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OBJECTIVES: This study aimed to evaluate the feasibility, safety, and efficacy of the transjugular mesenteric-caval shunt (TMCS) as a treatment for the cavernous transformation of the portal vein (CTPV) and recurrent variceal bleeding. METHODS: This retrospective case series was conducted with approval from the institutional review board. It involved seven patients diagnosed with CTPV and recurrent variceal bleeding who underwent the TMCS procedure. We analyzed the rate of procedural complications, incidents of rebleeding, stent stenosis, hepatic encephalopathy, and overall survival to assess treatment outcomes. RESULTS: The TMCS was successfully performed in all seven patients without any life-threatening complications. Postoperatively, one patient developed a lung infection and pleural effusion, which resolved with appropriate treatment. Additionally, two patients experienced an increase in total bilirubin levels, but there was no further deterioration in liver function. The median portal pressure gradient significantly decreased from a preoperative value of 27 mmHg (range 20-36 mmHg) to a postoperative value of 6 mmHg (range 4-11 mmHg). A notable improvement was observed in one cirrhotic patient, with liver function progressing from Child-Pugh class B (score 9) to class A (score 6). Over a median follow-up period of 14 months (range 7-18 months), none of the patients encountered rebleeding, stent stenosis, hepatic encephalopathy, or mortality. CONCLUSION: The TMCS appears to be a viable and effective alternative for managing CTPV with recurrent variceal bleeding. Its long-term outcome requires further evaluation. CLINICAL RELEVANCE STATEMENT: TMCS provides a promising treatment for patients with life-threatening CTPV complications when occluded portal vein cannot be recanalized and portal vein recanalization TIPS is not an option. KEY POINTS: Performing TIPS in patients with portal vein cavernoma is complex due to the requirement for recanalization of the occluded portal vein. Creating a mesenteric-caval shunt through a transjugular approach is a feasible technique. Establishing a TMCS provides a means to manage life-threatening complications arising from portal vein cavernoma.

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Purpose: To evaluate the feasibility and efficacy of a transmesenteric vein extrahepatic portosystemic shunt (TmEPS) for the treatment of cavernous transformation of the portal vein (CTPV). Materials and methods: The clinical data of 20 patients with CTPV who underwent TmEPS between December 2020 and January 2022 â€‹at Henan Provincial People's Hospital were retrospectively collected. The superior mesenteric vein (SMV) trunk was patent or partially occluded in these patients. An extrahepatic portosystemic shunt between the inferior vena cava and the SMV was established using a stent graft through an infraumbilical median longitudinal mini-laparotomy. The technical success, efficacy, and complication rates were evaluated, and the pre- and postoperative SMV pressures were compared. Patients' clinical outcomes and shunt patency were assessed. Results: TmEPS was successfully performed in 20 patients. The initial puncture success rate of the balloon-assisted puncture technique is 95%. The mean SMV pressure decreased from 29.1 â€‹± â€‹2.9 â€‹mmHg to 15.6 â€‹± â€‹3.3 â€‹mmHg (p â€‹< â€‹0.001). All symptoms of portal hypertension resolved. No fatal procedural complications occurred. During the follow-up period, hepatic encephalopathy occurred in two patients. The remaining patients remained asymptomatic. All shunts were patent. Conclusions: TmEPS is a feasible, safe, and effective treatment option for patients with CTPV.

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Introduction: Cavernous transformation of the portal vein, although a hepatic condition, may manifest with clinical features similar to those in patients with gastrointestinal pathologies. Particularly in young patients with no prior history of alcoholism or hepatic pathology, the diagnosis of cavernous transformation of the portal vein may be missed in an emergency setting where patients' reported symptoms may be mimicking those associated with bleeding peptic ulcer or other gastrointestinal pathologies. Case Report: We present a case of a 22-year-old male with no prior history of hepatic or pancreatic pathology who presented to the emergency room with some episodes of haematemesis, melena and slight dizziness, in whom a cavernous transformation of the portal vein was identified by abdominal duplex ultrasonography. Discussion: The clinical diagnosis of cavernous transformation of the portal vein can be challenging and easily missed especially in instances where a patient with no history of chronic alcoholism, liver cirrhosis, hepatoma, pancreatitis or prior abdominal surgery presents to the emergency room with haematemesis and anaemia. Ultrasonography presents as a reliable radiological tool in the diagnosis of rare and unforeseen conditions like cavernous transformation of the portal vein to allow for prompt management and prevention of adverse patients' outcomes. Conclusion: Abdominal duplex ultrasonography can reliably aid in the prompt diagnosis and management of patients with unforeseen rare hepatic pathologies such as cavernous transformation of the portal vein who present with upper gastrointestinal bleeding.

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Background: Cavernous transformation of the portal vein (CTPV) causes portal hypertension in children. Among Meso-Rex treatments, it is unclear whether the Meso-Rex bypass shunt (MRB) or the Meso-Rex transposition shunt (MRT) offers lower postoperative morbidity. Our objective was to evaluate postoperative outcomes, comparing MRB and MRT for children with CTPV. Methods: A retrospective study was conducted on children undergoing Meso-Rex for CTPV from January 2010 to December 2020. The primary outcome was shunt complications, including shunt stenosis and thrombus. The secondary outcome was re-operation. Results: Of the 43 patients included, 21 underwent MRT and 22 underwent MRB. MRT was associated with a higher rate of shunt complications when compared to MRB (23.8 vs. 9.1%, p = 0.191). The patients exhibited a higher rate of re-operation under the MRT than under the MRB (19 vs. 4.5%, p = 0.138). The operative time in the MRT group was significantly shorter than in the MRB group. Compared to MRT, the reduction in the length and thickness of the spleen was significantly greater in the MRB group. The increases in platelets were significantly higher in the MRB group than in the MRT group. The postoperative shunt velocity of MRB was notably faster than MRT. There was no significant difference in postoperative portal pressure between the two groups (p > 0.05). Conclusion: Both MRB and MRT result in acceptable postoperative outcomes, but MRT is associated with higher post-shunt complications, which often increase the re-operation rate. This study suggests that MRB may offer advantages for children with CTPV.

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The clinical outcomes differ between patients with cavernous transformation of the portal vein (CTPV) with and without cirrhosis. Therefore, invasive liver biopsy may be needed for the differential diagnosis of patients with CTPV with or without cirrhosis. The present study aimed to investigate the diagnostic efficacy of liver stiffness measurements (LSM) for the prediction of cirrhosis in patients with CTPV. A total of 20 patients with CTPV, 34 with chronic hepatitis B (CHB)-related cirrhosis and 20 healthy volunteers, were retrospectively recruited in the study. CTPV was diagnosed with contrast-enhanced computed tomography (CT) and ultrasound for the abdomen. LSM values were detected for each patient, while liver biopsy was performed in each patient in the CTPV and cirrhosis groups. The results demonstrated that LSM values were significantly lower in the CTPV group (12.5 kPa; range, 6.8-21.5 kPa) compared with the CHB-related cirrhosis group (21.0 kPa; range, 15.5-27.2 kPa; P=0.017). However, this was still higher compared with healthy volunteers (4.9 kPa; range 4.0-5.8 kPa; P<0.001). In addition, CTPV patients with cirrhosis (17.7 kPa; range, 13.9-30.8 kPa) exhibited significantly increased LSM values compared with those without cirrhosis (6.4 kPa; range, 5.7-7.8 kPa; P<0.001). Furthermore, LSM values in CTPV patients without cirrhosis were slightly higher compared with those of healthy volunteers (P=0.003), while no statistically significant difference was observed in LSM between CTPV patients with cirrhosis and CHB-related cirrhosis group. These findings indicated that LSM values could be used for the differential diagnosis of CTPV patients with or without cirrhosis. However, further validation studies are needed.

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Compression by hepatic hydatid lesions may invade the portal vein and cause cavernous transformation of the portal vein (CTPV), which has difficulties and high risks in surgery, and conventional hepatectomy cannot achieve radical treatment. This article reviews the etiology, classification, clinical manifestations, and auxiliary examinations of hepatic echinococcosis with CTPV and summarizes the treatment strategies for this disease. It is believed that the treatment of hepatic echinococcosis with CTPV should combine the resection of hydatid lesions with the effective relief of portal hypertension, and revascularization and ex-vivo liver resection with auto-transplantation play an important role in radical resection.

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OBJECTIVE: To assess the clinical application of preoperative retrograde portal venography for children with cavernous transformation of the portal vein (CTPV). METHODS: The clinical data of 8 cases of CTPV admitted in the Children's Hospital of Zhejiang University from January 2018 to September 2019 were retrospectively analyzed. Preoperative retrograde portography was performed to determine the corresponding vascular morphology and size of portal vein system. If the retrograde portography showed that the left branch of the shadow portal vein was unobstructed and its diameter was greater than 3 mm, Rex shunt would be performed after anatomic exploration of Rex recess; if retrograde portography showed that the diameter of left portal vein was less than 3 mm, but the diameter of left renal vein dissected during shunt operation was greater than 5 mm, Warren operation was selected. The patients were followed up for 1, 3 and 6 months after discharge, and then were followed up every 6 months. RESULTS: Retrograde portal venography was successfully performed in 8 child patients.The anatomical position and size of main portal vein and its left and right branches, left renal vein and other important vessels were determined. Among them, there was the well-developed left and right branches of portal vein in 4 child patients, in which the left and right branches of portal vein converged together, but did not communicate with the main portal vein. In addition, the left branch diameter of the portal vein was greater than 3 mm, and the anatomical exploration results during shunt were consistent with it, so Rex shunt was performed. In the other 4 cases, the left branch diameter of the portal vein was small (less than 3 mm) in 3 cases, and the right branch was not clearly developed. Moreover, the left branch of the portal vein was poorly developed and almost occluded in 1 case. However, the left renal vein in these 4 child patients was well developed, the blood flow was unobstructed and the diameter was greater than 5 mm, so Warren operation was performed. Seven patients recovered well after the operation, and the other one had digestive tract rudimentary one year after operation, and the condition was stable after conservative treatment. CONCLUSIONS: The preoperative retrograde portal venography can be used to evaluate the portal vein system in children with CTPV, which provides important clinical basis for making appropriate treatment plan before surgery.

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BACKGROUND: The study was to investigate the role of color Doppler ultrasonography in the evaluation of the effect of Rex-bypass shunt on the cavernous transformation of the portal vein (CTPV) in children. METHODS: Fifty children with symptomatic extrahepatic portal hypertension who received Rex-bypass shunt were retrospectively reviewed, and they were diagnosed with CTPV by ultrasonography. The clinical characteristics were analyzed before and after operation. RESULTS: Forty-five patients received color Doppler ultrasonography at 6 months after surgery, and good patency in the bypass vessels was displayed. The platelet count significantly increased (P < 0.001) and the esophagogastric varices were improved significantly (p < 0.001). The patency of bypass vessels on color Doppler ultrasonography was consistent with the changes in the platelet count and the degree of esophagogastric varices on gastroscopy before and after operation. The diameter of bypass vessels at 6 months was slightly larger than that at 7 days after operation, and there was no significant difference in blood flow velocity between two time points (P = 0.507). CONCLUSIONS: Color Doppler ultrasonography can display the patency, diameter, and flow velocity of bypass vessels. It plays an important role in evaluating the effect of Rex-bypass shunt on the CTPV.

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OBJECTIVE@#To assess the clinical application of preoperative retrograde portal venography for children with cavernous transformation of the portal vein (CTPV).@*METHODS@#The clinical data of 8 cases of CTPV admitted in the Children's Hospital of Zhejiang University from January 2018 to September 2019 were retrospectively analyzed. Preoperative retrograde portography was performed to determine the corresponding vascular morphology and size of portal vein system. If the retrograde portography showed that the left branch of the shadow portal vein was unobstructed and its diameter was greater than 3 mm, Rex shunt would be performed after anatomic exploration of Rex recess; if retrograde portography showed that the diameter of left portal vein was less than 3 mm, but the diameter of left renal vein dissected during shunt operation was greater than 5 mm, Warren operation was selected. The patients were followed up for 1, 3 and 6 months after discharge, and then were followed up every 6 months.@*RESULTS@#Retrograde portal venography was successfully performed in 8 child patients.The anatomical position and size of main portal vein and its left and right branches, left renal vein and other important vessels were determined. Among them, there was the well-developed left and right branches of portal vein in 4 child patients, in which the left and right branches of portal vein converged together, but did not communicate with the main portal vein. In addition, the left branch diameter of the portal vein was greater than 3 mm, and the anatomical exploration results during shunt were consistent with it, so Rex shunt was performed. In the other 4 cases, the left branch diameter of the portal vein was small (less than 3 mm) in 3 cases, and the right branch was not clearly developed. Moreover, the left branch of the portal vein was poorly developed and almost occluded in 1 case. However, the left renal vein in these 4 child patients was well developed, the blood flow was unobstructed and the diameter was greater than 5 mm, so Warren operation was performed. Seven patients recovered well after the operation, and the other one had digestive tract rudimentary one year after operation, and the condition was stable after conservative treatment.@*CONCLUSIONS@#The preoperative retrograde portal venography can be used to evaluate the portal vein system in children with CTPV, which provides important clinical basis for making appropriate treatment plan before surgery.

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Cavernous transformation of portal vein (CTPV) is the main cause of portal hypertension and its related variceal bleeding in children. Transjugular intrahepatic portosystemic shunt (TIPS) was not reported to treat CTPV for children younger than 5 years old. In this case report, the patient was a 26-month-old boy who presented with hematemesis and melena due to CTPV. Even after azygoportal disconnection, repeated hematemesis as well-melena still occurred. After careful evaluation, we performed TIPS under general anesthesia for him. The procedure was uneventful, and 6-mm stents were inserted. Six months after TIPS, there was no recurrence of bleeding, and no procedure-related event happened. The follow-up esophagogastroduodenoscopy proved dramatic remission of varices, indicating a successful outcome. We believe TIPS could be safely placed for young children to manage variceal bleeding due to CTPV.

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OBJECTIVE: To compare the efficacy of transjugular intrahepatic portosystemic shunts (TIPS) and endoscopic variceal ligations (EVL) plus propranolol in decreasing rebleeding and improving survival rates in cirrhotic patients with cavernous transformation of the portal vein (CTPV). METHODS: Cirrhotic patients with CTPV and a history of variceal bleeding who were treated for recurrent variceal bleeding between June 2010 and July 2016 were identified and classified based on the treatment they received (TIPS or EVL plus propranolol). Their characteristics and clinical data were recorded. The rebleeding and long-term survival rates between the two groups were analyzed. RESULTS: A total of 51 patients were included, of whom 25 were treated with TIPS and 26 with EVL plus propranolol. The mean duration of follow up was 21 months (range 1-47 months) in the former group and 27 months (range 6-73 months) in the latter group. The recurrent variceal bleeding-free rate increased remarkably in the TIPS group compared with the EVL + propranolol group (P = 0.047). Three (14.3%) patients died in the TIPS group, and one (3.8%) in the EVL plus propranolol group (P = 0.305). Hepatic encephalopathy occurred in 14.3% (3/21) of the patients in the TIPS group and in 3.8% (1/26) in the EVL + propranolol group (P = 0.202). CONCLUSION: TIPS appeared to be more effective in preventing rebleeding in cirrhotic patients with CTPV compared with EVL plus propranolol, without improving survival.

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Portal vein thrombosis is a common complication in patients with cirrhosis. Anticoagulation involves a high risk of bleeding secondary to portal hypertension, so placing transjugular intrahepatic portosystemic shunts (TIPS) has become an alternative treatment for portal vein thrombosis. Three strategies for TIPS placement have been reported: 1) portal recanalization and conventional implantation of the TIPS through the jugular vein; 2) portal recanalization through percutaneous transhepatic/transsplenic) access; and (3) insertion of the TIPS between the suprahepatic vein and a periportal collateral vessel without portal recanalization. We describe different materials that can be used as fluoroscopic targets for the TIPS needle and for portal recanalization. This article aims to show the success of TIPS implantation using different combinations of the techniques listed above, which is a good treatment alternative in these patients whose clinical condition makes them difficult to manage, and to show that portal vein thrombosis/cavernous transformation should not be considered a contraindication for TIPS.

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OBJECTIVE: To evaluate the effects of combined Rex-bypass shunt and pericardial devascularization on prehepatic portal hypertension secondary to cavernomatous transformation of portal vein (CTPV). METHODS: Forty-two patients aged from 3 years to 49 years (divided into 3 groups), 26 cases male and 16 female, with prehepatic vascular hepertention were treated with Rex-bypass shunt combined with pericardial devascularization. In each patient, preoperative assessment included ultrasound and computed tomographic angiography of the portal vein and blood analysis. The procedure was Rex-bypass shunt (with or without graft), and patients with moderate or severe gastroesophageal varices required additional paraesophagogastric devascularization. Splenectomy or subtotal splenectomy was performed if combined hypersplenism co-existed. All data were analyzed retrospectively. RESULTS: No intraoperative death occurred, blood routine analysis improved (P < 0.05), the blood flow velocity (P < 0.05) and diameter (P < 0.05) of the left portal vein (LPV) significantly increased, the esophageal and gastric varices significantly relieved in 34 patients (P < 0.05), and better effects of earlier operations were demonstrated than the delayed ones (P < 0.05). During the period of follow-up from 6 to 64 months, the overall patency rate was 85.7% and the younger the age the better of the effect. CONCLUSION: Rex-bypass shunt combined with pericardial devascularization is a safe and effective procedure for prehepatic portal hypertension caused by CTPV.

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Extrahepatic portal vein obstruction, although rare in children, is a significant cause of portal hypertension (PHT) leading to life-threatening gastrointestinal bleeding in the pediatric age group. PHT may also lead to other complications such as hyperesplenism, cholangyopathy, ascites, and even hepatopulmonary syndrome and portopulmonary hypertension that may require organ transplantation. Herein we report the case of an asymptomatic 11-month-old infant wherein a hepatomegaly and cavernous transformation of the portal vein was detected by liver ultrasound. Neither signs of thrombosis in arteriovenous system, nor affectation of biliary tract were identified in the magnetic resonance imaging study. A significant enlargement of the caudate lobe of the liver was reported. No risk factors were detected. The differential diagnosis performed was extensive. Inherited thrombophilia and storage disorders were especially considered. Liver biopsy was normal. Upper gastrointestinal esophagogastroduodenoscopy detected two small varicose cords on the distal third of the esophagus. Finding a cavernous transformation of the portal vein with evidence of collateral circulation in such an early age is a challenging condition for professionals, since PHT may lead to severe complications during childhood and can compromise growth and development. Evidence-based guidelines for the management of PHT in adults have been published. However, follow-up and treatment of pediatric patients have not yet been standardized. Moreover, management of PHT in infants faces particular difficulties such as technical restrictions that could hinder their treatment.

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Objective To explore the efficacy and safety of percutaneous transhepatic portal vein or transjugular intrahepatic portosystemie shunt (TIPS)to implant the portal vein metallic stent in treatment of cavernous transformation of portal vein (CTPV).Methods Clinical and imaging data of 8 patients with CTPV were retrospectively analyzed who were treated in our hospital.All patients were treated with metallic stent implantation in portal vein including 3 patients by TIPS and 5 by percutaneous transhepatic portal vein.Results All patients were successful in the stent implantation without any occurrence of serious complications such as intra-abdominal hemorrhage and so on.Intraoperative angiography showed blood circulated freely in these stents.1 day-2 weeks later,the patients symptoms of abdominal pain and gastrointestinal bleeding were obviously relieved or disappeared.Follow up 1 month-3 years,1 patient with stent occlusion after one year of operation,the blood flow recovery after stent reimplantation,and the remaining patients,color doppler ultrasound reflected patency of blood flows in their stents.No one suffered from gastrointestinal bleeding or abdominal pain again.Conclusion Implantation of portal vein metallic stent via percutaneous transhepatic portal vein or via TIPS in treatment of cavernous transformation of portal vein is safe and effective.

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Objective To study the therapeutic efficacy of Rex shunt in treating pediatric patients with prehepatic portal hypertension (PHPH).Methods Five children with PHPH who were admitted from October 2014 to May 2015 were reviewed.There were three boys and two girls,with age ranging from 50 to 95.5 months [(75.8 ± 1.9) months].They all suffered from recurrent upper gastrointestinal (GI) bleeding.Their red blood cell (RBC),white blood cell (WBC) and platelet (PLT) counts were decreased,but laboratory findings revealed no liver dysfunction.Ultrasound and CT scan diagnosed cavernous transformation of portal vein (CTPV) and splenomegaly.The mean splenic length was (42.8 ± 8.2) cm.Indirect portal venography revealed patent left portal vein.All patients underwent Rex shunt and were followed up for 3 ～ 7 months.Results The mean duration of operation was (566.0 ± 39.7) min.Intraoperative bleeding varied from 10 to 50 ml.The portal pressure significantly decreased after surgery from [(25.6 ± 1.5) mmHg,1 mmHg =0.133 kPa] preoperatively to (19.2 ± 3.3) mmHg postoperatively (P ＜ 0.05).Portal venography indicated patent left portal vein after the Rex shunt.The postoperative course was uneventful in the five patients with a mean hospital stay of (26 ± 9.3) days.There was no further GI bleeding.The RBC,WBC and PLT counts increased.Ultrasound indicated patent anastomotic stomas and decreased splenic size.Conclusion A Rex shunt in treating patients with PHPH is safe,feasible and efficacious.

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Extensive thrombosis of the portal and splenomesenteric veins combined with cavernous transformation of the portal vein (CTPV) has been considered to be a contraindication for living donor liver transplantation (LDLT) due to technical difficulties and perioperative risks. In recent years, several surgical innovations including cavoportal hemitransposition, renoportal anastomosis, and portal arterialization have been introduced to overcome diffuse portal vein thrombosis (PVT) and CTPV, but their outcomes were unsatisfactory with significant morbidity and mortality. Herein, we report two successful cases of adult LDLT in diffuse PVT with CTPV managed using the paracholedochal vein as portal inflow to the graft.

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Objective To evaluate the therapeutic efficacy of percutaneous transhepatic portal vein balloon angioplasty in treating cavernous transformation of portal vein following operation of congenital choledochal cyst. Methods From 2012 to 2014, a total of 6 patients with cavernous transformation of portal vein which occurred after the operation of congenital choledochal cyst were encountered at authors’ hospital. The clinical data were retrospectively analyzed. Before treatment, all patients presented symptoms of different degrees of hematemesis. Percutaneous transhepatic portal vein balloon angioplasty was carried out in all patients, and embolization of gastric coronary vein with coils was employed if angiography showed that coronary vein of stomach was pronouncedly dilated. The clinical manifestations, the imaging materials and the complications were analyzed. All the patients were followed up for 3 - 31 months. Results Of the 6 patients, portal vein main stem occlusion was found in 5 and severe localized stenosis was seen in one. Cavernous transformation of portal vein was revealed in all the 6 patients. Percutaneous transhepatic portal vein balloon angioplasty was successfully accomplished in 5 patients and failed in one patient. Embolization of gastric coronary vein with coils was performed in two patients. After the treatment, no treatment-related severe complications occurred in all patients. The follow-up period ranged from 3 to 31 months. During the follow-up period portal vein maintained patent in 5 patients. No recurrent hematemesis occurred in all patients. Conclusion For the treatment of cavernous transformation of portal vein occurring after the operation of congenital choledochal cyst, percutaneous transhepatic portal vein balloon angioplasty is a safe, effective and minimally - invasive therapeutic means.

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Extensive thrombosis of the portal and splenomesenteric veins combined with cavernous transformation of the portal vein (CTPV) has been considered to be a contraindication for living donor liver transplantation (LDLT) due to technical difficulties and perioperative risks. In recent years, several surgical innovations including cavoportal hemitransposition, renoportal anastomosis, and portal arterialization have been introduced to overcome diffuse portal vein thrombosis (PVT) and CTPV, but their outcomes were unsatisfactory with significant morbidity and mortality. Herein, we report two successful cases of adult LDLT in diffuse PVT with CTPV managed using the paracholedochal vein as portal inflow to the graft.

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