Peri-operative Anticoagulation Strategies, Bleeding and Thrombotic Complications in Pediatric Patients Undergoing Intervention for Congenital Portosystemic Shunts.

BACKGROUND: Congenital portosystemic shunts (CPSS) are rare congenital abnormalities causing abnormal blood flow between the portal vein and systemic circulation. This study reports on the peri-operative anticoagulation management of CPSS patients post closure, focusing on the incidence of thrombotic and bleeding complications. METHODS: This is a single-center retrospective analysis of CPSS patients who underwent surgery or endovascular intervention between 2005 and 2021. The protocol included unfractionated heparin (UFH) during and immediately after surgery, followed by either warfarin or low molecular weight heparin (LMWH) postoperatively. Outcomes assessed included postoperative thrombotic and bleeding complications. RESULTS: A total of 44 patients were included. Postoperatively, 89% received treatment-dose UFH, transitioning to warfarin or LMWH at discharge. Thrombotic complications occurred in 16% of patients, predominantly in the superior mesenteric vein. Surgical interventions and continuous infusion of tissue plasminogen activator (tPA) were used for clot resolution. Bleeding complications were observed in 64% of patients, primarily managed with transfusions and temporary UFH interruption. No deaths related to thrombotic, or bleeding events were reported. CONCLUSIONS: Our findings underscore the delicate balance required in anticoagulation management for CPSS patients, revealing an occurrence of both thrombotic and bleeding complications postoperatively. LEVELS OF EVIDENCE: Level II, retrospective study.

Clinical Implications and Management of Spontaneous Portosystemic Shunts in Liver Cirrhosis.

Portal hypertension from chronic liver disease leads to the formation of collateral blood vessels called spontaneous portosystemic shunts (SPSS). These shunts may form from existing vessels or through neo-angiogenesis. Their location affects clinical outcomes due to varying risks and complications. This review summarizes current knowledge on SPSS, covering their clinical impact and management strategies. Recent data suggest that SPSS increases the risk of variceal bleeding, regardless of shunt size. The size of the shunt is crucial in the rising incidence of hepatic encephalopathy (HE) linked to SPSS. It also increases the risk of portopulmonary hypertension and portal vein thrombosis. Detecting and assessing SPSS rely on computed tomography (CT) and magnetic resonance imaging. CT enables precise measurements and the prediction of cirrhosis progression. Management focuses on liver disease progression and SPSS-related complications, like HE, variceal bleeding, and portopulmonary hypertension. Interventional radiology techniques such as balloon-occluded, plug-assisted, and coil-assisted retrograde transvenous obliteration play a pivotal role. Surgical options are rare but are considered when other methods fail. Liver transplantation (LT) often resolves SPSS. Intraoperative SPSS ligation is still recommended in patients at high risk for developing HE or graft hypoperfusion.

Portal vein arterialization in 25 liver transplant recipients: A Latin American single-center experience.

BACKGROUND: Portal vein arterialization (PVA) has been used in liver transplantation (LT) to maximize oxygen delivery when arterial circulation is compromised or has been used as an alternative reperfusion technique for complex portal vein thrombosis (PVT). The effect of PVA on portal perfusion and primary graft dysfunction (PGD) has not been assessed. AIM: To examine the outcomes of patients who required PVA in correlation with their LT procedure. METHODS: All patients receiving PVA and LT at the Fundacion Santa Fe de Bogota between 2011 and 2022 were analyzed. To account for the time-sensitive effects of graft perfusion, patients were classified into two groups: prereperfusion (pre-PVA), if the arterioportal anastomosis was performed before graft revascularization, and postreperfusion (post-PVA), if PVA was performed afterward. The pre-PVA rationale contemplated poor portal hemodynamics, severe vascular steal, or PVT. Post-PVA was considered if graft hypoperfusion became evident. Conservative interventions were attempted before PVA. RESULTS: A total of 25 cases were identified: 15 before and 10 after graft reperfusion. Pre-PVA patients were more affected by diabetes, decompensated cirrhosis, impaired portal vein (PV) hemodynamics, and PVT. PGD was less common after pre-PVA (20.0% vs 60.0%) (P = 0.041). Those who developed PGD had a smaller increase in PV velocity (25.00 cm/s vs 73.42 cm/s) (P = 0.036) and flow (1.31 L/min vs 3.34 L/min) (P = 0.136) after arterialization. Nine patients required PVA closure (median time: 62 d). Pre-PVA and non-PGD cases had better survival rates than their counterparts (56.09 months vs 22.77 months and 54.15 months vs 31.91 months, respectively). CONCLUSION: This is the largest report presenting PVA in LT. Results suggest that pre-PVA provides better graft perfusion than post-PVA. Graft hyperperfusion could play a protective role against PGD.

Safety and efficacy of interventional embolization in cirrhotic patients with refractory hepatic encephalopathy associated with spontaneous portosystemic shunts.

This study aimed to assess the safety and efficacy of interventional embolization in cirrhotic patients with refractory hepatic encephalopathy (HE) associated with large spontaneous portosystemic shunts (SPSS). Inverse probability of treatment weighting (IPTW) was employed to minimize potential bias. A total of 123 patients were included in this study (34 in the embolization group and 89 in the control group). In the unadjusted cohort, the embolization group demonstrated significantly better liver function, a larger total area of SPSS, and a higher percentage of patients with serum ammonia levels > 60 µmol/L and the presence of hepatocellular carcinoma (HCC) (all P < 0.05). In the IPTW cohort, baseline characteristics were comparable between the two groups (all P > 0.05). Patients in the embolization group exhibited significantly longer HE-free survival compared to the control group in both the unadjusted and IPTW cohorts (both P < 0.05). Subsequent subgroup analyses indicated that patients with serum ammonia level > 60 µmol/L, hepatopetal flow within the portal trunk, the presence of solitary SPSS, a baseline HE grade of II, and the absence of HCC at baseline showed statistically significant benefit from embolization treatment (all P < 0.05). No early procedural complications were observed in the embolization group. The incidence of long-term postoperative complications was comparable to that in the control group (all P > 0.05). Hence, interventional embolization appears to be a safe and effective treatment modality for cirrhotic patients with refractory HE associated with large SPSS. However, the benefits of embolization were discernible only in a specific subset of patients.

Imaging features, classification and clinical features of intrahepatic congenital portosystemic shunts.

Congenital portosystemic shunts (CPSS) are a rare developmental anomaly diverting blood flow from the portal venous system and the liver to the systemic venous system. This case series examines the sonographic imaging findings, shunt classification, ultrasound shunt ratios, and outcomes in nine children (5 females, 4 males) admitted to our institution between 2015 and 2022 were included in this study. The shunts were initially categorized by the Parks classification and were followed by serial ultrasounds. Clinical presentation, clinical course, laboratory data, shunt ratios, and time to shunt closure were all followed on subsequent ultrasounds. The most common type of CPPS was the Type 3 shunt. In cases where shunt ratios were measured, the shunt ratio gradually decreased in tandem with decreasing ammonia levels until spontaneous closure was achieved. Predictors of lack of shunt closure included high shunt ratios and Type 4 shunts. Patients with CPPS can be followed with the shunt ratio calculation obtained from sonographic imaging, which may correlate to ammonia levels and indicate risk of hepatic encephalopathy as well as predict speed and timing of closure.

Incidental diagnosis of silent extrahepatic portosystemic shunt.

Extrahepatic portosystemic shunts are a very rare disease. Early diagnosis and appropriate treatment are needed to prevent serious complications. In asymptomatic patients, conservative treatment is an option.

Visión general de las várices gástricas y su tratamiento / Overview of gastric varices and their treatment

Las várices gástricas (VG) son un complejo de colaterales vasculares entre la circulación portal y sistémica, condición que se desarrolla como resultado de la presión elevada en el sistema venoso portal. Se encuentran en el 20 % de los pacientes con cirrosis, y son menos frecuentes que las várices esofágicas. Según la clasificación de Sarin, las VG se dividen en cuatro tipos según su ubicación en el estómago y su relación con las várices esofágicas (GOV1, GOV2, IGV1 e IGV2). Entender su hemodinámica con respecto a las rutas de drenaje de las VG es importante para guiar su tratamiento.

Gastric varices (GV) are a complex of vascular collaterals between portal and systemic circulation, a condition that develops as a result of elevated pressure in the portal venous system. They are found in 20% of patients with cirrhosis, and are less common than esophageal varices. According to the Sarin classification, GV are divided into four types based on their location in the stomach and their relationship with esophageal varices (GOV1, GOV2, IGV1, and IGV2). Understanding their hemodynamics with respect to GV drainage routes is important to guide their treatment.

Efficacy and safety of EUS-guided coil embolization in combination with cyanoacrylate injection versus conventional endoscopic cyanoacrylate injection in the treatment of gastric varices with spontaneous portosystemic shunts.

Background: Gastric varices (GV) with spontaneous portosystemic shunts (SPSS) pose considerable risks and challenges for administering endoscopic cyanoacrylate (CYA) injection. This study aimed to evaluate the efficacy and safety of EUS-guided coil embolization in combination with CYA injection compared to conventional endoscopic CYA injection for managing GV with SPSS. Methods: This retrospective analysis included patients with SPSS treated with either EUS-guided coil embolization in combination with CYA injection or conventional CYA injection for gastric variceal bleeding at Ningbo Medical Center Lihuili Hospital (Zhejiang, China) between January 2018 and March 2023. Patient demographics, procedural details, and follow-up results were reviewed. Results: The study evaluated 57 patients: 21 in the combined treatment group undergoing EUS-guided coil embolization in combination with CYA injection and 36 in the conventional group receiving conventional endoscopic CYA injection. Both cohorts achieved a 100% technical success rate. The mean volume of CYA used was significantly lower in the combined group (1.64 ± 0.67 mL) than in the conventional group (2.38 ± 0.72 mL; P < 0.001). Early GV rebleeding rates did not differ significantly between the groups; in contrast, the combined treatment group exhibited a considerably lower incidence of late GV rebleeding than the conventional group (4.8% vs 27.8%, P = 0.041). Conclusions: EUS-guided coil embolization in combination with CYA injection demonstrated superiority over conventional endoscopic CYA injection in reducing late GV rebleeding in treating GV with SPSS.

Prophylaxis of hepatic encephalopathy: current and future drug targets.

Hepatic encephalopathy is described by a broad spectrum of neurological and psychiatric aberrations resulting due to advanced liver dysfunction. It is a neurological disorder due to hepatic insufficiency and/or portosystemic shunts. Its clinical presentation includes neuropsychiatric dysfunction ranging from subclinical changes to comatose state. It is a sign of poor prognosis in cirrhotics with a high 1-year mortality. Each episode of hepatic encephalopathy leads to high hospitalization rate, poor prognosis and raised burden of healthcare. Primary prophylaxis is prevention of initial occurrence and secondary prophylaxis is prevention of reappearance of hepatic encephalopathy in subjects who had prior history. Early detection and management of triggers is very important in the treatment of hepatic encephalopathy. The initial choice of treatment is still lactulose, as it is effective in minimal, overt, and recurrent hepatic encephalopathy. Rifaximin is equally effective as lactulose in managing hepatic encephalopathy and is better tolerated. Branch chain amino acids are beneficial in subjects who are protein intolerant. L-ornithine L-aspartate and probiotics are also useful in the management of hepatic encephalopathy. Rifaximin along with lactulose is effective in managing overt and recurrent hepatic encephalopathy. Large portosystemic shunts embolization and liver transplant is efficacious in certain group of patients. Nutritional therapy and fecal microbiota transplantation are newer therapies for hepatic encephalopathy but the evidences are limited, more research is required to prove their efficacy. Involvement of hospital pharmacists, telemedicine, and providing education are also beneficial in managing hepatic encephalopathy.

Congenital Extrahepatic Portosystemic Shunt Complicated by the Development of Hepatoblastoma: A Case Report and Review of Literature.

Congenital portosystemic shunts (CPSS) or congenital extrahepatic portosystemic shunts (CEPS) is a rare malformation. This congenital anomaly presents with a diverse array of clinical manifestations, ranging from asymptomatic to severe complications such as cardiac failure, pronounced pulmonary hypertension, and widespread pulmonary arteriovenous malformations. CPSS increases the risk of developing benign or malignant liver tumors, including nodular regenerative hyperplasia, focal nodular hyperplasia, hepatic adenoma, hepatocellular carcinoma, and hepatoblastoma. We report a case of a 15-month-old boy, identified with Abernethy's malformation type Ib, who presented with an abdominal mass during a follow-up. A comprehensive assessment established a diagnosis of hepatoblastoma. The patient was transferred to a specialized liver transplant center for further treatment and management. This is a review of literature highlighting the complexity of Abernethy malformation and its associated risk of liver tumors.

Safety and efficacy of transjugular intrahepatic portosystemic shunts vs endoscopic band ligation plus propranolol in patients with cirrhosis with portal vein thrombosis: a systematic review and meta-analysis.

BACKGROUND: This systematic review and meta-analysis aimed to assess the efficacy and safety of transjugular intrahepatic portosystemic shunts (TIPS) against the combined treatment of endoscopic band ligation (EBL) and propranolol in managing patients with cirrhosis diagnosed with portal vein thrombosis (PVT). METHODS: A literature search from inception to September 2023 was performed using MEDLINE, the Cochrane Library, Web of Science, and Scopus. Independent screening, data extraction, and quality assessment were performed. The main measured outcomes were the incidence and recurrence of variceal bleeding (VB), hepatic encephalopathy, and overall survival. RESULTS: A total of 5 studies were included. For variceal eradication, there was initially no significant difference between the groups; however, after sensitivity analysis, a significant effect emerged (risk ratio [RR], 1.55; P < .0001). TIPS was associated with a significant decrease in the incidence of VB (RR, 0.34; P < .0001) and a higher probability of remaining free of VB in the first 2 years after the procedure (first year: RR, 1.41; P < .0001; second year: RR, 1.58; P < .0001). TIPS significantly reduced the incidence of death due to acute GI bleeding compared with EBL + propranolol (RR, 0.37; P = .05). CONCLUSION: TIPS offers a comprehensive therapeutic advantage over the combined EBL and propranolol regimen, especially for patients with cirrhosis with PVT. Its efficacy in variceal eradication, reducing rebleeding, and mitigating death risks due to acute GI bleeding is evident.

4D Flow MRI Reflects Physiological Hemodynamics for the Diagnosis and Management of Portosystemic Shunts.

A woman in her sixties with portosystemic shunt and hepatic encephalopathy underwent open mesenteric vein ligation, resulting in improved portal flow and blood ammonia. In this case, 4D flow MRI was a valuable diagnostic and follow-up tool, visualizing and quantifying physiological portal hemodynamics with features distinct from those of contrast-enhanced CT and digital subtraction angiography. Our case study highlights the value of 4D flow MRI for managing portosystemic shunts.

Umbilical-portal-systemic venous shunt and intrauterine growth restriction: an inquiry from a prospective study.

BACKGROUND: The investigation of the fetal umbilical-portal venous system is based on the premise that congenital anomalies of this system may be related to adverse perinatal outcomes. Several small retrospective studies have reported an association between umbilical-portal-systemic venous shunts and intrauterine growth restriction. However, the prevalence of portosystemic shunts in the fetal growth restricted population is yet to be determined. OBJECTIVE: The aims of this study were (1) to determine the prevalence of fetal umbilical-portal-systemic venous shunts in pregnancies complicated by intrauterine growth restriction and (2) to compare the perinatal and neonatal outcomes of pregnancies with intrauterine growth restriction with and without umbilical-portal-systemic venous shunts. STUDY DESIGN: This was a prospective, cross-sectional study of pregnancies diagnosed with intrauterine growth restriction, as defined by the Society for Maternal-Fetal Medicine intrauterine growth restriction guidelines. All participants underwent a detailed anomaly scan, supplemented with a targeted scan of the fetal portal system. Venous shunts were diagnosed using color Doppler mode. The perinatal outcomes of pregnancies with intrauterine growth restriction with and without umbilical-portal-systemic venous shunts were compared. RESULTS: A total of 150 cases with intrauterine growth restriction were recruited. The prevalence of umbilical-portal-systemic venous shunts in our cohort was 9.3% (n=14). When compared with the control group (intrauterine growth restriction without umbilical-portal-systemic venous shunts, n=136), the study group had a significantly lower mean gestational age at the time of intrauterine growth restriction diagnosis (29.7±5.6 vs 32.47±4.6 weeks of gestation; P=.036) and an earlier gestational age at delivery (33.50±6.0 vs 36.13±2.8; P=.005). The study group had a higher rate of fetal death (21.4% vs 0.7%; P<.001) and, accordingly, a lower rate of live births (71.4% vs 95.6%; P=.001). Additional associated fetal vascular anomalies were significantly more prevalent in the study group than in the control group (35.7% vs 4.4%; P≤.001). The rate of other associated anomalies was similar. The study group had a significantly lower rate of abnormal uterine artery Doppler indices (0% vs 40.4%; P=.011) and a higher rate of abnormal ductus venosus Doppler indices (64.3% vs 23%; P=.001). There were no cases of hypertensive disorders of pregnancy in the study group, whereas the control group had an incidence of 12.5% (P=.16). Other perinatal and neonatal outcomes were comparable. CONCLUSION: Umbilical-portal-systemic venous shunt is a relatively common finding among fetuses with growth restriction. When compared with pregnancies with intrauterine growth restriction with a normal portal system, these pregnancies complicated by intrauterine growth restriction and an umbilical-portal-systemic venous shunt are associated with a different Doppler flow pattern, an increased risk for fetal death, earlier presentation of intrauterine growth restriction, a lower gestational age at delivery, additional congenital vascular anomalies, and a lower rate of pregnancy-induced hypertensive disorders. Meticulous sonographic evaluation of the portal system should be considered in the prenatal workup of intrauterine growth restriction, as umbilical-portal-systemic venous shunts may affect perinatal outcomes.

[Reconstruction of total portosystemic shunt into selective portosystemic shunt in a child]. / Rekonstruktsiya total'nogo portosistemnogo shunta v selektivnyi portosistemnyi shunt u rebenka.

To date, side-to-side splenorenal shunt (SRS) and its analogues (splenosuprarenal shunts (SSRS)) are mainly used for portal hypertension. These are total portosystemic shunts characterized by total blood shunt from portal vein into inferior vena cava. The latter is fraught with a significant risk of complications such as pulmonary hypertension, decreased portal liver perfusion, liver failure and hepatic encephalopathy. Prevention of these complications is still an urgent problem in modern surgery. However, we proposed a new method of treatment, i.e. reconstruction of SRS and SSRS into selective shunt. This procedure was performed in 37 patients after 2020. We present laparoscopic reconstruction in an 11-year-old girl with portal hypertension and signs of hepatic encephalopathy identified after previous SSRS.

To evaluate the prevalence of spontaneous portosystemic shunts in decompensated cirrhosis patients and its prognostic significance.

INTRODUCTION: Spontaneous portosystemic shunts (SPSS) are frequent in liver cirrhosis and their prevalence increases as liver function deteriorates, probably as a consequence of worsening portal hypertension, but without achieving an effective protection against cirrhosis complications. This study was conducted to detect the prevalence of portosystemic shunts in liver cirrhosis patients and analyze its prognostic role. METHOD: We conducted a prospective observational study, where 92 patients with decompensated cirrhosis were evaluated based on history, physical examination, biochemical tests and abdominal computed tomography (CT) angiography findings. A follow-up was done after six months for the development of cirrhosis-related complications. RESULTS: Of the 92 cirrhotic patients, 57.6% had SPSS (large SPSS + small SPSS) detected by multi-detector computed tomographic angiography. Overall, we found large SPSS in 24 (26.1%) patients, small SPSS in 29 (31.5%) patients and no shunt in 39 (42.4%) patients. Among the shunts, the splenorenal shunt is the most frequent type (25, 27.2%) followed by the paraumbilical shunt (20.7%). Previous decompensating events, including hepatic encephalopathy, ascites, spontaneous bacterial peritonitis and gastrointestinal bleed, were experienced more frequently by the large SPSS group followed by the small SPSS and without SPSS groups. Regarding follow-up, decompensating episodes of hepatic encephalopathy developed more frequently in patients with large SPSS (41.7%) than in patients with small SPSS (24.1%) followed by patients without SPSS (12.8%). CONCLUSION: In summary, all cirrhotic patients should be studied with radiological imaging to detect the presence of portosystemic shunts. In several cases, patients with large SPSS had a more impaired liver function and more frequent complications of portal hypertension. So, these patients would probably benefit from a closer surveillance and more intensive therapy.

Repeated specific canine pancreatic lipase measurements do not identify multiple acquired portosystemic shunts in dogs after extrahepatic portosystemic shunt attenuation.

BACKGROUND: In dogs with portal hypertension (PH), spec cPL is suggested to be increased despite normal pancreatic histology. After attenuation of congenital extrahepatic portosystemic shunts (cEHPSS), multiple acquired portosystemic shunt (MAPSS) can develop as consequence of sustained PH. Presence of MAPSS affects future therapeutic options and prognosis. OBJECTIVE: Evaluate if spec cPL concentrations increase postoperatively in dogs that develop MAPSS and can thus serve as an indicator of PH. ANIMALS: Twenty-four dogs with cEHPSS. METHODS: Dogs classified according to surgical outcome after cEHPSS attenuation (8 with MAPSS [group M], 9 with closed cEHPSS [group C] and 7 with patent blood flow through the original cEHPSS, without evidence of MAPSS [group P]). Spec cPL was measured in preoperative samples (T0), 4 days (T1) and 1 (T2) and 3- to 6-months (T3) after surgery. RESULTS: Spec cPL was within reference interval (<200 µg/L) at all timepoints except at T1. At T1, 2 dogs in group M (321 and >2000 µg/L) and also 1 in group C (688 µg/L) and 1 in group P (839 µg/L) had increased spec cPL concentrations. No differences in spec cPL concentrations between groups or changes over time were identified. CONCLUSIONS AND CLINICAL IMPORTANCE: Spec cPL is not consistently increased in dogs that develop MAPSS after cEHPSS attenuation and has no potential as a biomarker for the identification of MAPSS after cEHPSS attenuation.

Pre-transjugular-intrahepatic-portosystemic-shunt measurement of hepatic venous pressure gradient and its clinical application: A comparison study.

BACKGROUND: It is controversial whether transjugular intrahepatic portosystemic shunt (TIPS) placement can improve long-term survival. AIM: To assess whether TIPS placement improves survival in patients with hepatic-venous-pressure-gradient (HVPG) ≥ 16 mmHg, based on HVPG-related risk stratification. METHODS: Consecutive variceal bleeding patients treated with endoscopic therapy + nonselective ß-blockers (NSBBs) or covered TIPS placement were retrospectively enrolled between January 2013 and December 2019. HVPG measurements were performed before therapy. The primary outcome was transplant-free survival; secondary endpoints were rebleeding and overt hepatic encephalopathy (OHE). RESULTS: A total of 184 patients were analyzed (mean age, 55.27 years ± 13.86, 107 males; 102 in the EVL+NSBB group, 82 in the covered TIPS group). Based on the HVPG-guided risk stratification, 70 patients had HVPG < 16 mmHg, and 114 patients had HVPG ≥ 16 mmHg. The median follow-up time of the cohort was 49.5 mo. There was no significant difference in transplant-free survival between the two treatment groups overall (hazard ratio [HR], 0.61; 95% confidence interval [CI]: 0.35-1.05; P = 0.07). In the high-HVPG tier, transplant-free survival was higher in the TIPS group (HR, 0.44; 95%CI: 0.23-0.85; P = 0.004). In the low-HVPG tier, transplant-free survival after the two treatments was similar (HR, 0.86; 95%CI: 0.33-0.23; P = 0.74). Covered TIPS placement decreased the rate of rebleeding independent of the HVPG tier (P < 0.001). The difference in OHE between the two groups was not statistically significant (P = 0.09; P = 0.48). CONCLUSION: TIPS placement can effectively improve transplant-free survival when the HVPG is greater than 16 mmHg.

Diagnostic and monitoring value of ultrasound, computed tomography angiography, and portal venography in surgical ligation of congenital extrahepatic portosystemic shunts.

PURPOSE: To investigate the diagnostic and monitoring value of ultrasound (US), computed tomography angiography (CTA), and portal venography in surgical ligation of congenital extrahepatic portosystemic shunts (CEPS) in children. METHODS: We retrospectively analysed different imaging examinations of 15 children with CEPS. Development of the portal vein before shunt occlusion, shunt location, portal vein pressure, main symptoms, diameter of the main portal vein, and location of secondary thrombosis after shunt occlusion were recorded. Final classification diagnosis was made by portal venography after shunt occlusion, and consistency with other imaging examinations in diagnosing development of the portal vein was calculated using Cohen's kappa. RESULTS: Compared with portal venography after shunt occlusion, US, CTA, and portal venography before shunt occlusion had poor consistency in showing the development of hepatic portal veins (Kappa value 0.091-0.194, P > 0.05). Six cases developed portal hypertension (40-48 cmH2O) during the temporary occlusion test, and US showed that portal veins gradually expanded after shunt ligation. Eight patients with haematochezia had inferior mesenteric vein (IMV)-iliac vein (IV) shunts. After surgery, secondary IMV thrombosis was observed in eight cases and secondary splenic vein thrombosis in four cases. CONCLUSION: Portal venography with occlusion testing is very important to accurately evaluate the development of the portal vein in CEPS. The portal vein needs to expand gradually, and partial shunt ligation surgery is necessary in cases diagnosed as portal vein absence or hypoplasia before occlusion testing to avoid severe portal hypertension. After shunt occlusion, US is effective in monitoring portal vein expansion, and both US and CTA can be used to monitor secondary thrombi. IMV-IV shunts can cause haematochezia and are prone to secondary thrombosis after occlusion.

Advanced Techniques for Accessing the Portal Vein during Transjugular Intrahepatic Portosystemic Shunt Creation.

Transjugular portosystemic shunt (TIPS) placement is a widely accepted method for reducing portal pressures in conditions including symptomatic varices, refractory ascites, hepatic hydrothorax, portomesenteric thrombosis, and Budd-Chiari syndrome. The traditional method for performing TIPS involves "blind" access into a portal vein branch from a hepatic vein, typically right portal vein to right hepatic vein, using preoperative imaging and intraoperative wedged portography for guidance. However, standard access technique may not always be feasible due to occluded portal or hepatic veins, distorted anatomy, or prior TIPS. In this article we discuss alternative techniques for accessing the portal vein for TIPS placement when standard methods are not sufficient.