Mesenteric and portal venous obstruction associated with primary antiphospholipid antibody syndrome

A 58-year-old Korean man who had a past history of appendicitis, superior mesenteric vein thrombosis and intestinal obstruction presented 7 years later suffering from colicky right upper quadrant pain, epigastric discomfort after fatty meals, nausea and vomiting. He was found to have thrombosis of the superior mesenteric and portal veins, portal hypertension with oesophageal varices, cholangitis, and a biliary stone. The serum anti-cardiolipin antibody (aCL) titres were 103 immunoglobulin (Ig)G antiphospholipid units (GPL) and 50 IgM antiphospholipid units (MPL) and the aCL-IgG titre was still high at 106 2 years after the initial diagnosis. No evidence of disease states known to be associated with antiphospholipid antibodies was found. We report a patient with mesenteric and portal venous obstruction associated with the primary antiphospholipid syndrome (APS).     

Congenital absence of the portal vein in an adult woman with liver tumor]

A 30-years-old Japanese woman with a liver tumor was found to have congenital absence of the portal vein (CAPV). Both three-dimensional CT and angiography revealed that the superior mesenteric vein and splenic vein flowed into inferior vena cava and there was us portal vein, CAPV is an extremely rare congenital anomaly and liver tumor. Most cases on diagnosed in childhood, although this case was found in on adult. We reviewed the literature on reported CAPV cases.     

Venous thrombosis in gastroenterology

Venous thrombosis involving the digestive tract affects the suprahepatic veins and the terminal part of the inferior vena cava, the portal vein and its roots. The etiology and diagnosis of this condition have made considerable progress. A thrombogenic disease can now be recognized in 90% of cases of involvement of the suprahepatic veins, and 75% of portal involvements. The most frequent causes are primary myeloproliferative syndromes, paroxysmal nocturnal hemoglobinuria, hereditary deficiency in coagulation proteins and circulating anticoagulants. The cause of involvement of the portal vein also include insults during biliary surgery and abdominal infections, particularly those caused by Bacteroides fragilis. Mechanical involvement due to compression finally plays a minor role in the etiology. Noninvasive techniques of diagnosis are now available, including ultrasound, computed tomography and magnetic resonance imaging. The expression of obstruction of the suprahepatic veins predominantly consists in ascites and hepatomegaly. Thrombosis of the portal vein preserving the mesenteric arches usually remains asymptomatic until the intrahepatic block is revealed by a digestive hemorrhage caused by portal hypertension. Isolate involvement of the splenic vein exceptionally causes the rupture of gastric or esophageal collateral veins. The treatment should combine the prevention of further thromboses by anticoagulants and the specific treatment of the venous obstruction. In case of suprahepatic obstruction, there are several methods of restoring a canal of drainage for hepatic blood. Their indications depend on the patency of the inferior vena cava and of the portal vein. In case of portal obstruction, portal-systemic bypass is feasible only if one of the major roots of the portal vein still is patent.(ABSTRACT TRUNCATED AT 250 WORDS)     

Pseudo-Budd-Chiari syndrome: extrinsic deformity of the intrahepatic inferior vena cava mimicking membranous obstruction

Budd-Chiari syndrome secondary to membranous obstruction of the intrahepatic inferior vena cava is a treatable form of chronic liver disease. I report a patient with portal hypertension in whom distortion of the inferior vena cava by cirrhosis and increased intraabdominal pressure initially suggested this condition. The correct diagnosis was made by obtaining lateral views during inferior vena cavography, which demonstrated a tapered, rather than membranous, obstruction, along with normal hepatic venous anatomy and pressure and markedly increased portal vein pressure during transhepatic puncture with a thin needle. That extrinsic deformity of the inferior vena cava may mimic membranous obstruction has not been emphasized recently. This distinction is important as surgical membranotomy is not indicated in patients with cirrhosis and secondary deformity of the inferior vena cava.     

Membranous obstruction of the inferior vena cava associated with a myeloproliferative disorder: a clue to membrane formation?

Although membranous obstruction of the inferior vena cava is frequently assumed to be a congenital defect, it has recently been hypothesized that it could be acquired and secondary to thrombosis at the ostium of hepatic veins. We report the case of a membranous obstruction of the inferior vena cava responsible for isolated hepatomegaly in a young Frenchman. No known cause of thrombosis was found but spontaneous formation of erythroid colonies occurred on bone marrow cultures. This indicated the existence of a latent myeloproliferative disorder, a frequent cause of hepatic vein thrombosis. We suggest that this association of a membranous obstruction of the inferior vena cava with a known, albeit often overlooked, cause of hepatic vein thrombosis provides strong support to the thrombosis theory of membrane formation.     

Idiopathic inferior mesenteric venous thrombosis demonstrated by angiography.

Idiopathic thrombosis of the inferior mesenteric vein, not associated with portal vein thrombosis, is an uncommonly diagnosed lesion. We are reporting a case of inferior mesenteric vein thrombosis diagnosed preoperatively by angiography. We believe this to be the first case in the literature of this particular lesion demonstrated conclusively by angiography before surgery.     

Postsurgical chylous ascites in sarcoidosis with portal hypertension

A patient with extensive thromboses of portal and mesenteric veins and sarcoid of the liver developed recurrent pulmonary emboli, which necessitated the insertion of an umbrella into the inferior vena cava. Chylous ascites appeared shortly thereafter; the ascitic fluid fat content was strikingly reduced after reduction of dietary long chain triglycerides, but the ascitic fluid volume was reduced only after insertion of a peritoneovenous shunt (LeVeen). The shunt was found to be nonfunctioning 4 months later, but ascites did not recur. Seven years later, while eating a normal diet and still having a nonfunctioning shunt, he remains free of ascites. We postulate that iatrogenic obstruction of the inferior vena cava in the presence of mesenteric and portal vein thromboses, as well as obstruction of mesenteric lymphatics by sarcoid lymphadenopathy, led to the formation of chylous ascites and that establishment of adequate mesenteric and portal venous and/or lymphatic collateral circulation was responsible for his sustained improvement.     

Early diagnosis and radical surgical treatment of Budd-Chiari syndrome

We report a 26-year-old woman who was diagnosed with Budd-Chiari syndrome following consultation for a skin nodule in the lower extremity. Histopathological examination of a biopsy specimen showed features of erythema induratum. As part of the diagnostic work-up, chest roentgenography performed to rule out possible tuberculosis showed enlarged right lower mediastinum. Computed tomography identified a dilated azygos vein and obstruction of the inferior vena cava near the liver. Liver function tests and blood cell counts were all within normal limit and no sign of portal hypertension was noted except for mild splenomegaly. Although angioplasty by balloon catheter resulted in recanalization of the obstructed inferior vena cava, obstruction of the inferior vena cava appeared again 2 months later. One-stage surgical reconstruction of the vascular abnormalities affecting inferior vena cava and hepatic vein using autologous pericardial patch was performed 11 months after angioplasty, which resulted in normalization of blood flow. Examination of a liver biopsy obtained intraoperatively revealed hepatic fibrosis compatible with early-stage Budd-Chiari syndrome. No complications were noted postoperatively and the nodular lesion in the lower extremity disappeared after surgery.     

Cavernous Transformation of the Portal Vein: A Common Manifestation of Behcet's Disease

Behcet's disease (BD) is a chronic, multisystem inflammatory disorder of unknown etiology, which is characterized by recurrent aphthous ulcers of the mouth and genitalia, uveitis with hypopyon, and a diffuse vasculitis that involves the arterial and venous systems.
From January 1968 to July 1993, 66 of 844 patients with BD seen at the Hacettepe University Hospital, Ankara, Turkey, experienced a vascular complication other than peripheral thrombophlebitis. The vascular complication in each case was identified based upon a combination of clinical data, digital subtraction angiography, CT, and ultrasonography findings. Six of these 66 (9.1%) had cavernous transformation of the portal vein. Five of these six had additional large vein involvement resulting in the Budd-Chiari syndrome with or without inferior vena caval obstruction.
Based upon this experience, it can be concluded that portal vein thrombosis is not a rare complication of BD. When patients with BD are found to have or develop splenomegaly, portal vein thrombosis should be suspected and investigated. If hepatomegaly and ascites are detected, Budd-Chiari syndrome due to hepatic vein thrombosis should be suspected. Finally, if hepato-splenomegaly, ascites, and dependent edema of the lower body are present, thrombosis of the inferior vena cava should be suspected.     

New type of asymptomatic congenital portosystemic shunt

Congenital extrahepatic portosystemic shunt (CEPS) is an extremely rare anomaly. In these malformations splanchnic blood bypasses the liver and drains into the systemic circulation through the inferior vena cava (IVC) or the left renal vein (LRV). Extrahepatic shunts may be divided into type 1 [end-to-side mesenterico-caval fistula with congenital absence of the portal vein (PV)] and type 2 (partial portocaval shunt caused by side-to-side mesenterico-caval fistula with normal or hypoplasic PV). Type 2 shunts typically are wholly extrahepatic between the PV or its right branch and the retrohepatic IVC. This report describes an asymptomatic case of CEPS not previously documented and not classifiable as type 1 or 2. CT revealed a normal PV with communication between the inferior mesenteric vein (IMV) and the LRV. The inferior mesenteric vein appeared tortuous, abnormally long and with a large calibre, and presented a connection with the LRV, in addition to a normal confluence into the splenic vein. Colour Doppler revealed hepatopetal normal flow in the PV and superior mesenteric vein with mild portal hypertension and an inversion of flow in the IMV directed to the LRV. Biochemical parameters showed a normal liver function without hyperammonaemia.     

Obliterative hepatocavopathy-inferior vena cava thrombosis at its hepatic portion

Budd-Chiari syndrome was formerly described as hepatic vein thrombosis within the liver, but it now includes inferior vena cava (IVC) thrombosis and other conditions that cause hepatic vein outflow obstruction. The author consider that primary hepatic vein thrombosis and primary IVC thrombosis represent two different clinical disorders.     

Anomalies of the inferior vena cava in patients with iliac venous thrombosis

BACKGROUND: Cases of deep venous thrombosis in the lower extremities triggered by abnormalities of the vena cava have been reported. OBJECTIVE: To describe anomalies of the inferior vena cava in patients with deep venous thrombosis. DESIGN: Prospective, consecutive case series. SETTING: University Hospital, Graz, Austria. PATIENTS: 97 patients with deep venous thrombosis. INTERVENTION: Sonography, venography, or both to diagnose deep venous thrombosis; magnetic resonance angiography to image the inferior vena cava. MEASUREMENTS: Anomalies of the inferior vena cava imaged by magnetic resonance angiography. RESULTS: 31 of 97 patients showed thrombotic occlusion of iliac veins (common and external iliac vein [ n = 29] or external iliac vein [ n = 2]). Five of 31 patients (3 men, 2 women) had an anomaly of the inferior vena cava. Anomalies were missing inferior vena cava, hypoplastic hepatic segment, and missing renal or postrenal segments. Patients with anomalies were significantly younger than the 92 patients without (mean age+/-SD, 25+/-6 years vs. 53+/-19 years; P = 0.002). In 2 patients with anomalies, the thrombotic occlusion was recurrent. CONCLUSIONS: An anomaly of the inferior vena cava should be suspected if thrombosis involving the iliac veins is seen in patients 30 years of age or younger. Patients with both an anomaly and thrombosis may be at higher risk for thrombotic recurrence.     

Acute thrombosis of pelvic and leg veins in agenesis of the renal segment of the inferior vena cava

A 19-year-old, otherwise asymptomatic man presented to the hospital of orthopaedic surgery with acute severe pain like lumbago. Symptomatic treatment was performed after extensive orthopaedic diagnostic procedures. On the third day after admission he showed clinical signs of deep vein thrombosis with painful swelling and livid discoloration of both legs. Colour duplex ultrasound revealed complete thrombosis of the leg and pelvic veins bilaterally, but the cranial extent was not clear. Contrast-enhanced helical computer tomography of the abdomen and the pelvis confirmed deep pelvic vein thrombosis and showed extension into the inferior vena cava. Moreover, the study revealed the agenesis of the renal segment of the inferior vena cava with collateral flow through dilated lumbar veins to enlarged azygous and hemiazygous, through vertebral and paravertebral venous plexus. The renals were drained via dilated capsular veins. The agenesis of renal vena cava is a very rare anomaly causing acute thrombosis of the deep leg and pelvic veins. Other risk factors of thromboembolic disease were not found. The patient was treated successfully with systemic thrombolysis. Therefore we used ultra-high streptokinase infusion (9 million units over 6 hours). Colour duplex ultrasound revealed good flow into deep leg and pelvic veins after three cycle of lysis. Magnetic resonance angiography of the abdomen and pelvis was performed to evaluate the successful fibrinolysis with complete recanalisation of the pelvic veins and to demonstrate the venous anatomy. Permanent oral anticoagulation with phenprocoumon is indicated to decrease the high rate of recurrent thrombosis. Compression stockings were prescribed. To prevent thrombosis, additional risk factors like smoking, immobilization and unusual physical activity should be strictly avoided.     

Transjugular intrahepatic portosystemic shunt in combination with oral anticoagulant for Budd-Chiari syndrome.

The antiphospholipid antibody syndrome is characterized by arterial and venous thrombosis including hepatic veins. Although transjugular intrahepatic portosystemic shunt or liver transplantation have been considered for Budd-Chiari syndrome, treatment options for patients with complete obstruction of three hepatic veins including the junction with the inferior vena cava are limited. We describe a 27-year-old female, who suffered thrombotic obliteration of hepatic veins including the portion confluent with the inferior vena cava (Budd-Chiari syndrome) associated with marked ascites and liver dysfunction. Transjugular intrahepatic portosystemic shunt using a Wall-stent (10 mm in diameter) between inferior vena cava and intrahepatic portal vein was performed. Intrastent coagulation and recurrence of thrombosis were prevented by combination therapy with warfarin potassium and ticlopidine hydrochloride. These treatments induced loss of ascites and improvement of liver function, and she has been able to resume daily life. The portosystemic shunt described above in addition to combination therapy with warfarin potassium and ticlopidine hydrochloride appeared to be one of the options for treating Budd-Chiari syndrome associated with antiphospholipid antibody syndrome.     

Transformation of inferior vena caval thrombosis to membranous obstruction in a patient with the lupus anticoagulant

A 24-yr-old woman with hemolytic anemia developed multiple thrombosis of the hepatic vein and inferior vena cava. She was found to have circulating lupus anticoagulant that could have been causally related to the thrombosis and hence the Budd-Chiari syndrome. On her first admission to the hospital vena cava and hepatic vein catheterizations revealed partial thrombotic occlusion of the cava at the level of the diaphragm, which was subsequently transformed into complete membranous obstruction. The right hepatic vein, which was patent on the first admission, was also completely occluded. These observations support the theory that membranous obstruction of the inferior vena cava is a sequela to inferior vena caval thrombosis rather than a congenital anomaly.     

Deep vein thrombosis caused by congenial interruption of the inferior vena cava--a case report

Congenital interruption of the inferior vena cava is an uncommon vascular anomaly. In this setting, the appearance of deep vein thrombosis is very rare because associated azygous or portal continuation develops as a collateral system for venous return. The authors present a case of infrahepatic interruption of the inferior vena cava in a 21-year-old man who presented with symptoms of deep vein thrombosis. Clinical features and prognosis of this entity are discussed.     

In-Hospital Risks and Management of Deep Venous Thrombosis According to Location of the Thrombus

BackgroundWhether deep venous thrombosis involving the pelvic veins or inferior vena cava is associated with higher in-hospital mortality or higher prevalence of in-hospital pulmonary embolism than proximal or distal lower extremity deep venous thrombosis is not known.MethodsThis was a retrospective cohort study based on administrative data from the Nationwide Inpatient Sample, 2016, 2017. Patients hospitalized with a primary diagnosis of deep venous thrombosis at known locations were identified by International Classification of Diseases-10-Clinical Modification codes.ResultsIn-hospital all-cause mortality with deep venous thrombosis involving the inferior vena cava in patients treated only with anticoagulants was 2.2% versus 0.8% with pelvic vein deep venous thrombosis (p<0.0001), 0.7% with proximal deep venous thrombosis (p<0.0001) and 0.2% with distal deep venous thrombosis (p<0.0001). Mortality with anticoagulants was similar with pelvic vein deep venous thrombosis compared with proximal lower extremity deep venous thrombosis, 0.8% versus 0.7% (p=0.39). Lower mortality was shown with pelvic vein deep venous thrombosis treated with thrombolytics than with anticoagulants, 0% versus 0.8% (p<0.0001). In-hospital pulmonary embolism occurred in 11% to 23%, irrespective of the site of deep venous thrombosis.ConclusionPatients with deep venous thrombosis involving the inferior vena cava had higher in-hospital mortality than patients with deep venous thrombosis at other locations. Pelvic vein deep venous thrombosis did not result in higher mortality or more in-hospital pulmonary embolism than proximal lower extremity deep venous thrombosis. The incidence of in-hospital pulmonary embolism was considerable with deep venous thrombosis at all sites.     

Surgical treatment for an extrahepatic portosystemic shunt: a case report

An extrahepatic portosystemic shunt that has neither liver cirrhosis nor portal hypertension is rare. A 60-year-old Japanese woman who had been suffering chronic liver disease and anemia with mild disorientation was admitted to investigate general fatigue with dizziness and disorientation. The laboratory data revealed mild pancytopenia and liver dysfunction including hyperammoniemia, an increased Indocyanine Green 15-min retention rate, and a decreased Fischer's ratio. Color Doppler ultrasonography, computed tomography, and arterial portography revealed an extrahepatic portosystemic shunt that extended tortuously from the superior mesenteric vein into the inferior vena cava, and decreased blood flow in the main portal vein. Judging from intraoperative measurement of portal pressure and intraoperative portography, shunt ligations were performed at both the efferent portion of shunt from the superior mesenteric vein and the afferent portion of the shunt into the inferior vena cava, and resection of the spleen was also performed. On the postoperative laboratory data, pancytopenia disappeared, and liver function improved. Postoperative abdominal imaging showed increased blood flow in the main portal vein and disappearance of the shunt vessel. Moreover, symptoms present before surgery also disappeared. In conclusion, surgical treatment of extrahepatic portosystemic shunts may result in better postoperative quality of life if it is performed in carefully selected patients.     

Unusual collateral vessel from right subclavian vein to left atrium,a rare complication of superior vena cava obstruction

The most commonly reported collateral systems in the setting of superior vena cava obstruction are azygos venous system, vertebral venous system, external and internal thoracic venous system based on McLntire and Sykes classification. A 49‐year‐old female with renal disease complained dyspnea on exertion. Transesophageal echocardiography showed significant mitral annular calcification, large multi‐lobulated mass at posterior aspect of RA, and complete obstruction of superior vena cava by thrombus formation. Computed tomography angiography showed a collateral vein to the left atrium (LA) roof. This case report is the first one which shows development of collateral vein from right subclavian to LA.     

A Case of Liver Cirrhosis with Large Portacaval Shunt from the Superior Mesenteric Vein: Normalization of Reversed Portal Blood Flow with Medical Treatment

A case of liver cirrhosis with large portacaval shunt from the superior mesenteric vein is reported. The portal blood flow was diagnosed as reversed on admission, but normalized with treatment. Blood flow was measured noninvasively and repeatedly using an ultrasonic Doppler duplex system. The reversed portal flow led to the superior mesenteric vein, right (and some left) internal inguinal vein, and inferior vena cava. This rare collateral pathway was clearly demonstrated by percutaneous transhepatic portalgraphy. A link between shunt and an appendectomy carried out 22 yr ago is considered, and the possibility ofthe temporarily reversed portal blood flow on admission being due to deteriorated hepatichemodynamic conditions is discussed.