Budd-Chiari syndrome with thrombosis of the inferior vena cava: long-term patency of meso-caval and cavoatrial prosthetic bypass

Portacaval or mesocaval shunts may relieve ascites that is caused by chronic forms of primary Budd-Chiari syndrome. When inferior vena cava stenosis is severe or is the site of thrombosis, another procedure has to be used. Portoatrial or cavoatrial shunting has been suggested, and a few reports have been made after only a short follow-up period. The case of a young woman with long-standing ascites caused by primary occlusion of the hepatic veins illustrates the incomplete relief of ascites after mesocaval shunt, which was caused by severe stenosis of the inferior vena cava. At 2 1/2-year follow-up, thrombosis of the retrohepatic inferior vena cava was documented and the mesocaval shunt remained patent. Cavoatrial bypass performed with the use of a long Dacron graft was successful as noted at a 4 1/2-year postoperative follow-up, and there was angiographic proof of patency. Budd-Chiari syndrome with stenosis or thrombosis of the inferior vena cava may be cured by prosthetic bypasses to the right atrium. Combined mesocaval and cavoatrial shunt should be encouraged in this specific situation.     

Portoatrial shunt in Budd-Chiari syndrome

It is now well accepted that mesoatrial bypass is an efficient treatment of Budd-Chiari syndrome and that it is indicated when the inferior vena cava is obstructed. This report presents a patient in whom the superior mesenteric vein was thrombosed after a previous mesocaval shunt. A bypass was constructed between the left side of the portal vein and the right atrium with a 16 mm diameter reinforced polytetrafluoroethylene prosthesis. The prosthesis passed between the left lobe of the liver and the caudate lobe and had a direct trajection. The procedure was simple, the postoperative course was uneventful, and the patient was well 20 months later. This observation suggests that portoatrial shunt is a good alternative to mesoatrial shunts in patients with Budd-Chiari syndrome and unavailable inferior vena cava and superior mesenteric vein.     

VENOUS ALLOGRAFTS: A USEFUL ALTERNATIVE TO VENOUS AUTOGRAFTS IN DIGESTIVE SURGERY

Over a 16 month period seven patients underwent surgery using venous allografts either to reconstruct the portal vein, or to construct a mesocaval ‘H’ graft or a shunt between the coronary vein and the subhepatic inferior vena cava. The allografts were harvested during multiorgan procurement from the bifurcation of the inferior vena cava, the common iliac vein and the external iliac vein and kept in a preservation solution at 4°C for a mean time of 6 days (range 1–29) before use. Subsequent thrombosis was clinically evident in only two patients. The use of venous allografts appears to be a useful alternative to other venous replacements.     

Anomalous dual drainage of the right pulmonary veins in a patient with cor triatriatum: report of a case without scimitar sign

The majority of patients with partial anomalous drainage of pulmonary veins are asymptomatic during infancy and childhood. Patients with significant left-to-right shunt develop symptoms and benefit from early corrective surgery. Anomalous pulmonary veins draining into inferior vena cava is very rare and frequently encountered in association with scimitar syndrome. The purpose of this case report is to describe a non-scimitar patient with cor triatriatum who had anomalous dual drainage of right pulmonary veins into inferior vena cava/left atrium and anomalous connection of persistent left superior vena cava with a common pulmonary venous chamber. The patient underwent an operation with redirection of anomalous pulmonary venous drainage into left atrium and ligation of persistent left superior vena cava.     

Successful laparoscopic division of a patent ductus venosus: report of a case

Patent ductus venosus (PDV) is a rare condition of a congenital portosystemic shunt from the umbilical vein to the inferior vena cava. This report presents the case of an adult patient with PDV, who was successfully treated with laparoscopic shunt division. A 69-year-old male was referred with hepatic encephalopathy. Contrast-enhanced CT revealed a large connection between the left portal vein and the inferior vena cava, which was diagnosed as PDV. The safety of a shunt disconnection was confirmed using a temporary balloon occlusion test for the shunt, and the shunt division was performed laparoscopically. The shunt was carefully separated from the liver parenchyma with relative ease, and then divided using a vascular stapler. Portal flow was markedly increased after the operation, and the liver function of the patient improved over the 3-month period after surgery. Although careful interventional evaluation for portal flow is absolutely imperative prior to surgery, a minimally invasive laparoscopic approach can be safely used for treating PDV.     

Hemodynamics of the interposition mesocaval shunt.

Eighty interposition mesocaval shunts, using a knitted Dacron large diameter prosthesis, have been performed during the past five and one-half years. Patients were evaluated from the standpoint of protection from recurrent esophageal hemorrhage, shunt patency, encephalopathy and cumulative survival analysis. In a selected group of patients, hemodynamic measurements were also obtained in the pre, intra, and postoperative periods. These included measurements of wedged hepatic vein pressure, superior mesentric venous blood flow, and residual superior mesenteric, hepatic sinusoidal and inferior vena cava pressures following the shunt procedure. Additionally, direct shunt flow measurements utilizing a square wave of electromagnetic flowmeter were also performed. Results indicate that the shunt patency is 95%; adequate decompression of the portal system was accomplished; recurrent variceal hemorrhage did not occur if the shunt remained patent; the incidence of encephalopathy was low (11%); and the operative mortality for the entire series was 9%. Continued perfusion of the liver was documented in 44% of patients and appears to be a function of the residual total portal resistance largely controlled by inferior vena caval pressure at the level of graft replacement. Life survivhat the interposition mesocaval shunt appears to be an effective technique for the control of variceal hemorrhage, has important hemodynamic advantages and can be applied to most patients for the control of variceal hemorrhage due to portal hypertension.     

系-腔分流术治疗门静脉高压症术后再出血的临床研究

目的探讨系-腔C形、H形架桥术对门脉高压症再出血的临床疗效及肠系膜上静脉外科干解剖变异时的临床处理.方法总结2002年1月至2004年8月36例门脉高压症术后再出血病例资料,其中脾切除、断流术后再出血21例,近端脾肾分流术后再出血9例,远端脾肾分流术后再出血4例,近端脾肾分流术＋断流术后2例;再出血后行系-腔C形架桥术18例,系-腔H形架桥术12例,肠系膜上静脉外科干解剖变异改行肠系膜下静脉-下腔静脉分流术4例,改行冠腔分流术2例.通过术中测压、术后B超测定吻合口血流量以及胃镜、肝功能随访评价系-腔分流术临床疗效.结果术后门脉降压明显,随访6个月至3年,吻合口通畅,胃底静脉曲张减轻,无一例再出血,无严重并发症,无一例死亡.结论系-腔分流术能有效的治疗门脉高压症术后再出血,其中C形架桥术降压效果最明显;当肠系膜上静脉外科干解剖变异时,应及时选择其它分流方法.     

体外肝切除联合自体肝移植治疗肝泡型包虫病

Ex-vivo liver resection and liver autotransplantation offers a chance to cure extensive regional liver diseases which are otherwise deemed untreatable in selected cases. A 33-year-old male patient with advanced alveolar echinococcosis (AE) which invaded the right lobes and adjacent retrohepatic inferior vena cava, while the left lobes were free from lesion and had proliferated to over 1200 ml in size except for a 1 cm × 1 cm solid lesion in the segment Ⅳ. No extrahepatic metastases were found in this patient. A lesion with a size of 4300 ml and other small lesions in the right lobes were removed extracorporeally,and the retrohepatic vena cava was repaired, then the remaining AE-free left lobes (more than 700 ml in size) and the retrohepatic inferior vena cava were re-implanted in situ. A temporary end-to-end cavo-caval shunt with interposition of a blood vessel prosthesis and end-to-side portocaval shunt were established to keep the blood flow during the four-hour anhepatic phase. The patient was followed up for six months, and he had no signs of residual liver disease with good hepatic function.     

Results of portal systemic shunts in Budd-Chiari syndrome.

Nine patients with Budd-Chiari syndrome (BCS) were treated by a portal systemic shunt. One had thrombosis of the superior mesenteric vein (SMV) and another had complete obstruction of the retrohepatic inferior vena cava (IVC). All other patients had a marked stenosis of the retrohepatic IVC with caval pressure ranging from 12 to 24 mmHg (mean: 17 mmHg). Seven patients had an interposition mesocaval shunt using an autologous jugular vein. The patient with a thrombosed SMV had a portoatrial shunt. The patient with an obstructed IVC had a cavoatrial shunt after an erroneous portacaval shunt had failed to relieve ascites. There were no operative deaths and no major postoperative complications. One patient died 19 months after operation of acute leukemia complicating polycythemia rubra vera. All other patients were alive and well 8 months to 6 years after operation. None of them had encephalopathy. These results suggest several comments: Portal systemic shunts are a good treatment for BCS and have a low operative risk. The mesocaval shunt is an efficient procedure, even when there is stenosis of the IVC with high caval pressure; shunts to the right atrium should be performed only in the case of complete obstruction or inaccessibility of the IVC. The long-term prognosis is excellent, except in patients with potential malignancies. Therefore, portal systemic shunts should be indicated early in patients with symptomatic BCS.     

经皮肾镜术中误入下腔静脉伴血栓形成一例并文献复习

目的探讨经皮肾镜碎石取石术（percutaneous nephrolithotomy, PCNL）中穿刺误入下腔静脉,留置肾造瘘管后出现下腔静脉血栓的处理办法。 方法结合文献复习,回顾性分析2017年5月我院收治的1例左肾铸型结石患者的临床资料。患者男,59岁,于全麻下行左PCNL。术中穿刺建立通道后出血汹涌,视野不清,中止手术,留置并夹闭肾造瘘管。术后第3日复查CT提示肾造瘘管经左肾静脉、下腔静脉至肝脏,下腔静脉内血栓形成。行经皮下腔静脉及左肾动脉造影,置入下腔静脉滤器及溶栓导管,透视监视下拔出左肾造瘘管。 结果拔出肾造瘘管后,患者无不良反应,血流动力学状态稳定,反复经导管造影,未见造影剂外溢。经溶栓治疗后,术后第11日再次行下腔静脉造影未见充盈缺损,顺利回收滤器。 结论PCNL术中穿刺误入下腔静脉,留置肾造瘘管伴下腔静脉血栓形成时,在血管造影辅助下分步缓慢拔出肾造瘘管并行溶栓治疗的方法安全、可靠,可避免外科手术的二次伤害及血栓相关并发症的发生。     

Balloon Venoplasty for Liver Failure Due to Stenosis of the Left Hepatic Vein After Right Tri-Segmentectomy

A 41-year-old male patient with hepatitis B underwent right tri-segmentectomy and total caudate lobectomy for a huge hepatocellular carcinoma associated with complete occlusion of the inferior vena cava with thrombosis of the infrahepatic inferior vena cava due to tumor compression. Five months later, he was readmitted for ascites and hyperbilirubinemia. Venography revealed stenosis and tortuosity of the left hepatic vein and the inferior vena cava, for which balloon angioplasty of the left hepatic vein and the inferior vena cava was performed using an 8-mm and 10-mm balloon, respectively. The left hepatic venous pressure decreased from 65 mmHg to 25 mmHg after dilatation. The patient made a satisfactory recovery thereafter and remains well with normal liver functions and without ascites. Balloon angioplasty may be useful for liver failure due to hepatic vein stenosis after hepatic resection.     

Meso-atrial shunt in the surgical treatment of Budd-Chiari syndrome

Three patients with idiopathic Budd-Chiari syndrome with occlusion of the hepatic veins and significant obstruction of the hepatic segment of the inferior vena cava are presented. The inferior vena caval obstruction precluded the use of conventional side-to-side portacaval or mesocaval shunts. Dramatic regression in liver size and resolution of ascites occurred in all three patients following the construction of meso-atrial shunts using wide-lumen woven Dacron grafts. Shunt patency was confirmed by angiography in two patients and Doppler studies in one. One patient died five months after surgery and another after 16 months. The shunts were widely patent at autopsy and the full length of the grafts were lined by neo-intima. The third patient is asymptomatic 20 months after surgery and is free of ascites. Our experience in these three cases suggests that patency can be expected in long synthetic grafts between the portal and systemic venous system provided the shunt is of adequate diameter and there is a pressure gradient between the two ends.     

Accessory hepatic vein complicating extra-cardiac total cavopulmonary connection.

We encountered unexpected, severe hypoxia after the right heart bypass operation in a patient with isomerism. A 2-year-old girl with polysplenia had a complex cardiac anomaly consisting of a single atrium, single ventricle, pulmonary stenosis, absence of the right superior vena cava, hemiazygos continuation of the left inferior vena cava, and d-malposition of the great arteries. After a total cavopulmonary shunt, we performed an extra-cardiac total cavo-pulmonary connection with a 14 mm tube graft. The postoperative course was complicated by severe hypoxia. Angiography performed 20 days after the operation showed that contrast medium in the conduit poured into the hepatic vein, and through the intrahepatic communications, it passed into a left-sided accessory hepatic vein, which was connected directly to the left side of the aspect of the atrium. As the intrahepatic communication was adequate, we ligated the accessory hepatic vein within the pericardial cavity. The SpO2 returned to normal and no hepatic dysfunction was detected. We conclude that surgeons performing extra-cardiac total cavopulmonary connection need to pay closer attention to the possibility that an accessory hepatic vein might exist.     

门静脉-下腔静脉吻合术预防活体肝移植术后小肝综合征3例报道

目的探讨门静脉-下腔静脉吻合术用于预防活体肝移植术后小肝综合征(small-for-size liver syndrome,SFSS)的效果。方法 3例活体肝移植均采用不含肝中静脉的右半肝作为移植物。术中发现实测移植物(肝)重量/受体的体质量(体重)的比值(graft to recipient weight ratio,GRWR)为0.58%、0.77%及0.71%,均<0.8%,符合小移植物的诊断。处理:首先吻合肝静脉流出道,其次吻合门静脉,将受体门静脉右支与移植肝门静脉右支端端吻合,将受体门静脉左支与下腔静脉行端侧吻合达到门腔分流的作用,之后按顺序吻合动脉和胆道。术中均未行脾静脉结扎或脾切除等处理。术后定期随访。结果 3例患者术后均未发生SFSS并顺利出院,出院时间分别为术后25d、34d及56d。移植肝功能逐步好转,术后1d门静脉流速理想。移植肝增长良好。门静脉-下腔静脉短路通畅时间:除1例通畅持续仅104d,其余2例持续通畅。结论 LDLT术中进行门静脉-下腔静脉吻合术可以及时有效预防小移植物背景下的SFSS,受体门静脉左支与下腔静脉行端侧吻合的分流技术安全可靠。     

Mesoatrial shunt: a new treatment for the Budd-Chiari syndrome.

A patient is presented with the Budd-Chiari Syndrome. Because of a thrombosed inferior vena cava, none of the standard portal-systemic shunts could be utilized for decompression of the engorged liver. A new shunt constructed by interposing a Dacron graft between the superior mesenteric vein and the right atrium was performed. Portal pressure was reduced by the shunt from 30 cm of H2O to 10 cm of H2O. Patency has been confirmed post-operatively by catheterization and with angiography. The patient is asymptomatic with normal liver function tests nine months following the procedure. A surgical approach is outlined for symptomatic patients with the Budd-Chairi Syndrom.     

A radical operation of Chiari's disease using the internal shunt tube of the inferior vena cava

A case with the Budd-Chiari syndrome due to the right hepatic occlusion who was successfully operated by using the internal shunt tube of the inferior vena cava was reported. The patients was 33 year old male. Midsternal incision and right subcostal oblique incision were made and the internal shunt tube was inserted through the right auricle to the inferior vena cava. The hepatic vein was approached through the longitudinal suprahepatic caval venotomy. Then membranous obstruction in the right hepatic vein ostia was resected. Mesenteric venous pressure dropped immediately from 360mm H2O to 160mm H2O by direct manometry. All the blood bled from the right hepatic vein was transfused through the internal shunt tube to the right atrium. The patient tolerated the procedure and his postoperative course was free from the complication. The resected membrane was congenital anomaly and an operative liver biopsy was demonstrated the evidence of congestive liver with fibrosis.     

Anomalies of the Systemic Venous Return: A Review

Congenital anomalies of the systemic venous connection to the heart represent a rather wide and heterogeneous group of malformations, whose physiological consequences may vary from nil to the most severe form of systemic arterial desaturation. The malformations may be summarized as follows: (1) Left superior vena cava connected to the coronary sinus, interrupted inferior vena cava and absent right superior vena cava that do not indicate surgical repair 'per se', but require some technical attention during open heart surgery performed for other anomalies; (2) Left superior vena cava connected to the left atrium, due to incorporation of the coronary sinus into the left atrial cavity, resulting in a right-to-left-shunt; (3) Right superior vena cava or inferior vena cava draining into the left atrium, both are extremely rare and require treatment for the ensuing right-to-left shunt; (4) Total anomalous systemic venous connection to the left atrium, usually combined with atrial isomerism and other very complex heart malformations; (5) Cor triatriatum dexter, which has been frequently diagnosed as an anomalous venous connection for its similar hemodynamic consequences. Such anomalies are reviewed with particular respect to their surgical implications.     

A case of left inferior vena cava discovered by chance at nephrectomy for renal transplantation

A case of left inferior vena cava, in a 35-year-old woman, discovered by chance at nephrectomy for renal transplantation is reported. She was admitted to our hospital as a donor of the kidney to her son. She had no remarkable physical signs on chest or abdomen. All laboratory tests were within normal limits. Intravenous pyelogram showed no major abnormality except for the lower position of left kidney than right one. Abdominal aortogram and selective renal arteriogram revealed no abnormality. Bilateral renal arteries were single. Left nephrectomy for transplantation was performed on April 13, 1983. In this operation the left sided inferior vena cava was discovered by chance. Inferior vena cavography was taken postoperatively. It showed the left sided inferior vena cava, type C. Knowledge of the abnormalities of the left renal vein and inferior vena cava is of surgical importance for the urologist when renal transplantation is being considered.     

Peri-operative comparison of different transient external shunt techniques in bidirectional cavo-pulmonary shunt.

OBJECTIVE: In patients with functional single ventricular physiology, the avoidance of cardiopulmonary bypass offers many advantages including earlier extubation, decreased necessity of inotropic support, improved hemodynamical status and reduced likelihood of post-operative prolonged pleural effusion. We believe that the bidirectional cavopulmonary anastomosis operations may be performed with transient external shunt techniques. The purpose of this prospective study is the peri- and post-operative comparison of different transient external shunt methods used in bidirectional cavopulmonary shunt operations. METHODS: Between years 1997 and 2000, 30 patients have undergone bidirectional cavo-pulmonary shunt operation by using three different types of external shunt. The mean patient age was 13 months (range, 3 months-3 years). Previous operations had been performed in ten patients (33%). All patients were divided into three groups according to type of external shunt used. In group A (ten patients), the transient external shunt was constructed between superior vena cava and right atrium by uniting two standard venous cannulas with a Y-connector. In group B (ten patients), the external shunt was performed with a single short venous cannula constructed between superior vena cava and right atrium. In group C (ten patients), the external shunt was constructed between superior vena cava and left pulmonary artery by using a single short venous cannula. During operation, central venous pressure (CVP), arterial O(2) saturation and mean arterial blood pressure were recorded continuously. RESULTS: All operations are completed without the establishment of cardiopulmonary bypass. Hospital mortality was 3.3%. One patient in group A died because of low cardiac output at the end of postoperative day 2. All patients were extubated within 4h. In groups A-C mean superior vena caval pressures were measured 28, 24 and 21 mmHg, respectively during superior vena cava-right pulmonary artery anastomosis. In both groups A and B patients, arterial O(2) saturation decreased to a minimum 53+/-2 and 53+/-2%, respectively during the operation. In the group C, minimum arterial O(2) saturation was measured 82+/-2%. Although mean arterial pressure decreased in all groups during clampage; in group C patients, this drop is not significant. CONCLUSION: Based on the study presented here, bidirectional cavo-pulmonary anastomosis can be carried out by using different types of transient external shunt. The best hemodynamical condition and arterial O(2) levels were achieved with the shunt constructed between superior vena cava and left pulmonary artery.     

One-stage total hepatectomy in the rat using microvascular anastomoses

A method is described for one-stage total hepatectomy in the rat using microvascular techniques. The operation consists of creation of a side-to-side mesocaval shunt performed just distal to the renal veins, total removal of the liver with ligation of the portal vein, hepatic arteries, and the bile duct. The vena cava is reconstructed with a segment of vena cava taken from a donor animal. The procedure takes 32 +/- 5 min to complete. Blood glucose concentration was maintained by infusions of balanced salt solution containing from 0.625% to 2.5% dextrose. Mean survival time was 10.5 hr (range, 5.5 to 21.5 hr).