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.     

Anatomical basis of the liver hanging maneuver

BACKGROUND/AIMS: The anterior approach to right hepatectomy using the liver hanging maneuver without liver mobilization claims to be anatomically evaluated. During this procedure a 4 to 6-cm blind dissection between the inferior vena cava and the liver is performed. Short subhepatic veins, entering the inferior vena cava could be torn and a hemorrhage, difficult to control, could occur. METHODOLOGY: On 100 corrosive casts of livers the anterior surface of the inferior vena cava was studied to evaluate the position, diameter and draining area of short subhepatic veins and inferior right hepatic vein. The width of the narrowest point on the planned route of blind dissection was determined. RESULTS: The average value of the narrowest point on the planned route of blind dissection was 8.7+/-2.3mm (range 2-15mm). The ideal angle of dissection being 0 degrees was found in 93% of cases. In 7% we found the angle of 5 degrees toward the right border of inferior vena cava to be the better choice. CONCLUSIONS: Our results show that liver hanging maneuver is a safe procedure. With the dissection in the proposed route the risk of disrupting short subhepatic veins is low (7%).     

Phrenic vein dissection in partial liver graft harvesting

BACKGROUND/AIMS: Despite the impressive results of living donor liver transplantation, hepatic venous reconstruction remains a controversial component. METHODOLOGY: A total of 211 consecutive donor hepatectomies were performed. The proximal route of the hepatic vein was exposed by dissection of the connective tissue around the hepatic vein and by dividing and ligating all of the inferior phrenic veins that open into the hepatic vein, into the confluence of the hepatic vein and inferior vena cava, or directly into the inferior vena cava. RESULTS: In the 114 left-side hepatectomy procedures, the number of divided left inferior phrenic veins ranged from 1 to 4 and the diameters of the left and middle hepatic veins ranged from 7 to 33mm. For the 97 right-side procedures, the number of divided right inferior phrenic veins ranged from 1 to 4 and the diameters of right hepatic veins ranged from 9 to 34mm. This maneuver safely allowed for the safe exposure of all trunks and routes of the hepatic veins and the suprahepatic portion of the inferior vena cava. CONCLUSIONS: Our technique is useful for obtaining a wide ostium and a sufficient length of the hepatic vein for grafts obtained from living donors.     

Liver hanging maneuver for right hemiliver in situ donation--anatomical considerations

Background. An anatomical study was carried out to evaluate the safety of the liver hanging maneuver for the right hemiliver in living donor and in situ splitting transplantation. During this procedure a 4–6 cm blind dissection is performed between the inferior vena cava and the liver. Short subhepatic veins entering the inferior vena cava from segments 1 and 9 could be torn with consequent hemorrhage. Materials and methods. One hundred corrosive casts of livers were evaluated to establish the position and diameter of short subhepatic veins and the inferior right hepatic vein. Results. The average distance from the right border of the inferior vena cava to the opening of segment 1 veins was 16.7±3.4 mm and to the entrance of segment 9 veins was 5.0±0.5 mm. The width of the narrowest point on the route of blind dissection was determined, with the average value being 8.7±2.3 mm (range 2–15 mm). Discussion. The results show that the liver hanging maneuver is a safe procedure. A proposed route of dissection minimizes the risk of disrupting short subhepatic veins (7%).     

Wedged hepatic venous pressure in congential heart disease.

Seventy cases of congential heart disease including the most frequent types were studied, and wedge hepatic venous pressure (WHVP) was measured in each. The mean pressure was determined in the "jammed position" and in the free hepatic veins, inferior vena cava, and low right atrium. The average mean WHVP was 7.0 mm Hg, 5.0 in inferior vena cava, and 3.4 in the right atrium. A direct relationship was found between wedge hepatic venous pressure of the inferior vena cava and the low right atrium, but not other parameters. Ten patients had a mean pressure above 10 mm Hg. We believe that in many circumstances in patients with congenital heart disease, liver function may be abnormal and high values of wedge hepatic venous pressure may also be found.     

Membranous obstruction of the inferior vena cava and invasion of the inferior vena cava by tumour in hepatocellular carcinoma

A 28 yr old Zulu presented with a painful swelling in the right hypochondrium and severe swelling of the legs of short duration. The serum alpha-fetoprotein concentration was over 2 X 10(5) ng/ml and imaging showed a large hepatic mass-lesion. Radionuclide venography revealed no flow through the inferior vena cava but flow through a large collateral vessel. Contrast venography showed the upper portion of the inferior vena cava to be occluded: large collateral vessels arose from the lower vena cava and the iliac veins. The histological features were those of longstanding hepatic venous outflow obstruction with irregular centrizonal and portal fibrosis: severe acute centrizonal congestion was not seen. This combination of findings indicates the presence of both membranous obstruction of the inferior vena cava, a rare developmental abnormality which predisposes to hepatocellular carcinoma formation, and invasion by the tumour of the inferior vena cava via the hepatic veins, an uncommon complication of hepatocellular carcinoma.     

Sonospirometry: a new method for noninvasive estimation of mean right atrial pressure based on two-dimensional echographic measurements of the inferior vena cava during measured inspiration

A noninvasive method of estimating mean right atrial pressure would be useful in evaluating hemodynamics and calculating pulmonary pressures by Doppler echocardiography. An electronic pressure gauge was built and tested for measurement of inspiratory pressures during two-dimensional echocardiography to quantitate the diameter of the inferior vena cava. Thirty-one studies were made in 27 alert, informed, consenting patients with an in-place pulmonary artery catheter having right atrial ports. Inferior vena cava diameter was measured in successive 10 mm segments distal to the right atrial-inferior vena cava junction on images obtained while the patient suspended breathing at full inspiration and during each 4 mm Hg increment of a calibrated inspiratory maneuver. Results show that the segment between 5 and 30 mm distal to the right atrial-inferior vena cava junction was the region most responsive to increasing inspiratory pressure. In this segment, the inspiratory pressure required to decrease the inferior vena cava diameter to greater than or equal to 85% of the difference between its maximal (suspended full inspiration) and minimal (over the entire inspiratory maneuver) values was similar or equal to the mean right atrial pressure (measured from the pulmonary artery catheter) (r = 0.87, SEE = 2.9 mm Hg). Minimal inferior vena cava diameter was directly related to mean right atrial pressure (r = 0.56); the minimal to maximal inferior vena cava diameter ratio was inversely related to mean right atrial pressure (r = -0.57). Maximal inferior vena cava diameter and the absolute (measured) amount of inferior vena cava diameter decrease correlated weakly with mean right atrial pressure.(ABSTRACT TRUNCATED AT 250 WORDS)     

Measurements of central blood vessels in infants and children: normal values.

In order to provide physicians interpreting vascular radiographic studies with normal data regarding central blood vessel size in children and to facilitate the design and adaptation of intravascular devices for pediatric use, we measured lengths and diameters of central blood vessels in 141 radiographic studies in 136 children. The diameters of the following vessels were determined: right and left internal jugular veins and common carotid arteries; the inferior vena cava and the descending thoracic aorta; right and left iliac veins; and right and left femoral veins and arteries. In addition, the lengths of the inferior vena cava and the descending aorta were also determined. Blood vessel dimensions were highly correlated with age, height, weight, and body surface area. The linear regression equations for each measured dimension against age, weight, height, and surface area are provided, along with a table of predicted vessel size as a function of age.     

Measurements of central blood vessels in infants and children: Normal values

In order to provide physicians interpreting vascular radiographic studies with normal data regarding central blood vessel size in children and to facilitate the design and adaptation of intravascular devices for pediatric use, we measured lengths and diameters of central blood vessels in 141 radiographic studies in 136 children. The diameters of the following vessels were determined: right and left internal jugular veins and common carotid arteries; the inferior vena cava and the descending thoracic aorta; right and left iliac veins; and right and left femoral veins and arteries. In addition, the lengths of the inferior vena cava and the descending aorta were also determined. Blood vessel dimensions were highly correlated with age, height, weight, and body surface area. The linear regression equations for each measured dimension against age, weight, height, and surface area are provided, along with a table of predicted vessel size as a function of age.     

Retrohepatic vena cava replacement of hepatic malignancies without using total hepatic vascular exclusion or extracorporeal bypass.

Total hepatic vascular exclusion and venovenous bypass are frequently used surgical procedures when concomitant resection of the inferior vena cava is required during surgery of liver cancer involving the retrohepatic inferior vena cava close to the hepatic veins. However, the duration of total hepatic vascular exclusion is limited due to the risk of hepatic ischemia. Three patients presented with severely compressed inferior vena cava and/or hepatic veins due to liver cancer. The surgical procedure involved initial taping of the inferior vena cava just below the hepatic veins by extrahepatic division and taping of the hepatic veins. After taping the inferior vena cava, hepatectomy with caval resection was performed by simply clamping the retrohepatic inferior vena cava, without the need for total hepatic vascular exclusion or venovenous bypass. In all patients the retrohepatic inferior vena cava were safely replaced with a prosthetic graft under stable hemodynamics. Duration of the inferior vena cava clamping was 31, 66, 75 minutes, respectively. No graft-related complications occurred, but 2 of the 3 patients showed temporal renal dysfunction associated with renal congestion postoperatively. The surgical procedure described herein is effective for the treatment of retrohepatic inferior vena cava in some patients. However, when the case is complicated by chronic nephropathy or simultaneous nephrectomy is required, venovenous bypass should be performed.     

Congenital familial vascular anomalies: a study of patients with an anomalous inferior vena cava, and of their first-degree relatives

With magnetic resonance angiography and computed tomography, congenital anomalies of the inferior vena cava are diagnosed more frequently than they used to be. Accessory renal arteries identified by magnetic resonance angiography in a patient with an anomalous inferior vena cava indicated a combination of arterial and venous abnormalities. The study was initiated to screen consecutive patients with an anomalous inferior vena cava for concomitant abdominal and pelvic arterial abnormalities, and their first-degree relatives for congenital vascular anomalies. Magnetic resonance angiography identified in 2 of 5 patients with an anomalous inferior vena cava concomitant accessory renal arteries and in 5 of 11 first-degree relatives major abdominal vascular anomalies including accessory renal arteries, accessory renal veins, and anomalies of the hepatic artery. None of the relatives showed abnormalities of the inferior vena cava. The familial occurrence of vascular anomalies strongly suggests an underlying pathogenetic component in affected family members. In patients with a congenital anomaly of the inferior vena cava, concomitant arterial abnormalities should be considered. First-degree relatives may be at risk for congenital vascular anomalies.     

Chylothorax and chylopericardium as the initial clinical manifestation of Behcet’s disease

Behcet’s disease (BD) is a chronic relapsing systemic vasculitic disorder affecting the arteries, veins, and vessels of any size. Large vein thrombosis in BD is not commonly developed and most commonly observed in the veins in the lower extremities and inferior or superior vena cava. In this report, a 18-year-old male patient with large vein thrombosis involving superior vena cava was presented. He was treated due to chylothorax and chylopericardium with SVC syndrome before diagnosis of BD. SVC thrombosis complicated by chylothorax and chyolpericardium can be a rare presenting initial symptom of BD. H. Moon and Y. J. Lee contributed equally.     

多排螺旋CT在检出右侧肾上腺静脉应用价值的初步探讨

目的:探讨多排螺旋CT(MDCT)对右侧肾上腺静脉检出率及右侧肾上腺静脉解剖结构显示情况。方法:402例行MDCT腹部三期增强扫描的患者,由两个不同的影像科医师观察其右侧肾上腺静脉轴位像和三维重建图像,进而评价右侧肾上腺静脉的情况。评价要点:可视化程度;右侧肾上腺静脉的直径与长度;与副肝静脉和其他静脉间的关系;右侧肾上腺静脉的位置及与周围结构间的关系,与下腔静脉的方向关系。结果:402例患者检出右侧肾上腺静脉338例(84.1%),其中,有31例(9.2%)右侧肾上腺静脉与副肝静脉共干,右侧肾上腺静脉开口位于胸11~腰1之间。另307例患者中,在横断面上右侧肾上腺与下腔静脉横方向关系为向后和向右的占282例(91.9%),向后和向左的占25例(8.1%);在垂直面上右侧肾上腺朝向下腔静脉尾侧有292例(95.1%),头侧为15例(4.9%)。在这338例患者中,右侧肾上腺静脉的长度和直径分别为平均(3.8±1.7)mm和(1.7±0.6)mm。结论:MDCT有较高检出右侧肾上腺静脉的能力,并能大致显示其解剖特征,包括它的位置和与周围结构的关系。     

Hepatic autotransplantation with ex situ neoplasm extirpation and vena cava replacement

The treatment of a 64-year-old man with a retrohepatic neoplasm deemed not accessible by conventional in situ surgical techniques is presented to illustrate the potential benefit offered by techniques adapted from liver transplantation and vascular surgery. A computed tomography scan performed for uncharacteristic abdominal discomfort revealed a hepatic or retrohepatic tumor compressing the inferior vena cava. Biopsies were interpreted as probably leiomyoma or malignant schwannoma. The liver with neoplasm and retrohepatic inferior vena cava was removed en bloc and taken to the back table where the neoplasm invading the inferior vena cava wall was removed together with the inferior vena cava. The inferior vena cava was then replaced by a 22-mm polytetrafluoroethylene graft and the 3 hepatic veins were reconstructed with anastomoses to this graft. The liver was then autotransplanted by standard transplantation technique. The postoperative course was uneventful and the patient is in good health more than 2 years after surgery.     

Histopathology of membranous obstruction of the inferior vena cava in the Budd-Chiari syndrome.

It is generally believed that membranous obstruction of the inferior vena cava in the Budd-Chiari syndrome is caused by congenital malformation. However, it does not explain the late onset of the disease. In the current study, hepatic portion of the inferior vena cava and hepatic veins were studied in 17 autopsy cases of the Budd-Chiari syndrome, 16 of which had no demonstrable cause (idiopathic). A sufficient amount of vena cava tissue was available for evaluation in 15 cases. Nine had membranous obstruction, with thickness varying from 3 to 8 mm. Thrombus formation was recognized in 7 of 9 cases. Occlusion of hepatic vein orifices of varying degree was present in 8 cases. In these occluding lesions, the basic structure of the venous wall was maintained. The intima was transformed into a fibrous laminar structure, and organized thrombi of varying ages were recognized; they were a mixture of fresh thrombi, organized thrombi, fibrous tissues, recanalizations, and calcifications. It is concluded that in these cases of the Budd-Chiari syndrome, occluding and stenosing lesions in the inferior vena cava and hepatic veins were thrombosis and its sequelae. There was no indication of congenital malformation.     

Budd-Chiari syndrome as a late complication following closure of an atrial septal defect

A case of a Budd-Chiari syndrome in a 19-year-old female patient is reported who had undergone surgical closure of a secundum atrial septal defect 13 years before. 8 months before the development of the Budd-Chiari syndrome she started to take oral contraceptives. The clinical picture of the Budd-Chiari syndrome developed within several days. The inferior vena cava did not fill with contrast dye when an angiography was performed using the right vena iliaca approach. The contrast dye disappeared through collateral veins (vena azygos, vena hemiazygos). After 4 days of treatment with systemic streptokinase she underwent open-heart surgery. The orifice of the inferior vena cava was occluded to a diameter of 6 mm. No thrombi were found. The lesion was corrected with two patches, one in the right atrium and the other in the inferior vena cava. This case report demonstrates that a Budd-Chiari syndrome is a possible late complication after closure of an atrial septal defect which should be treated by surgery.     

Cholangiocellular carcinoma in a patient who has survived for 6 years after extended left hepatic lobectomy and caudate lobe resection with pancreatoduodenectomy

We describe a case of cholangiocellular carcinoma in a 66-year-old woman. A well-defined, hypoechoic tumor, 9 cm in greatest diameter, was detected in the left lobe of the liver by ultrasonography in December 1988. Celiac angiography showed a faintly stained tumor at the same location, with interruption of the left portal vein. Computed tomography revealed invasion of the inferior vena cava and lymph node enlargement around the head of the pancreas. In January 1989, the patient underwent extended left hepatic lobectomy with caudate lobe resection, pancreatoduodenectomy, partial resection of the inferior vena cava, and lymph node dissection around the hepatoduodenal ligament and the common hepatic artery. Postoperative histopathological examination revealed a moderately differentiated tubular adenocarcinoma. Cancer cells had invaded only the portal region, unlike the features of most cholangiocellular carcinomas. There was no evidence of lymph node metastasis in the dissected specimens. Six years after operation, there have been no signs of recurrence, and the patient is still alive and well.     

Retrohepatic vena cava deroofing in living donor liver transplantation for caudate hepatocellular carcinoma

The removal of tumor together with the native liver in living donor liver transplantation for hepatocellular carcinoma is challenged by a very close resection margin if the tumor abuts the inferior vena cava. This is in contrast to typical deceased donor liver transplantation where the entire retrohepatic inferior vena cava is included in total hepatectomy. Here we report a case of deroofing the retrohepatic vena cava in living donor liver transplantation for caudate hepatocellular carcinoma. In order to ensure clear resection margins, the anterior portion of the inferior vena cava was included. The right liver graft was inset into a Dacron vascular graft on the back table and the composite graft was then implanted to the recipient inferior vena cava. Using this technique, we observed the no-touch technique in tumor removal, hence minimizing the chance of positive resection margin as well as the chance of shedding of tumor cells during manipulation in operation.     

Immunohistochemical evaluation for outflow reconstruction using opened round ligament in living donor right posterior sector graft liver transplantation: A case report

Utilizing the opened round ligament as venous grafts during liver transplantation is useful but controversial,and there are no pathological analyses of this procedure. Herein,we describe the first reported case of a pathological analysis of an opened round ligament used as a venous patch graft in a living donor liver transplantation(LDLT). A 13-year-old female patient with biliary atresia underwent LDLT using a posterior segment graft from her mother. The graft had two hepatic veins(HVs),which included the right HV(RHV; 15 mm) and the inferior RHV(IRHV; 20 mm). The graft RHV and IRHV were formed into a single orifice using the donor's opened round ligament(60 mm × 20 mm) as a patch graft during bench surgery; it was then anastomosed end-to-side with the recipient inferior vena cava. The recipient had no post-transplant complications involving the HVs,but she died of septic shock with persistent cholangitis and jaundice 86 d after LDLT. The HV anastomotic site had no stenosis or thrombus on autopsy. On pathology,there was adequate patency and continuity between the recipient's HV and the donor's opened round ligament. In addition,the stains for CD31 and CD34 on the inner membrane of the opened round ligament were positive. Hepatic venous reconstruction using the opened round ligament as a venous patch graft is effective in LDLT,as observed on pathology.