Congenital absence of portal vein with multiple hyperplastic nodular lesions in the liver

Congenital absence of the portal vein is an extremely rare anomaly, in which enteric blood bypasses the liver and drains into the inferior vena cava. A 16-year-old girl was referred to our hospital presenting with liver tumor. Although she had suffered from galactosemia soon after birth, the galactosemia had improved spontaneously 1 year later. Between the ages of 8 and 12 years, chronic hepatitis with a mild elevation of aspartate transaminase (AST) and alanine transaminase (ALT) was observed, but liver tumor had not been detected on computed tomography (CT) in regular medical examinations. However, at age 16, liver tumors, 10 cm in diameter, were found. Abdominal angiography indicated complete absence of the portal vein, suggesting that enteric blood was bypassing the liver and draining into the inferior vena cava. In biopsy specimens obtained under ultrasonographic guidance, liver tumors were confirmed histologically as hyperplastic nodules. In addition to this case report, the clinical features of 25 reported cases of congenital absence of the portal vein are reviewed. Received: October 24, 2001 / Accepted: February 8, 2002 Reprint requests to: Y. Tanaka Editorial on page 308     

Congenital absence of the portal vein.

Absence of the terminal portion of the portal vein and its intrahepatic branches was demonstrated in a 22-year-old woman with focal nodular hyperplasia (FNH) of the liver. Various imaging studies and angiography showed that the portal vein entered into the inferior vena cava just above the renal veins. The clinical and radiological features of this patient and nine previously reported cases of this entity are herein reviewed.     

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.     

Congenital absence of the portal vein complicating hepatic tumors

Congenital absence of the portal vein (CAPV) is a rare malformation that is often accompanied by other anomalies such as cardiac and skeletal malformations and/or hepatic tumors. We describe here a case of CAPV complicating hepatic tumors in a 16-year-old Japanese girl. Abdominal ultrasonography revealed a hyperechoic tumor in the liver and dilatation of the portal vein that appeared to be connected directly with the inferior vena cava. Subsequent abdominal computed tomography (CT) revealed tumors and magnetic resonance angiography confirmed that the portal vein entered directly into the inferior vena cava just above the liver. In addition, there was absence of the right portal vein and the left intrahepatic branch except for the presence of left portal vein only within the porta hepatis. These findings led to a diagnosis of CAPV complicated hepatic tumors. Careful monitoring of these hepatic tumors is ongoing due to the possibility of malignant transformation.     

Multiple focal nodular hyperplasia of the liver associated with congenital absence of the portal vein

We report the 32nd case of congenital absence of portal vein in an 18-year-old female adult associated with multiple focal nodular hyperplasia of the liver. The association of various hepatic tumors has been observed in half of the publications about congenital absence of portal vein. Hepatic tumors seem to result from systemic diversion of portal vein flow with a resultant increase of arterial flow causing important vascular and nutritif changes the liver and consequent parenchymal transformation.     

Preduodenal portal vein in the adult

Preduodenal portal vein (PDPV) is a rare developmental anomaly. We recently encountered this anomaly in a 73-year-old woman who had gastric cancer and colonic cancer with liver metastasis. The PDPV was diagnosed preoperatively by computed tomography and angiography. To the best of our knowledge, there are only 19 adult cases reported in the world literature, with this additional case being reported here. In infants the anomaly has often been associated with duodenal obstruction, but in adults it is often symptomless. We discuss the disease process, embryology, diagnosis, association with other anomalies, and surgical treatment.     

Hydatid disease of the liver with portal vein invasion mimicking portal vein thrombosis

Hydatid cyst disease is a zoonosis caused by the parasite Echinococcus. It may infest any organ of the body, but it most frequently involves the liver, lungs, and nervous system. Portal vein involvement by hydatid cyst disease is extremely rare with only five cases published in the English literature to our knowledge. We present the ultrasonography (US) and computed tomography (CT) findings of a 77-year-old male with hydatid disease of the liver with portal vein invasion mimicking portal vein thrombosis. Colour Doppler US confirmed the lack of blood flow within the portal vein and stigmata of cavernomatosis. CT clearly demonstrated a communication between the multiloculated lesion and the portal vein and the multiple daughter vesicles obstructing the portal vein. The consideration of this complication will make it possible to distinguish this entity from portal vein thrombosis and, thus, the management of the patients with hydatid cyst disease particulary in endemic regions.     

肝癌门静脉癌栓的介入治疗

肝癌合并门静脉癌栓(PVTT)的治疗仍是医学难题,既往一直被视为手术禁忌,近年随着介入医学的进步,发展了多种微创治疗方法,取得了可喜的疗效,使肝癌并PVTT由不可治变为部分可治,展现了介入方法治疗肝癌并PVTT的乐观前景.本文就当前针对门静脉癌栓的介入治疗方法,技术要点及各种介入治疗方法的初步疗效进行了综述.为进一步的临床规范化综合治疗肝癌并门静脉癌栓提供参考.     

Cardiac anomalies associated with congenital absence of the portal vein.

The authors discovered congenital absence of the portal vein, with visceral venous return to the right atrium, in a 5-year-old girl with aortic valvar stenosis. Interestingly, of the 19 patients, it was discovered that 11 reported with portal venous agenesis also had cardiac defects. We have, therefore, investigated the hypothesis that the congenital absence of the portal vein and the associated cardiac malformations may result from a similar embryologic insult, and that cardiac development may be affected by the systemic diversion of portal venous flow.     

Congenital complete absence of pericardium in a young woman with non-specific symptoms

Congenital absence of the pericardium is a very rare entity that is usually asymptomatic and hence difficult to diagnose. However, cases of sudden death have been reported in patients with partial pericardial defects (even asymptomatic ones), and such patients require surgical treatment.We report the case of a 17-year-old patient with complete pericardial agenesis (diagnosed by chance during a cardiological consultation) and briefly review the radiological findings of this entity.     

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.     

Macroscopic portal vein tumor thrombi of liver metastasis from colorectal cancer

We present a case of multiple colorectal liver metastases with macroscopic portal vein thrombi. A 55-year-old woman presented to us with rectosigmoid cancer and presented with two liver metastases. The tumor in the posterior sector was associated with invasion of first order branches of the portal vein. We performed low anterior resection, hepatic posterior sectorectomy and partial left anterior sectorectomy. Both the colorectal cancer and liver tumors exhibited histological characteristics of moderately differentiated adenocarcinoma with a substantial amount of mucin production. The liver metastases were associated with prominent tumor thrombi in many branches of the portal vein. Stronger staining for endoglin (CD 105) than for Fas ligand (Fas L) and matrix metalloproteinase (MMP-2) was observed in both the colorectal cancer and metastatic liver tumor cells. Expression of the vascular endothelial growth factor within the tumor cells was seen in both the colorectal cancer as well as the metastatic liver tumor cells. Six months after the operation, she was diagnosed to have multiple, more than about 20 liver metastases, and in 9 months after the operation, the patient died. The colorectal cancer with liver metastases associated with portal vein tumor thrombosis was poor prognosis, found neoplastic microvessel formation.     

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

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