pediatric patients with isolated EHPVT who were poor candidates for a Rex shunt and who underwent a fSRS procedure at our institution between 2003 and 2020 were reviewed. The pre/post shunt portosystemic gradient change, rates of early and late complications, postoperative shunt patency, and mortality were evaluated. RESULTS: Twelve EHPVT patients (mean age of 6.1 years) underwent a fSRS procedure. The mean portosystemic gradient change for the cohort was -11.7 mmHg (±4.9). There were no cases of recurrent variceal bleeding or episodes of shunt thrombosis reported after fSRS procedures. CONCLUSIONS: Surgical shunts continue to be an important adjunct in the treatment of complications related to EHPVT. The functional Side-to-Side Splenorenal Shunt is a safe alternative that is easy to perform, involves minimal dissection and requires only a single anastomosis.

Optimal Rex shunt procedures as a treatment for pediatric extrahepatic portal hypertension.

PURPOSE: To assess the long-term results after Rex bypass (RB) shunt and Rex transposition (RT) shunt and determine the optimal approach. METHODS: Between 2010 and 2019, traditional RB shunt was performed in 24 patients, and modified RT shunt was performed in 23 children with extrahepatic portal hypertension (pHTN). A retrospective study was conducted based on comparative symptoms, platelet counts, color Doppler ultrasonography and computed tomographic portography of the portal system, and gastroscopic gastroesophageal varices postoperatively. The portal venous pressure was evaluated intraoperatively. RESULTS: The operation in the RB group was notably more time-consuming than that in the RT group (P < 0.05). Compared to RT shunt, the reduction in gastroesophageal varix grading, the increases in platelets, and the caliber of the bypass were greater in the RB group (P < 0.05). Although not statistically significant, higher morbidity of surgical complications was found after RT shunt (17.4%) compared with RB shunt (8.3%) with patency rates of 82.6 and 91.7%, respectively. Additionally, patients exhibited a lower rate of rebleeding under the RB procedure (12.5%) than under the RT procedure (21.7%). CONCLUSIONS: The RT procedure is an alternative option for the treatment of pediatric extrahepatic pHTN, and RB shunt is the preferred procedure in our center.

Surgical shunts for extrahepatic portal vein obstruction in pediatric patients: a systematic review.

BACKGROUND: Extrahepatic portal vein obstruction (EHPVO) causes portal hypertension in noncirrhotic children. Among surgical treatments, it is unclear whether the meso-Rex shunt (MRS) or portosystemic shunt (PSS) offers lower post-operative morbidity and superior patency over time. Our objective was to evaluate long-term outcomes comparing MRS and PSS for pediatric patients with EHPVO. METHODS: A systematic review was conducted of articles reporting children undergoing surgical shunts for EHPVO from 1/2000-2/2020. Of 87 articles screened, 22 were eligible for inclusion. The primary outcome was shunt thrombosis and secondary outcomes included non-operative complications, stenosis, and re-operation. RESULTS: Eighteen of 22 studies were of good quality and four had fair quality. Of 461 patients included, 340 underwent MRS and 121 underwent PSS. MRS were associated with a higher rate of post-operative thrombosis when compared to PSS (14.1% vs 5.8%, p = 0.021). There were 40/340 MRS patients (11.8%) that required at least one re-operation for either shunt thrombosis or stenosis, versus 5/121 PSS patients (4.1%), p = 0.019. CONCLUSION: Both MRS and PSS result in acceptable long-term patency rates, but the more technically demanding MRS is associated with higher post-shunt thrombosis, often requiring further operative intervention. This study suggests that PSS may offer advantages for pediatric patients with EHPVO.

Malignant Peritoneal Mesothelioma Arising in Young Adults With Long-standing Indwelling Intra-abdominal Shunt Catheters.

Only 50% to 70% of patients with mesothelioma report asbestos exposure. Other exposures (eg, radiation) play a role in some cases, but some patients have no obvious cause. We describe a series of patients with long-standing indwelling intra-abdominal shunt catheters who developed malignant peritoneal mesothelioma, suggesting a novel association. We identified 7 patients who had shunts and subsequently developed mesothelioma (5 women; median age: 31 y, range: 18 to 45 y). Clinical history and pathology materials were reviewed, and RNA sequencing was performed. Clinical presentations varied; 6 patients had hydrocephalus and a ventriculoperitoneal shunt, and 1 patient had portal hypertension and a portoatrial shunt. The median duration of shunt therapy in 5 cases was 29 years (range: 12 to 35 y); the remaining 2 patients also had shunts for many years, but specific details were unavailable. Two patients had radiotherapy for malignancies in childhood. One had an alleged exposure to asbestos and 1 had prior exposure to talc. The rest had no known risk factors. Histologically, all tumors were purely epithelioid. Treatments included surgical debulking, chemotherapy, and palliative care. All 7 died of disease (median survival: 7 mo, range: 1 to 18 mo). Molecular testing showed loss of NF2 and CDKN2A/B and a BAP1 mutation in 1 case, and no genomic alterations associated with mesothelioma in 2 cases. Peritoneal mesothelioma may represent a complication of long-standing indwelling shunt catheters. The mechanism is unknown, but chronic peritoneal irritation may play a role. Albeit rare, mesothelioma should be considered in patients with a shunt who present with new ascites.

Residual Pulmonary Hypertension more than 20 Years after Repair of Shunt Lesions.

Background and Objectives: After successful surgical repair of a congenital shunt lesion, pulmonary hypertension (PH) often disappears. However, PH can persist long-term after the closure. This study aimed to assess the prevalence of PH long-term after surgical repair of congenital heart disease (CHD), and to evaluate the outcomes and preoperative factors related to residual PH. Materials and Methods: In this retrospective cohort study, we reviewed patients who underwent right heart catheterisation in Vilnius University Hospital Santaros Klinikos during the period of 1985-2007. Among 4118 right heart catheterisations performed, 160 patients underwent congenital systemic-to-pulmonary shunt repair at a young age (<18 years) and had pre-operative PH. Half of the patients were foreigners whose follow-up data were unavailable. Eventually, 88 patients with available follow-up data were included in this study. Results: The median age at diagnosis of CHD with PH was 0.8 (0.6-3.0) and 1.1 (0.6-3.9) years at surgery (50% females). Residual PH was assessed 9.5 years after surgery and observed in 30.7% (n = 27) of the patients. It was associated with having more than one shunt (44.4% (n = 12), p = 0.016) and higher median pulmonary vascular resistance (3.4 (2.5-6.5) vs. 2.2 (1.0-3.7), p = 0.035) at baseline. After a median follow-up of 21 (15-24) years, 9.1% of the patients were deceased. Kaplan-Meier survival analysis revealed significantly higher mortality in the residual PH group (p = 0.035). Conclusions: Residual PH affects a significant proportion of patients after surgical repair of a shunt lesion and is associated with worse long-term outcome.

Portosystemic Shunt Leading to Encephalopathy in a Non-Cirrhotic Patient.

While portal-systemic encephalopathy is a common entity in cirrhotic patients, it is less frequent in non-cirrhotic patients. We are reporting a case of a 68-year-old female who presented with unresponsiveness for the second time in six months. She underwent extensive evaluation for liver disease with ultrasonography and computerized tomography of the abdomen, testing for causes of liver disease including testing for viral hepatitis was negative. A liver biopsy was done clearing any doubt about the presence of significant liver disease or clinically significant portal hypertension. With absence of liver disease hence lower likelihood of portal-systemic encephalopathy (PSE) we evaluated for other causes of encephalopathy with unremarkable neuroimaging including brain MRI and head CT, unremarkable CSF analysis and EEG showing no seizure activity. Given the negative workup and the high ammonia level with the significant clinical response to ammonia lowering therapy we further evaluated the patient for other causes of PSE with Doppler ultrasonography of the liver and eventually angiography of the portal system with the high suspicion for a portosystemic shunt as a cause of her encephalopathy. A shunt from the inferior mesenteric vein to the left renal vein was diagnosed and successfully occluded utilizing coil embolization. The patient recovered normal mentation and was eventually discharged home. This case sheds light on the importance of diagnosing portosystemic shunts leading to encephalopathy, as occlusion of the shunt can correct the encephalopathy and help prevent further episodes.

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

BACKGROUND: Portal 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. METHODS: All patients who underwent surgical shunt at a single institution from 2008 to 2017 were reviewed. The primary outcome was intervention-free shunt patency. RESULTS: In 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. CONCLUSION: Children 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.

The efficacy of surgical shunts to treat severe portal hypertension after a Kasai procedure for biliary atresia.

BACKGROUND: To assess the outcome of patients with biliary atresia (BA) who underwent a surgical shunt (SS) for severe portal hypertension (PH) following a Kasai procedure. METHODS: We collected and analyzed the data and outcomes of patients with BA who underwent SS for severe PH following a Kasai procedure between 1974 and 2014, focusing on complications related to the procedure, overall survival (OS), and transplant-free survival (TFS). RESULTS: SS was performed at a median age of 5.5 years [2-13.5] in 38 patients. Conjugated bilirubin level (cBL) was ≤20 µmol/l in 24 patients at time of SS. Median follow-up was 15 years [1-32]. OS at 5 and 10 years was 91% and 87% respectively. TFS at 5 and 10 years was 84% and 70% respectively. Long-term complications included hepatic encephalopathy in 9 patients, and hepatopulmonary syndrome in 3. At last follow-up, 10/14 patients without LT and 18/ 24 who had a delayed LT at a median delay of 11 years [1.5-22] were alive. CONCLUSION: Surgical shunt for severe portal hypertension in biliary atresia may delay the need for liver transplantation. However complications are indications for transplantation. LEVEL OF EVIDENCE: Type of study: Therapeutic. Level of evidence III.

Surgical portosystemic shunts versus transjugular intrahepatic portosystemic shunt for variceal haemorrhage in people with cirrhosis.

BACKGROUND: Variceal haemorrhage that is refractory or recurs after pharmacologic and endoscopic therapy requires a portal decompression shunt (either surgical shunts or radiologic shunt, transjugular intrahepatic portosystemic shunt (TIPS)). TIPS has become the shunt of choice; however, is it the preferred option? This review assesses evidence for the comparisons of surgical portosystemic shunts versus TIPS for variceal haemorrhage in people with cirrhotic portal hypertension. OBJECTIVES: To assess the benefits and harms of surgical portosystemic shunts versus transjugular intrahepatic portosystemic shunt (TIPS) for treatment of refractory or recurrent variceal haemorrhage in people with cirrhotic portal hypertension. SEARCH METHODS: We searched the Cochrane Hepato-Biliary Group Controlled Trials Register, CENTRAL, MEDLINE, Embase, LILACS, Science Citation Index Expanded, and Conference Proceedings Citation Index - Science. We also searched on-line trial registries, reference lists of relevant articles, and proceedings of relevant associations for trials that met the inclusion criteria for this review (date of search 8 March 2018). SELECTION CRITERIA: Randomised clinical trials comparing surgical portosystemic shunts versus TIPS for the treatment of refractory or recurrent variceal haemorrhage in people with cirrhotic portal hypertension. DATA COLLECTION AND ANALYSIS: Two review authors independently assessed trials and extracted data using methodological standards expected by Cochrane. We assessed risk of bias according to domains and risk of random errors with Trial Sequential Analysis (TSA). We assessed the certainty of the evidence using the GRADE approach. MAIN RESULTS: We found four randomised clinical trials including 496 adult participants diagnosed with variceal haemorrhage due to cirrhotic portal hypertension. The overall risk of bias in all the trials was judged at high risk. All the trials were conducted in the United States of America (USA). Two of the trials randomised participants to selective surgical shunts versus TIPS. The other two trials randomised participants to non-selective surgical shunts versus TIPS. The diagnosis of liver cirrhosis was by clinical and laboratory findings. We are uncertain whether there is a difference in all-cause mortality at 30 days between surgical portosystemic shunts compared with TIPS (risk ratio (RR) 0.94, 95% confidence interval (CI) 0.44 to 1.99; participants = 496; studies = 4). We are uncertain whether there is a difference in encephalopathy between surgical shunts compared with TIPS (RR 0.56, 95% CI 0.27 to 1.16; participants = 496; studies = 4). We found evidence suggesting an increase in the occurrence of the following harms in the TIPS group compared with surgical shunts: all-cause mortality at five years (RR 0.61, 95% CI 0.42 to 0.90; participants = 496; studies = 4); variceal rebleeding (RR 0.18, 95% CI 0.07 to 0.49; participants = 496; studies = 4); reinterventions (RR 0.13, 95% CI 0.06 to 0.28; participants = 496; studies = 4); and shunt occlusion (RR 0.14, 95% CI 0.04 to 0.51; participants = 496; studies = 4). We could not perform an analysis of health-related quality of life but available evidence appear to suggest improved health-related quality of life in people who received surgical shunt compared with TIPS. We downgraded the certainty of the evidence for all-cause mortality at 30 days and five years, irreversible shunt occlusion, and encephalopathy to very low because of high risk of bias (due to lack of blinding); inconsistency (due to heterogeneity); imprecision (due to small sample sizes of the individual trials and few events); and publication bias (few trials reporting outcomes). We downgraded the certainty of the evidence for variceal rebleeding and reintervention to very low because of high risk of bias (due to lack of blinding); imprecision (due to small sample sizes of the individual trials and few events); and publication bias (few trials reporting outcomes). The small sample sizes and few events did not allow us to produce meaningful trial sequential monitoring boundaries, suggesting plausible random errors in our estimates. AUTHORS' CONCLUSIONS: We found evidence suggesting that surgical portosystemic shunts may have benefit over TIPS for treatment of refractory or recurrent variceal haemorrhage in people with cirrhotic portal hypertension. Given the very low-certainty of the available evidence and risks of random errors in our analyses, we have very little confidence in our review findings.

[Standardization of intensive therapy tactics for acute hepatic insufficiency in patients with liver cirrhosis after portosystem shunting]. / Standartizatsiia taktiki intensivnoi terapii pri ostroi pechenochnoi nedostatochnosti u patsientov s tsirrozom pecheni posle portosistemnogo shuntirovaniia.

AIM: Here we analyze efficiency of the algorithm for intensive therapy of acute hepatic insufficiency in patients with cirrhosis after portosystemic shunting, based on the experience of the Republican Specialized Scientific and Practical Medical Center of Surgery named after academician V.V. Vakhidov and considering recommendations of the world's leading hepatology schools. MATERIAL AND METHODS: Algorithm efficiency was analyzed based on the results of portosystemic shunting of 556 patients with cirrhosis and acute liver insufficiency. Treatment effectiveness was assessed by presence and severity of postoperative complications, such as hepatic insufficiency and hepatic encephalopathy. Initial decompensation of liver cirrhosis significantly increases the risk of postoperative complications of portosystemic shunting due to edematous ascites syndrome - liver insufficiency (13.5% vs 6.9% in patients without ascites p≥0.05) and hepatic encephalopathy (64.9% vs. 51,7%, respectively, p≥0.05). In group of patients with preoperative ascites syndrome mortality rate was 3.8% (10 patients) and in the group without ascites - 2.3% (6 patients). The main cause of early postoperative lethality was anastomotic thrombosis development with recurrence of bleeding from esophagus and stomach varicose veins. To reduce the risk of postoperative complications and lethality, a therapeutic-tactical algorithm was developed which was effective in combination with a complex of conservative and biophysical methods of treatment and aimed at eliminating the 'resolving factors' of their developmen. RESULTS: One of the most important components of this algorithm is pharmacotherapy with hepatoprotective drugs use in patients with liver cirrhosis in the postoperative period to maintain liver functional state. Considering the antihypoxic, detoxifying and antioxidant properties of active components of remaxol and revealed positive effect of its use in patients (decrease in bilirubin level, increase in albumin fraction of total blood protein and decrease in activity of ALAT and ASAT in 2 times), this drug can be recommended for inclusion in the postoperative pathogenetic standard therapy in this category of patients.

Surgical portosystemic shunts versus devascularisation procedures for prevention of variceal rebleeding in people with hepatosplenic schistosomiasis.

BACKGROUND: Hepatosplenic schistosomiasis is an important cause of variceal bleeding in low-income countries. Randomised clinical trials have evaluated the outcomes of two categories of surgical interventions, shunts and devascularisation procedures, for the prevention of variceal rebleeding in people with hepatosplenic schistosomiasis. The comparative overall benefits and harms of these two interventions are unclear. OBJECTIVES: To assess the benefits and harms of surgical portosystemic shunts versus oesophagogastric devascularisation procedures for the prevention of variceal rebleeding in people with hepatosplenic schistosomiasis. SEARCH METHODS: We searched the Cochrane Hepato-Biliary Group Controlled Trials Register, CENTRAL, MEDLINE, Embase, Science Citation Index Expanded, LILACS, reference lists of articles, and proceedings of relevant associations for trials that met the inclusion criteria (date of search 11 January 2018). SELECTION CRITERIA: Randomised clinical trials comparing surgical portosystemic shunts versus oesophagogastric devascularisation procedures for the prevention of variceal rebleeding in people with hepatosplenic schistosomiasis. DATA COLLECTION AND ANALYSIS: Two review authors independently assessed the trials and extracted data using methodological standards expected by Cochrane. We assessed risk of bias according to domains and risk of random errors with GRADE and Trial Sequential Analysis. We assessed the certainty of evidence using the GRADE approach. MAIN RESULTS: We found two randomised clinical trials including 154 adult participants, aged between 18 years and 65 years, diagnosed with hepatosplenic schistosomiasis. One of the trials randomised participants to proximal splenorenal shunt versus distal splenorenal shunt versus oesophagogastric devascularisation with splenectomy, and the other randomised participants to distal splenorenal shunt versus oesophagogastric devascularisation with splenectomy. In both trials the diagnosis of hepatosplenic schistosomiasis was made based on clinical and biochemical assessments. The trials were conducted in Brazil and Egypt. Both trials were at high risk of bias.We are uncertain as to whether surgical portosystemic shunts improved all-cause mortality compared with oesophagogastric devascularisation with splenectomy due to imprecision in the trials (risk ratio (RR) 2.35, 95% confidence interval (CI) 0.55 to 9.92; participants = 154; studies = 2). We are uncertain whether serious adverse events differed between surgical portosystemic shunts and oesophagogastric devascularisation with splenectomy (RR 2.26, 95% CI 0.44 to 11.70; participants = 154; studies = 2). None of the trials reported on health-related quality of life. We are uncertain whether variceal rebleeding differed between surgical portosystemic shunts and oesophagogastric devascularisation with splenectomy (RR 0.39, 95% CI 0.13 to 1.23; participants = 154; studies = 2). We found evidence suggesting an increase in encephalopathy in the shunts group versus the devascularisation with splenectomy group (RR 7.51, 95% CI 1.45 to 38.89; participants = 154; studies = 2). We are uncertain whether ascites and re-interventions differed between surgical portosystemic shunts and oesophagogastric devascularisation with splenectomy. We computed Trial Sequential Analysis for all outcomes, but the trial sequential monitoring boundaries could not be drawn because of insufficient sample size and events. We downgraded the overall certainty of the body of evidence for all outcomes to very low due to risk of bias and imprecision. AUTHORS' CONCLUSIONS: Given the very low certainty of the available body of evidence and the low number of clinical trials, we could not determine an overall benefit or harm of surgical portosystemic shunts compared with oesophagogastric devascularisation with splenectomy. Future randomised clinical trials should be designed with sufficient statistical power to assess the benefits and harms of surgical portosystemic shunts versus oesophagogastric devascularisations with or without splenectomy and with or without oesophageal transection.

Postoperative enlargement and prognostic effects of portal venous bypass grafts in children undergoing Rex shunt.

OBJECTIVE: Patency of the bypass vein after a Rex shunt is an important indicator of prognosis. However, there is no report about the change of caliber of the bypass vein after a Rex shunt. The aim of this study was to identify postoperative changes of the bypass vein and to assess the relationship with prognosis. METHODS: Between October 2008 and October 2016 in our center, 114 children were diagnosed with extrahepatic portal venous obstruction. The portal cavernoma-portal bypass with interposition of grafted portal vessels was performed in 31 children, the gastroportal shunt was performed in 54 children, and other Rex shunts were performed in another 29 children. At follow-up, the patency and diameter of the bypass vein were assessed with ultrasound and computed tomography. The intraoperative and postoperative diameters of the bypass vein were compared to identify postoperative changes of the bypass vein. Prognosis was compared between children with and children without an enlarged bypass vein. RESULTS: The caliber of the bypass vein was enlarged in 50% of children (40/80) at 6 months postoperatively. The postoperative incidences of rebleeding and esophageal varices were significantly lower in children with an enlarged bypass vein than in those without (P < .05). Postoperatively, the reduced splenic size was significantly higher in children with an enlarged bypass vein than in those without (P < .05). The postoperative increase in platelet count in children with an enlarged bypass vein was significantly higher than in those without (P = .006). There was no significant difference in the flow velocity of the bypass vein between children with and children without an enlarged bypass vein (P = .133). The portal pressure was significantly reduced after surgery in children with an enlarged bypass vein than in children without an enlarged bypass vein (P = .017). CONCLUSIONS: The caliber of the bypass vein increases in 50% of children after a Rex shunt using a grafted portal vessel, which is related to a better prognosis.

Surgical treatment for rebleeding caused by bypass failure after Rex shunt: re-Rex shunt or Warren shunt?

PURPOSE: Although Rex shunt is an effective treatment for extrahepatic portal venous obstruction (EHPVO), 4-20% children develop rebleeding postoperatively. This study was used to evaluate the surgical treatment of rebleeding after Rex shunt in our center. METHOD: From June 2008 to Jan 2017, 12 of 122 children with EHPVO underwent a second operation due to graft stenosis and occlusion after Rex shunt in our center. The abdominal ultrasound and computed tomography (CT) showed the occlusion of bypass vein in nine children, stenosis of bypass vein in two children, and the patency of bypass vein in one child with dysplasia of intrahepatic portal vein. A re-Rex shunt was performed in eight children, of which one child required conversion to Warren shunt due to postoperative rebleeding. Five children underwent Warren shunt due to a narrowed left portal vein shown by intraoperative portal angiography and surgical exploration. All patients were followed up in this study. RESULTS: The postoperative incidences of re-bleeding and esophageal varices in children with Warren shunt were significantly lower than those in children undergoing re-Rex shunt (P = 0.027 and 0.015). After a second operation, the rate of bypass vein patency in children with re-Rex shunt was lower than that in children with Warren shunt (50 vs. 100%). The postoperative reduced size of spleen in children undergoing Warren shunt was significantly higher than that of children undergoing re-Rex shunt (P < 0.05). CONCLUSIONS: Comparing to re-Rex shunt, Warren shunt was a better treatment for rebleeding caused by bypass failure after Rex shunt.

Incidence of Postoperative Neurologic Complications in Pugs Following Portosystemic Shunt Attenuation Surgery.

Postoperative seizures occur in 5-12% of dogs following surgical attenuation of congenital extrahepatic portosystemic shunts (CEPSS) and are often refractory to treatment. Because pugs are predisposed to necrotizing meningoencephalitis, they may be at higher risk of developing neurologic complications after CEPSS attenuation. We hypothesized that pugs have a higher prevalence of postoperative neurologic complications and that pugs who died due to neurologic complications would have evidence of encephalitis at necropsy. Records were searched for pugs that had undergone surgical correction of a single extrahepatic CEPSS. Fourteen pugs met the inclusion criteria and were compared with a control group of 30 dogs of varying breeds who also underwent surgical attenuation for a single CEPSS. Four of 14 pugs (28.6%) died or were euthanized within 1 mo after surgery for neurologic complications, compared with only 1 of 30 dogs in the control group (P < .029). No evidence of necrotizing meningoencephalitis was seen on necropsy. Pugs may be at an increased risk of developing fatal neurologic complications following surgical attenuation for CEPSS. Further studies are indicated to investigate reasons for this increased risk, as well as to determine any factors that may indicate which pugs are at higher risk.

EUS-guided intrahepatic portosystemic shunt with direct portal pressure measurements: a novel alternative to transjugular intrahepatic portosystemic shunting.

BACKGROUND AND AIMS: Transjugular intrahepatic portosystemic shunting involves the creation of a low-resistance channel between the portal vein (PV) and the hepatic vein (HV), and is routinely performed under angiography. The aims were to evaluate (1) safety and technical feasibility; (2) procedural duration; and (3) subjective workload assessment of EUS-guided intrahepatic portosystemic shunt (EIPS). METHODS: Five Yorkshire pigs were used in the study. The HV or inferior vena cava (IVC) was identified using a linear-array echoendoscope and accessed with a 19-G FNA needle preloaded with a digital pressure wire. Mean pressure was recorded. The needle was advanced into the PV, where pressure measurements were again taken, and ultimately exchanged over a guidewire. A lumen-apposing metal stent was deployed under EUS and fluoroscopic guidance, with distal and proximal ends positioned inside the PV and HV (IVC), respectively. Dilation was performed, and pressure measurements repeated. The National Aeronautics and Space Administration (NASA) Task Load Index (TLI) was scored. Animals survived 2 weeks before necropsy. RESULTS: EIPS was successful in 5 out of 5 pigs. Mean time required for EUS identification, needle access, pressure measurement, and stent placement was 43 (31-55) minutes. NASA TLI scores revealed moderate workload. Mean baseline pressure measurements were PV 7.0 (5-9) and HV/IVC 5.0 (3-7) mm Hg and PV 6.3 (5-7) and HV/IVC 6.0 (4-7) mm Hg after. All animals survived 2 weeks. No bleeding was seen on necropsy. CONCLUSIONS: This study demonstrates the technical feasibility of EIPS using LAMS, with direct portal pressure measurement, in a survival model. In addition, the procedure was performed quickly with moderate technical demand.

Portosystemic Shunt Surgery in Patients with Idiopathic Noncirrhotic Portal Hypertension.

BACKGROUND Idiopathic noncirrhotic portal hypertension (INCPH) is a rare disease characterized by increased portal venous pressure in the absence of cirrhosis and other causes of liver diseases. The aim of the present study was to present our results in using portosystemic shunt surgery in patients with INCPH. MATERIAL AND METHODS Patients who had been referred to our Liver Transplantation Institute for liver transplantation and who had undergone surgery from January 2010 to December 2015 were retrospectively analyzed. Patients with INCPH who had undergone portosystemic shunt procedure were included in the study. Age, sex, symptoms and findings, type of portosystemic shunt, and postoperative complications were assessed. RESULTS A total of 1307 patients underwent liver transplantation from January 2010 to December 2015. Eleven patients with INCPH who did not require liver transplantation were successfully operated on with a portosystemic shunt procedure. The mean follow-up was 30.1±19 months (range 7-69 months). There was no mortality in the perioperative period or during the follow-up. Two patients underwent surgery again due to intra-abdominal hemorrhage; one had bleeding from the surgical site except the portacaval anastomosis and the other had bleeding from the h-graft anastomosis. No patient developed encephalopathy and no patient presented with esophageal variceal bleeding after portosystemic shunt surgery. Shunt thrombosis occurred in 1 patient (9.9%). Only 1 patient developed ascites, which was controlled medically. CONCLUSIONS Portosystemic shunt surgery is a safe and effective procedure for the treatment of patients with INCPH.