Congenital Absence Of The Portal Vein Research Articles

Unraveling the mystery: congenital absence of the portal vein and its cardiac implications.

A 25-year-old male with tachycardia and right heart enlargement was diagnosed with atrial-ventricular nodal reentrant tachycardia (AVNRT) and successfully treated with radiofrequency ablation. Cardiac MRI confirmed right heart enlargement but ruled out cardiomyopathy. Further imaging revealed a rare congenital absence of the portal vein (CAPV) with an associated portosystemic shunt. This case highlights the importance of considering CAPV in unexplained cardiac symptoms and structural heart changes.

Living donor liver transplantation with pericholedochal collateral vein anastomosis in a pediatric patient with congenital absence of the portal vein

Living donor liver transplantation with pericholedochal collateral vein anastomosis in a pediatric patient with congenital absence of the portal vein

Living donor liver transplantation with direct collateral portal vein anastomosis in a pediatric patient with congenital absence of the portal vein

Living donor liver transplantation with direct collateral portal vein anastomosis in a pediatric patient with congenital absence of the portal vein

Living donor liver transplantation with graft dextro-rotation and vein interposition in a pediatric patient with congenital absence of the portal vein

Living donor liver transplantation with graft dextro-rotation and vein interposition in a pediatric patient with congenital absence of the portal vein

Focal Nodular Hyperplasia and Focal Nodular Hyperplasia-like Lesions.

Focal nodular hyperplasia (FNH) is a benign lesion occurring in a background of normal liver. FNH is seen most commonly in young women and can often be accurately diagnosed at imaging, including CT, MRI, or contrast-enhanced US. In the normal liver, FNH frequently must be differentiated from hepatocellular adenoma, which although benign, is managed differently because of the risks of hemorrhage and malignant transformation. When lesions that are histologically identical to FNH occur in a background of abnormal liver, they are termed FNH-like lesions. These lesions can be a source of diagnostic confusion and must be differentiated from malignancies. Radiologists' familiarity with the imaging appearance of FNH-like lesions and knowledge of the conditions that predispose a patient to their formation are critical to minimizing the risks of unnecessary intervention for these lesions, which are rarely symptomatic and carry no risk for malignant transformation. FNH is thought to form secondary to an underlying vascular disturbance, a theory supported by the predilection for formation of FNH-like lesions in patients with a variety of hepatic vascular abnormalities. These include abnormalities of hepatic outflow such as Budd-Chiari syndrome, abnormalities of hepatic inflow such as congenital absence of the portal vein, and hepatic microvascular disturbances, such as those that occur after exposure to certain chemotherapeutic agents. Familiarity with the imaging appearances of these varied conditions and knowledge of their association with formation of FNH-like lesions allow radiologists to identify with confidence these benign lesions that require no intervention. Online supplemental material is available for this article. ©RSNA, 2022.

Living donor liver transplantation with proximal splenic vein ligation in a pediatric patient with congenital absence of the portal vein

Living donor liver transplantation with proximal splenic vein ligation in a pediatric patient with congenital absence of the portal vein

The effectiveness of intraoperative mesenteric portography for preventing misdiagnosis of congenital absence of the portal vein in dog with extrahepatic portosystemic shunt: a case report

A 5-month-old mixed-breed dog and a 10-month-old Welsh corgi dog were presented for evaluation with signs of congenital portosystemic shunt. In both dogs, computed tomography angiography revealed a single extrahepatic portosystemic shunt with the absence of the portal vein cranial to the shunt origin. Intraoperative mesenteric portography (IOMP) was performed after temporary complete occlusion of the shunt, revealing the portal branches faintly opacified with contrast medium. A cellophane band was applied around the shunt vessel without intraoperative attenuation. Postoperatively, both the dogs showed significant clinical improvement and the portal veins, not observed on preoperative ultrasonography, were visualized on postoperative ultrasonography. This study suggests the efficacy of IOMP, enabling the appropriate surgical procedure to be determined.

A 3-year-old boy with congenital absence of the portal vein presenting fasting hypoglycemia and postprandial hyperglycemia with hyperinsulinemia

A 3-year-old boy developed hypoglycemia with convulsions, rectal bleeding and hyperammonemia. Three-dimensional computed tomography scanning revealed the missing main branch of the portal vein, which confirmed the diagnosis of congenital absence of the portal vein (CAPV). Oral glucose tolerance test showed hyperglycemia with hyperinsulinemia at 30 min, whereas hypoglycemia was observed during fasting and at 120 min. Postprandial hyperglycemia was observed repeatedly. Repetitive hypoglycemia and rectal bleeding were uncontrollable. Living-donor liver transplantation, which was successfully performed, was apparently effective in preventing fasting hypoglycemia and improving hyperammonemia; rectal bleeding ceased after the surgery. Considerably, CAPV could result in hyperinsulinemia with partial failure of hepatic insulin degradation and hypoglycemia due to the lack of glycogen accumulation. Postprandial hyperglycemia could result from the fact that glucose-abundant splanchnic blood directly flows into the systemic circulation through the portosystemic shunt. Therefore, it is important to be aware of CAPV as a cause of glucose metabolism abnormalities including fasting hypoglycemia and postprandial hyperglycemia with hyperinsulinemia.

A226 INCIDENTAL FINDING OF A LARGE GASTRIC VARIX ASSOCIATED WITH NEONATAL UMBILICAL VEIN CANALIZATION

Abstract Results A 41-year old Asian male, who immigrated to Canada many years ago, and who had previously been successfully treated for Helicobacter pylori infection underwent gastroscopy for investigation of dyspepsia. His gastroscopy was normal except for a large subepithelial abnormality that was noted close to the gastroesophageal junction. Routine gastric biopsies from the antrum and body were normal. Subsequent endoscopic ultrasound revealed flow through the anechoic tortuous lesion and confirmed it was a very large isolated gastric varix type 1. Abdominal CT scan revealed chronic occlusion of the portal vein, splenic vein, and the portal confluence with extensive collateralization in the upper abdomen. There was complete cavernous transformation of the portal vein. Of the numerous varices in the upper abdomen, a very large varix drained into the left renal vein and indented into the posterior wall of the fundus of the stomach which accounted for the endoscopic finding. Multiple mesenteric veins were identified that connected to varices adjacent to the inferior aspect of the pancreas and duodenum. Notably, there was no evidence of cirrhosis or chronic pancreatitis. Liver enzymes, albumin, and INR were normal. Further collateral history revealed that he was hospitalized as a neonate for pneumonia with catheterization of the umbilical vein, which is known to be associated with thrombosis of the portal vein. Conclusions Detection of congenital absence of the portal vein (CAPV) is recognized more often due to advances in diagnostic imaging. Radiologically, the absence of the portal vein in CAPV is distinguished from portal vein thrombosis by the lack of venous collaterals or sequalae of portal hypertension, such as ascites or splenomegaly. A more gradual thrombosis of the portal vein may permit collaterals to develop without acute changes and is not equivalent to portal vein aplasia or agenesis as intrahepatic bile ducts are normal. The gold standard for diagnosis of CAPV is histologic absence of the portal vein in the liver on catheter angiography. CAPV is associated with abnormal embryologic development of the portal vein and frequently presents with complications of portal hypertension or portosystemic encephalopathy or the sequalae of venous shunts, hepatic or cardiac abnormalities found on imaging. Our case is an incidentally discovered absence of the portal venous system due to chronic thrombosis with extensive collateralization and an enlarged gastric varix protruding into the proximal stomach. It is well documented that canalization of the umbilical vein in infancy is associated with portal vein thrombosis, with incidences up to 68%. This case highlights the importance of eliciting a childhood hospitalization history in cases of non-cirrhotic portal hypertension. Funding Agencies None

The Congenital Absence of the Portal Vein

Congenital absence of the portal vein (CAPV) is a rare defect often accompanied by other abnormalities such as heart defect, skeletal anomalies and / or liver tumors [1]. We describe here a case of CAPV revealed by an upper gastrointestinal tract hemorrhage in a child aged 02 years and 8 months. The esogatro-duodenal fibroscopy revealed the presence of Esophageal varices grade II, abdominal ultrasound revealed a large liver with no visualization of the spleen. Subsequent abdominal computed tomography revealed the presence of a spleno-mesaraic trunk measuring 7 mm in anteroposterior diameter receiving the inferior and superior mesenteric vein and a small splenic vein draining by dilated peri-gastric and peri-esophageal leads. Associated with splenic hypoplasia and portal trunk atresia. The rest of the malformative assessment was negative

Abernethy Malformation in Combination with Gilbert’s Syndrome

Aim. To present a clinical case of the Abernethy syndrome.Key points. Abernethy syndrome is a rare vascular anomaly associated with a congenital absence of the portal vein, as a result of which portal blood from the intestines and spleen drains directly into the systemic circulation bypassing the liver though a complete or partial shunt. In the vast majority of cases, Abernethy syndrome is manifested during the newborn period by jaundice syndrome, hypergalactosemia and encephalopathy. In rare cases, this vascular malformation is diagnosed in older patients during ultrasound screening. A 31 year-old patient sought medical attention with the complaints of sleep disturbance and fatigue. The conducted instrumental observation revealed echo-signs of malformation (agenesia) of the portal vein, which was further confirmed by both X-ray-contrast computed tomography and the pathohistological analysis of liver biopsy slides. The genotype UGT1A1•28 confirmed Gilbert's syndrome. Neutropenia (0.8 × 109/L) with a drop in the level of segmented neutrophils up to 27% was regarded as shunt neutropenia. Number connection test confirmed shunt encephalopathy. Conservative therapy for correcting hepatic encephalopathy was prescribed, followed by a dynamic monitoring of the patient’s condition.Conclusion. Diagnosis of Abernethy malformation is important for choosing the right treatment for the timely correction of complications of the disease and early detection of adenoma or hepatocellular carcinoma.

The first report of living donor liver transplantation for abernethy malformation (congenital absence of the portal vein) in Vietnam

Abstract Abernethy malformation, also known as Congenital absence of the portal vein (CAPV), is a very rare malformation. There are some reports of liver transplantation as a treatment for CAPV, and our report is the first case in Vietnam. A 10-year-old boy, diagnosed with Abernethy type 1b malformation (portal vein draining directly into the inferior vena cava) and liver tumor. Living donor liver transplantation was performed using a right liver graft from his mother. Histopathological examination of the native liver showed a huge mass lesions within the right liver and absence of portal vein in portal space. Postoperatively, the patient attained optimal liver function and at a 13-month follow-up has returned to normal life.

Pediatric Living Donor Liver Transplantation for Congenital Absence of the Portal Vein With Pulmonary Hypertension: A Case Report

Few reports of liver transplantation exist in patients with congenital absence of the portal vein and pulmonary hypertension. Living donor liver transplantation is usually performed before exacerbation of pulmonary hypertension. A 7-year-old girl (height: 131.5 cm; weight: 27.4 kg) with congenital absence of the portal vein was diagnosed with pulmonary hypertension (mean pulmonary artery pressure 35 mm Hg), and liver transplantation was planned before exacerbation of pulmonary hypertension. We successfully managed her hemodynamic parameters using low-dose dopamine and noradrenaline under monitoring of arterial blood pressure, central venous pressure, cardiac output, and stroke volume variation. Anesthesia was maintained using air-oxygen-sevoflurane and remifentanil 0.1 to 0.6 μg∙kg-1∙min-1. It is necessary to understand the potential perioperative complications in such cases and to adopt a multidisciplinary team approach in terms of the timing of transplantation and readiness to deal with exacerbation of pulmonary hypertension.

Anomalies of the Portal Venous System in Dogs and Cats as Seen on Multidetector-Row Computed Tomography: An Overview and Systematization Proposal

This article offers an overview of congenital and acquired vascular anomalies involving the portal venous system in dogs and cats, as determined by multidetector-row computed tomography angiography. Congenital absence of the portal vein, portal vein hypoplasia, portal vein thrombosis and portal collaterals are described. Portal collaterals are further discussed as high- and low-flow connections and categorized in hepatic arterioportal malformation, arteriovenous fistula, end-to-side and side-to-side congenital portosystemic shunts, acquired portosystemic shunts, cavoportal and porto-portal collaterals. Knowledge of different portal system anomalies helps understand the underlying physiopathological mechanism and is essential for surgical and interventional approaches.

Congenital absence of the portal vein: translated version.

Congenital portosystemic shunt (CPS) is classified into type I (congenital absence of the portal vein) and type II, depending on the presence or absence of the intrahepatic portal vein. Reports still exist on cases in which psychiatric diseases or mental retardation was diagnosed or cases in which diagnosis and treatment are not performed until severe pulmonary complications occur, indicating the need to recognize CPS. Herein, we reviewed epidemiology, etiology, classification, symptom, diagnosis, and treatment based on clinical view points of CPS. For clinical view points, classification according to (1) the presence or absence of the intrahepatic portal venous system (IPVS) and (2) the extrahepatic or intrahepatic of shunt sites, facilitates the understanding of pathophysiological conditions and is useful in selecting a treatment for symptomatic CPS. Radiological and pathological examinations are important in IPVS evaluations, and IPVS evaluations are currently essential to make diagnoses by portography with balloon occlusion and liver biopsy. Symptomatic CPS (hepatic nodular lesions, portosystemic encephalopathy, and pulmonary complications, etc.) is an indication of treatment by shunt closure, but an indication of treatment for asymptomatic CPS is the challenge going forward.

An adult case of primary biliary cholangitis accompanied by a patent ductus venosus showing type-I congenital absence of the portal vein

An adult case of primary biliary cholangitis accompanied by a patent ductus venosus showing type-I congenital absence of the portal vein

Congenital absence of the portal vein complicated by hepatocellular carcinoma in the liver of an adult woman: review of imaging, literature and management

Congenital absence of the portal vein complicated by hepatocellular carcinoma in the liver of an adult woman: review of imaging, literature and management

Abernethy malformation type I (congenital absence of the portal vein) in a patient with chronic kidney disease

Abernethy malformation type I (congenital absence of the portal vein) in a patient with chronic kidney disease

Extensive Gastroesophageal Varices in a Non-Cirrhotic Female with Left Isomerism: A Case Report and Literature Review

: Heterotaxy syndrome with polysplenia is a congenital abnormality with malposition of the visceral organs and multiple associated abnormalities. The majority of affected individuals die during childhood due to severe cardiovascular anomalies. However, affected individuals who reach adulthood are usually asymptomatic. Anomalies of the abdominal venous system, including interruption of the inferior vena cava with azygos or hemiazygos continuation, congenital absence of the portal vein with a portosystemic shunt, preduodenal portal vein, and abnormalities of renal veins are frequent findings. Heterotaxy syndrome usually has no clinical significance except in the setting of planned vascular intervention or surgery. We are reporting an extremely rare case of heterotaxy syndrome (polysplenia) that presented with massive variceal hemorrhage due to associated venous malformation and systemic to portal venous system collaterals.

Congenital Extrahepatic Portosystemic Shunt (Abernethy Malformation Type Ib) with Associated Hepatocellular Carcinoma: Case report and Literature Review

Abernethy malformation, also termed congenital portosystemic shunt and congenital absence of portal vein (CAPV) is the result of malformation of the splanchnic venous system. Congenital portosystemic shunts are divided into extra-and intrahepatic shunts. Two shunts have been defined: type I is characterized by the complete diversion of portal blood into the vena cava with an associated congenital absence of the portal vein. Type II is defined by an intact but diverted portal vein through a side-to-side, extrahepatic connection to the vena cava. The clinical manifestations of Abernethy malformation are diverse with a typical presentation consisting of hypoxia and hepto-pulmonary syndrome. Histologically, focal nodular hyperplasia, nodular regenerative hyperplasia, liver adenoma, hepatoblastoma, and hepatocellular carcinoma have all been reported. Herein we report a case of Abernethy malformation, type Ib, in a 12 month old male who was found to have a small hepatocellular carcinoma at the time of explant. The immunohistochemical characteristics in relation to the genetic aspects are discussed. To our knowledge, this is the first reported case of hepatocellular carcinoma developing in a patient who is under the age of 5 years old with Abernethy Malformation.