Noncirrhotic portal hypertension (NCPH) comprises a diverse group of vascular liver disorders characterized by elevated portal pressure without cirrhosis. Due to overlapping clinical features, distinguishing NCPH from cirrhosis and porto-sinusoidal vascular disorder (PSVD) remains challenging. This review explores recent advancements in diagnosis, differentiation, and evolving treatment strategies.

NCPH is characterized by preserved liver function and near-normal hepatic venous pressure gradients (HVPG). It shares risk factors with PSVD, including infections, drugs, toxins, and prothrombotic conditions. Diagnostic advancements, such as liver stiffness measurement (LSM) and splenic stiffness measurement (SSM), offer noninvasive differentiation from cirrhosis, while liver biopsy remains crucial for confirming PSVD and noncirrhotic portal fibrosis (NCPF). Imaging is reliable for diagnosing extrahepatic portal vein obstruction (EHPVO). Transjugular intrahepatic portosystemic shunts (TIPS) for refractory variceal bleeding or ascites, achieving rebleeding control in 72-80% of cases. Surgical shunts and splenectomy remain essential for uncontrolled bleeding and portal biliopathy, demonstrating excellent variceal control (93-95%).

NCPH requires a high index of suspicion for diagnosis. Differentiation from cirrhosis and PSVD relies on clinical, histological, and hemodynamic assessments. Management focuses on endoscopic, interventional, and surgical strategies tailored to disease severity. Future research should standardize diagnostic criteria, explore targeted therapies, and refine prognostic tools to improve outcomes.

Portal cavernoma cholangiopathy (PCC) is a complex condition associated with portal hypertension, particularly in patients with extrahepatic portal vein obstruction (EHPVO). Herein, we present a case of liver failure with PCC in a 55-year-old male successfully treated with living-donor liver transplantation (LDLT). The patient had a history of gastrointestinal bleeding and recurrence of cholangitis. Imaging studies confirmed cavernous transformation and pericholedochal varices. Preoperative angiography verified hepatopetal flow in the pericholedochal varix, which facilitated successful anastomosis with the donor's portal vein during LDLT. Histological examination of the explanted liver confirmed vanishing bile duct syndrome (VBDS) and secondary bile stasis was considered to have caused liver failure. No postoperative complications were observed within 13 months of LDLT. We report the first case of VBDS in the PCC resulting from EHPVO that was successfully managed with LDLT. Careful management of similar cases should involve angiography and long-term postoperative monitoring of portal vein complications.

Patients with obstructive jaundice are conventionally described as hypocoagulable due to vitamin K malabsorption. However, associated underlying malignancy and synthetic liver dysfunction are mediators of hypercoagulability. The actual effect of biliary obstruction on the coagulation profile is not well characterised. This study aimed to define the coagulation status of patients with established biliary obstruction using rotational thromboelastometry (ROTEM).

This prospective cohort study, conducted in an Australian metropolitan hospital, included patients with a total bilirubin level of >150 umol/L and biliary obstruction on imaging. The primary outcome was the coagulation profile assessed using ROTEM.

20 patients were included (median age 74.5 years), 15 were male and 17 had a malignant cause for biliary obstruction. The median bilirubin level was 209 umol/L (IQR: 175.0 umol/L - 255.8 umol/L). On ROTEM, all patients had normal or reduced clot formation times, and normal or increased maximum clot firmness. This confirmed all patients had a normal or hypercoagulable clotting profile, and none were auto-anticoagulated. Vitamin K administration before ROTEM did not vary the coagulation profile.

Patients with established biliary obstruction and jaundice, predominantly due to malignancy, were normo or hypercoagulable. The belief that obstructive jaundice is associated with a hypocoagulable state should be questioned.

Ectopic varices and associated bleeding, although rare, pose a significant risk to patients with portal hypertension, carrying a relatively high mortality rate. These varices can occur in various anatomical regions, excluding the gastroesophageal region, which is typically associated with portal vein drainage. The limited data available in the literature, derived mostly from case reports and series, make the diagnosis and treatment of ectopic variceal bleeding particularly challenging. Furthermore, it is crucial to recognize that ectopic varices in different sites can exhibit variations in key decision-making factors such as aetiology and vascular anatomy, severity and bleeding risk and hepatic reserve. These factors significantly influence treatment strategies and underscore the importance of adopting individualized management approaches. Therefore, the objective of this review is to provide a comprehensive overview of the fundamental knowledge surrounding ectopic varices and to propose site-oriented, stepwise diagnosis and treatment algorithms for this complex clinical issue. A multidisciplinary treatment approach is strongly recommended in managing ectopic varices. In addition, to enhance clinical reference, we have included typical case reports of ectopic varices in various sites in our review, while being mindful of potential publication bias.

More than 20% of patients with extrahepatic portal vein obstruction (EHPVO) may be deemed as nonshuntable due to lack of a suitable vein. The role of "makeshift shunts" or "lesser shunts" assumes importance in such cases. In this report, the authors have shared their experience with the makeshift shunts in the management of portal hypertension in children with emphasis upon anatomic considerations, resolution of symptoms, outcomes after surgery, and shunt patency.

During the period 1983-2018, 138 children with portal hypertension were managed under the care of a single surgeon (VB). Of them, 134 were EHPVO. Children with EHPVO were treated with splenectomy and proximal lienorenal shunt (n = 107), splenectomy and devascularization (n = 21), and makeshift shunts (n = 6). Makeshift shunts comprised (i) side-to-side right gastroepiploic vein (Rt-GEV) to left renal vein (LRV) shunt (n = 1), (ii) superior mesenteric vein (SMV) to inferior vena cava (IVC) shunt using a spiral saphenous venous graft (n = 1), (iii) side-to-side inferior mesenteric vein (IMV) to LRV shunt (n = 2), (iv) side-to-side IMV to IVC shunt (n = 1), (v) end-to-side IMV to IVC shunt (n = 1), and (vi) side-to-side IMV to LRV shunt (n = 1) in a case of crossed fused renal ectopia.

Following the creation of portosystemic shunt, a decline in portal pressure was demonstrated in all six patients. There was resolution of symptoms including hematemesis, melena, and anorectal variceal bleed. None of the patients demonstrated the features of hepatic encephalopathy. The associated portal cavernoma cholangiopathy (n = 1) also resolved following Rt-GEV to LRV shunt. Shunt patency was documented for the entire duration of follow-up (1.5-4 years) in five of six patients; the sixth patient demonstrated shunt block at 6-month follow-up but without recurrence of symptoms.

Makeshift shunts offer a viable alternative to standard portosystemic shunting in pediatric patients with a nonshuntable vein. The selection of such shunts is, however, subject to surgeon's preferences and has to be individualized to local anatomy.

Splanchnic vein thrombosis includes Budd-Chiari syndrome and portal vein thrombosis. These diseases share common features: (i) they are rare diseases and (ii) they can lead to portal hypertension and its complications. Budd-Chiari syndrome and portal vein thrombosis in the absence of underlying liver disease share many risk factors, the most common being myeloproliferative neoplasms. A rapid and comprehensive workup for thrombosis risk factors is necessary in these patients. Long-term anticoagulation is indicated in most patients. Portal vein thrombosis can also develop in patients with cirrhosis, and is associated with a worse course of cirrhosis. Indications for anticoagulation in patients with cirrhosis are increasing. Transjugular intrahepatic portosystemic shunt is a second-line procedure in this setting. Because of the rarity of these diseases, high-level evidence studies are rare. However, collaborative studies have provided a better understanding of their natural history and allowed to improve the management of these patients. This review focuses on the causes, diagnosis, and management of patients with Budd-Chiari syndrome, patients with portal vein thrombosis without underlying liver disease, and patients with cirrhosis and portal vein thrombosis.

Transjugular intrahepatic portosystemic shunting (TIPS) is an established strategy for the management of complications of portal hypertension. Endoprosthetic infection ("endotipsitis") is a rare but serious and difficult-to-treat complication of TIPS placement. Here we report the occurrence of an infected thrombus complicating TIPS placement in a patient with extra-hepatic portal vein obstruction, recurrent variceal bleeding and portal biliopathy accompanied by recurrent cholangitis. Infected thrombotic material within TIPS could be removed only by employing rotational thrombectomy. This procedure revealed the presence of a biliary fistula which carried pathogens in the systemic circulation. The multiple episodes of sepsis did no longer recur following exclusion of the biliary fistula. This case highlights the possibility to use rotational thrombectomy for the management of complex cases of TIPS dysfunction.

There are different therapeutic approaches for biliary strictures and reducing portal hypertension in patients with symptomatic portal cavernoma cholangiopathy (PCC). Endoscopic treatment includes endoscopic biliary sphincterotomy (EST), dilation of stricture with a biliary balloon, placement of plastic stent(s) and stone extraction. Fully covered self-expandable metal stent (FCSEMS) is placed as a rescuer in case of haemobilia seen after EST, dilation of stricture and removal of plastic stent rather than the stricture treatment itself. In this retrospective observational study, we sought to assess the clinical outcomes of FCSEMS as the initial treatment for PCC-related biliary strictures.

Twelve symptomatic patients with PCC both clinically and radiologically between July 2009 and February 2019 were examined. Magnetic resonance cholangiopancreatography (MRCP) and cholangiography were employed as the diagnostic imaging methods. Chandra-Sarin classification was used to distinguish between biliary abnormalities in terms of localization. Llop classification was used to group biliary abnormalities associated with PCC. Endoscopic partial sphincterotomy was performed in all the patients. If patients with dominant strictures 6-8-mm balloon dilation was first performed. This was followed by removal of the stones if exist. Finally, FCSEMS placed. The stents were removed 6-12 weeks later.

The mean age of the patients was 40.9 ± 10.3 years, and 91.6% of the patients were male. Majority of the patients (n = 9) were noncirrhotic. Endoscopic retrograde cholangiopancreatography (ERCP) findings showed that 11 of the 12 patients were Chandra Type I and one was Chandra Type IIIa. All the 12 patients were Llop Grade 3. All patients had biliary involvement in the form of strictures. Stent placement was successful in all patients. FCSEMSs were retained for a median period of 45 days (30-60). Seven (58.3%) patients developed acute cholecystitis. There was no occurrence of bleeding or other complications associated with FCSEMS replacement or removal. All patients were asymptomatic during median 3 years (1-10) follow up period.

FCSEMS placement is an effective method in biliary strictures in case of PCC. Acute cholecystitis is encountered frequently after FCSEMS, but majority of patients respond to the medical treatment. Patients should be followed in terms of the relapse of biliary strictures.

Ectopic variceal bleeding is a rare cause of gastrointestinal bleeding that can occur in settings of cirrhotic and non-cirrhotic portal hypertension and is characterised by its development at locations remote from the oesophagus and stomach. Ectopic varices can be difficult to identify and access, and, although a relatively uncommon cause of portal hypertensive bleeding, can represent a difficult diagnostic and therapeutic challenge associated with considerable mortality. Low incidence and variance in variceal anatomy preclude large randomised controlled trials, and clinical practice is based on experience from case reports, case series, and specialist centre expertise. Optimisation of survival outcomes relies on understanding a patient's portal venous anatomy and functional hepatic reserve to guide timely and targeted endoscopic and endovascular interventions to facilitate the rapid control of ectopic variceal bleeding.

Portal biliopathy (PB) is an abnormality of the biliary tree wall due to extrahepatic portal hypertension. Among the complications of portal biliopathy are digestive bleeding, jaundice, and cholangitis. Surgical treatment is an exception when medical management is not possible.

This is a case series study of four patients with severe PB complications requiring surgical management in our center from 2005 to 2016. Two of them had previous surgical procedures related to portal hypertension. All presented with severe biliary stenosis and recurrent cholangitis, and two also had massive upper gastrointestinal bleeding. Because of endoscopic management failure, a Roux-en-Y hepaticojejunostomy was performed in all cases. Two patients presented morbidity Clavien-Dindo>IIIA, requiring reoperation. During follow-up, no one developed other complications related to PB.

Surgical treatment for PB complications is a challenge and mainly implies a portosystemic shunt as a first step. When it fails, an alternative is perform a biliodigestive anastomoses, with high risk of bleeding given the prominent collaterals present in the hepatoduodenal pedicle secondary to portal cavernomatosis.

Our patients after YRGB didn't present new complications due to PB. The surgery could be a definite solution for PB complications. It has only been made for selective cases because it implies high complexity and risk.

The expression splanchnic vein thrombosis encompasses Budd-Chiari syndrome and portal vein thrombosis. These disorders have common characteristics: they are both rare diseases which can cause portal hypertension and its complications. Budd-Chiari syndrome and portal vein thrombosis in the absence of underlying liver disease share many risk factors, among which myeloproliferative neoplasms represent the most common; a rapid comprehensive work-up for risk factors of thrombosis is needed in these patients. Long-term anticoagulation is indicated in most patients. Portal vein thrombosis can also develop in patients with cirrhosis and in those with porto-sinusoidal vascular liver disease. The presence and nature of underlying liver disease impacts the management of portal vein thrombosis. Indications for anticoagulation in patients with cirrhosis are growing, while transjugular intrahepatic portosystemic shunt is now a second-line option. Due to the rarity of these diseases, studies yielding high-grade evidence are scarce. However, collaborative studies have provided new insight into the management of these patients. This article focuses on the causes, diagnosis, and management of patients with Budd-Chiari syndrome, portal vein thrombosis without underlying liver disease, or cirrhosis with non-malignant portal vein thrombosis.

Polycythemia vera (PV) and non-cirrhotic portal hypertension (NCPH) are relatively independent diseases, and few studies have linked them. However, in clinical settings, there may be a causal relationship. The aim of the present study was to analyze the clinical data of five patients with portal hypertension caused by PV and summarize the characteristics of PV with portal hypertension, to enhance the knowledge of this disease. The clinical data of five patients with PV and portal hypertension treated at Beijing You'an Hospital (Beijing, China) from January 2010 to March 2022 were retrospectively collected. The characteristics of these patients were then summarized and analyzed, including general information, laboratory tests, imaging and gastroscopy data. Overall, four patients were diagnosed with PV earlier compared with those with NCPH (ranging between days and years), whereas one patient was diagnosed with NCPH at the time of PV diagnosis. These four patients had blood cell elevations of 2-3 categories (red blood cells, white blood cells or platelets). The Child classification of liver functions in all five patients were found to be grades A-B. All five patients had splenomegaly, where three patients had portal vein thrombosis and cavernous degeneration. In addition, four patients had moderate or severe esophageal varices. In conclusion, to the best of our knowledge, this was the first case series of NCPH caused by PV. Among the patients, it was revealed that: i) NCPH caused by PV had milder liver function damage compared with cirrhosis-induced portal hypertension; ii) splenomegaly, ascites and esophageal varicose veins were prominent symptoms of NCPH caused by PV; iii) If PV is diagnosed, esophagogastroduodenoscopy should be performed as early as possible and regularly, where primary prevention measures for esophageal variceal hemorrhage are recommended; and iv) patients with PV with portal hypertension are at risk of thrombosis and bleeding, but it remains to be determined whether early antithrombotic therapy can reduce complications.

Sclerosing cholangitis is a chronic cholestatic disease characterized by stricturing, beading, and obliterative fibrosis of the bile ducts. Sclerosing cholangitis is considered primary (PSC) if no underlying etiology is identified or secondary (SSC) if related to another identifiable cause. In this article, we will review the clinical features, pathogenesis, diagnosis, and imaging findings of PSC and SSC, with an emphasis on features that may aid in the distinction of these entities. We will also discuss various etiologies of SSC including recurrent pyogenic cholangitis, other infectious etiologies, ischemic damage, toxic insults, and immunologic, congenital, and miscellaneous causes, highlighting the unique imaging findings and clinical context of each diagnosis.

Portal cavernoma cholangiopathy is defined as an obstruction of the biliary system due to distended veins surrounding bile ducts that mainly occur in patients with extrahepatic portal venous obstruction. The periductal venous plexuses encircling the ducts can cause morphological changes which may or may not become symptomatic. Currently, non-invasive techniques such as ultrasonography, computed tomography, magnetic resonance cholangiopancreatography, and dynamic contrast enhanced magnetic resonance images are being used to diagnose this disorder. Only a few patients who have symptoms of biliary obstruction require drainage which might be accomplished using endoscopic stenting, decompression of the portal venous system usually via a lienorenal shunt, a difficult direct hepaticojejunostomy, and rarely a liver transplant.

Shunt surgery is the definitive treatment for symptomatic patients with portal cavernoma cholangiopathy (PCC), but few patients are non-surgical candidates or fail to improve even after surgery. This study aims to analyze the long-term outcomes of endoscopic therapy in these patients.

Retrospective review of a prospectively maintained database of all patients with symptomatic PCC managed with endoscopic retrograde cholangiography (ERC) followed by stent placement. Outcomes studied included number of biliary interventions, complications, resolution of stricture, development of decompensation and mortality.

Thirty-five patients (68.6% males, median age = 35 years) with a median follow-up duration of 46 months (12-112) were included in the analysis. Presentation was only jaundice in 51.4% cases while one-third (37.1%) of the patients presented with cholangitis. Patients underwent a total of 363 endoscopic sessions with a median of 9 procedures (3-29) per patient. Hemobilia was the most common complication of the procedure (6.06%). Ten (28.5%) patients required frequent stent exchanges. Patients who required frequent stent exchanges had higher number of cholangitis episodes and hospitalization. Secondary biliary cirrhosis developed in 4 (11.4%) patients and 2 (5.7%) patients had mortality. Of the 5 (14.3%) patients who were given a stent free trial, 3 patients required restenting due to redevelopment of symptoms.

Patients with PCC without shuntable veins for surgery or those who failed to improve after surgery can be managed long-term with repeated endoscopic intervention with a slightly increased risk of non-fatal hemobilia.

Portal hypertensive biliopathy (PHB) was caused by anatomical and functional abnormalities in the intrahepatic and extrahepatic bile ducts secondary to portal hypertension. Currently, there is no consensus regarding to the optimal treatment for PHB. Transjugular intrahepatic portosystemic shunt (TIPS) is the treatment choice for the management of symptomatic PHB, however, it could be very difficult in patients with PHB and cavernous transformation of portal vein.

We report a case of PHB, successfully managed with TIPS. A 23-year-old man with liver cirrhosis presented with jaundice. Magnetic resonance cholangiopancreatography (MRCP) showed multiple tortuous hepatopetal collateral vessels compressing the common bile duct (CBD) and leading to the dilated proximal bile duct. He was diagnosed with PHB and treated with TIPS. A guidewire was inserted into the appropriate collateral vessel through transsplenic approach to guide intrahepatic puncture and TIPS was performed successfully. After the operation, portal vein pressure decreased and the symptoms of biliary obstruction were relieved significantly. In addition, the patient showed no jaundice at a follow-up of one year.

For PHB patients presenting for cavernous transformation of the portal vein, which precludes the technical feasibility of TIPS, a combined transjugular/transsplenic approach could be an alternative option.

Intestinal ischemia/reperfusion (I/R) injury has high morbidity and mortality rates. Gut microbiota is a potential key factor affecting intestinal I/R injury. Populations exhibit different sensitivities to intestinal I/R injury; however, whether this interpopulation difference is related to variation in gut microbiota is unclear. Here, to elucidate the interaction between the gut microbiome and intestinal I/R injury, we performed 16S DNA sequencing on the preoperative feces of C57BL/6 mice and fecal microbiota transplantation (FMT) experiments in germ-free mice. The transwell co-culture system of small intestinal organoids extracted from control mice and macrophages extracted from control mice or Toll-like receptor 2 (TLR2)-deficient mice or interleukin-10 (IL-10)-deficient mice were established separately to explore the potential mechanism of reducing intestinal I/R injury.

Intestinal I/R-sensitive (Sen) and intestinal I/R-resistant (Res) mice were first defined according to different survival outcomes of mice suffering from intestinal I/R. Fecal microbiota composition and diversity prior to intestinal ischemia differed between Sen and Res mice. The relative abundance of Lactobacillus murinus (L. murinus) at the species level was drastically higher in Res than that in Sen mice. Clinically, the abundance of L. murinus in preoperative feces of patients undergoing cardiopulmonary bypass surgery was closely related to the degree of intestinal I/R injury after surgery. Treatment with L. murinus significantly prevented intestinal I/R-induced intestinal injury and improved mouse survival, which depended on macrophages involvement. Further, in vitro experiments indicated that promoting the release of IL-10 from macrophages through TLR2 may be a potential mechanism for L. murinus to reduce intestinal I/R injury.

The gut microbiome is involved in the postoperative outcome of intestinal I/R. Lactobacillus murinus alleviates mice intestinal I/R injury through macrophages, and promoting the release of IL-10 from macrophages through TLR2 may be a potential mechanism for L. murinus to reduce intestinal I/R injury. This study revealed a novel mechanism of intestinal I/R injury and a new therapeutic strategy for clinical practice. Video Abstract.

Portal cavernoma cholangiopathy (PCC) refers to abnormalities of the extra- and intrahepatic bile ducts in patients with portal cavernoma. The literature on PCC in children is very scarce. This study aimed at characterizing PCC in children with extrahepatic portal venous obstruction (EHPVO) using endoscopic ultrasound (EUS) and magnetic resonance cholangiography/portovenography (MRC/MRPV).

A total of 53 consecutive children diagnosed with EHPVO were prospectively evaluated for PCC using MRC/MRPV and EUS. Chandra classification was used for type of involvement and Llop classification for grading of severity.

All 53 children (100%) had PCC changes on MRC/EUS, but none were symptomatic. Extrahepatic ducts (EHDs) and intrahepatic ducts were involved in majority (85%), and 58.5% had severe changes. Periductal thickening/irregularity (71%) was the commonest change in intrahepatic ducts, whereas irregular contour of the duct with scalloping (68%); common bile duct (CBD) angulation (62.3%) were the frequent changes in the EHDs. Increased CBD angulation predisposed to CBD strictures (P = 0.004). Both left and right branches of portal vein were replaced by collaterals in all children. Among the EUS biliary changes, para-pericholedochal, intrapancreatic, and intramural gall bladder collaterals had significant association with severity, with higher frequency of occurrence in children with the most severe Llop Grade.

PCC develops early in the disease course of EHPVO, in children, but is asymptomatic despite severe changes. EUS biliary changes are more likely to be observed with increasing severity of PCC.

Altemeier-Klatskin tumor is a perihilar cholangiocarcinoma that occurs within 2 cm of the confluence of the right and left hepatic duct at the hepatic hilum and accounts for 50-70% of all cholangiocarcinomas cases. Although imaging techniques have come very far today, this entity can still be very challenging to diagnose as there are many lesions that can mimic Klatskin tumor. In this review, we will present the most common Klatskin-mimicking lesions.

Extrahepatic portal vein thrombosis (PVT) is the most common cause of portal hypertension (PH), particularly in children. PH-related manifestations include refractory variceal bleeding, splenomegaly and ascites. Albeit more rarely performed, the distal splenorenal shunt (Warren's shunt) has proven to be effective in selectively decompressing the collateral circulation. The aim of our study was to describe our experience with the distal splenorenal shunt and to determine the long-term effect on PH-related side-effects.

Distal splenorenal shunt operations performed at our institution between 2000 and 2014 were reviewed for: age, male/female ratio, children/adults ratio, body mass index, indications, grade of PVT (Yerdel classification), maximal shunt-flow velocity, shunt patency and thrombosis, re-intervention for variceal bleeding and survival. Complications of PH (esophageal variceal bleeding and ascites) were compared pre- versus post-operatively (last follow-up). Paired student t-test and fisher's exact were applied for pre- versus post-operative comparison. Results are reported as median [range].

Fourteen patients with PVT and refractory complications of PH underwent distal splenorenal shunt surgery. Age was 15 years [4.5-66]. Male/female ratio was 7/7. PVT -grade was 2 [1-4]. Follow-up was 3 [0.5-14]. All shunts were patent (100%) with no shunt thrombosis (0%) at last follow-up. There was no re-intervention for variceal bleeding (0%) and survival at last follow-up was 100%. Occurrence of esophageal variceal bleeding was higher pre-operatively (57%) than postoperatively (0%) (p = .0032) and also the incidence of ascites was higher pre-operatively (79%) than postoperatively (0%) (p < .0001).

Based on our experience, the distal splenorenal shunt can be considered a valuable surgical technique for PVT-induced PH, with excellent post-operative prevention of complications of PH.

Laparoscopic cholecystectomy (LC) in patients with extrahepatic portal vein obstruction causing portal cavernoma (PC) is considered high risk because of portosystemic collateral veins in the hepatocystic triangle. The literature is limited to isolated case reports. We describe our experience of LC in patients with PC.

Data of patients with PC who underwent LC for symptomatic gallstones or related complications was reviewed. Patients with simultaneous cholecystectomy with splenorenal shunt and open cholecystectomy were excluded. Pre-operative evaluation consisted of complete blood count, international normalisation ratio and liver function tests, ultrasound of the abdomen with Doppler, contrast-enhanced computerised tomography, magnetic resonance cholangiopancreatography and esophagogastroscopy as indicated. A standard four-port LC was performed. The technical principles followed were to avoid injury to the collateral veins, liberal use of energy sources and division of dominant collateral veins between clips.

Seven adult patients including three females underwent LC. Three patients had thrombosis of previous surgical shunt with persistent PC. The remaining four patients did not have any indication for shunt surgery. Successful LC was performed in six patients. The median duration of surgery was 170 (130-250 min). Blood transfusion was not required. All the patients had uneventful post-operative recovery. The histopathology of gall bladder consists of acute cholecystitis in three patients and chronic cholecystitis in four.

LC is feasible in patients with PC at a centre with experience in both laparoscopic and portal hypertension surgeries. Excellent outcome with low rate of conversion to open surgery can be achieved.

While portal vein thrombosis (PVT) is a frequently encountered complication in the cirrhosis population, its management can be challenging for even the most experienced clinicians. Multiple factors must be considered with regards to management, including the degree of underlying portal hypertension and liver dysfunction, risks of therapies including anticoagulation and transjugular intrahepatic portosystemic shunt placement, and extent of the thrombosis. Interpreting the available literature to determine the best treatment strategy for any individual patient can be especially challenging given the lack of prospective, randomized controlled trials and the heterogeneity of cohorts studied. This review will provide an overview of PVT in the cirrhosis population, including necessary steps in evaluation and the potential benefits and drawbacks of different treatment approaches.

Nontumoral portal vein thrombosis (PVT) is an increasingly recognized complication in patients with cirrhosis. Substantial evidence shows that portal flow stasis, complex thrombophilic disorders, and exogenous factors leading to endothelial dysfunction have emerged as key factors in the pathogenesis of PVT. The contribution of PVT to hepatic decompensation and mortality in cirrhosis is debatable; however, the presence of an advanced PVT increases operative complexity and decreases survival after transplantation. The therapeutic decision for PVT is often determined by the duration and extent of thrombosis, the presence of symptoms, and liver transplant eligibility. Evidence from several cohorts has demonstrated that anticoagulation treatment with vitamin K antagonist or low molecular weight heparin can achieve recanalization of the portal vein, which is associated with a reduction in portal hypertension-related events and improved survival in cirrhotic patients with PVT. Consequently, interest in direct oral anticoagulants for PVT is increasing, but clinical data in cirrhosis are limited. Although the most feared consequence of anticoagulation is bleeding, most studies indicate that anticoagulation therapy for PVT in cirrhosis appears relatively safe. Interestingly, the data showed that transjugular intrahepatic portosystemic shunt represents an effective adjunctive therapy for PVT in cirrhotic patients with symptomatic portal hypertension if anticoagulation is ineffective. Insufficient evidence regarding the optimal timing, modality, and duration of therapy makes nontumoral PVT a challenging consequence of cirrhosis. In this review, we summarize the current literature and provide a potential algorithm for the management of PVT in patients with cirrhosis.

Single-stage biliary decompression without prior shunt surgery, although risks for catastrophic bleeding, it is feasible to perform upfront hepaticojejunostomy in a splenectomized patient where shuntable vein is not available in patient with portal biliopathy.

Portal cholangiopathy is one of the complications of the chronic portal vein thrombosis (PVT). Chronic PVT can occur in a patient with acute PVT that usually does not resolve regardless of the treatment. There is a development of collateral blood vessels that bring blood from the portal system towards the liver around the obstruction area, known as the cavernous transformation of the portal vein or portal cavernoma, in a patient with chronic PVT. The appearance and location of collateral channels depends on the extent and location of thrombus in the portomesenteric venous system. If the portomesenteric venous system is occluded near the formation of the portal vein, blood tends to flow through collateral channels that form varices in and around the common bile duct. Portal cholangiopathy (also referred to as portal biliopathy) is common in patients with long-standing chronic PVT. It is due to compression of the large bile ducts by the venous collaterals that form in patients with chronic PVT. Most of the patients with long-standing PVT have portal cholangiopathy. Typically, symptoms of portal cholangiopathy include jaundice, biliary colic, and pruritus. Portal cholangiopathy is a rare complication of chronic portal hypertension, and it is an important differential diagnosis of biliary colic secondary to cholelithiasis. The patient can also present with the sharp right upper quadrant pain, which is atypical by nature for biliary colic.

Chronic non cirrhotic extrahepatic portal vein obstruction (EHPVO) refers to the cavernomatous transformation of the portal vein (the so-called "portal cavernoma") which occurs following acute thrombosis of the portal vein in the absence of recanalization. In adults, EHPVO mainly occurs following thrombosis, while in children it may be related to congenital malformations and/or neonatal umbilical venous catheterization. However, 50% of the cases of EHPVO remain idiopathic [1]. Risk factors and associated diseases should be investigated (chapter 1). Indeed, the presence of a thrombophilic alteration, in particular myeloproliferative neoplasm impacts prognosis and determine a causal treatment.

Cholangiopathies describe a group of conditions affecting the intrahepatic and extrahepatic biliary tree. Impairment to bile flow and chronic cholestasis cause biliary inflammation, which leads to more permanent damage such as destruction of the small bile ducts (ductopaenia) and biliary cirrhosis. Most cholangiopathies are progressive and cause end-stage liver disease unless the physical obstruction to biliary flow can be reversed. This review considers large-duct cholangiopathies, such as primary sclerosing cholangitis, ischaemic cholangiopathy, portal biliopathy, recurrent pyogenic cholangitis and Caroli disease.

To investigate the liver parenchymal changes on MRI in patients with extrahepatic portal venous obstruction (EHPVO).

This was a retrospective evaluation of the MRI studies in patients with EHPVO between January 2016 and April 2018. The diagnosis of EHPVO in each case had been established on the basis of clinical and Doppler parameters. Following findings were recorded on MRI: liver size; outline; overall volume redistribution; volume redistribution of caudate, right and left lobes; signal heterogeneity, intrahepatic biliary radicle dilatation, focal liver lesions, gallstones and ascites.

A total of 164 MRI studies were evaluated. Median age was 27 years (range, 6-70). There were 90 (54.9%) males and 74 (45.1%) females. The median liver size was 14 cm (range 5-17). The median spleen size was 15.9 cm (range, 6-28). A spleen size ≥12 cm was seen in 136 (82.9%) patients. The liver outline was nodular in 32 (19.5%) patients. Volume redistribution was seen in 63 (38.4%) patients. Caudate lobe hypertrophy was seen in 49 (29.9%) patients. Right lobe atrophy and hypertrophy was seen in 4 (2.4%) and 1 (0.6%) patients respectively. Left lobe atrophy and hypertrophy was seen in 15 (9.1%) and 10 (6.1%) patients respectively. 30 MRI (18.3%) showed heterogeneous signal intensity. Focal lesions were seen in 22 (13.4%) patients. Intrahepatic biliary radicle dilatation, gallstones and ascites were seen in 120 (73.2%), 54 (32.9%) and 26 (15.9%) patients respectively.

Liver morphological changes are common in EHPVO.

The morphological changes in liver in patients with EHPVO have not been described previously. This information will prevent misdiagnosis of this condition as cirrhosis.

Portal vein thrombosis unrelated to solid malignancy is common in patients with cirrhosis, but less frequently observed in patients without cirrhosis. Prompt diagnosis and management of acute symptomatic portal vein thrombosis are essential. Failure to detect and treat thromboses can result in mesenteric ischemia, chronic cavernous transformation, and complications of portal hypertension. In patients with cirrhosis, development of portal vein thrombosis is often insidious and remains undetected until its incidental detection. Management of portal vein thrombosis in patients with cirrhosis is more controversial. However, there are data to support treatment of specific patients with anticoagulation agents. We review the common and distinct features of portal vein thromboses in patients without liver tumors, with and without cirrhosis.

The hepatoduodenal ligament is frequently involved by conditions affecting the portal triad and surrounding structures, including a vast array of non-neoplastic conditions. Due its unique location between the retroperitoneum and the peritoneal space, the hepatoduodenal ligament is also targeted by inflammatory conditions involving the retroperitoneum and the liver. Finally, the presence of lymphatics and of the biliary tracts makes the hepatoduodenal ligament a route of spread for a variety of infections. The purpose of this pictorial essay is twofold: to review the cross-sectional radiological anatomy and variants of the structures within the hepatoduodenal ligament, and to illustrate the non-neoplastic conditions that may arise within the hepatoduodenal ligament.

Familiarity with these specific entities and their cross-sectional imaging findings is fundamental for a more accurate diagnosis.

Portal hypertensive biliopathy may occur in patients with noncirrhotic hepatic fibrosis. Portal hypertensive biliopathy treatment should be focused on management of portal hypertension and relief of biliary obstruction. In patients with noncirrhotic portal fibrosis and symptomatic portal hypertensive biliopathy, portal decompression surgery by proximal splenorenal shunt is one successful treatment option.

Portal cavernoma cholangiopathy (formerly portal biliopathy) is a type of biliary injury that occurs in association with a portal vein thrombus or cavernoma. Although the radiographic features of portal cavernoma cholangiopathy have been enumerated in the literature, its histologic features have not been described in detail.

We describe the histologic findings in liver specimens from three patients with radiologically confirmed portal cavernoma cholangiopathy.

Of the three patients, one underwent surgical resection due to a clinical suspicion for cholangiocarcinoma, one had a liver biopsy sample obtained for evaluation of possible cirrhosis, and one had a clinically suspicious "hilar mass" at the time of orthotopic liver transplant. Histologic features common among the three liver specimens included portal venous abnormalities, where the portal veins were obliterated or small relative to the portal tract size, and obstructive biliary changes, such as ductular reaction and reactive epithelial atypia accompanied by a mixed inflammatory cell infiltrate with neutrophils.

This case series provides clinicopathologic characteristics of portal cavernoma cholangiopathy. Histologic changes are reminiscent of hepatoportal sclerosis and/or bile duct obstruction. Attention to portal veins can provide helpful diagnostic clues, especially when biopsy samples are obtained from patients with a known portal vein thrombus or cavernoma.

This is a case with associated radiologic images for a pediatric patient who developed portomesenteric and splenic vein thrombosis (PMSVT) after Roux-en-Y gastric bypass with subsequent development of portal cavernoma and gallbladder varices (GBV). This case highlights both the importance of post-operative prophylactic anti-coagulation after gastric bypass and detailed imaging following a diagnosis of PMSVT. This case is relevant for pediatric surgeons as they are performing this operation more frequently with the increase in pediatric obesity.

Portal cavernoma cholangiopathy (PCC), a surgical-endoscopic dilemma, has not been studied comprehensively, more so in children. Our study aimed to evaluate PCC in children using a combination of magnetic resonance cholangiography-portovenography (MRC-MRPV) and endoscopic ultrasonography (EUS).

In this prospective cross-sectional study, recruited children with extrahepatic portal venous obstruction (EHPVO) underwent MRC-MRPV and radial array EUS. PCC was categorized as asymptomatic PCC, symptomatic and no-PCC. Modified Llop grading was used to grade the MRC changes.

Sixty-six of 72 (92%) children had PCC (85% asymptomatic; 7% symptomatic) on MRC. Age at study and duration of disease had significant correlation (r = 0.588, P < 0.001). 63% had grade III MRC changes. MRC grades and superior mesenteric vein block (64%) on MRPV significantly corresponded with EUS changes (intracholedochal varices, choledochal perforators, intramural cholecystic collaterals and biliary calculi). Superior mesenteric vein non-patency was a strong predictor of MRC biliary changes (P = 0.003, odds ratio 46.4, 95% confidence interval 4.91-623.6).

A majority of EHPVO children have asymptomatic cholangiopathy and should be routinely evaluated for PCC at the time of first presentation by MRC-MRPV. Additional superior mesenteric vein block with portal cavernoma results in significantly higher changes of cholangiopathy on MRC and EUS.

Hemobilia refers to bleeding from and/or into the biliary tract and is an uncommon cause of gastrointestinal hemorrhage. Hemobilia has been documented since the 1600s, but due to its relative rarity, it has only been more critically examined in recent decades. Most cases of hemobilia are iatrogenic and caused by procedures involving the liver, pancreas, bile ducts, and/or the hepatopancreatobiliary vasculature, with trauma and malignancy representing the two other major causes. A classic triad of right upper quadrant pain, jaundice, and overt upper gastrointestinal bleeding has been described, but this is present in only 25-30% of patients with hemobilia. Historically, the gold standard for diagnosis and treatment has been angiography and interventional radiologic intervention, respectively. However, the paradigm is shifting, at least in select cases, towards first-line reliance on noninvasive imaging (e.g., computed tomography) and therapeutic endoscopy, owing to advances in and the less invasive nature of both, while saving interventional radiological and/or surgical intervention for refractory or imminently life-threatening cases.

Portal vein thrombosis (PVT) is a common complication in cirrhosis. The aim of this study was to determine risk factors for PVT, assess the efficacy of anticoagulant therapy, and evaluate the effects of PVT on patients with cirrhosis undergoing elective transjugular intrahepatic portosystemic shunt (TIPSS). A total of 101 patients with cirrhosis undergoing elective TIPSS were prospectively studied. After TIPSS, all patients received preventive therapy for PVT and were followed up at 3, 6, 12, and 24 months. Clinical outcomes were compared between patients who developed PVT after TIPSS and those who did not. Multivariate analysis showed that white blood cell count (relative risk [RR]: 0.377; 95% confidence interval [CI]: 0.132-0.579; P = .001), Child-Turcotte-Pugh score (RR: 1.547; 95% CI: 1.029-2.365; P = .032), and ascites (RR: 1.264; 95% CI: 1.019-1.742; P = .040) were independent predictors for PVT. Warfarin treatment within 12 months achieved significantly higher rates of complete recanalization than aspirin or clopidogrel in patients with PVT (54.5% vs 31.3%; P = .013), although adverse events were similar between the 2 groups ( P > .05). Patients without PVT had significantly lower 2-year cumulative rates of variceal rebleeding (15.9% vs 36.6%; P = .023), shunt dysfunction (27.0% vs 46.8%; P = .039), hepatic encephalopathy (24.1% vs 42.6%; P = .045), and hepatocellular carcinoma (11.4% vs 31.2%; P = .024) and markedly higher 2-year cumulative survival rates (89.8% vs 72.9%; P = .041) than those with PVT. The PVT is associated with poorer clinical outcomes in TIPSS-treated patients, and warfarin is both safe and more effective in recanalizing PVT than aspirin or clopidogrel.