Hypoplastic Portal Vein Research Articles

Retro-pancreatic pull-through reconstruction of the hypoplastic portal vein using the autologous mesosystemic shunt vessel in adult living donor liver transplantation: a case report

BackgroundIn liver transplant patients with hypoplastic portal vein (PV), when the narrowed segment is extended too deep into the dorsal side of the pancreas, it is difficult and dangerous to reconstruct the interposition graft from the upper part of the pancreas. Herein, we present a case of PV reconstruction with the autologous mesosystemic shunt vessel from the caudal side of the pancreas in a situation where the narrowed PV was deep, and we discuss the technical details.Case presentationA 25-year-old woman presented with cholestatic liver cirrhosis due to biliary atresia after Kasai procedure. Since her jaundice progressed, she was referred to our hospital for liver transplantation. Laboratory tests showed that her total bilirubin was elevated to 7.6 mg/dL. The Model for End-Stage Liver Disease score was 18, and the Child–Pugh score was 9 (Grade B). She underwent living donor liver transplantation (LDLT) using a right hemi-liver graft procured from her 54-year-old mother. The conventional approach from the cephalad side to the superior mesenteric vein (SMV) and splenic vein (SpV) confluence behind the pancreas was extremely difficult in this case because the confluence of SMV and SpV was close to the lower edge of the pancreas. Therefore, we decided to perform PV reconstruction from the caudal side. The main trunk of PV was documented as narrow (5 mm in diameter), for which retro-pancreatic pull-through PV reconstruction was successfully performed using her own mesosystemic shunt vessel. A contrast computed tomography (CT) scan was performed on postoperative day 5 because of an elevation of D-dimer and found a partial thrombus in the left pulmonary artery, as well as in the PV and left renal vein. Thereafter, thrombolytic therapy with low-molecular-weight heparin was started immediately and switched to a direct oral anticoagulant. The follow-up CT taken 3 months after liver transplantation revealed a patent PV without thrombus; therefore, anticoagulant therapy was discontinued. Currently, the patient has been well and active with a patent PV without anticoagulant therapy for 3 years after LDLT.ConclusionsRetro-pancreatic pull-through reconstruction of the hypoplastic PV is a feasible and effective method when conventional reconstruction is not indicated.

Modified patch-conduit venoplasty for hypoplastic portal vein in pediatric liver transplantation

Modified patch-conduit venoplasty for hypoplastic portal vein in pediatric liver transplantation

PSUN338 A 2 year-old Girl with a Congenital Portosystemic Shunt and Hypoglycemia Due to Secondary Hyperinsulinism Treated with Uncooked Cornstarch

Abstract Background Hepatic portosystemic shunts (Abernethy malformation) are rare congenital malformations in children, which result in direct connections of splanchnic venous drainage to systemic circulation, bypassing hepatic uptake and metabolism. Congenital portosystemic shunts (CPSS) can also rarely lead to secondary hyperinsulinism and hypoglycemia. We describe a case of a 2-year-old girl with a CPSS presenting with hypoglycemia due to reactive hyperinsulinism successfully treated with uncooked cornstarch. Clinical Case A 2-year-old girl presents for evaluation of altered mental status and hypoglycemia. Three months earlier she was treated for Lemierre syndrome and found to have a CPSS (severely hypoplastic portal vein with direct drainage of splanchnic vasculature into the IVC) with hyperammonemia treated with lactulose and rifaximin. This admission, she presented with lethargy and altered mental status as she was difficult to arouse. EMS detected a glucose of 19 mg/dl and treated her with a bolus of 25% dextrose fluids. The day before, she had eaten a typical dinner of cookies and mashed potatoes without concern. On admission, she had no elevation in urine ketones and her blood gas measurements, alcohol, and ammonia levels were within normal limits, as such a diagnosis of secondary hyperinsulinism was suspected. A 3-hour OGTT with 2 g/kg glucose was performed after 2 days of stable oral intake and normoglycemia. Her fasting glucose was 74 mg/dl with a baseline insulin of 9.3 mcIU/ml. Within 1 hour, her glucose rose to 223 mg/dl with an insulin of 247.14 mcIU/ml. At 3 hours (2-hour levels not obtained) her glucose dropped to 67 mg/dl and with an insulin of 24.83 mcIU/ml. Thirty minutes after completion of the OGTT (3.5 hours from glucose load) she became lethargic with a glucose of 45 mg/dl confirming suspicion for secondary hyperinsulinism induced hypoglycemia. Education on glucose monitoring, hypoglycemia management and dietary changes were made to include meals with sufficient fat and protein to help reduce postprandial hyperglycemia. Additionally, uncooked cornstarch 1.4 g/kg was started to provide an overnight source of carbohydrates while avoiding rapid absorption. At 3 months her mother reported no hypoglycemic episodes with fasting glucose levels 80-100 mg/dl. Clinical Lessons Hypoglycemia can occur in CPSS patients due to secondary hyperinsulinism. The vascular malformation causes postprandial hyperglycemia, from the absence of first-pass uptake of glucose by the liver, which leads to insulin secretion. However, insulin similarly bypasses hepatic uptake and degradation after release (normally clears up to 80% of insulin), and the systemic circulation and peripheral tissues are exposed to elevated levels leading to hypoglycemia. While surgical correction provides definitive treatment, uncooked cornstarch can maintain euglycemia both by decreasing postprandial hyperglycemia and by providing a slowly absorbed source of carbohydrate. Presentation: Sunday, June 12, 2022 12:30 p.m. - 2:30 p.m.

Transcatheter closure of congenital portosystemic shunts - A multicenter experience.

Background:Congenital portosystemic shunts (CPSS) are rare and present variably with hepatic encephalopathy, pulmonary arteriovenous malformations (PAVMs), and pulmonary hypertension (PH).Objective:The objective of the study was to see the feasibility of transcatheter closure of CPSS and their outcome.Materials and Methods:We analyzed the data of 24 patients of CPSS who underwent transcatheter closure from five institutions (March 2013 to April 2019). Baseline evaluation included echocardiography with bubble contrast study, ultrasound examination of the abdomen, computed tomography angiogram, and cardiac catheterization with test balloon occlusion of the CPSS. The evaluation showed cyanosis due to PAVM in 12, PH in 8, and respiratory distress in 2. Two had both cyanosis and PH. Criteria for eligibility for complete catheter closure of CPSS included demonstration of intrahepatic portal vein (PV) radicals together with a PV pressure of ≤18 mmHg on occlusion.Results:The median age and weight were 8 years (0.5–21) and 19.5 kg (4.2–73), respectively. Transcatheter closure was performed in 21 patients (22 procedures) using a variety of occlusive devices and stent-graft exclusion was done in one patient. Closure was not done in 3 in view of high portal venous pressures and hypoplastic PVs. During the follow-up (median: 42 months and range: 61 days–4.8 years), saturation normalized in 14 patients with PAVM. PH declined in all eight patients who underwent the procedure. Respiratory distress improved in two patients.Conclusions:Early and short-term follow-up results of catheter closure of CPSS appear promising. However, further, follow-up is needed to demonstrate long-term effectiveness.

A case of two shunts in the endovascular treatment of type II Abernethy syndrome

BackgroundAbernethy malformation is a rare condition defined by a congenital extrahepatic portosystemic shunt, often leading to absence or hypoplasia of the intrahepatic portal venous system. Although there are no consensus treatment guidelines, interventional techniques now offer minimally invasive treatment options for Abernethy malformations. This case report describes a case of Abernethy Syndrome Type II where the patient had two separate extrahepatic portosystemic shunts treated with endovascular occlusion with two Amplatzer plugs and demonstrates the feasibility of this treatment for this rare condition. This case was in a young adult, adding to the scarce literature of treatment for Abernethy syndrome in the adult population.Case presentationWe report a case of a 20-year-old female patient with neurocognitive behavioral difficulty, voracious appetite, and chronic encephalopathy secondary to type II Abernethy malformation with not one, but two extrahepatic portosystemic shunts. The patient had failed medical management and was not a liver transplant candidate. Therefore, she presented to us for an endovascular treatment option. The two shunts were treated with endovascular occlusion using Amplatzer vascular plugs. Following embolization, flow into the hypoplastic portal vein improved with near complete occlusion of flow into the portosystemic shunts, thus restoring blood flow into the native portal system. At 3 month follow up, a CT demonstrated complete occlusion of the two portosystemic shunts, and a portal vein diminutive in caliber. The portal vein measured 7 mm in diameter on both pre and post-procedure CT scans. The total volume of the liver was found to be 843 cm3 on pre-procedure CT & 1191 cm3 on post-procedure CT.ConclusionsThis report demonstrates the feasibility of using endovascular embolization to treat Abernethy II malformations. The management strategy of Type II Abernethy Syndrome should be to redirect blood flow into the hypoplastic native portal system, allowing for physiologic hepatic metabolism of splanchnic blood, hypertrophy of the portal system, and growth of the liver from the increased trophic flow.

Comparative study of open and laparoscopic Kasai portoenterostomy in children undergoing living donor liver transplantation for biliary atresia.

The effectiveness of laparoscopic Kasai portoenterostomy (Laparoscopic-KPE) for biliary atresia (BA) has been reported but remains controversial. We reviewed our own cases and cases described in previous studies of liver transplantation (LT) for BA after Laparoscopic-KPE to investigate the efficacy of Laparoscopic-KPE. Subjects were children of ≤ 2years old with LT for BA after KPE who underwent Laparoscopic-KPE (n = 10) or Open-KPE (n = 115) between 2009 and 2020. Propensity score matching was performed to reduce the effect of treatment selection bias. The clinical data regarding the preoperative characteristics and surgical results were compared. The rates of hypoplastic portal vein and retrograde portal vein flow were lower in the Laparoscopic-KPE group than in the Open-KPE group (0 vs. 40.0%, p = 0.02 and 0 vs. 35.0%, p = 0.04). There was no marked difference in the operation time or duration of hepatectomy. For portal vein reconstruction, a vein graft was not required in the Laparoscopic-KPE group (0 vs. 35.0%, p = 0.03). No patients in the Laparoscopic-KPE group developed portal vein complications or required re-laparotomy for bowel perforation or re-bleeding, in contrast to the Open-KPE group (0 vs. 15.0% and 0 vs. 10.0%, respectively). Laparoscopic-KPE may reduce postoperative complications that necessitate re-laparotomy in LT.

Outcome of Portal Vein Reconstruction Using Branch Patch Anastomosis in Pediatric Living Related Liver Transplant Recipients Weighing Less than 10 kg

Pediatric living related liver transplantation (PLRLT) in children less than 10 Kg of weight with Portal vein (PV) reconstruction in atretic and hypoplastic portal vein is challenging. Portal venoplasty using branch patch anastomosis is one of the techniques that are being advocated for these groups of patients.

The Outcomes of Portal Vein Reconstruction With Vein Graft Interposition in Pediatric Liver Transplantation for Small Children With Biliary Atresia

Several technical modifications in portal vein (PV) reconstruction have shown excellent outcomes in pediatric liver transplantation (LT); however, which procedure is the best for PV reconstruction of the hypoplastic PV in pediatric LT remains unclear. One hundred sixteen pediatric patients aged 1 or younger with biliary atresia (BA) undergoing living donor LT (LDLT) at our center were enrolled in the present study to investigate the outcomes of the different types of PV reconstruction. We compared the results between patients with and without vein graft (VG) interposition (VG group, n = 33; non-VG group, n = 83) to analyze the risk factors for PV complications (PVCs). The median follow-up period was 4.7 years. PVCs occurred in 10 cases (7.2%), including 5 cases in the non-VG group and 5 in the VG group. Stenosis and thrombosis occurred in 10 and 3 cases, respectively, and 3 cases suffered from both. Three patients were surgically treated for PVCs, and 2 underwent stent insertion to treat short-term recurrence after the initial treatment. The incidence of PVCs in the 2 groups did not differ to a statistically significant extent. Although retrograde PV flow was 1 of the significant risk factors in a univariate analysis, a multivariate analysis revealed that early transplant era was the only independent risk factor for PVCs. VG interposition for PV reconstruction in LDLT appears to be a feasible alternative option with acceptable outcomes for patients with BA. Achieving sufficient PV flow is essential to preventing PVCs after LDLT.

Improved portal vein venoplasty with an autogenous patch in pediatric living donor liver transplantation.

A stenotic or hypoplastic portal vein (PV) represents a challenge for PV reconstruction in pediatric living donor liver transplantation (LDLT). Several PV venoplastic techniques have been developed. However, we still seek improved venoplastic techniques with better efficacy and compatibility. From June 2016 to July 2017, 271 LDLT procedures were performed at the Department of Liver Surgery, Renji Hospital. A total of 16 consecutive children with stenotic and sclerotic PVs underwent a novel technique-the autogenous PV patch plastic technique. Vessel patches were procured from the left branch (LB), or the bifurcation of the right branch and LB of the PV in the native liver. Then, the PVs were enlarged by suturing the patches along the longitudinal axis from the confluence of the PV and coronary vein (CV). In this series, 15/16 achieved good intraoperational PV flow, and 1 showed low PV flow but was treated with stent placement. Within a median follow-up of 11 months (1-18 months), 15 patients were alive and had normal graft function, whereas 1 child died from lung infection 1 month after transplantation. No PV complications were detected. In conclusion, the autogenous patch venoplasty technique using the PV-CV confluence is simple and safe. This novel venoplastic reconstruction technique could serve as a surgical option to achieve satisfactory outcomes, especially those with stenotic PV (<4.5 mm) and dilated CV (>3.0 mm). Liver Transplantation 2018 AASLD.

Challenging HCC surveillance in a case with a history of peliosis hepatis and liver cirrhosis CHILD A due to a hypoplastic portal vein

Challenging HCC surveillance in a case with a history of peliosis hepatis and liver cirrhosis CHILD A due to a hypoplastic portal vein

Simple ellipsoid reconstruction technique for a hypoplastic portal vein during pediatric liver transplantation.

Simple ellipsoid reconstruction technique for a hypoplastic portal vein during pediatric liver transplantation.

Catheter interventional treatment of congenital portosystemic venous shunts in childhood.

The study aimed on the catheter interventional treatment of congenital portosystemic venous shunt (CPSVS) in childhood and the impact on vascular growth of hypoplastic portal veins. Clinical course and follow up of partial or complete closure of CPSVS are described. CPSVS is a very rare vascular malformation of the portal venous drainage resulting in potentially life threatening abdominal, pulmonary, cerebral, and cardiac complications. The anatomic type, clinical course, and diagnosis must be determined for optimal management. Single centre case series. Eight (6 female) children with extrahepatic (n = 5) and intrahepatic (n = 3) CPSVS were diagnosed invasively by catheterization, including test balloon occlusion of the shunt and simultaneous retrograde angiography, and treated by catheter interventions (n = 5) with partial (n = 2) and complete (n = 3) occlusion of CPSVS at a median age of 3.9 years (range 0.7-21). Congenital heart disease (CHD) was the most frequent associated organ manifestation (n = 5) followed by mild to severe pulmonary arterial hypertension (n = 4), hepatopulmonary syndrome (n = 2), and portosystemic encephalopathy (n = 1). CHD was simple (n = 3) or complex type (n = 2). Three patients were untreated so far, because they were in excellent clinical condition at an age <1 year, refused treatment, or planned for later treatment. Accurate invasive diagnosis of CPSVS with test balloon occlusion of the shunt is mandatory to depict the anatomic situation. Catheter interventional treatment of CPSVS offers a feasible and safe approach with complete or partial closure of the vascular malformation inducing potentially significant vascular growth of a former hypoplastic portal venous system. © 2015 Wiley Periodicals, Inc.

Preoperative hepatic hemodynamics in the prediction of early portal vein thrombosis after liver transplantation in pediatric patients with biliary atresia

Portal vein thrombosis (PVT) is one of the main vascular complications after liver transplantation (LT), especially in pediatric patients with biliary atresia (BA). This study aimed to assess the preoperative hepatic hemodynamics in pediatric patients with BA using Doppler ultrasound and determine whether ultrasonographic parameters may predict early PVT after LT. One hundred and twenty-eight pediatric patients with BA younger than 3 years of age underwent Doppler ultrasound within seven days before LT, between October 2006 and June 2013. The preoperative hepatic hemodynamic parameters were then compared between patients with early PVT (within 1 month following LT) and those without PVT. Receiver operating characteristic analysis was performed to determine the optimal cutoff value for predicting early PVT. Of the 128 transplant recipients, 41 (32.03%) had a hypoplastic portal vein (PV), 52 (40.63%) had hepatofugal PV flow and 40 (31.25%) had a high hepatic artery resistance index (HARI) of ≥1. Nine cases (7.03%) experienced early PVT. A PV diameter ≤4 mm (sensitivity 88.89%, specificity 72.27%), and a hepatofugal PV flow (sensitivity 77.78%, specificity 62.18%) with a high HARI ≥1 (sensitivity 77.78%, specificity 72.27%) were hepatic hemodynamic risk factors for early PVT. Hepatic hemodynamic disturbances in pediatric recipients with BA were more common. Small PV diameter (≤4 mm) and hepatofugal PV flow combined with high HARI (≥1) are strong warning signs of early PVT after LT in pediatric patients with BA. Intense monitoring of vascular patency and prophylactic thrombolytic therapy should be considered in pediatric patients undergoing LT for BA.

Computational Simulation-Based Vessel Interposition Reconstruction Technique for Portal Vein Hypoplasia in Pediatric Liver Transplantation

BackgroundTo enhance the technical feasibility of portal vein (PV) interposition grafting for pediatric PV hypoplasia, we performed a computational simulation to establish a customized surgical technique allowing a secure anastomosis of an iliac vein graft to a severely hypoplastic PV stump. MethodsBased on the literature and on our own experience with reconstruction of PV hypoplasia, we devised three types of recipient PV stump preparations and three types of interposition vein grafts, yielding five technically feasible combinations. ResultsThe computational simulation model for PV reconstruction using an interposition vein graft revealed the most feasible combination to be a sequential inverted-T incision to the confluence of the superior mesenteric vein and splenic vein with a longitudinal slit in the transverse vein graft end, the technical feasibility was validated by an artificial suture model. This reconstruction was clinically applied to treat a 7.2-kg 10-month-old female patient with biliary atresia and a severely hypoplastic PV. The PV reconstruction was successful; the patient recovered uneventfully. ConclusionsWe have presented a simplified surgical technique for PV interposition that is applicable to pediatric PV hypoplasia, which also appears to be a feasible option for pediatric liver transplantation.

Congenital Extrahepatic Portosystemic Shunts (Abernethy Malformation)

Congenital extrahepatic portosystemic shunt, also known as Abernethy malformation, is a rare malformation in which intestinal and splenic venous blood bypasses the liver and drains into systemic veins. Aside from the complete or near-complete absence of portal veins, other histologic features of Abernethy malformation have not been evaluated in the literature. The goal of this study was to detail the hepatic histopathology in 5 patients with Abernethy malformation diagnosed at our institution. Paraffin-embedded tissue sections from 1 explant, 2 liver tumor resections, and 2 liver biopsies were evaluated using hematoxylin and eosin stains, reticulin, elastic, and trichrome stains, and immunohistochemistry for D2-40. Histologic findings included absence of portal veins in small portal tracts, absent or hypoplastic portal veins in medium-sized and large-sized portal tracts, isolated capillaries and arterioles in the lobules, hypertrophy of hepatic artery branches, remodeling of the liver architecture, and nodular regenerative hyperplasia in 1 case. Two patients had hepatocellular carcinoma without cirrhosis, and 2 had focal nodular hyperplasia. In addition to loss of portal veins, Abernethy malformation is characterized by multiple abnormalities due to remodeling of the hepatic vasculature. Abernethy malformation may also be associated with hepatocellular carcinoma and focal nodular hyperplasia in some patients.

A Case of Congenital Extra Hepatic Portocaval Shunt (Abernethy Malformation Type 2) with a very Large Liver Mass and an Atrial Septal Defect

저자들은 간의 거대 종괴와 심방 중격 결손으로 수술을 받았던 환아에서 복부 CT와 간 조직 검사를 통해 확인된 Abernethy 기형 2형 1예를 경험하였기에 문헌 고찰과 함께 보고하는 바이다. 【Extrahepatic portosystemic shunts, known as Abernethy malformations, were first reported by John Abernethy in 1793. They are classified into two types: Type I refers to a congenital absence of the portal vein and Type II refers to a shunt involving a side-to-side anastomosis with reduced portal blood flow into the liver parenchyma. This malformation is so rare that less than 100 cases have been reported in the medical literature. We report the case of a 13-month-old boy who had a congenital extrahepatic portocaval shunt with a hypoplastic portal vein. This case was complicated with an atrial septal defect and a large hyperplastic nodule in the liver. The patient was diagnosed with a Type II Abernethy malformation. We planned on surgical occlusion of the extrahepatic portocaval shunt. However, six months later, the patient had a sudden onset of a fever of unknown origin and developed hepatic encephalopathy. Although he underwent a liver transplantation, he died of acute hepatic failure.】

Congenital portocaval fistula associated with hepatopulmonary syndrome: ligation vs liver transplantation

A 4-year-old boy underwent pulmonary testing for diagnosis of exercise-induced dyspnea and subsequent cyanosis. Findings demonstrated the presence of multiple pulmonary arteriovenous fistulas resulting in oxygen desaturation owing to shunting (Pa o 2, 44 mm Hg). Abdominal ultrasound, abdominal computer tomography, and mesenteric angiography revealed an extrahepatic portocaval fistula (PCF), absence of a patent portal vein, and no evidence of portal hypertension. Because these findings were consistent with hepatopulmonary syndrome (HPS), liver transplantation was initially considered. However, subsequent workup using cavofistulography revealed the presence of a hypoplastic portal vein that selective catheterization showed to be threadlike but patent. Based on this finding, a definitive diagnosis of a congenital PCF with hypoplasia of the portal vein (type 2 Abernethy malformation) was made and surgical ligation with transection of the fistula was performed at the age of 5. Treatment was successful without subsequent development of portal hypertension and pulmonary symptoms disappeared. Follow-up examination 4 years later showed that the boy was asymptomatic and that the intrahepatic portal system was patent with normal hepatopetal flow. This is the first reported case of HPS because of portal type 2 Abernethy malformation. Anatomical types of PCF and corresponding therapeutic options in case of HPS are discussed.

Portal Vein Cavernoma Associated with Goldenhar Syndrome

Portal Vein Cavernoma Associated with Goldenhar Syndrome

Congenital extrahepatic portocaval shunt (Abernethy Type 2), huge liver mass, and patent ductus arteriosus-a case report of its rare clinical presentation in a young girl

The authors report a case of congenital extrahepatic portocaval shunt with a hypoplastic portal vein. This type of shunt is called an Abernethy type 2 shunt, where the portal vein and inferior vena cava are connected side to side, and a hypoplastic portal flow exists in the liver parenchyma. This case of a young girl is complicated with a cardiac anomaly and a large hyperplastic nodule in the liver. This phenotype usually is seen in the Abernethy type 1 shunt, which is a side-to-end shunt with the complete absence of a portal vein. In this context, the current case is very unique in its clinical manifestation, and this phenotype has never been reported. Therefore, it is important to speculate the etiology and treatment of this kind of abnormality and its associated hepatic mass.

Improved technique of portal vein reconstruction in pediatric liver transplant recipients with portal vein hypoplasia.

Children with small or hypoplastic portal veins represent a challenge for liver transplantation. Graft loss of up to 70% has been reported in these patients in the past. A variety of techniques has been used in both cadaveric and living related transplants in an effort to overcome this problem. Variability arises as to whether to use a vascular graft and where on the portal system to attach the graft. We present our usage of a simple and straightforward interposition iliac vein allograft fashioned in a manner to achieve large anastomotic cross-sectional area on the confluence of the superior mesenteric/splenic veins. The procedure also overcomes problems of graft vein/portal vein size mismatch in the cases where liver and vein grafts are procured from much larger donors. A total of 14 children presented with hypoplastic portal vein (diameter<5 mm), of a total of 30 consecutive patients requiring cadaveric liver transplants, and benefited from this technique. Median recipient age was 10.5 months. Revascularization times ranged from 22 to 43 min with a mean of 33 min. All patients are alive and well at a mean follow-up of 329 days (10 months). All liver grafts are well and functioning. No portal vein problem was detected. Results from this technique are clearly encouraging. Because portal vein hypoplasia is a common problem in pediatric transplant candidates, we believe this alternative technique is of interest and should be added to the transplant surgeon's armamentarium.