Adolescent ; Arteriovenous Malformations ; Female ; Humans ; Portal Vein ; abnormalities

We present here the radiological findings of a rare case of multiple focal nodular hyperplasia that was associated with portal vein atresia and portopulmonary hypertension in a young woman. This case illustrates and supports the pathophysiological hypotheses that were previously proposed for the coexistence of these three abnormalities.
Adult ; Female ; Focal Nodular Hyperplasia/*radiography ; Humans ; Hypertension, Portal/*complications ; Hypertension, Pulmonary/*complications ; Portal Vein/*abnormalities

No abstract available.
Hepatic Encephalopathy/*congenital ; Humans ; Liver Neoplasms/*congenital ; Male ; Portal Vein/*abnormalities ; *Septal Occluder Device

Cavernous transformation of the portal vein is a rare condition probably arising secondary to extrahepatic portal vein thrombosis or obstruction with recannalization and/or collateral veins formation to bypass the obstruction. It is believed that cavernous transformation of the portal vein is caused by a variety of diseases associated with periportal collateral development and hepatopedal flow. It is known that portal vein occlusion, which is the actual cause of cavernous transformation, has a wide variety of etiologies, such as congenital abnormalities, omphalitis, pancreatitis, various carcinoma, and liver cirrhosis. In most cases, the revealing symptom is upper gastrointestinal bleeding. Rarely, however, diagnosis is made from obstructive jaundice. Extensive collateral veins due to portal vein occlusion may compress and narrow the biliary tract. A 39-year-old man was admitted due to jaundice and abdominal discomfort for 1 month. He was confirmed to have obstructive jaundice due to collateral vessels of cavernous transformation of the portal vein. We report a case of obstructive jaundice caused by cavernous transformation of the portal vein.
Adult ; Biliary Tract ; Congenital Abnormalities ; Diagnosis ; Hemorrhage ; Humans ; Jaundice ; Jaundice, Obstructive* ; Liver Cirrhosis ; Pancreatitis ; Portal Vein* ; Veins ; Venous Thrombosis

Cavernous transformation of the portal vein that is resulted as a collateral vessel formation from a portal hypoplasia is very rarely to be observed in human being. Such an abnormal transformation can be caused by congenital defect of vein formation in the early embryonal development or congenital hematologic hypercoagulability which gives rise to the cavernous development of cavernous collateral branches. Acute thrombosis of portal vein can also be the cause of acquired cavernous transformation. In most cases it extends into the parenchyme of liver, and often has porto-systemic shunt (with left gastropiploic vein and varices around spleen) or porto-portal shunt (with varices around gallbladder). A 48 years old male patient underwent splenectomy at the age of 24 owing to splenomegaly. He had liver cirrhosis without hepatitis and alcohol history. Intrahepatic stone in left lateral lobe of liver was diagnosed in the course of medical treatment. The cavernous transformation of portal vein with right lobe hypoplasia was confrimed during the operation.
Congenital Abnormalities ; Hepatitis ; Humans ; Liver Cirrhosis ; Liver* ; Male ; Middle Aged ; Portal Vein* ; Splenectomy ; Splenomegaly ; Thrombophilia ; Thrombosis ; Varicose Veins ; Veins

in the leg and the toe and was treated with angioplasty and thrombolytic therapy with phlebotomy.
Female ; Humans ; Middle Aged ; Polycythemia Vera/complications/*diagnosis/ultrasonography ; Portal Vein/*abnormalities ; Tomography, X-Ray Computed ; Venous Thrombosis/etiology

OBJECTIVETo discuss the techniques for excision and reconstruction of anomalous portal venous branches (APVB) in adult-to-adult right lobe living donor liver transplantation (A-A RL LDLT).

METHODSFrom February 2002 to April 2007, 70 cases of A-A RL LDLT were performed. Preoperative three-dimensional computed tomography of the donor revealed the configurations of hepatic artery, portal vein and hepatic vein. Nine donors had anomalous portal venous branching (APVB). The APVB were type II (trifurcation) in 7 cases and type III in two. Except the excision of APVB with a common opening by a narrow bridge of main portal vein tissue in one type II donor, all the right APVB were transected on the principal of donor priority: right APVB being excised approximately 2-3 mm from the confluence while leaving the donor's portal vein intact. In type II APVB, the donor portal venous branches were transected with separate two openings and reconstructed as double anastomoses in 4 cases, with separate two openings joined as a common orifice at the back table and reconstructed as single anastomoses in 2 cases, and with one common opening with narrow-bridge of tissue and reconstructed as single anastomoses in 1 case. In type III APVB, the APVB were transected with separate two openings and were reconstructed by double anastomoses in 1 case and by a new technique named U-shaped vein graft interposition in the another one.

RESULTSThere were no vascular complications such as portal vein stricture or thrombosis, hepatic artery stricture or thrombosis and hepatic vein outflow stricture in all 9 recipients transplanted with grafts with APVB. Only the type II APVB donor undergoing a excision of APVB with a common opening by a narrow bridge of main portal vein tissue developed portal vein thrombosis on the third postoperative day and underwent thrombectomy followed by repair with vein patch plasty. The velocity of blood flow in the U-graft was normal.

CONCLUSIONSIt is feasible and safe of APVB excision on the principal of donor priority and reconstruction including double anastomoses and the novel U-graft interposition in A-A RL LDLT, and has a good outcome without increasing the management difficulty.

Adult ; Female ; Follow-Up Studies ; Humans ; Liver Transplantation ; methods ; Living Donors ; Male ; Portal Vein ; abnormalities ; surgery ; Retrospective Studies ; Treatment Outcome

OBJECTIVETo investigate the ultrasonographic features of congenital intrahepatic portosystemic venous shunt (CIPSVS) and to assess the clinical value of ultrasonography in the diagnosis of CIPSVS.

METHODSSix cases of CIPSVS diagnosed in our hospital between March 2010 and March 2012 and confirmed by enhanced computed tomography (CT) were retrospectively reviewed. Five of the six cases had follow-up data that was included in the analysis.

RESULTSAmong the six CIPSVS cases, only one was classified as Park's type II and the rest were classified as Park's type III. Five cases involved the right lobe of the liver and only one case involved the left lobe. The lesion shapes included cystic, tubular, and irregular with clear contour and appeared to be anechoic on CT scan. The lesions ranged in size from 1.1*0.6 cm to 2.0*1.7 cm. For all cases, the color Doppler ultrasound images showed blood flowing from the portal vein to the hepatic vein, and single-phase spectrum was detected in the diversion channel. The differences observed in level of lesion size and blood flow velocity at the shunt from the time of examinations at diagnosis and subsequent follow-up did not reach statistical significance (P = 0.223 more than 0.05 and P = 0.930 more than 0.05 respectively).

CONCLUSIONAlthough cases of CIPSVS are rare, they share some specific sonographic features that may help in diagnosis. Color Doppler ultrasound findings have high diagnostic accuracy and may represent a preferred modality for follow-up monitoring.

Aged ; Female ; Humans ; Male ; Middle Aged ; Portal Vein ; abnormalities ; diagnostic imaging ; Retrospective Studies ; Ultrasonography ; Vascular Malformations ; diagnostic imaging

Klippel-Trenaunay-Weber syndrome is a congenital vascular disorder consisted of a variety of vascular malformations, enlargement of the involved limb, and varicose veins. We report a case of Klippel-Trenaunay-Weber syndrome presenting portal hypertension and varices bleeding caused by hypoplasia of the portal vein. Portal hypertension was caused by portal vein hypoplasia associated with Klippel-Trenaunay-Weber syndrome. There were three episodes of variceal bleeding, and hemostasis were achieved by endoscopic band ligation, Sugiura operation, and splenic artery embolization respectively. Although successful hemostasis was achieved, an additional procedures to reduce portal hypertension were needed to prevent repeated episodes of variceal bleeding.
Adult ; English Abstract ; Esophageal and Gastric Varices/*complications ; Gastrointestinal Hemorrhage/*etiology ; Humans ; Hypertension, Portal/complications ; Klippel-Trenaunay-Weber Syndrome/*complications ; Male ; Portal Vein/abnormalities

A 67-year-old woman presented with memory impairment and behavioral changes. Brain MRI indicated hepatic encephalopathy. Abdominal CT scans revealed an intrahepatic portosystemic venous shunt that consisted of two shunt tracts to the aneurysmal sac that communicated directly with the right hepatic vein. The large tract was successfully occluded by embolization using the newly available AMPLATZERTM Vascular Plug II and the small tract was occluded by using coils. The patient's symptoms disappeared after shunt closure and she remained free of recurrence at the 3-month follow-up evaluation.
Aged ; Embolization, Therapeutic/*instrumentation/methods ; Female ; Hepatic Encephalopathy/etiology/*therapy ; Hepatic Veins/abnormalities/radiography ; Humans ; Liver Circulation ; Portal Vein/abnormalities/radiography ; *Septal Occluder Device