Arterio-portal shunt in liver rescued by hepatectomy after arterial embolization

Arterio-portal shunts are generally treated with transcatheter arterial embolization, as a therapeutic measure for bleeding of esophageal varices. However, transcatheter arterial embolization is frequently associated with reestablishment of arterio-portal shunts. We now report our experience with partial hepatectomy to remove the arterio-portal shunt associated with esophageal varices, which recurred after transcatheter arterial embolization. The patient was a 60-year-old female, who had massive hematemesis caused by rupture of esophageal varices. Doppler sonography and arteriography demonstrated an arterio-portal shunt in the right anterior superior segment of the liver. Temporary hemostasis was achieved with transcatheter arterial embolization, however, hemorrhage recurred one month later. The second transcatheter arterial embolization failed to manage the shunt and varices. The patient developed hepatic coma. After recovery from coma, she was referred to our hospital. We carried out partial hepatectomy, which provided remarkable hemodynamic improvement; the portal vein flow changed from hepatofugal to hepatopetal. Esophageal varices and hepatic coma have totally disappeared. This patient has had no complaint and has remained free of esophageal varices, for 3 years postoperatively. She is having a normal life. The partial hepatectomy to remove the arterio-portal shunt induced complete resolution of the arterio-portal shunt, as well as dramatic improvement in portal flow and hepatic coma. Our experience in the present case suggests that partial hepatectomy should be considered as a radical therapy for arterio-portal shunt, without insistence on transcatheter arterial embolization.     

Portal vein obstruction complicating intra-arterial chemo-infusion for hepatic metastases

In three patients with colon cancer and liver metastases who had received intra-arterial chemo-infusion of fluorouracil (5FU) and mitomycin C and/or cis-diamminedichloroplatinum (CDDP), intrahepatic portal vein thrombosis (PVT) was incidentally demonstrated by computerized tomography (CT) 6, 1 and 7 months respectively after the cessation of administration of anti-cancer agents. One patient developed complete PVT in the whole liver as shown by follow-up CT 6 months after a diagnosis of pre-existing sclerosing cholangitis, and died from rupture of oesophageal varices. In the remaining two patients, PVT was found incidentally by follow-up CT in the right portal vein (Case 2) and the right anterior portal vein (Case 3) respectively; it spontaneously recanalized in Case 2 and was still present in Case 3 2 months later. PVT seems to be one of the complications of hepatic arterial chemo-infusion and its possibility must be borne in mind in such patients, even though the exact interval between the arterial chemo-infusion and occurrence of PVT could not be determined.     

Median liver lobe of woodchuck as a model to study hepatic outflow obstruction: a pilot study

Background: Hepatic vein outflow obstruction represents an important clinical problem in living‐liver transplantation. An animal model is required to study the influence of outflow obstruction on the intrahepatic regulation of liver perfusion and the subsequent effects on liver injury and recovery during liver regeneration. The size of woodchucks enables the use of standard clinical imaging procedures. Aim: This study aims at describing hepatic vascular and territorial anatomy of the woodchuck liver based on a virtual three‐dimensional (3D) visualization of the hepatic vascular tree. Methods: Woodchucks (n=6) were subjected to an all‐in‐one computed tomography (CT) after contrasting the vascular and the biliary tree. CT‐images were used for 3D‐reconstruction of hepatic and portal veins and calculation of the corresponding portal and hepatic vein territories and their respective volume using hepavision (MeVisLab). A virtual resection was performed following the Cantlie‐line and territories at risk were calculated. Results: The median lobe of the woodchuck liver has a similar vascular supply and drainage as the human liver with two portal (right and left median portal vein) and three hepatic veins (left, middle and right median hepatic vein). The corresponding portal and hepatic vein subterritories are of a similar relative size compared with the human liver. Virtual splitting of the median lobe of the woodchuck liver revealed areas at risk of focal outflow obstruction, as observed clinically. Conclusion: The median liver lobe of the woodchuck represents, to a small extent, the hepatic vascular anatomy of the human liver and is therefore a suitable potential model to correlate repeated imaging of impaired liver perfusion with histomorphological findings of liver damage and regeneration.     

Human hepatic infarction: histopathological and postmortem angiological studies

ABSTRACT— Twenty hepatic infarction cases selected from 5420 consecutive autopsy cases were investigated to clarify the pathogenetic aspects of this disease. Additional postmortem angiological studies of 24 normal human livers obtained at autopsy were also further performed to analyse the effects of blocking vascular structures on lesion development. Seventeen of the 20 cases (85%) were clinically associated with systemic circulatory insufficiency, especially hepato- and/or renal failure. Histopathologically, there was a significantly closer relationship between the location of infarcted regions and portal vein thrombosis than with either hepatic vein thrombosis or hepatic arterial damage. The borders between infarcted regions and surviving hepatic parenchyma were located around central veins, corresponding with the microcirculatory periphery of the portal venous system. Postmortem angiographic studies revealed that hepatic lobuli mainly consist of portal vein branches. Moreover, postmortem embolization studies of six normal livers using glass beads and bariumgelatin injection showed that physical occlusion of portal vein branches produced defects in broad areas of the hepatic parenchyma. Therefore, it is suggested that the development of hepatic infarction principally depends on disturbances of the portal venous system. In addition, systemic circulatory insufficiency, which reduces the intrahepatic blood flow, probably contributes greatly to the development of hepatic infarction.     

Comparison of hemodynamic changes in two veno-venous bypass techniques modified at the portal cannulation site

Veno-venous bypass under total vascular exclusion is a useful technique to permit safer resection of hepatic malignancy. We describe here a retrospective study of two modified venous bypass techniques as alternatives to the conventional end-on portal cannulation technique. Portal decompression via inferior mesenteric vein access was performed in eight patients (group A), and portal decompression via a passive shunt between a branch of the mesenteric vein and the right saphenous vein was performed in a second group (group B;n = 8). Both techniques were used in hepatic resection for malignancy under total vascular exclusion. To assess the efficacy of these bypass techniques, we compared the hemodynamic changes in the two groups. There were no differences in the bypass flow between the two groups. Neither group showed any significant changes in hemodynamic parameters (including mean arterial pressure, cardiac index, systemic vascular resistance index, and pulmonary artery pressure) between the pre-bypass and bypass phases. The heart rate in the bypass phase was significantly increased compared to that in the pre-bypass phase in both groups. All hemodynamic parameters in each phase were similar in the two groups. We conclude that both techniques maintained adequate venous return and stabilized the hemodynamic changes during hepatic resection under total vascular exclusion, and that either technique can be selected according to the intraoperative situation.     

Is portal vein cavernous transformation a component of congenital hepatic fibrosis？

Congenital hepatic fibrosis （CHF） is an autosomal recessive disorder that belongs to the family of fibropolycystic liver diseases. This family includes a spectrum of disorders which are usually found in combination with each other and are usually inherited. Clinically fibropolycystic diseases have three effects being present in different proportions, those of a space occupying lesion, of portal hypertension and of cholangitis. In most patients, the first manifestations of CHF are signs and symptoms related to portal hypertension such as splenomegaly and varices. Portal hypertension in these patients has been attributed to the hypoplasia or compression of the portal vein radicles in the fibrous bands. Cavernous transformation of the portal vein （CTPV） is a relatively rare condition resulting from extrahepatic portal vein obstruction with recanalization or collateral vein formation to bypass the obstruction. It has been found that patients with CHF having an accompanying CTPV have relatively large splenomegaly and suffers more frequent episodes of bleeding from esophageal varices.We believe that CTPV is a congenital component of CHF and also one of the important causative factors of portal hypertension in these patients.     

The incidence of portal vein thrombosis at liver transplantation.

The incidence of portal vein thrombosis was examined in 885 patients who received orthotopic liver transplantations for various end-stage liver diseases between 1989 and 1990. The thrombosis was classified into four grades. Grade 1 was thrombosis of intrahepatic portal vein branches, grade 2 was thrombosis of the right or left portal branch or at the bifurcation, grade 3 was partial obstruction of the portal vein trunk, and grade 4 was complete obstruction of the portal vein trunk. Among the 849 patients without previous portosystemic shunt, 14 patients (1.6%) had grade 1, 27 patients (3.2%) had grade 2, 27 patients (3.2%) had grade 3 and 49 patients (5.8%) had grade 4 portal vein thrombosis. The incidence of portal vein thrombosis was highest (34.8%) in the patients with hepatic malignancy in the cirrhotic liver, followed by those with Budd-Chiari syndrome (22.2%) and postnecrotic cirrhosis of various causes (15.7%). The patients with encephalopathy, ascites, variceal bleeding, previous splenectomy and small liver had significantly higher incidences of portal vein thrombosis than the others. The total incidence of portal vein thrombosis among the 36 patients with previous portosystemic shunt was 38.9%, which was significantly higher than that (13.8%) of those without shunt.     

In vivo assessment of the hepatic microcirculation after mesenterico‐portal bypass (REX‐shunt) using orthogonal polarization spectral imaging

Background: Extrahepatic portal vein thrombosis, not associated with cirrhosis or tumours, is the second most frequent cause of portal hypertension worldwide. Especially in children, anatomic mesenterico‐portal interposition (REX‐shunt) has become an established treatment. The changes in hepatic microcirculation after reperfusion of the shunt have not been investigated so far. Aims: This study investigates the hepatic microcirculation before and after REX‐shunt interposition using orthogonal polarization spectral imaging (OPS). Patients and methods: Since 2004, three consecutive patients with extrahepatic portal vein thrombosis underwent REX‐shunt interposition. We measured the hepatic microcirculation by OPS before and directly after REX‐shunt reperfusion and analysed the capillary vessel diameter, red blood cell velocity, functional capillary density and volumetric blood flow. Furthermore, we compared our values with the physiological values of the hepatic microcirculation defined previously by other investigators. Results: All shunts showed an excellent function in the follow‐up investigations. The intra‐individual microcirculatory analysis revealed a reduction in the red blood cell velocity after shunt reperfusion in particular. Conclusions: Our results provide preliminary evidence for the reversal of the hepatic arterial buffer response following the restoration of the portal venous blood flow. This may be a short‐term effect because of the restored portal venous blood flow.     

A simple modification in operative technique can reduce the incidence of nonanastomotic biliary strictures after orthotopic liver transplantation

Nonanastomotic strictures after liver transplantations are a source of significant morbidity, often necessitating retransplantation. The purpose of this study was twofold: first to identify features associated with the development of this lesion; second, to make technical modifications that will decrease the incidence of this problem. In the first part of this study, 15 of 131 patients were diagnosed with nonanastomotic biliary stricture. A stepwise logistic-regression analysis associated donor cold ischemic time and dopamine dose with the development of nonanastomotic biliary strictures. All these patients had arterial reconstruction after partial revascularization of the liver with portal venous blood. Because the bile duct receives its blood supply from only the hepatic artery, we hypothesized that the prolonged period of warm ischemia from staged reconstruction of the vascular supply would promote the development of this lesion. In a second part of this study, the stricture rate in 45 patients with simultaneous revascularization using both the hepatic artery and portal vein was compared with that in 83 patients from the first part of this study initially revascularized with portal venous blood. All patients in the second study had grafts preserved using UW solution. Only 1 patient with simultaneous revascularization developed a nonanastomotic biliary stricture. Because we were unable to identify any significant complications related to this method of revascularization, we propose that the hepatic artery and portal vein should be released simultaneously, especially in patients receiving a graft with prolonged storage time.     

Location and function of intrahepatic shunts in anaesthetised rats

BACKGROUND: In the present study we determined the proportion of shunt flow due to patent intrahepatic portal systemic shunts in the normal rat liver and its relationship with microsphere induced portal hypertension. METHODS: Systemic and hepatic haemodynamics were measured continuously before, during, and after intraportal injection of 15 micro m diameter microspheres in anaesthetised male Wistar rats. Functional hepatic blood flow and intrahepatic shunt flow were determined by the use of constant intraportal infusion of sorbitol and simultaneous measurements in the portal vein, hepatic vein, and carotid artery. The percentage of large shunts of diameter >15 micro m were estimated by intraportal injection of (51)Cr labelled 15 micro m diameter microspheres. RESULTS: Hepatic sorbitol uptake was 97.9 (0.5)% in normal control rats, with functional hepatic blood flow equalling total hepatic blood flow (2.52 (0.23) ml/min/100 g body weight). Microsphere injection decreased sorbitol uptake to 12.8 (4.3)% and further to 4.1 (0.7)% when followed by hepatic arterial ligation. In the latter two groups, intrahepatic shunt flow (1.46 (0.15) and 1.16 (0.19) ml/min/100 g body weight, respectively) was not significantly different from portal venous flow (1.36 (0.20) and 1.20 (0.20) ml/min/100 g body weight, respectively). Portal venous flow remained at 70% of basal values and portal venous pressure only increased by 50% from baseline. (51)Cr labelled microsphere shunt fraction through large shunts (>15 micro m) was less than 1.0%. CONCLUSION: The site of confluence between the hepatic artery and portal vein is in zone II. Intrahepatic shunts originate in presinusoidal regions in zone I in the normal liver and, when activated by intraportal injection of microspheres, divert 70% of the total portal blood flow away from zone III and thereby reduce acute increases in portal venous pressure.     

Is there an Alternative to TIPS? Ultrasound-guided Direct Intrahepatic Portosystemic Shunt Placement in Budd-Chiari Syndrome

Budd-Chiari syndrome is a spectrum of manifestations which develops as a result of hepatic venous outflow obstruction. Transjugular intrahepatic portosystemic shunt (TIPS) is a minimally invasive vascular and interventional radiological procedure indicated in the management of refractory ascites in such patients. Conventional TIPS requires the presence of a patent hepatic vein and reasonable accessibility to the portal vein, and in patients with totally occluded hepatic veins, this procedure is technically challenging. Direct intrahepatic portosystemic shunt (DIPS) or so called “percutaneous TIPS” involves ultrasound-guided percutaneous simultaneous puncture of the portal vein and inferior vena cava followed by introduction of a guidewire through the portal vein into the inferior vena cava, as a deviation from conventional TIPS. Described here is our experience with DIPS. Three patients with BCS who had refractory ascites but were unsuitable for conventional TIPS due to occlusion of the hepatic veins were chosen to undergo the DIPS procedure. Our technical success was 100%. The shunts placed in two patients remain patent to date, while the shunt in a third patient with underlying antiphospholipid syndrome was occluded a month after the procedure. The percutaneous TIPS procedure seems to be technically feasible and effective in the management of refractory ascites as a result of BCS, particularly in the setting of occluded hepatic veins.     

肝硬化非常见侧支循环临床特点研究

目的 探讨肝硬化患者门体循环之间非常见侧支循环形成的临床特点及意义。方法 对临床确诊为肝硬化的患者运用64排螺旋CT和三维血管成像结合电子胃镜检查,观察其门体循环之间非常见侧支循环的形成。结果 ①700例肝硬化患者中118例(16.86%)存在非常见侧支循环,依次为脾肾静脉分流、胃肾静脉分流、椎旁静脉分流、腹膜后静脉分流、胃脾分流和心膈角静脉分流。②非常见侧支循环形成与肝硬化Child-Pugh分级相关(P<0.01)。③与常见侧支循环形成组比较,非常见侧支循环组较少出现重度食管和(或)胃底静脉曲张、重度门静脉高压性胃病及大量腹水(P<0.01)。④非常见侧支循环组中肝性脑病和慢性血氨升高的发生率高于常见侧支循环组(P<0.01)。结论 ①肝硬化患者中非常见侧支循环并不"非常见";②非常见侧支循环形成与肝功能Child-Pugh分级有关;③非常见侧支循环形成可缓解门静脉高压引起的相关并发症,但增大了肝性脑病和慢性血氨升高的发病率。     

Splenic-intrahepatic left portal shunt in an adult patient with extrahepatic portal vein obstruction without recurrence after pancreaticoduodenectomy

In the last decade, a superior mesenteric-intrahepatic left portal shunt (Rex shunt) has been reported for successful management of extrahepatic portal vein obstruction in children. However, in adults, a mesocaval shunt has been generally performed for the surgical management of extrahepatic portal vein obstruction because of the complexity of the underlying disease and the difficulty of the superior mesenteric-intrahepatic left portal shunt. We herein report an adult patient who was successfully treated by splenic-intrahepatic left portal shunt with an artificial graft (6-mm polytetrafluoroethylene) for complete obstruction of the extrahepatic portal vein following pancreaticoduodenectomy. The shunt procedure not only relieved portal hypertension but also restored hepatic portal flow. In the near future, the Rex shunt should be considered for a beneficial management of extrahepatic portal vein obstruction, even in adults.     

Portal biliopathy

In patients with portal hypertension, particularly with extrahepatic portal vein obstruction, portal biliopathy producing biliary ductal and gallbladder wall abnormalities are common. Portal cavernoma formation, choledochal varices and ischemic injury of the bile duct have been implicated as causes of these morphological alterations. While a majority of the patients are asymptomatic, some present with a raised alkaline phosphatase level, abdominal pain, fever and cholangitis. Choledocholithiasis may develop as a complication and manifest as obstructive jaundice with or without cholangitis. Endoscopic sphincterotomy and stone extraction can effectively treat cholangitis when jaundice is associated with common bile duct stone(s). Definitive decompressive shunt surgery is sometimes required when biliary obstruction is recurrent and progressive.     

Influence of Selective Hepatic Vascular Perfusion on Ammonia Metabolism in Dog

In trying to evaluate the influence of vascular pathways in ammonia clearance by the liver, continuous ammonium chloride perfusions were made separately through the hepatic artery and the portal vein in 20 dogs. Continuous ammonium chloride perfusion (45 mg/kg/hr) through the portal vein results in a highly significant increase in peripheral venous and arterial levels of ammonia, in spite of an ammonia concentration remaining normal in the hepatic vein. On the other hand, continuous ammonium chloride perfusion through the hepatic artery does not significantly change the peripheral ammonia concentration nor the hepatic vein ammonia concentration. From these figures, it must be assumed that during portal perfusion. a fraction of ammonium chloride solution is shunted before reaching the sinusoids. During hepatic artery perfusion, the entire amount of the solution is cleared by the liver and no shunts can be detected. The existence and the level of such shunts are discussed.     

Effects of nifedipine on hepatic venous pressure gradient and portal vein blood flow in patients with cirrhosis

Abstract We investigated the effects of nifedipine on splanchnic haemodynamics in 13 patients with cirrhosis and portal hypertension, and in 10 control subjects using hepatic venous catheterization and pulsed Doppler ultrasound. There were no significant changes in systemic or splanchnic haemodynamics in control patients. In contrast, systemic vascodilatation, evidenced by significant decreases in mean arterial pressure and systemic vascular resistance, was observed in patients 20 min after sublingual application of 10 mg nifedipine. Moreover, hepatic venous pressure gradient and portal vein blood flow significantly increased after nifedipine administration. There was a significant correlation between the percentage increases in portal vein blood flow and in hepatic venous pressure gradient. However, no correlation was found between the percentage change in cardiac output and that in portal vein blood flow. Thus the increase in portal vein blood flow appears to be related to splanchnic arterial vasodilatation by nifedipine. Consequently, nifedipine has deleterious effects on portal haemodynamics in patients with cirrhosis. As nifedipine may potentially increase the risk of variceal haemorrhage in patients with less advanced varices, this drug should be used with caution in patients with chronic liver disease.     

Development of portal vein thrombosis complicating idiopathic portal hypertension. A case report

A 58-yr-old woman with biopsy-proven idiopathic portal hypertension presented with ascites and pretibial pitting edema. On admission, ultrasonic Doppler flowmetry demonstrated hepatopetal flow of a markedly reduced velocity in the portal vein, hepatofugal flow in the splenic vein, and a large spontaneous splenorenal shunt. The patient spontaneously developed hepatic encephalopathy 1 mo later. Percutaneous transhepatic portography demonstrated mural thrombi at the porta hepatis after the catheter had penetrated the mural thrombi without resistance; there was also a long retention of contrast medium in the portal vein. 99mTc-Macroaggregated albumin instilled into the superior mesenteric vein was caught in the lungs, and no activity entered the liver. Measurements of ammonia and immunoreactive insulin clearly indicated that superior mesenteric venous blood was shunted through the splenic vein and the splenorenal shunt. Subsequent ultrasonic examination with Doppler flowmetry suggested further growth of the thrombi and lack of blood flow in the portal vein. Although the procedure of percutaneous transhepatic catheterization could have contributed to the growth of thrombi, it is more likely that the thrombosis in the portal vein was a sequela to idiopathic portal hypertension, and was growing at the time of catheterization. This case may be of significance in the understanding of the relationship between idiopathic portal hypertension and extrahepatic portal obstruction.     

Therapeutic approaches for portal biliopathy: A systematic review

Portal biliopathy(PB) is defined as the presence of biliary abnormalities in patients with non-cirrhotic/nonneoplastic extrahepatic portal vein obstruction(EHPVO) and portal cavernoma(PC). The pathogenesis of PB is due to ab extrinseco compression of bile ducts by PC and/or to ischemic damage secondary to an altered biliary vascularization in EHPVO and PC. Although asymptomatic biliary abnormalities can be frequently seen by magnetic resonance cholangiopancreatography in patients with PC(77%-100%), only a part of these(5%-38%) are symptomatic. Clinical presentation includes jaundice, cholangitis, cholecystitis, abdominal pain, and cholelithiasis. In this subset of patients is required a specific treatment. Different therapeutic approaches aimed to diminish portal hypertension and treat biliary strictures are available. In order to decompress PC, surgical porto-systemic shunt or transjugular intrahepatic porto-systemic shunt can be performed, and treatment on the biliary stenosis includes endoscopic(Endoscopic retrograde cholangiopancreatography with endoscopic sphincterotomy, balloon dilation, stone extraction, stent placement) and surgical(bilioenteric anastomosis, cholecystectomy) approaches. Definitive treatment of PB often requires multiple and combined interventions both on vascular and biliary system. Liver transplantation can be considered in patients with secondary biliary cirrhosis, recurrent cholangitis or unsuccessful control of portal hypertension.     

CEUS： A new imaging approach for postoperative vascular complications after right-lobe LDLT

AIM： To investigate contrast-enhanced ultrasound （CEUS） for early diagnosis of postoperative vascular complications after right-lobe living donor liver transplantation （RLDLT）. METHODS： The ultrasonography results of 172 patients who underwent RLDLT in West China Hospital, Sichuan University from January 2005 to June 2008 were analyzed retrospectively. Among these 172 patients, 16 patients＇ hepatic artery flow and two patients＇ portal vein flow was not observed by Doppler ultrasound, and 10 patients＇ bridging vein flow was not shown by Doppler ultrasound and there was a regional inhomogeneous echo in the liver parenchyma upon 2D ultrasound. Thus, CEUS examination was performed in these 28 patients. RESULTS： Among the 16 patients without hepatic artery flow at Doppler ultrasound, CEUS showed nine cases of slender hepatic artery, six of hepatic arterial thrombosis that was confirmed by digital subtraction angiography and/or surgery, and one of hepatic arterial occlusion with formation of lateral branches. Among the two patients without portal vein flow at Doppler ultrasound, CEUS showed one case of hematoma compression and one of portal vein thrombosis,and both were confirmed by surgery. Among the 10 patients without bridging vein flow and with liver parenchyma inhomogeneous echo, CEUS showed regionally poor perfusion in the inhomogeneous area, two of which were confirmed by enhanced computed tomography （CT）, but no more additional information about bridging vein flow was provided by enhanced CT. CONCLUSION： CEUS may be a new approach for early diagnosis of postoperative vascular complications after RLDLT, and it can be performed at the bedside.     

Pediatric non-cirrhotic portal hypertension:Endoscopic outcome and perspectives from developing nations

Non-cirrhotic portal hypertension(NCPH) forms an important subset of portal hypertension in children. Variceal bleed and splenomegaly are their predominant presentation. Laboratory features show cytopenias(hypersplenism) and preserved hepatic synthetic functions. Repeated sessions of endoscopic variceal ligation or endoscopic sclerotherapy eradicate esophageal varices in almost all cases. After variceal eradication, there is an increased risk of other complications like secondary gastric varices, cholangiopathy, colopathy, growth failure,especially in extra-hepatic portal vein obstruction(EHPVO). Massive splenomegaly-related pain and early satiety cause poor quality of life(QoL). Meso-Rex bypass is the definitive therapy when the procedure is anatomically feasible in EHPVO. Other portosystemic shunt surgeries with splenectomy are indicated when patients present late and spleen-related issues predominate. Shunt surgeries prevent rebleed, improve growth and QoL. Non-cirrhotic portal fibrosis(NCPF) is a less common cause of portal hypertension in children in developing nations.Presentation in the second decade, massive splenomegaly and patent portal vein are discriminating features of NCPF. Shunt surgery is required in severe cases when endotherapy is insufficient for the varices. Congenital hepatic fibrosis(CHF)presents with firm palpable liver and splenomegaly. Ductal plate malformation forms the histological hallmark of CHF. CHF is commonly associated with Caroli's disease, renal cysts, and syndromes associated with neurological defects.Isolated CHF has a favourable prognosis requiring endotherapy. Liver transplanta-tion is required when there is decompensation or recurrent cholangitis, especially in Caroli's syndrome. Combined liver-kidney transplantation is indicated when both liver and renal issues are present.