Congenital Absence Of The Portal Vein Research Articles

A 57 Year Old with Recurrent Hepatic Encephalopahthy

59 year old Caucasian male presents with 3–4 days of encephalopathy. He has a past medical history of celiac sprue, hypertension, asthma, and GERD with history of recurrent encephalopathy for the past 3 years with consistently elevated ammonia levels despite lactulose therapy. He was complaint with a gluten free diet. He has had a complete neurological workup including MRI/CT of brain that showed mild degenerative changes and an EEG that showed generalized slowing of waves not consistent with his symptoms. Hepatic workup for elevated serum ammonia included liver biopsy and an ERCP that was unremarkable. A mesenteric angiogram with transhepatic portagraphy displayed a portal vein to inferior vena caval shunt as a cause of his elevated ammonia levels. Hepatic Encephalopathy is neuro-psychiatric syndrome caused by liver disease. It occurs most often in patients with cirrhosis but is also seen in acute hepatic failure.[1] Liver failure and portasystemic shunting of blood are two important factors underlying hepatic encephalopathy.[1] Portasystemic shunts are of two major types spontaneous and surgical.[1] Surgical porto-caval shunts have been widely used in the treatment of Cirrhosis and recurrent variceal bleeding, however little is known about the congenital formation of these anomalies. Congenital intrahepatic portal-systemic venous shunt is defined as a communication between the portal and the systemic-venous circulation, measuring more than 1 mm in diameter and at least partially located inside the liver.[3] It is a very rare condition with uncertain etiopathogenesis.[3] There are 5 different types. Type I (intrahepatic type) designates cases in which shunts are located between the portal and systemic veins. Type II designates a type of intra/extrahepatic shunt that originates from the umbilical part of the portal vein and serpentines in the liver, then leaves the liver. Type III (extrahepatic type) occurs most frequently. Type IV (extrahepatic) is accompanied by shunts similar to those in type III, but hepatic pathology presents as idiopathic portal hypertension. Type V (extrahepatic) represents the congenital absence of the portal vein, where the superior mesenteric vein joins the intrahepatic inferior vena cava or the left renal vein.[2] Only 47 cases have been reported in the entire French and English literature.[3]

Portal Vein Cavernoma Associated with Goldenhar Syndrome

Portal Vein Cavernoma Associated with Goldenhar Syndrome

Successful liver transplantation for congenital absence of the portal vein complicated by intrapulmonary shunt and brain abscess

A 27-day-old boy had convulsion associated with brain abscesses and severe hypoxemia at the age of 3 months. Congenital absence of the portal vein (CAPV) and some associated anomalies were detected by radiological examinations. Brain abscess and hypoxemia were thought to be serious complications resulting from CAPV and were successfully corrected by living donor liver transplantation at the age of 4 months. This is the first report of a successful transplantation indicated for intrapulmonary shunt and brain abscess in an infant with CAPV.

Congenital absence of the portal vein with splenomegaly in a young woman (Case Report)

Congenital Absence of the Portal Vein (CAPV) is a rare anomaly in which the intestinal and splenic venous drainage bypasses the liver and drains into the systemic veins through various venous shunts. We present a case of a 32-year-old woman with this malformation, the patient experienced hematemesis and melena repetitively and had splenomegaly and hypersplenism. The angiography demonstrated absence of portal vein. Splenectomy was performed to reduce the pressure of the veins around stomach and to correct the hypersplenism. In endoscopy examination six months after surgery, the esophageal varices had disappear and the size of gastric varices had decrease. (Med J Indones 2005; 14: 122-7) Keywords: Congenital absence of portal vein, splenomegaly, hypersplenism, hematemesis and melena

Congenital absence of the portal vein in an adult woman: A case report

Congenital absence of the portal vein is a rare malformation. Mesenteric and splenic venous blood bypasses the liver and drains into the renal veins or directly into the inferior vena cava. The malformation occurs predominantly in females and children and is often associated with other anomalies such as hepatic tumors, cardiac malformations and skeletal abnormalities. We describe a 23-year-old female with congenital absence of the portal vein associated with variant origin of the hepatic artery from the superior mesenteric artery. Additional anomalies were not present. The patient is in good condition. Laboratory tests showed elevated total bilirubin; other liver parameters were within normal range. The absence of the portal vein was confirmed by MR angiography, which revealed that the upper part of the superior mesenteric vein and the splenic vein drained into the left renal vein. A liver biopsy showed no signs of cirrhosis. Yearly follow-up with laboratory tests and ultrasonography was recommended to our patient. According to the literature, the prognosis of this patient depends on the presence of cardiac defects and liver tumors. There is little information on the outcome of patients with congenital absence of the portal vein; only long-term follow-up investigations will provide answers to the numerous remaining questions.

Living domino liver transplantation in an adult with congenital absence of portal vein

Congenital absence of the portal vein (CAPV) is a rare malformation of the splanchnic venous system. Although CAPV is usually detected in the pediatric age group, our patient was a 35-year-old woman. She had been diagnosed with CAPV in 1996 when she was 27 years old. In 1998, she was placed on hemodialysis due to chronic renal failure. After several episodes of encephalopathy in 2002, liver transplantation (LT) was recommended to her and her family. Since there was no suitable living donor candidate, she was put on the waiting list for a deceased donor liver transplant in Japan. In 2004, her ammonia level increased to around 300 microg/dl, and she went into a coma lasting for three days. After recovering from this event, she underwent a living domino transplantation using a whole liver donated by a familial amyloid polyneuropathy (FAP) patient. Her portal vein, which had drained directly into the inferior vena cava (IVC), was transected together with a cuff of the IVC wall and anastomosed to the graft liver portal vein in an end-to-end fashion. In conclusion, liver transplantation proved to be a safe and effective way to save this patient and improve her quality of life.

A case of congenital absence of the portal vein

We experienced a girl with congenital absence of the portal vein. She was examined by computed tomography (CT), three-dimensional computed tomographic angiography (3DCTA), digital subtraction angiography and liver biopsy. Nodular regenerative hyperplasia of the liver was detected, presumably due to an abnormal hepatocellular response to absent portal flow. 3DCTA showed that the splenic vein and superior mesenteric vein joined to form a common trunk, which directly entered the right atrium. 3DCTA may be a valuable noninvasive tool for identifying portal malformations.

Congenital absence of portal vein presenting as hepatopulmonary syndrome

A 9-year-old girl presented with cyanosis and decreased exercise tolerance. Chest X-ray, lung function and echocardiogram were normal. Contrasted echocardiogram showed intrapulmonary right to left shunt and computerized tomography of the thorax showed dilated pulmonary vasculature. There was no arteriovenous malformation. Computerized tomography of the abdomen revealed absence of the intrahepatic portion of the portal vein. The superior mesenteric and splenic veins joined as a common trunk, bypassed the liver and drained into the right atrium. We concluded that the patient had hepatopulmonary syndrome secondary to absence of the portal vein. This is the first report of hepatopulmonary syndrome in a female paediatric patient with a congenital absence of the portal vein. As all portal blood entered directly into systemic circulation, the condition was equivalent to congenital portosystemic shunt. Cases of congenital portosystemic shunt complicated by hepatopulmonary syndrome were also reviewed.

Hepatic Nodular Lesions Associated with Abnormal Development of the Portal Vein

We reviewed the medical records including pathologic descriptions, CT images, MR images, and digital subtraction angiograms of three patients with hepatic lesions that were associated with abnormal development of the portal vein-patent ductus venosus or congenital absence of the portal vein-to clarify the imaging characteristics of these abnormalities. Two-phase helical CT and MRI, including MR angiography and dynamic studies, are useful for the diagnosis of patent ductus venosus and congenital absence of the portal vein. MRI may more effectively reveal hepatic lesions than two-phase helical CT under such abnormal conditions in which the liver has only the arterial but not the portal blood supply.

Sinusoidal Dilatation and Congestion in Liver Biopsy: Is It Always Due to Venous Outflow Impairment?

Impairment of venous outflow manifests as zone 3 sinusoidal dilatation and congestion (SDC) in liver biopsy. However, the finding of SDC is not specific for venous outflow impairment.Objectives.-To determine the specificity of SDC in liver biopsies for venous outflow impairment and to seek an explanation for SDC in patients without clinical or radiologic features of venous outflow impairment. Liver biopsies from 51 patients with sinusoidal dilatation were reviewed. Biopsies from transplant recipients, patients with cirrhosis, and patients with hepatic neoplasms (primary or metastatic) were not included. Clinical records were reviewed for laboratory tests and final clinicopathologic diagnosis. Thirty-four patients (66.7%) had confirmed venous outflow impairment. Of the 17 cases (33.3%) without clinical and/or radiologic evidence of venous outflow impairment, vascular causes were present in 5 cases (9.8%; nodular regenerative hyperplasia in 2 cases and portal vein thrombosis, congenital absence of the portal vein, and sickle cell anemia in 1 case each). Systemic inflammatory disorders were identified in 6 patients (11.8%). These included 2 cases of Castleman disease and 1 each of sarcoidosis, Crohn disease, rheumatoid arthritis, and Still disease. Three patients (5.9%) had tumors without direct involvement of the liver (1 case each of Hodgkin lymphoma, renal cell carcinoma, and pancreatic serous pseudopapillary tumor). In the remaining 3 patients, SDC was identified in wedge liver biopsies performed at the time of surgery, including gastric bypass surgery, cholecystectomy, and splenectomy. No other disease association was apparent in these cases. Sinusoidal dilatation and congestion in liver biopsy is associated with venous outflow impairment in two thirds of the cases. In the absence of clinical and/or radiological evidence of venous outflow, diagnostic considerations include other vascular conditions, such as portal vein insufficiency and nodular regenerative hyperplasia. Sinusoidal dilatation and congestion can also occur in the setting of systemic inflammatory diseases, granulomatous disorders, and neoplasms, as well as in wedge biopsies obtained during abdominal surgery.

Congenital absence of the portal vein and interruption of the inferior vena cava with end-to-end portosystemic shunt, hepatopulmonary syndrome and focal nodular hyperplasia of the liver

Congenital absence of the portal vein and interruption of the inferior vena cava with end-to-end portosystemic shunt, hepatopulmonary syndrome and focal nodular hyperplasia of the liver

Congenital absence of the portal vein: clinical and radiologic findings.

Congenital absence of the portal vein (CAPV) is a rare anomaly in which the intestinal and splenic venous drainage bypasses the liver and drains into the systemic veins through various venous shunts. In patients with CAPV, the portosystemic shunting causes disruption of the enterohepatic circulation and leads to various clinical manifestations. CAPV can be diagnosed without invasive techniques. This article illustrates the clinical and radiologic findings (including ultrasound, CT, and MRI) of CAPV.

Congenital absence of the portal vein and role of liver transplantation in children

Background/Purpose: Congenital absence of the portal vein (CAPV) is a subtype of congenital portosystemic shunt, which can cause a broad spectrum of clinical manifestations. The authors report on 4 patients with CAPV including a boy with CAPV-associated encephalopathy, which was resolved effectively by liver transplantation (LT). Methods: The case records of 4 pediatric patients with CAPV who were referred to the author's institution between 1984 and 1999 were reviewed. Results: The patients (3 boys and 1 girl) ranged in age at diagnosis from 0.8 to 14 years. Two patients had growth retardation or disturbed consciousness, and the other 2 had no specific manifestations. Not only high serum levels of bile acids, ammonia, and transaminases but also low plasma levels of branched-chain amino acids were common laboratory findings. The absent portal vein was replaced by a large portosystemic shunt, which connected the splanchnic vein to the inferior vena cava or the left renal vein. Two patients survived without any symptoms, but 1 with growth retardation died of hepatic failure. The other with encephalopathy did not respond to medical therapy and underwent LT, which resolved symptoms and metabolic disorders effectively. Conclusions: Patients with CAPV do not always have a good prognosis. They should be followed up with careful observation of their symptoms, hepatic function, and metabolic abnormalities. LT might be indicated for patients with symptomatic CAPV unresponsive to medical therapy. J Pediatr Surg 36:1026-1031. Copyright © 2001 by W.B. Saunders Company.

Congenital absence of the portal vein associated with focal nodular hyperplasia of the liver and congenital choledochal cyst: A case report

A congenital absence of the portal vein (CAPV) is a rare malformation, which almost is always associated with other anomalies such as hepatic tumors and cardiac malformations. This case report describes a 3-year-old girl with a congenital absence of the portal vein, focal nodular hyperplasia (FNH) of the liver, and a congenital choledochal cyst (CCC). Angiography findings showed the mesenteric vein and splenic vein to be joined together to form a common trunk that entered the inferior vena cava directly above the liver. This is the first known reported case of CAPV with concurrent CCC. J Pediatr Surg 36:622-625. Copyright © 2001 by W.B. Saunders Company.

Living related partial liver transplantation for hyperammonemia due to congenital absence of the portal vein

Living related partial liver transplantation for hyperammonemia due to congenital absence of the portal vein

Portal-systemic encephalopathy in non-cirrhotic patients: classification of clinical types, diagnosis and treatment.

Hepatic encephalopathy is suspected in non-cirrhotic cases of encephalopathy because the symptoms are accompanied by hyperammonaemia. However, the cause of the large portal-systemic shunt formation observed in these cases is not clear, as cirrhosis and portal hypertension are absent. The frequency of such cases reported in the literature is increasing with progress and spread of abdominal imaging diagnostic techniques. Some cases have been misdiagnosed as psychiatric diseases (dementia, depression and others) and consequently patients have been hospitalized in psychiatric institutions or geriatric facilities. Some paediatric cases have also been misdiagnosed. Therefore, the importance of accurate diagnosis of this disease should be strongly emphasized. Some paediatric cases have also been misdiagnosed. When psychoneurological symptoms are suggestive of hepatic encephalopathy but objective and subjective symptoms or abnormal values of liver function tests are not sufficiently indicative of liver cirrhosis, portal-systemic encephalopathy should be suspected. Abnormal angiograms of the portal vein, superior mesenteric vein or splenic vein are conclusive evidence of portal-systemic encephalopathy. Transrectal portal scintigraphy also provides information useful for detection of shunts and a quantitative estimation of shunt index. We classified the disease into five types based on whether the shunt is formed inside or outside the liver. Type I (intrahepatic type) designates cases in which shunts are located between the portal and systemic veins. Type II designates a type of intra/extrahepatic shunt that originates from the umbilical part of the portal vein and serpentines in the liver, then leaves the liver. Type III (extrahepatic type) occurs most frequently. Type IV (extrahepatic) is accompanied by shunts similar to those in type III, but hepatic pathology presents as idiopathic portal hypertension. Type V (extrahepatic) represents the congenital absence of the portal vein, where the superior mesenteric vein joins the intrahepatic inferior vena cava or the left renal vein. The prevalence of each type in our country was examined by a nationwide investigation. In addition to the conventional diet or drug treatments, obliteration by less invasive interventional radiology using a metallic coil and ethanol has recently been used more frequently than surgical occlusion of shunts. Shunt-preserving disconnection of portal and systemic circulation and partial splenic artery embolization are also performed. International investigation of the disease status and establishment of diagnostic and therapeutic methods for the disease are awaited and investigation of long-term prognosis after therapy is also necessary.

A case of congenital absence of the portal vein associated with hyperplastic nodule in the liver with special reference to their relevancy.

A case of congenital absence of the portal vein associated with hyperplastic nodule in the liver with special reference to their relevancy.

HEPATIC STEATOSIS DUE TO CONGENITAL ABSENCE OF THE PORTAL VEIN

Congenital portosystemic shunts are rare and known to cause various metabolic derangements. We report a child with congenital absence of the portal vein and portosystemic shunt who presented with hypergalactosemia, and was found to have other biochemical abnormalities as well as hepatocellular steatosis. A nine month old Caucasian girl was born at 38 weeks after an uncomplicated pregnancy. Her newborn metabolic screen on day of life 4 detected a high level of free galactose at 15 mg/dL (normal < 4). The RBC activities of the three major galactose metabolic enzymes were normal. She developed normally and achieved developmental milestones in a timely fashion. At 4 months the spleen was palpable 2 cm beneath the left costal margin. The elevated galactose level with normal enzymatic activity prompted investigation for evidence of a portosystemic shunt. A magnetic resonance angiogram of the abdomen demonstrated a small liver, with a virtually absent left lobe. The liver was of normal signal intensity, and without focal lesions. Mild splenomegaly was identified. No portal venous flow was identified either intra or extrahepatically. The superior mesenteric and splenic veins were patient, and drained via a large shunt to the left renal vein, and subsequently to the IVC. No other collaterals were identified. There was a small accessory retro-aortic left renal vein. Urine contained a trace amount of reducing substances. Serum chemistries revealed moderately elevated AST, ALT, and alkaline phosphatase. Blood ammonia was twice normal. Serum glucose, bilirubin, albumin, CBC, PT, and PTT were all normal. Subsequent evaluation at 8 months revealed an alpha-fetroprotein of 52.9 IU/ml (normal <6.4), a serum bile acid level of 3855 mcg/dl (normal <60), and hepatitis A and B serologies that were negative. The patient underwent liver biopsy at 9 months. The general hepatic architecture was well preserved, and the hepatocytes had normal size and orientation. There was depletion of glycogen and moderate diffuse microvesicular fatty change of hepatocytes. There was focal pericellular fibrosis with no evidence of necroinflammatory activity. Ultrastructural exam revealed occasional mitochondrial enlargement, but was otherwise normal. Peroxisomes were normal. This case illustrates histologic abnormalities of the liver, as well as biochemical abnormalities, that result from congenital absence of the portal vein and abnormal hepatic blood supply.

Congenital extrahepatic portocaval shunts—The Abernethy Malformation

The first account of an absent portal vein and a congenital mesenterico-caval shunt was given by John Abernethy in 1793. Five new cases of congenital extrahepatic portocaval shunts are described in this report. One end-to-side shunt with congenital absence of the portal vein (type 1 shunt) is the 13th case in the literature. Four are side-to-side (type 2) shunts of which there have been only two previous reports. Type 1 shunts are associated with other congenital abnormalities and have only been recognized in girls. Five of these cases developed liver tumours. Type 2 shunts are rarer, and four of five have been boys. They are not usually associated with other anomalies. Encephalopathy may be associated with these shunts in adults, and surgical closure of the side-to-side shunts is therefore recommended. End-to-side shunts are not correctable but the abnormal anatomy may create a problem for hepatic transplantation, should this be necessary for associated disorders such as biliary atresia. “The Abernethy Malformation” would seem to be a suitable eponym for congenital extrahepatic portocaval shunts.

Congenital absence of the portal vein: Two cases and a proposed classification system for portasystemic vascular anomalies

Congenital absence of the portal vein (CAPV) is an unusual condition that often is associated with other anomalies. This is the first report of reduced-size liver transplant in a patient with CAPV. Because the presence of this rare congenital portasystemic shunt, there was no portal-systemic pressure differential, and thus an absence of collateral vessels in the pretransplant state. As a result, surgery was complicated by severe mesenteric edema caused by an increase in portal pressure when the allograft was implanted. The morbidity associated with CAPV usually results from associated conditions, but if transplantation is necessary, careful management of mesenteric congestion is crucial to success. The authors' experience and a review of the literature indicate that the CAPV can be classified into one of two groups of portasystemic anomalies.