青少年布加综合征临床特点及介入治疗

目的 探讨青少年布加综合征(Budd-Chiari syndrome,BCS)临床特点并评估介入治疗的疗效.方法 徐州医学院附属医院自1990年1月至2012年4月收治227例年龄在29岁以下的BCS患者,均经彩超及血管造影证实,其中下腔静脉型87例、肝静脉型105例、混合型35例.通过经皮血管腔内血管成形术(percutaneous transluminal angioplasty,PTA)、血管内支架置入术及置管溶栓术开通闭塞血管.术后给予抗凝治疗、定期随访.结果 227例患者均以门脉高压的症状和体征为最初临床表现.210例患者初次介入手术取得成功,其中下腔静脉阻塞型成功率100％,肝静脉阻塞型85.7％,混合型94.3％.介入治疗成功后的下腔静脉平均压力由术前的(26.52±8.16) cm H2O下降至术后(14.28 ±4.08) cm H2O(P＜0.05).肝静脉平均压力由术前(35.70±13.26) cm H2O下降至术后(18.36±8.16) cm H2O(P＜0.05).术后随访1个月至15年,平均(46±37)个月.再狭窄发生率为21.4％(45/210),其中下腔静脉型狭窄率为13.8％(12/87),肝静脉阻塞型31.1％ (28/90),混合型15.2％ (5/33),肝静脉型患者再狭窄发生率明显高于其他两型.再狭窄患者介入治疗方法同初次治疗,44例再狭窄患者再次介入治疗取得成功.结论 青少年布加综合征患者以肝静脉阻塞型最多见,门脉高压症状和体征为主要临床表现特点,肝静脉型介入治疗后复发率高于其他两型.     

Postoperative MR imaging features after superior semicircular canal plugging in Minor syndrome

Purpose

To report the postoperative magnetic resonance imaging (MRI) features after superior semicircular canal plugging in patients with Minor syndrome.

Resectability of large focal liver lesions

Despite modern imaging techniques evaluation of the resectability of large focal liver lesions is often difficult or impossible until the time of operation. Based on experience with 54 primary or secondary focal liver tumours, a simple morphological classification has been found to be reasonably predictive of resectability. All tumours were classified before operation using computed tomography, ultrasound and angiography; 38 patients underwent laparotomy. Dependent, 'hanging' tumours (n = 7) were resected in six cases, expansively growing 'pushing' tumours (n = 19) were resected in 18 cases, and infiltrating, 'invasive' tumours (n = 17) were not resected because of involvement with major vascular structures. Eleven small tumours (less than 5 cm) were not classifiable by this system. This simple classification may be a useful clinical concept in preoperative assessment of resectability of focal liver lesions. Hanging tumours should always be resected, and large expansile tumours are generally resectable despite their size. Invasive tumours can only be resected in exceptional cases.     

侵袭性肺炎克雷伯菌肝脓肿综合征临床及影像学特征

目的 观察侵袭性肺炎克雷伯菌肝脓肿综合征（IKPLAS）临床及影像学特征。方法 回顾性纳入68例肺炎克雷伯菌肝脓肿（KPLA）患者,根据是否发生肝外侵袭性感染分为IKPLAS组（n=25）及非IKPLAS组（n=43）,比较组间临床资料及CT和/或MRI表现。结果 IKPLAS组患者年龄低于而糖化血红蛋白、D-二聚体均高于非IKPLAS组（P均＜0.05）。IKPLAS组18例、非IKPLAS组6例可见肝静脉血栓性静脉炎,IKPLAS组10例、非IKPLAS组28例可见动脉期脓肿周围异常高灌注,组间差异均有统计学意义（P均＜0.05）。IKPLAS组肝外感染主要累及肺（19/25,76.00%）。结论 IKPLAS患者年龄低于而糖化血红蛋白、D-二聚体高于其他KPLA;较常见肝静脉血栓性静脉炎及肝外感染主要累及肺为IKPLAS影像学特征。     

Laparoscopic procedures in focal lesions of the liver

Seventy-three laparoscopic procedures were performed for the treatment of focal lesions of the liver. Fifty-four patients had operations for non-parasitic cysts of the liver and cystic disease. Polycystic disease of the liver was in 8 patients. Multiple cysts were in 19 patients, solitary - in 27. A total of 139 cysts were treated. Surgery was performed with the standard technique. Atypical marginal resection of the liver was performed in 16 patients for hemangioma (n=8), nodular hyperplasia (n=2), hepatic metastases (n=5), hamartoma (n=1). Atypical marginal resection by type of peritumor resection was conducted in 5 cases, and it was similar to segmentectomy in 11 cases. Laparoscopic cryodestruction of benign tumors and hepatic metastases was performed in 3 patients including in combination with atypical marginal resection in 2 cases. There were no intrasurgical complications which required laparotomies. There were no hemotransfusions. Long-term results were followed-up from 1 to 3 years in 25 patients with hepatic cysts and from 3 months to 3 years in 7 patients after marginal resection for hemangiomas and fibronodular hyperplasia. Ultrasonic examination revealed no recurrences of the disease.     

Left-side hemihepatectomy in focal lesions of the liver

An analysis of results of the radical surgical treatment of 12 patients with focal diseases of the left portal lobe of the liver is presented. The succession of performing the surgical measures for anatomical hemihepatectomy is discussed. The method of hemostasis in resection of the liver by means of compression of hepatoduodenal ligament is described.     

The resectability of large focal liver lesions

This paper presents a simple clinical concept of tumor morphology considered to be of value in the preoperative assessment of focal liver tumours. Based on preoperative clinical and radiological investigation and laparotomy in a large number of liver lesions, we propose a classification of liver tumours of special value in the preoperative assessment of resectability of large primary and secondary liver tumours.     

Budd-Chiari syndrome: portacaval shunt and subsequent liver transplantation

The Budd-Chiari syndrome (BCS) is caused by hepatic venous outflow obstruction, which often leads to death as a result of portal hypertension and liver failure. Therapeutic approaches vary widely from conventional medical therapy to liver transplantation. If and when a patient suffering with BCS needs surgery remains a matter of contention. However, it is well accepted that portacaval shunt surgery and orthotopic liver transplantation represent efficient surgical treatments of this condition. We report on a patient with an eventful course after BCS was diagnosed. After portacaval shunt surgery the patient had acute liver failure and had a successful orthotopic liver transplantation.     

MR弥散峰度成像平均弥散率鉴别肝脏良、恶性病变:Meta分析

目的 采用Meta分析方法评估MR弥散峰度成像（DKI）平均弥散率（MD）鉴别肝脏良、恶性病变的效能。方法 于Pubmed、Cochrane Library、Embase、中国知网、维普和万方医学网中检索自建库至2021年3月20日以DKI参数MD值鉴别诊断肝脏良、恶性病变的相关文献;根据标准筛选文献,提取相关资料,以Stata 15.1和Meta-Disc 1.4软件分析MD值鉴别肝脏良、恶性病变的效能。结果 共纳入6篇文献,含493例患者、225个良性及362个恶性共587个病灶。汇总分析结果显示,肝脏恶性病变的MD值小于良性[标准均数差（SMD）=-1.31,95%CI（-1.54,-1.09）,Z=11.34,P<0.000 1];以MD值鉴别肝脏良、恶性病变的合并敏感度、特异度、阳性似然比、阴性似然比及诊断比值比分别为0.78[95%CI（0.74,0.82）]、0.73[95%CI（0.67,0.79）]、2.74[95%CI（2.22,3.39）]、0.31[95%CI（0.25,0.38）]及9.71[95%CI（6.63,14.21）];汇总受试者工作特征曲线的曲线下面积（AUC）为0.823 8。以MD值判断肝脏恶性病变为恶性的验后概率为76%,判断良性病变为恶性的验后概率为22%。结论 DKI的MD值鉴别肝脏良、恶性病变的效能较高。     

Surgical treatment of Budd-Chiari syndrome and veno-occlusive disease]

We operated 5 patients with the Budd-Chiari syndrome or veno-occlusive disease between 1984 and 1989 (2 porto-caval shunts, one Warren distal spleno-renal shunt, one meso-atrial shunt and one Le Veen peritoneo-jugular shunt). As liver transplantation is now part of the therapeutic armamentarium for these conditions, this paper reviews retrospectively the indications for surgery in these 5 patients.     

MRI in the differential diagnosis of liver focal lesions

51 patients with focal liver abnormalities at ultrasound were examined by MRI. The cases with liver metastasis of known origin submitted for staging and cholangiocarcinomas of the external bile ducts or gall bladder with liver invasion were not included in the study. Most of the examinations were performed for suspected hepatocarcinoma. MRI studies, using 2D T1 and T2 weighted FSE sequences and T1 weighted 3D SPGRE contrast enhanced sequences were able to differentiate between different kinds of benign lesions e.g. large hemangiomas or FNH or even complicated hydatic cysts from hepatocarcinoma.     

Laparoscopic surgery of focal lesions of the liver

In this work the laparoscopic surgical approach of two benignant hepatic lesions (focal nodular hyperplasia [1st case], and hepatic cyst with simultaneous cholecystolithiasis [2nd case]) will be discussed. The authors present the steps of the laparoscopic procedures: non-anatomical liver resection in the first, resection and cholecystectomy in the latter case. During the operations no surgical complications occurred, the postoperative course was also uneventful. The patients were discharged in the fifth day after the surgery, without any complains. During a short follow-up period, our patients were asymptomatic and free of recurrences. In our opinion, the laparoscopic hepatic surgery might be a feasible technique in the hands of surgeons with hepatic and laparoscopic experience, but careful selection criteria should be followed in the selection of such cases.     

Value of three-dimensional sonography in biopsy of focal liver lesions

Background. The aim of this study was to evaluate the efficacy of three-dimensional (3D) sonography in liver biopsies. Primarily, the diagnosis of hepatic tumors is based on 2D sonography. This method allows the noninvasive evaluation of the anatomical structure and tumor localization. Interpretation difficulties emerge most often during the assessment of tumor boundaries and their relationships to the blood vessels. Methods. The study included 19 patients, aged 40–75 years (average, 57.5 years) admitted to the Department of General Surgery due to diagnosed liver tumors. Results. The livers of all the examined patients displayed focal changes. In order to obtain material for histopathologic examinations, percutaneous needle biopsies were performed, with no complications. Conclusions. 3D sonography generated more information about hepatic changes, due to imaging of multiplanar reconstructions in oblique presentations (which are impossible to present in classical 2D), and thus facilitated design of the biopsy. Evaluation of the biopsy needle visualization inside the tumor with the use of both the 2D and the 3D presentations depends on the subjective approach of the examiner. Based on our knowledge and experience in ultrasonography-guided needle biopsy, we believe 3D sonography-guided biopsy has high efficacy. Received: March 31, 2002 / Accepted: December 17, 2002 RID="*" ID="*" Offprint requests to: J.R. Ladny     

Recurrence of Budd-Chiari syndrome after liver transplantation in paroxysmal nocturnal hemoglobinuria

Venous thrombembolism is a major complication of paroxysmal nocturnal hemoglobinuria (PNH). Often, veins of atypical localization are afflicted, resulting in cerebral, mesenteric, or hepatic venous thrombosis. We present a patient who received an orthotopic liver graft for chronic Budd-Chiari syndrome in 1988. PNH was the only thrombophilic predisposition identified in this patient. After transplantation, he repeatedly suffered from hemorrhage. Subsequently, the patient discontinued prophylactic anticoagulation nearly 10 years after transplantation. Within 6 months Budd-Chiari syndrome recurred, but stabilized after anticoagulation therapy with low-molecular-weight heparin was reinstituted. The patient is clinically stable 14 years after receiving the liver graft. Eleven cases of relapsing Budd-Chiari syndrome have been reported in the literature. Of these, four patients suffered from PNH. All patients transplanted for PNH-associated Budd-Chiari syndrome in these reports suffered from either major bleeding or thrombosis. In conclusion, patients afflicted with PNH appear to be at high risk of incurring complications after liver transplantation.