First Report of Two-stage Living Donor Liver Transplantation to Avoid Futility and Ensure Double Equipoise in Acute Liver Failure Complicated by Toxic Liver Syndrome.

Acute hepatic failure may occasionally be complicated by toxic liver syndrome. Emergency hepatectomy for stabilization followed by delayed graft implantation is a recognized strategy in such cases in the setting of deceased donor liver transplantation. Living donor liver transplantation adds additional complexity to this scenario as the donor liver is a directed donation and failure to stabilize the patient after emergency hepatectomy can lead to a futile live donor hepatectomy, hepar-divisum, or an orphan graft. We report a case where the two-stage strategy was utilized to circumvent this situation. A patient with toxic liver syndrome underwent emergency hepatectomy and was closely monitored in the operating theater. A live donor hepatectomy was started after the recipient demonstrated cardiovascular and neurological stabilization. Graft implantation was completed after an anhepatic period of 9.45 h. To our knowledge, this is the first reported instance of using the two-stage strategy in living-donor-liver-transplantation for toxic liver syndrome to prevent futile donor surgery and achieve double equipoise.

Sarcopenia is a risk factor for post-transjugular intrahepatic portosystemic shunt hepatic encephalopathy and mortality: A systematic review and meta-analysis.

BACKGROUND/AIMS: Transjugular intrahepatic portosystemic shunt (TIPS) is a commonly performed procedure in patients with liver cirrhosis to treat portal hypertension-related conditions, including variceal bleeding and refractory ascites. However, while the increased risk of hepatic encephalopathy (HE) after TIPS is important to consider when determining whether a patient is a good candidate for TIPS, currently there is no widely used method to predict the development of post-TIPS HE, although the model for end-stage liver disease (MELD) score is used to predict post-TIPS mortality. We conducted a systematic review and meta-analysis to evaluate sarcopenia as a risk factor for HE and mortality in patients undergoing TIPS. METHODS: A comprehensive search strategy was used to identify reports of post-TIPS HE and mortality in sarcopenia vs. non-sarcopenia patients with liver cirrhosis who received TIPS in March 2023. Open Meta Analyst was used to compute the results. RESULTS: Twelve studies with 2056 patients met inclusion criteria and were included in the final meta-analysis. Sarcopenia was associated with a significantly higher post-TIPS HE rate than non-sarcopenia (risk ratio [RR]: 1.68, 95% CI: 1.48-1.92, p < 0.00001, I2 = 65%), as well as a significantly higher post-TIPS mortality rate (RR: 1.73, 95% CI: 1.14-2.64, p < 0.00001, I2 = 87%). CONCLUSION: Patients with sarcopenia have a significantly increased risk of post-TIPS HE and mortality. Presence of sarcopenia should be considered when weighing the risks and benefits of performing TIPS in patients with cirrhosis. Further studies are needed to determine the clinical utility of important risk factors such as sarcopenia on post-TIPS outcomes.

Splenic Artery Ligation: An Ontable Bail-Out Strategy for Small-for-Size Remnants after Major Hepatectomy: A Retrospective Study.

It has been reported that the prevention of acute portal overpressure in small-for-size liver grafts leads to better postoperative outcomes. Accordingly, we aimed to investigate the feasibility of the technique of splenic artery ligation in a case series of thirteen patients subjected to major liver resections with evidence of small-for-size syndrome and whether the maneuver results in the reduction of portal venous pressure and flow. The technique was successful in ten patients, with splenic artery ligation alleviating portal hypertension significantly. Three patients required the performance of a portocaval shunt for the attenuation of portal hypertension. Portal inflow modulation via splenic artery ligation is a technically simple technique that can prove useful in the context of major hepatectomies as well as in liver transplantations and the early evaluation and modification of portal venous pressure post hepatectomy can be used as a practical tool to guide the effect of the intervention.

Novel technique in treating portal cavernous transformation with portal Biliopathy.

We present a case report of a patient who is a non-cirrhotic with portal cavernous transformation secondary to previous trauma. The patient presents with portal biliopathy requiring ERCP/EUS with biliary stenting. The patient was referred to Interventional Radiology (IR) for portal vein recanalization. The patient underwent a novel technique of transplenic access with portal vein recanalization via a gunsight technique, ultimately receiving a direct intrahepatic portocaval shunt (DIPS). Subsequently, his symptoms resolved, and the biliary stent was successfully removed.

Which cava anastomotic techniques are optimal regarding immediate and short-term outcomes after liver transplantation: A systematic review of the literature and expert panel recommendations.

BACKGROUND: It has long been debated whether cava anastomosis should be performed with the piggyback technique or cava replacement, with or without veno-venous bypass (VVB), with or without temporary portocaval shunt (PCS) in the setting of liver transplantation. OBJECTIVES: To identify whether different cava anastomotic techniques and other maneuvers benefit the recipient regarding short-term outcomes and to provide international expert panel recommendations. DATA SOURCES: Ovid MEDLINE, Embase, Scopus, Google Scholar, and Cochrane Central. METHODS: A systematic review following PRISMA guidelines and recommendations using the GRADE approach derived from an international expert panel (CRD42021240979). RESULTS: Of 3205 records screened, 307 publications underwent full-text assessment for eligibility and 47 were included in qualitative synthesis. Four studies were randomized control trials. Eighteen studies were comparative. The remaining 25 were single-center retrospective noncomparative studies. CONCLUSION: Based on existing data and expert opinion, the panel cannot recommend one cava reconstruction technique over another, rather the surgical approach should be based on surgeon preference and center dependent, with special consideration toward patient circumstances (Quality of evidence: Low | Grade of Recommendation: Strong). The panel recommends against routine use of vevo-venous bypass (Quality of evidence: Very Low | Grade of Recommendation: Strong) and against the routine use of temporary porto-caval shunt (Quality of evidence: Very Low | Grade of Recommendation: Strong).

Direct intrahepatic portocaval shunt (DIPS) or transjugular transcaval intrahepatic portosystemic shunt (TTIPS) to treat complications of portal hypertension: Indications, technique, and outcomes beyond Budd-Chiari syndrome.

Transjugular intrahepatic portosystemic shunt (TIPS) is nowadays the benchmark treatment of severe portal hypertension complications. However, besides usual contraindication to the procedure (namely recurrent hepatic encephalopathy, severe liver dysfunction, right heart failure and/or pulmonary hypertension), TIPS appears regularly unfeasible due to abnormal and/or distorted anatomy. In this situation, the only non-surgical approaches to treat severe portal hypertension consist in the creation of an intrahepatic portocaval shunt from percutaneous (direct intrahepatic portocaval shunt - DIPS) or transjugular route (transjugular transcaval intrahepatic portosystemic shunt - TTIPS). These procedures have been rapidly adopted in patients with Budd-Chiari syndrome but are only poorly reported in patients with cirrhosis and without BCS. Considering the broadening landscape of TIPS indication in patients with cirrhosis within the last ten years, we aimed to describe the techniques, safety and efficacy of DIPS and TTIPS procedures as an alternative to TIPS in case of unfavourable anatomy.

Temporary portal decompression during liver transplantation: a video review of the different techniques.

PURPOSE: Temporary portal decompression (TPD) during liver transplantation (LT) remains a divisive technical issue in the liver transplant community. In this video-based article, we show the technical details of the different techniques used for TPD during LT. METHODS: An early portal section, before liver mobilization, should be preferred in order to achieve hepatectomy of a totally devascularized liver. Portal decompression can be achieved through direct right portocaval shunts and indirect portosystemic shunts (i.e., mesentericosaphenous and portosaphenous shunts). RESULTS: The preference for direct portocaval or indirect portosystemic shunts is tailored on patients and anatomical characteristics. Each of these three techniques presents specific indications, limitations, and advantages. CONCLUSION: TPD during LT can be achieved through different techniques that aim to facilitate the recipient hepatectomy, reduce the blood loss, and maintain hemodynamic stability.

Neurological Symptoms of Congenital Portosystemic Shunt Reversed by Venous Endovascular Intervention: A Six Years Follow-Up Study.

Congenital portosystemic shunt (CPSS) is a rare disorder characterized by a diversion of Porto-mesenteric blood into systemic veins. Type I is an end to side fistula between the portal vein and the inferior vena cava. Type II is a side to side fistula between the main portal vein or its branches and mesenteric, splenic, gastric, and systemic veins. Clinical presentation of these patients varies. Treatment and management are guided by the type of malformation and clinical presentation. Herein, we present a case of CPSS Type IIb with neurological symptoms, treated with endovascular occlusion with 6-year follow-up without remission.

Update on Shunt Surgery.

BACKGROUND: Bleeding from esophagogastric varices is a life-threatening complication from portal hypertension. It occurs in 15% of patients and has a mortality rate of 20-35%. SUMMARY: The primary therapy for variceal bleeding is medical. In cases of recurrent bleeding, a definitive therapy is required. In cases of parenchymal decompensation, liver transplantation is the causal therapy, but if liver function is preserved, portal decompression is the therapy of choice. The use of the transjugular intrahepatic portosystemic shunt (TIPS) has achieved widespread acceptance, although evidence for surgical shunts is comparable or better in patients with good hepatic reserve. The type of surgical shunt depends on the patent veins of the portomesenteric system. If total occlusion is present, a devascularization procedure might be indicated. KEY MESSAGES: Therapy, taking into account liver function, morphology of the portovenous system, and imminent liver transplantation, should be performed by an interdisciplinary team of gastroenterologists, interventional radiologists, and gastrointestinal surgeons.

Living donor liver retransplantation for primary non-function of liver graft following multivisceral transplantation in a pediatric patient.

Multivisceral organ transplantation (MVOT) includes transplantation of three or more abdominal organs, generally including the small bowel, duodenum, stomach, liver, pancreas, and colon. We here presented the detailed procedures of repeat living donor liver transplantation for primary non-function of the first liver graft following MVOT in a pediatric patient. A 6-year-old girl with chronic intestinal pseudo-obstruction underwent MVOT with 5-year-old donor organs. However, the primary non-function of the liver graft developed, and an emergency living donor liver transplantation operation using a left lateral section graft was performed on the third day after MVOT. The donor was the patient's father. Portal flow interruption induced ischemic congestion of the whole small bowel, thus we used a series of porto- caval shunt to reduce the risk of ischemic splanchnic congestion during recipient hepatectomy and graft implantation. Other surgical procedures were the same as the standardized procedures for left liver graft implantation. The graft-recipient weight ratio was 2.15. The patient was managed conservatively for 3 months and discharged in an improved condition at 4 months after MVOT. She finally passed away at 22 months after MVOT. We think that our experience will be helpful for surgeons to cope with portal vein clamping-associated splanchnic congestion during liver transplantation and other abdominal surgeries.

Pulmonary arterial hypertension caused by congenital extrahepatic portocaval shunt: a case report.

BACKGROUND: Congenital extrahepatic portocaval shunt (CEPS), also known as Abernethy malformation, is an extremely rare anomaly of the splanchnic venous system, especially when accompanied by pulmonary arterial hypertension. CASE PRESENTATION: We report a case of a 15-year-old female who was diagnosed with CEPS (Abernethy type Ib) accompanied by pulmonary arterial hypertension. This case was incidentally identified during abdominal ultrasound examination and confirmed by mesenteric and splenic arteriography. During more than 4 years of follow-up, after receiving sildenafil (80 mg/day), the patient's condition improved in the first year after discharge. However, one year later, the patient's conditions start to deteriorate. CONCLUSION: This article presents a rare case of Abernethy malformation accompanied by pulmonary arterial hypertension, which can be diagnosed by using abdominal ultrasonography, portal vein computed tomography angiography or mesenteric and splenic arteriography. This malformation had limited treatment and poor prognosis.

Percutaneous extrahepatic splenomeso-caval shunt creation in a patient with portal vein thrombosis after Whipple procedure.

A 72-year-old male presented with refractory ascites secondary to portal vein occlusion with cavernomatous transformation following pancreaticoduodenectomy (Whipple procedure). Due to the unfavorable anatomy, transjugular intrahepatic portosystemic shunt was not an option. However, given patency of the spleno-mesenteric confluence and absence of the pancreatic head after the Whipple procedure, a splenomeso-caval shunt was successfully created using a transjugular-transsplenic rendezvous technique.

Congenital intrahepatic portocaval shunts and hypoglycemia due to secondary hyperinsulinism: a case report and review of the literature.

BACKGROUND: Congenital portosystemic shunts present with various associated complications, such as other congenital malformations, hyperammonemia, or hepatopulmonary syndrome. Few cases of associated hypoglycemia have been reported so far and our case, to the best of our knowledge, describes the most severe extent of hypoglycemia. CASE PRESENTATION: We describe the case of a newborn Arab boy with two intrahepatic portosystemic shunts, resulting in severe and persistent hypoglycemia, due to which one of the shunts was closed by interventional radiology whereas the other shunt had already closed spontaneously. CONCLUSIONS: Because he showed elevated levels for insulin and prolonged high insulin levels in an oral glucose tolerance test, our case supports the theory that portocaval shunts cause a reduced hepatic insulin reduction due to the high blood volume bypassing the liver. This case provides further insights into glucose regulation mechanisms of the liver and we suggest a consistent screening for hypoglycemia in patients with congenital portosystemic shunts.

Cerebral Blood Flow and Metabolism in Hepatic Encephalopathy-A Meta-Analysis.

Hepatic Encephalopathy (HE) is associated with abnormalities in brain metabolism of glucose, oxygen and amino acids. In patients with acute liver failure, cortical lactate to pyruvate ratio is increased, which is indicative of a compromised cerebral oxidative metabolism. In this meta-analysis we have reviewed the published data on cerebral blood flow and metabolic rates from clinical studies of patients with HE. We found that hepatic encephalopathy was associated with reduced cerebral metabolic rate of oxygen, glucose, and blood flow. One exemption was in HE type B (shunt/by-pass) were a tendency towards increased cerebral blood flow was seen. We speculate that HE is associated with a disturbed metabolism-cytopathic hypoxia-and that type specific differences of brain metabolism is due to differences in pathogenesis of HE.

Stepwise management of hepatocellular carcinoma associated with Abernethy syndrome.

Patients with congenital agenesis of the portal vein may develop hepatocellular tumors due to enhanced arterial blood flow. These tumors may be benign (FNH, adenomas) or malignant (hepatoblastoma, HCC). Liver resection can be proposed, and preoperative arterial embolization may decrease blood loss during surgery. Liver transplantation with PV reconstruction is also an option.

Transjugular liver biopsy after transjugular intrahepatic portosystemic shunt (TIPS) or direct intrahepatic portocaval shunt (DIPS): Is it feasible, effective, and safe?

PURPOSE: To evaluate our experience with transjugular liver biopsies (TJLB) in patients with previously placed transjugular intrahepatic portosystemic shunt (TIPS) or direct intrahepatic portocaval shunt (DIPS). MATERIAL AND METHODS: A single-institution retrospective review was performed looking at all TJLBs in patients with previously placed TIPS or DIPS over the past seven years. There were six men and one woman (mean age 57.9±8.8 [SD] years; range: 47-71 years). Patient demographics, indications, procedural details, laboratory data, complications, and pathology were recorded. Patients with occluded TIPS were excluded from this study. RESULTS: Seven TJLBs were performed, five in patients who had a TIPS and two who had a DIPS. Of the patients with TIPS, biopsies were performed from the same hepatic vein as TIPS in three procedures and from a different hepatic vein in two procedures. In DIPS patients, both biopsies were performed from the right hepatic vein. The reasons for the transjugular rather than the percutaneous approach to liver biopsy included ascites, coagulopathy, or need for concurrent TIPS/DIPS evaluation and/or revision. All procedures were technically successful with adequate samples obtained. There were no immediate or delayed complications. CONCLUSION: Our results suggest that if needed a TJLB can safely and effectively be performed in patients with previously placed TIPS or DIPS.

Prenatally diagnosed congenital portosystemic shunts.

AIM: Congenital portosystemic shunts (CPSS) are rare, congenital malformations that are increasingly often discovered during the fetal period, and for which, the manifestations and evolution are poorly understood. The objective of this review is to describe the phenotype and evolution of forms diagnosed in the antenatal period. MATERIALS AND METHODS: We performed a systematic review of the literature cited in Pubmed between 1982 and 2016 for CPSS cases diagnosed during the fetal period. RESULTS: We identified 123 cases. The median age at diagnosis was 25 GA (14-38 weeks GA). Eighty patients had 128 associated congenital anomalies. The congenital abnormalities most frequently associated with antenatal diagnosis of CPSS were congenital cardiac disease (30 cases), intrauterine growth restriction (21 cases), vascular anomalies (14 cases), and trisomy 21 (7 cases). Seventy-five complications were reported in the literature. The most frequent were antenatal hemodynamic abnormalities (27 cases), neonatal cholestasis (11 cases), and hyperammonemia (10 cases). Twenty-nine patients had no complications. The choice of treatment was conservative in 29/56 cases, interventional radiology in 15 cases and surgery in 15 cases (three of the latter after failure of embolization). CONCLUSION: From this review, we propose an algorithm for the perinatal management of this congenital abnormality.

Outcome of intrahepatic portosystemic shunt diagnosed prenatally.

We analyzed the characteristics of the population with congenital portosystemic shunt diagnosed during the antenatal period and the organization of their perinatal care. This multicentric retrospective study included all the patients with a prenatal diagnosis of congenital portosystemic shunt. Between 1999 and 2015, 12 patients were included. Prenatal diagnosis was done at a median 26.5 weeks of gestation (21-34). All the patients presented intrahepatic CPSS, three of them had associated congenital cardiopathy, and one a Bannayan-Zonana syndrome. Ten patients had simple outcome on conservative treatment, eight of them having a spontaneous closure of their portosystemic shunt within the first 2 years of life. One patient had surgical treatment which failed and he developed a focal nodular hyperplasia. Another patient had radiological interventional closure of his shunt which was complicated by a venal portal thrombosis. CONCLUSION: Outcome of intrahepatic portosystemic shunt diagnosed prenatally is good in the majority of cases. What is known: • Multiples studies exist on congenital porto systemic shunt but when the diagnosis is done after birth. • The evolution, management, and complication are well known. What is new: • There is very few studies with only patients diagnosed in antenatal and it is a large series of cases. • Outcome of intrahepatic portosystemic shunt diagnosed prenatally is good in the majority of cases.

Ultrasonic examination for patients with liver cirrhosis after transjugular intrahepatic portosystemic shunt / 局解手术学杂志

Objective To investigate the application of ultrasonic examination for patients with liver cirrhosis after transjugular intrahepatic portosystemic shunt (TIPS) in the stents in artificial channel (SIAC) and portal vein (PV).Methods The clinical data of 28 patients who were admitted into our hospital from January 2013 to February 2017 and received TIPS were analyzed.Scanned the stents in artificial channel and portal vein and their inner diameter through transabdominal probe,and then measured the blood flow velocity by doppler.ResultsAfter TIPS,the SIAC of cirrhosis patients showed parallel tubular high echo,smooth wall and clear vessel lumen.And the inner diameter of SIAC was (5.93±0.76)mm,the blood flow velocity was (97.14±28.46)cm/s,and the 95% reference value was 4.45 mm~7.41 mm and 41.36~152.92 cm/s respectively.And the inner diameter of PV was (12.40 mm±2.90)mm,the blood flow velocity was (38.33 cm±16.01)cm/s,and the 95% reference value was 6.72~18.08 mm and 22.32~49.34 cm/s respectively.Conclusion Ultrasonography can give objective evaluation of whether SIAC and PV is unobstructed after TIPS,and it can get their parameters of inner diameter and velocity.As a result, ultrasonography can be regard as a perfect means for follow-up checkup.