Creation of a percutaneous mesocaval shunt to control variceal bleeding in a child.

Although transjugular intrahepatic portosystemic shunt (TIPS) placement is the standard procedure for the treatment of portal hypertension, it is often impossible to perform in patients with extrahepatic portal vein occlusion. In these patients, options for decompressing the liver are few. In this report, we present a novel solution for managing gastro-esophageal hemorrhage in a child with portal hypertension and extrahepatic portal vein occlusion, through the creation of a percutaneous mesocaval shunt.

Rex shunt for portal vein thrombosis after adult living donor liver transplantation.

Portal vein thrombosis (PVT) after liver transplantation is a relatively common but serious complication which could lead to portal hypertension or a direct graft loss. A "Rex" shunt created between the superior mesenteric vein (SMV) and the umbilical portion of the left portal vein can be a useful option to treat PVT after pediatric liver transplantation, however, its application to adult patients has not been reported so far because appropriate vein grafts are hardly available. Herein we present a case of PVT after left lobe living donor liver transplantation (LDLT) who underwent the procedure using the own inferior jugular vein and the gonadal vein as a shunt graft. The shunt was patent immediately after the procedure but was thrombosed 2 days after probably due to the insufficient inflow from the SMV and the absence of anticoagulation therapy, for which emergent thrombectomy and ligation of the significant hepatofugal collateral veins followed by full anti-coagulation therapy were performed. The shunt remains open at 8 month after the procedure with a normal anmonia level and liver function. In conclusion, the Rex shunt using recipient's autologous vein grafts is a feasible and valuable option for adult patients to treat PVT after LDLT.

Direct puncture of the recanalized paraumbilical vein for portal vein targeting during transjugular intrahepatic portosystemic shunt procedures: assessment of technical success and safety.

PURPOSE: To assess the success of direct percutaneous puncture of the recanalized paraumbilical vein (RPUV) for access and visualization of the portal vein (PV) to guide transhepatic puncture during transjugular intrahepatic portosystemic shunt (TIPS) creation. The predictive value of successful catheterization based on preprocedural vein diameter was analyzed. MATERIALS AND METHODS: A retrospective review of all TIPS procedures from 2002 to 2008 performed at a single institution was conducted, and a subset of procedures in which portal venography was attempted via the paraumbilical vein were identified. In this subset, TIPS outcomes and diameters of the RPUV near the skin puncture site and left PV junction were measured and analyzed with a two-tailed Student t test. RESULTS: During the study period, 114 TIPSs were created. RPUV punctures were found in 22 patients (19.3%) and portal venography was successful in 14 of the 22 patients (64%), all without complications. In the remainder (n = 8), access via the RPUV failed secondary to a small vein diameter (< 0.3 cm; n = 3), moderate to severe vessel tortuosity (n = 4), and distal thrombosis (n = 1). Puncture, catheterization, and portal venography was successful when the paraumbilical vein measured a mean of 0.75 cm at the skin and a mean of 0.84 cm at the junction with the left PV when analyzed against the failed attempts. CONCLUSIONS: Portal venography via the RPUV is a feasible and probably safe alternative to other methods of PV opacification during TIPS procedures.

Intractable Hepatic Encephalopathy with a Large Portosystemic Shunt Successfully Treated Using Shunt-preserving Disconnection of the Portal and Systemic Circulation.

Hepatic encephalopathy (HE) is a significant symptom of decompensated liver cirrhosis. Occlusion of portosystemic shunts is used to treat refractory HE. Nevertheless, these treatments often cause adverse events, such as ascites and esophageal varices. We treated a 57-year-old man with refractory HE using shunt-preserving disconnection of the portal and systemic circulation (SPDPS). After SPDPS, there were no obvious complications, and the patient's ammonia level significantly decreased. To date, the patient has not experienced recurrent HE. SPDPS appears to be a safe and effective treatment method for portosystemic encephalopathy.

Mesocaval shunt as an alternative treatment for persistent ascites after liver transplantation: case reports.

Refractory ascites after liver transplantation is a relatively rare complication. If the initial medical treatment fails, more invasive techniques may be required. The TIPS procedure has emerged as a major treatment option for decompression of the portal venous system. Mesocaval shunt can be an alternative to TIPS in selected cases. We describe two patients who underwent mesocaval shunt construction for refractory ascites.

Intraoperative Surgical Portosystemic Shunt in Liver Transplantation: Systematic Review and Meta-Analysis.

BACKGROUND Expanded clinical and surgical techniques in liver transplantation can markedly improve patient and graft survival. The main purpose of this study was to evaluate the efficacy of intraoperative portocaval shunts in liver transplantation. MATERIAL AND METHODS Searches were conducted in Cochrane, MEDLINE, and EMBASE databases, and updated in January 2018. The following specific outcomes of interest were defined and evaluated separated using 2 different reviews and meta-analyses for 1) hemi-portocaval shunt (HPCS) and 2) temporary portocaval shunt (TPCS). Comparative studies were analyzed separately for both surgical portocaval shunt modalities. RESULTS Only 1 well-designed randomized controlled trial was found. Most studies were retrospective or prospective. Initially, we found 1479 articles. Of those selected, 853 were from PubMed/MEDLINE, 32 were from Cochrane and 594 were from EMBASE. Our meta-analysis included a total of 3232 patients for all the included studies. Results found that 41 patients with HPCS experienced increased 1-year patient survival (OR 16.33; P=0.02) and increased 1-year graft survival (OR 17.67; P=0.01). The TPCS analysis with 1633 patients found patients had significantly shorter intensive care unit length of stay (days) (P=0.006) and hospital length of stay (P=0.02) and had decreased primary nonfunction (PNF) (OR 0.30, P=0.02) and mortality rates (OR 0.52, P=0.01). CONCLUSIONS Intraoperative surgical portosystemic shunt in relation to liver transplantation with TPCS was able to prevent PNF, decrease hospital length of stay and unit care length of stay. Furthermore, in analyzing data for patients with HPCS, we observed increases in the 1-year graft and patient survival rates. More prospective randomized trials are needed to arrive at a more precise conclusion.

Portal hypertension and its management in children.

Portal hypertension (PHT), defined as raised intravascular pressure in the portal system, is a complication of chronic liver disease or liver vascular occlusion. Advances in our ability to diagnose and monitor the condition but also predict the risk of gastrointestinal bleeding have enabled us to optimise the management of children with PHT either at a surveillance or at a postbleeding stage. A consensus among paediatric centres in the classification of varices can be beneficial in streamlining future paediatric studies. New invasive (endoscopic and surgical procedures) and non-invasive (pharmacotherapy) techniques are currently used enabling clinicians to reduce mortality and morbidity in children with PHT.

Portal-to-right portal vein bypass for extrahepatic portal vein obstruction.

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 34.6cmH2O (28-45 cmH2O) before the bypass to 19.6cmH2O (14-24cmH2O) 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.

[Clinical reasoning and decision making in practice. A 9-year-old boy with isolated splenomegaly]. / Klinisch denken en beslissen in de praktijk. Een 9-jarige jongen met een geïsoleerde splenomegalie.

Splenomegaly was discovered by chance in a 9-year-old boy who had no further complaints. Apart from splenomegaly and mild thrombocytopenia, no other pathological condition was found in the first instance. Ultrasound revealed a spleen with a median length of 16.7 cm. Blood tests remained stable and a bone marrow biopsy showed no pathology either. Doppler ultrasound of the splenic vessels was normal; screening for a coagulation disorder was not performed. A wait-and-see policy was instituted. Later, the patient developed haematemesis because ofoesophageal varices. It turned out that he had portal hypertension caused by thrombosis of the portal vein. The underlying cause was a heterozygous protein-C deficiency. Treatment consisted of anticoagulant therapy and a mesocaval shunt procedure. Splenomegaly is an atypical clinical manifestation in childhood. Coagulation disorders should be considered in children with splenomegaly.

Hepatocellular carcinoma in a patient with congenital porto-systemic shunt.

Congenital porto-caval shunt is a rare anomaly that can result in direct shunt-related complications (encephalopathy, pulmonary hypertension) or indirect complications such as the development of benign or malignant hepatic neoplasms. Treatment consists of management of the complications and occlusion of the porto-caval shunt in one or two stages by either a surgical or an interventional radiology approach.

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.

[Obstructive jaundice caused by portal varicosis]. / Obstructie-icterus door portale varicosis.

In a 66-year-old woman with pruritus, jaundice, dark-brown urine and light-colored faeces obstructive jaundice was diagnosed. Despite extensive investigations, it was not possible to clearly distinguish if varicosis or cholangiocarcinoma was the cause of the obstruction. During laparotomy the right lobe of the liver was seen to be greatly underdeveloped. The portal system showed a varicose deformation with compression of the bile ducts and portal hypertension. The right lobe of the liver was removed and the portal hypertension was treated by creating a shunt between the hepatic portal vein and the right ovarian vein. The jaundice disappeared and the patient recovered. Histological investigation showed atrophy, secondary biliary fibrosis, cirrhosis and a biliary cystadenoma. There were no signs of malignancy. The varicose deformation can be considered to be a result of the portal hypertension caused by fibrosis and cirrhosis with possibly a history of thrombosis and insufficient recanalization.

Recurrent urinary conduit bleeding in a patient with portal hypertension: management with a transjugular intrahepatic portosystemic shunt.

OBJECTIVE: To determine if a transjugular intrahepatic portosystemic shunt can control recurrent urinary conduit bleeding in a patient with portal hypertension. METHODS: Following transjugular catheterization of the right hepatic vein, a long curve Colapinto needle was advanced through the liver parenchyma into the portal vein near its bifurcation. After a guide wire exchange, a catheter was advanced into the portal system and venogram was obtained. Following another guide wire exchange, a balloon angioplasty catheter was used to create the shunt by dilating the parenchymal tract between the hepatic and portal veins. A self-expandable stent was used to ensure patency of the shunt. RESULTS: After shunt placement, bleeding from the ileal conduit and stroma decreased significantly. A duplex ultrasound at five-month follow-up demonstrated the shunt to be completely patent. CONCLUSIONS: Based on this limited experience, it appears that the transjugular, intrahepatic, portosystemic shunt is an acceptable method to control massive, recurrent urinary conduit bleeding in patients with portal hypertension.

Medium aperture meso-caval shunts reliably prevent recurrent variceal hemorrhages.

BACKGROUND: Objectives of partial medium aperture mesocaval shunts (MCS) include reduction of portal hypertension to prevent recurrent variceal hemorrhage, preservation of portal flow through liver while maintaining an intact porta hepatis to facilitate a future liver transplant (OLTx). PATIENTS AND METHODS: Fifteen patients were retrospectively analyzed to review the indications for the procedure, its short- and long-term complications as well as patency and functional status of the shunt. They were followed for a period of 21 months. RESULTS: The perioperative and long-term mortality rate was 0%. Rebleeding rate perioperatively and in follow-up was 0%. Early shunt nonfunction was 13% and post-shunt encephalopathy (PSE) was 20%. The encephalopathy was grade I to II and controlled medically. Abdominal ultrasound and Doppler confirmed 13 patent shunts (2 patients did not agree to ultrasound) with preserved hepatopetal flow in 10. CONCLUSIONS: Medium aperture MCS utilizing ringed polytetrafluoroethylene (PTFE) grafts safely and reliably prevent recurrent variceal hemorrhage. Encephalopathy is infrequent and mild. This technique preserves the portal venous anatomy making a future OLTx technically easier.

Temporary splenic artery occlusion during construction of the distal splenorenal shunt.

A technique for temporary occlusion of the splenic artery during construction of the distal splenorenal shunt is described. This maneuver is proposed to reduce intraoperative blood loss associated with mobilization of the splenic vein in the cirrhotic patient.

Splenoadrenal shunt. An original portosystemic decompressive technique.

Management of gastrointestinal hemorrhage from rupture of esophageal and gastric varices due to portal hypertension remains a debated question. In patients with sclerotherapy-resistant esophagogastric varices, and preserved hepatic function, a surgical shunt is considered the treatment of choice. A 63-year-old male was admitted in our Department with a diagnosis of idiopathic fibrosis of the liver, portal hypertension, esophageal and gastric varices and previous history of variceal bleeding. A distal splenorenal shunt was planned. During the isolation, a large diameter left adrenal vein was identified. An end-to-end anastomosis utilizing the distal splenic vein and the proximal adrenal stump was performed. The procedure was uneventful. An ultrasound color-Doppler on the 3rd postoperative day, showed normal intrasplenic resistance index, demonstrating the efficacy of the shunt. A splenic angiography carried out on the 8th postoperative day showed the complete patency of the splenoadrenal shunt. At the 15th postoperative day, the patient was discharged. In patients with portal hypertension, sclerotherapy-resistant esophagogastric varices and preserved hepatic function, a surgical portosystemic shunt is mandatory. Splenoadrenal shunt, utilizing a left adrenal vein represent an excellent option in selected cases.