Diagnosis of intravenous leiomyomatosis extending to heart with emphasis on magnetic resonance imaging

Background  Intravenous leiomyomatosis is a rare neoplasm, and its cardiac extension is often overlooked or misdiagnosed. The purpose of this study was to explore the imaging features of intravenous leiomyomatosis with cardiac extension, especially the value of magnetic resonance imaging in differential diagnosis.

Methods  Between July 2005 and August 2008, 4 cases of intravenous leiomyomatosis with cardiac extension were resected in Cangzhou Central Hospital. Three cases had echocardiography performed, two had post contrast scans of CT performed, and two had MRI performed. Between July 2005 and May 2010, 25 cases of histopathologically proven other kinds of tumors involving the inferior vena cava and right atrium were compared for discussion of differential diagnosis.

Results  Intravenous leiomyomatosis with cardiac extension demonstrated a hyperechoic elongated mobile mass extending from the inferior vena cava to the right atrium with or without evidence of protruding into the right ventricle on echocardiography. The lesion was enhanced heterogeneously on post contrast scans of CT and was of relatively lower density compared to the enhanced blood in the inferior vena cava and right atrium, with common iliac vein and the ipsilateral internal iliac and ovarian veins involved in some cases. The untreated uterus myoma demonstrated enlargement of the uterus with heterogeneous contrast enhancement. On MRI, the lesion looked like a luffa vegetable sponge on FIESTA coronal images and a sieve pore on T2-weighted axial images. All four tumors were removed successfully, and follow up of one to four years revealed no recurrence. The 25 cases of histopathologically proven other kinds of tumors involving inferior vena cava and right atrium had their own imaging features different from those seen on intravenous leiomyomatosis with cardiac extension. With reference to their medical history, differential diagnosis can often be made.

Conclusion  The imaging appearance of intravenous leiomyomatosis has some unique features, and the luffa vegetable sponge and sieve pore like appearance on MRI may be helpful for differential diagnosis.

     

Piggyback liver transplant techniques in the surgical management of urological tumors with inferior vena cava tumor thrombus

Background An important characteristic of renal cell carcinomas and adrenal tumors is that these tumors may expand into the renal vein and inferior vena cava, and transform into tumor thrombi. This study was to evaluate the use of piggyback liver transplant techniques for surgical management of urological tumors with inferior vena cava tumor thrombus. Methods Nineteen patients with renal cell carcinomas or adrenal tumors with inferior vena cava tumor thrombus were treated from November 1995 to April 2008. Their ages ranged from 29 years to 76 years （mean 54 years）. The extent of tumor thrombus was infrahepatic （level Ⅰ） in 2, retrohepatic （level Ⅱ） in 7, suprahepatic （level Ⅲ） in 6, and intra-atrial （level Ⅳ） in 4 patients. We used cardiopulmonary bypass with deep hypothermic circulatory arrest to remove the thrombi in 3 cases of level IV and in 2 cases of level Ⅲ. In all level Ⅱ, 4 level Ⅲ, and 2 level IV cases, we used piggyback liver transplant techniques to mobilize the liver off of the inferior vena cava and to separate the inferior vena cava from the posterior abdominal wall. Results Mean operative time was 5.1 hours, mean estimated blood loss was 2289 ml and mean blood transfusion was 12.84 U. One patient with adrenal cortical carcinoma and level Ⅳ thrombus died in the immediate postoperative period. Three patients were lost to follow up, and the other 15 survivors were followed from 5 months to 56 months. Eight of these 15 patients died due to metastasis; however 7 were still alive at the last follow-up. Conclusions An aggressive surgical approach is the only hope for curing patients diagnosed with urological tumors combined with inferior vena cava tumor thrombus. The use of piggyback liver transplant techniques to mobilize the liver off of the inferior vena cava provides excellent exposure of the inferior vena cava. Patients with a level Ⅱ or level Ⅲ inferior vena cava thrombus may be treated without using cardiopulmonary bypass.     

Management of retrohepatic inferior vena cava injury during hepatectomy for neoplasms

The aim of this article is to discuss the management of retrohepatic inferior vena cava injury during hepatectomy for neoplasms. Step-by-step hepatic vascular exclusion, digital compression, finger pinching, and surface-to-surface suturing were used in the management of retrohepatic inferior vena cava injury during hepatic resection in 16 cases： 12 patients underwent exclusion of the hepatic artery and portal vein by portal triad clamping （PTC） only;     

Intravenous leiomyomatosis with right heart involvement-a report of 4 cases and literature review

Intravenous leiomyomatosis(IVL) is a rare benign neoplasm which originates from the smooth muscle cells and is usually confined to the pelvic venous system.Rarely,intracaval and intracardiac extension has been described.Death can occur as a result of intracardiac involvement.We reported 4 cases of IVL with right heart involvement(intracardiac leiomyomatosis,ICL).Three of them suffered recurrent sudden syncope,and the other one was totally asymptomatic.All of them were successfully treated through one-stage operation under extracorporeal circulation.     

Orthotopic heart transplantation with prolonged donor ischemic time: report of 3 cases and literature review

Heart transplantation has become an effective therapy for patients with end stage heart failure. The preservation of the donor heart is an important factor that affects the results of the operation. We performed 3 cases of orthotopic heart transplantation and obtained some experience in the preservation of the donor heart. Methods： Three male patients with end stage heart failure received the operation in our department successfully. Doppler echocardiography showed left ventrieular end diameter （LVED） of the patients were 91, 87, and 83 mm, and ejection fraction （EF） were 24%, 20%, 12.9%, respectively. Once the declaration of brain death had been made, the median stemotomy was performed with a sternal saw. Haparin at a dose of 300 U/kg of body weight was administered. After at least 2-min heparin circulation, the procurement proceeded. The superior vena cava and the inferior vena cava were nearly completely divided. When the heart was empty, the ascending aorta was cross-clamped and the St. Thomas solution was infused by gravity. The heart was excised by transection of the inferior vena cava, the superior vena cava and all pulmonary veins. After donor heart was removed, it was infused with University of Wisconsin （UW） solution by gravity at a temperature of 4-6℃, then placed in UW solution for storage during transportation. The temperature of solution was maintained at about 4-6℃. The ischemic times of donor heart were 9, 8 and 6 h, respectively. The bicaval anastomotic heart transplantation was adopted. The left atrial anastomoses were constructed using 3.0 polypropylene. The inferior vene cava anastomosis was constructed, the donor and native aorta were cut to an appropriate length. Then the aorta and main pulmonary artery anastomosis were performed respectively. The superior vene cava anastomosis was usually constructed during the rewarming phase. The intraoperative course with a cardiopulmonary bypass of the 3 patients was 96, 44 and 49 min, respectively. Standard triple immunosuppression therapy was commenced in the immediate post-operative period. Results： The operation procedure was smooth and no perioperative death occurred. The follow-up was carried out carefully. The patient＇s condition was fine in 25, 30 and 32 months after operation. The blood pressure was 130/90, 140/95 and 120/80 mmHg, respectively, and LVED was 51, 49 and 53 mm; EF was 50%, 54% and 60%, respectively. Cardiothoracic ratio was 0.63, 0.55, and 0.64, respectively. Conclusion： Preservation time of donor heart with St. Thomas solution infusion and UW solution storage at 0-4℃ may exceed 6 h, and receive comparable middle-term outcomes.     

Radical correction of Budd-Chiari syndrome

Background Interventional therapy is widely accepted as the first choice for the treatment of the Budd-Chiari syndrome but the use of radical correctional therapy should not be discarded. This study describes radical correction by controlling bleeding from distal end of pathological segment of the inferior vena cava （IVC） and discusses potential surgical errors and postoperative complications. Methods Of the 216 patients in the study, 78 were treated with simple membranectomy, 64 with dissection of the pathological segment of the IVC and vascular prosthesis or pericardial patch plasty, 60 with resection of the pathological segment of the IVC and orthotopic graft transplantation with vascular prosthesis, and 14 with resection of the occlusive main hepatic vein and its upper IVC, hepatic venous outflow plasty and vascular prosthesis orthotopic graft transplantation from the hepatic venous entrance to the IVC of right atrial ostium. Results Except 14 cases who were discharged after hepatic vein outflow plasty, four cases died postoperatively, and 198 patients were discharged without complications. The symptoms of 15 patients were relieved partially and 2 without any change. There were no deaths intraoperatively. Of the 112 cases who were followed up for 72 months, 13 suffered from a relapse. Conclusions Radical correction is a beneficial therapy in the treatment of Budd-Chiari svndrome.     

Vascular surgery：Surgical treatment of tumor invading impor tant vessel

Objective To sum up preliminary experience of successful resection of tumor involving importnat vessel in 77 cases. Methods Seventy-seven cases were treated in this series including 47 cases in male and 30 cases in female. The ages ranged from 18 to 75 years with the average of 57. The tumor involving thoraco-abdominal cavity was in one case with malignant neuroblastoma. The tumor form retroperitoneum invaded into the whole inferior vena cava （IVC）, and into two-thirds of the right atrium. Tumor located in thoracic cavity were in 13 cases including IVC leiomyosarcoma in 2 cases,     

Prevention of tumor emboli from the inferior vena cava by the Tempofilter II during resection of nephroblastoma with level III tumor thrombus

The optimal treatment for patients with nephroblastoma and inferior vena cava （IVC） minor thrombus is radical nephrectomy and minor thrombectomv, but the operation for patients with level III tumor thrombus is usually at high risk of puhnonary, embolism （PE）. We report one case of nephroblastoma with level III thrombus in our hospital in 2007, the vena cava tumor thrombectonly was safely performed under the protection of Tempofilter II inferior vena cava filter.     

Prevention of tumor emboli from the inferior vena cava by the Tempofilter II during resection of nephroblastoma with level Ⅱ tumor thrombus

The optimal treatment for patients with nephroblastoma and inferior vena cava (IVC) tumor thrombus is radical nephrectomy and tumor thrombectomy, but the operation for patients with level Ⅲ tumor thrombus is usually at high risk of pulmonary embolism (PE). We report one case of nephroblastoma with level Ⅲ thrombus in our hospital in 2007, the vena cava tumor thrombectomy was safely performed under the protection of Tempofilter Ⅱ inferior vena cava filter.     

Multidisciplinary treatment for renal cell carcinoma involving inferior vena cava

<正>Objective To evaluate the surgical treatment for renal cell carcinoma with inferior vena cava tumor thrombus and the clinical significance of muhidisciplinary treatment. Methods Two cases of renal cell carcinoma with inferior vena cava thrombus diagnosed by Doppler     

静脉内平滑肌瘤病累及下腔静脉六例的处理经验

目的评价静脉内平滑肌瘤病累及下腔静脉时的诊断和处理方式。方法回顾性分析了北京协和医院血管外科自2000年7月至2006年2月收治的6例累及下腔静脉的静脉内平滑肌瘤病。结果6例患者中3例行Ⅰ期,2例行Ⅱ期手术,手术均获得成功,无围手术期死亡或并发症,其中4例患者的原发肿瘤和静脉内瘤栓完整切除,1例患者因多次手术,肿瘤与盆腔粘连严重,残余少许肿瘤,但术后给予抗雌性激素治疗后,瘤体有明显缩小。所有患者术后均复发。结论静脉内平滑肌瘤病极为少见,血管外科临床医师应当对该病有足够的重视,该病累及下腔静脉时可以选择多种治疗方式,手术是最佳选择,能否完整切除瘤体是防止复发的关键。     

伴有心脏受累的静脉内平滑肌瘤病一例

静脉内平滑肌瘤病是一种特殊类型的子宫肌瘤,其生长方式类似恶性肿瘤,达下腔静脉甚至累及右心腔者非常罕见,具有潜在的致命性.本文报道1例伴有心脏受累的静脉内平滑肌瘤病患者.患者41岁,女性,因"间断右上腹疼痛,伴双下肢轻度肿胀20天"入院.既往有子宫肌瘤及子宫次全切除病史.超声心动示:右心房内可见团块,与心腔无黏连,左室射血分数60.00%.腹部CT示:下腔静脉全程、左侧髂总、髂内静脉内软组织肿物,病变卜极达右心房,增强扫描示肿物有明显强化.妇科及盆腔血管彩超可见下腔静脉下段、左侧髂总静脉内占位,左侧附件区髂内、外静脉之间可见一分叶状团块,其内见营养血管,子官次全切除术后.初步诊断:下腔静脉、右心房肿瘤.行胸腹联合心脏、下腔静脉伞程肿瘤切除,左侧髂内静脉结扎术,术后未予激素治疗,病理示肿瘤细胞呈梭形,未见核分裂相,无凝固性坏死,免疫组化SMA(+++),desmin(+),PR(+),ER(+),S100(-),CD117散在个别细胞(+),CD34(-),Ki67<50%.随访6个月未见肿瘤复发.该病临床表现无特异性,术前诊断率低,确诊主要依靠病理,鉴别心脏、下腔静脉原发肿瘤和血栓后,临床治疗主要是肿瘤彻底手术切除,术后短期未见复发.     

腔静脉节段切除术在处理侵犯腔静脉的肾肿瘤瘤栓中的应用

目的：探讨腔静脉节段切除术治疗侵犯腔静脉的肾肿瘤瘤栓的可行性、安全性和手术经验。方法：2015年5月至2017年7月,北京大学第三医院共收治92例肾肿瘤伴静脉瘤栓的患者,其中17例患者因瘤栓侵犯腔静脉壁需行腔静脉节段切除术。本组病例的特点为：男性15例、女性2例,平均年龄为（59.2±12.9）岁（31~84岁）;左侧6例,右侧11例;Mayo静脉瘤栓分级Ⅱ级10例、Ⅲ级３例、Ⅳ级４例;肾肿瘤最大径平均为（9.1±3.7）cm（3.0~14.5 cm）。结果：所有患者均成功完成手术,5例腹腔镜下完成（中转开放2例）,12例开放手术完成（伴体外循环2例）,平均手术时间为（430.4±120.7） min（284~694 min）,术中平均失血量为（2 918.8±2 608.2）mL（300~10 000 mL）。腔静脉内瘤栓中位长度为10 cm（3~21 cm）,手术切除范围是从瘤栓顶端水平到瘤栓底部水平。发生术后并发症患者11例,分别为Clavien分级Ⅰ级1例、Ⅱ级7例、Ⅳ级2例和Ⅴ级1例（术后第2天因大出血死亡）。术后肌酐中位数为116 μmol/L（79~645 μmol/L）,其中2例需术后床旁血液滤过或透析。术后病理为肾透明细胞癌10例、乳头状细胞癌2型5例、尿路上皮癌1例、梭形细胞肉瘤1例。中位随访时间为8个月（1~28个月）,1例围术期死亡,1例术后9个月因多发转移死亡,3例患者发现远处转移,2例术后随访3~6个月仍有双下肢水肿,其余患者恢复情况和治疗效果良好。结论：对于某些侵犯腔静脉的肾肿瘤瘤栓的患者,可行腔静脉节段切除完成手术,短期随访手术效果良好。     

心脏不停跳连续缝合法行二尖瓣置换术97例分析

目的:探讨浅低温体外循环心脏不停跳连续缝合行二尖瓣置换术的应用效果。方法:对97例风湿性心脏病二尖瓣病变患者,在浅低温体外循环心脏不停跳的情况下,采用连续缝合方法进行人工机械二尖瓣置换术。术中仅阻断上、下腔静脉,不阻断主动脉,在心脏缓慢空跳下,采用右心房-房间隔切口径路,加强左、右心吸引,保持术野清晰,用一根2-0 Prolene双头针带小垫片的缝线连续缝合行二尖瓣置换。同期合并手术有:三尖瓣环De Vega环缩术29例,左心房血栓清除术5例。结果:术后瓣周漏1例,再次手术修补成功,其余患者手术顺利,术后无低心排血量综合征、严重心律失常、气栓、血栓、卡瓣等发生,均治愈出院,随访2~24个月,效果满意。结论:浅低温体外循环心脏不停跳连续缝合法行二尖瓣置换术,缩短手术时间,采用较接近生理状态的心肌保护方法,减少手术并发症,具有良好的心肌保护效果。     

累及右心的下腔静脉瘤栓手术的麻醉处理

下腔静脉瘤栓主要来源于静脉内平滑肌瘤病和肝肾肿瘤。瘤体向上蔓延可累及右心、甚至肺动脉,造成严重循环梗阻。体外循环支持下的瘤体摘除术是治疗累及右心的下腔静脉瘤栓的最为有效方法。由于这种累及右心的下腔静脉瘤栓极为罕见,文献报道也多局限于手术处理的个例介绍,目前尚无临床研究或者文献综述来讨论这种累及右心的下腔静脉瘤栓手术的麻醉处理,即便是有关麻醉处理的个案报道也极为罕见。本文将在简述这类疾病的行为特点和手术方式的基础上,结合笔者在这类手术上的初步经验,探讨如何对这类手术实施麻醉和术中管理。     

胎儿期超声诊断左位下腔静脉及双下腔静脉

目的: 总结胎儿期左位下腔静脉及双下腔静脉的超声声像图特征。方法: 回顾性分析于浙江大学医学院附属妇产科医院行超声检查显示左位下腔静脉（18例）和双下腔静脉胎儿（16例）的临床资料及超声图像表现,总结其特征。结果: 左位下腔静脉超声表现为胎儿上腹部横切面下腔静脉和腹主动脉位置表现正常,即下腔静脉位于腹主动脉右前方;而于肾门水平下方下腔静脉位于腹主动脉左后方,于肾门水平跨越腹主动脉前方,向右上方斜行,形成右侧的下腔静脉,最后流入右心房。双下腔静脉超声表现为胎儿下腹部横切面脊柱前方见三根血管横断面。腹部冠状切面腹主动脉两侧均见静脉伴行,并均延续自同侧髂静脉。34例下腔静脉异常胎儿中,合并其他系统异常17例,其中心脏异常13例。结论: 左位下腔静脉和双下腔静脉有典型产前超声表现,产前超声容易诊断。左位下腔静脉和双下腔静脉常合并其他系统异常（尤其是心脏畸形）,须仔细排查。     

肾癌根治性切除加癌栓取出术治疗MayoⅢ级下腔静脉癌栓的手术技术及临床经验

目的：探讨肾癌根治性切除加下腔静脉癌栓取出术治疗Mayo Ⅲ级下腔静脉癌栓（inferior vena cava tumor thrombus,IVCTT）的有效性和安全性,总结手术技术及临床经验。方法：回顾性分析2014年10月至2016年9月北京大学第三医院泌尿外科收治的8例肾癌合并Mayo Ⅲ级（美国Mayo医学中心分级法）IVCTT患者的临床资料。8例患者中男性3例,女性5例,年龄18~77岁,平均（58.0±18.7）岁,体重指数（body mass index,BMI）为15.2~30.8 kg/m2,平均(22.7±4.4) kg/m2。8例患者的肿瘤均位于右侧,肿瘤直径(7.9±2.5) cm。行开放肾癌根治性切除加下腔静脉癌栓取出术者5例,行腹腔镜下肾癌根治性切除加下腔静脉癌栓取出术者3例,其中1例患者行中转开放手术。结果：8例患者手术均顺利完成,无围术期死亡病例。手术时间272~567 min,平均(370.3±101.6) min。下腔静脉阻断时间17~55 min,平均(41.0±12.1) min。术中出血量200~3 000 mL,平均(1 181.3±915.7) mL。术中输注悬浮红细胞者5例,输入量800~2 000 mL,平均(850.0±783.8) mL。术中输注血浆者3例,输入量400~1 000 mL。术后住院时间9~23 d,平均(14.1±4.0) d。8例患者中4例因术中发现癌栓侵犯下腔静脉壁而行下腔静脉壁切除术。8例患者术前血肌酐值60~101 μmol/L,平均(76.4±15.3) μmol/L,术后1周血肌酐值74~127 μmol/L,平均(100.8±21.1) μmol/L。术后组织病理检查诊断为肾透明细胞癌（renal clear cell carcinoma,RCC）6例,肾乳头状腺癌（papillary adenocarcinoma）1例,Fuhrman分级为Ⅲ级6例、Ⅳ级1例,尤文肉瘤（Ewing’s sarcoma）1例。8例患者中,5例发生术后早期并发症,均为ClavienⅡ级并发症,未见严重并发症发生。8例患者均获随访,随访时间2~24个月,平均11.3个月。术前未发现远处转移的7例患者中,出现远处转移1例,为肺转移。结论：肾癌根治性切除加下腔静脉癌栓取出术治疗Mayo Ⅲ级下腔静脉癌栓较为有效、安全。Ⅲ级静脉癌栓延伸范围广,手术技术难度较大,充分的术前准备、丰富的解剖学知识和手术操作经验可提高手术安全性。     

下腔静脉变异并发下肢深静脉血栓形成的临床诊断及治疗

  目的  探讨先天性下腔静脉变异(inferior vena cava malformation,IVCM)合并下肢深静脉血栓形成(deep vein thrombosis,DVT)的临床诊断与治疗方法。  方法  回顾性分析2011年8月—2021年7月蚌埠医学院第一附属医院血管外科收治的16例下腔静脉变异患者的临床资料,分析其临床表现及诊治方法。  结果  双下腔静脉(double inferior vena cava,DIVC)变异12例,其中9例合并下肢DVT(5例合并左下肢DVT,均行下腔静脉滤器植入+导管溶栓术,3例合并右下肢DVT,行右位IVC滤器植入术,1例合并双下肢DVT, 分别于左右位IVC各置入1枚滤器),3例合并不明原因双下肢肿胀,给予消肿治疗;左位下腔静脉(left inferior vena cava,LIVC)变异4例,其中2例因下肢肿胀行左下肢深静脉造影发现,给予消肿治疗,2例合并右下肢DVT术中经左股静脉穿刺造影发现,行下腔静脉滤器植入术,其中1例同时行置管溶栓术。放置永久性滤器1例,可回收滤器10例,所有DVT患者术后均给予抗凝治疗。  结论  IVCM是静脉血栓栓塞症的重要危险因素,合适位置的下腔静脉滤器置入具有重要的临床意义。