经腹腔镜开窗术治疗多囊肝

成人多囊性肝病（APLD简称多囊肝）并不多见，且常无症状；但也有症状明显甚至出现严重并发症。治疗方法有经皮硬化剂注射，手术开窗术，肝切除以及肝移植术等。开腹手术后常引起广泛粘连，因此一旦囊肿复发就难以再次开窗；而腹腔镜开窗术除创伤小，恢复快，还可反复多次开窗。作者自1991年1月至1994年7月治疗15例有症状的APLD患者，全部为女性。其中13例（中数年龄49岁）作了16次经腹腔镜开窗术。其余2例则一例作了左肝外侧叶切除，另一例因并发严重肾衰及顽固性腹水而作了肝肾移植术。13例均有明显上腹痛或不适症状。全组15例同时均…     

肝部分切除结合囊肿开窗术治疗严重成人多囊肝的疗效分析

目的 探讨肝部分切除结合囊肿开窗术治疗严重成人多囊肝的疗效.方法 回顾性分析近期2例严重成人多囊肝的临床、影像学资料及手术疗效.结果 2例多囊肝采用肝部分切除结合囊肿开窗术治疗术后即时及远期疗效良好.结论 肝部分切除结合囊肿开窗术治疗严重成人多囊肝安全有效,远期疗效好,可作为该类疾病的首选治疗方法.     

肝移植治疗肝硬化肝癌切除后肝功能衰竭

目的　评估肝硬化肝癌局部切除后肝功能衰竭 (肝衰 )行肝移植的疗效。方法　 1例重度肝硬化肝癌病人行局部切除后肝衰 ,行原位肝移植术 ;术后尽早予足量化疗 ;免疫抑制剂予最低有效剂量。结果　肝移植术使肝衰、门静脉高压症得到有效治疗 ,肿瘤得到根治性切除 ,术后 6个月未见复发征象。结论　肝硬化肝癌切除后肝衰行肝移植疗效较好。合并重度肝硬化的大肝癌可能是肝移植的手术指征     

Bismuth Ⅳ型肝门部胆管癌的外科治疗

目的探讨BismuthⅣ型肝门部胆管癌的外科治疗方法。方法对近5年来经手术和病理确诊的22例BismuthⅣ型肝门部胆管癌患者的临床资料进行回顾性分析。结果22例患者中,男13例,女9例,男女之比1.4∶1。常规手术方法治疗16例(常规手术治疗组),包括手术切除5例(31.3%),其中根治性切除2例,姑息切除3例;内或外引流术11例。经典式原位肝移植术6例(肝移植组)。常规手术方法治疗组1,2年累积生存率分别为32.1%,0;肝移植组除1例术后11个月死于慢性排异反应外,余均健康存活,现已分别存活28,19,17,12个月和9个月,未见肿瘤复发和转移,术后1,2年累积生存率分别为80.0%(4/5),50.0%(1/2)。两组生存率比较差异有显著性(P=0.041)。结论BismuthⅣ型肝门部胆管癌应行积极的外科治疗,其中根治性切除术是提高患者存活率的关健;常规手术无法根治切除者是原位肝移植的适应证,术后疗效满意。     

严重多囊肝的外科处理

目的　严重多囊肝外科处理上较困难 ,治疗后症状再发率较高 ,本文探讨目的为提高对严重多囊肝的处理水平。方法　对 2 2例获随访的严重多囊肝病例按治疗方法不同 ,对疗效进行分析 ,讨论了治疗方法的选择与疗效的关系。结果　肝部分切除加开窗术、剖腹肝囊肿开窗术、B超下穿刺治疗及腹腔镜肝囊肿开窗术在术后 0 5～ 7年 (平均 3年 )内症状再发 ,再发率分别为 0 ,2 8.5 % ,6 5 .5 %及 10 0 %。结论　肝部分切除加开窗术是治疗严重肝囊肿的有效方法 ;B超下穿刺治疗更适合作为手术前后暂时缓解症状的辅助手段 ;腹腔镜开窗术应慎用于严重多囊肝病人     

肝移植治疗肝内胆管结石和肝包虫病

目的探讨肝移植治疗合并胆汁性肝硬化的广泛肝内胆管结石和不能手术治疗的肝包虫病的效果。方法对2例广泛肝内胆管结石伴胆汁性肝硬化和4例不能手术切除的肝包虫病(泡球蚴病)患者施行原位肝移植，术后定期随访，评价疗效。结果2例胆管结石患者已存活2年多：4例肝包虫病患者，除1例术后3个月死于心力衰竭外，其余3例已分别存活9个月、15个月及2年；存活患者均恢复了正常生活和工作。结论肝移植可作为治疗广泛肝内胆管结石和不能手术治疗的肝包虫病的有效手段。     

原位肝移植治疗肝脏上皮样血管内皮瘤(附2例报告及文献复习)

目的探讨原位肝移植治疗肝脏上皮样血管内皮瘤(EHE)的疗效及相关治疗进展。方法回顾性分析2例肝脏EHE患者的临床资料。例1为肝脏广泛多发EHE合并脾脏多发梗死灶的患者,实施了原位肝移植+脾切除术;例2为肝脏多发EHE患者,仅实施了原位肝移植。结合国内外文献,分析肝脏EHE患者的病理学特点、肝移植治疗及预后情况。结果两例患者手术过程顺利,均康复出院。病理结果证实为肝脏EHE,例1合并脾脏EHE。例1术后1个月抗排斥反应药由他克莫司换为西罗莫司,术后4个月EHE复发,给予卡培他滨口服化学药物治疗(化疗);术后6个月考虑肝脏EHE复发并多发性骨转移,予索拉非尼口服抗肿瘤治疗,术后7个月患者死于恶病质、肝衰竭。例2随访至投稿日(术后8个月),未见术后相关并发症及肿瘤复发迹象。国内外文献报道提示肝脏EHE首选手术切除,肝内广泛多发而无法手术切除者,甚至有肝外病灶者,肝移植疗效最佳。另外,化疗、经皮穿刺肝动脉化疗栓塞和抗血管生成治疗对本病也有一定疗效。结论肝脏EHE治疗首选手术切除,对于肝内广泛多发者,肝移植被认为是最佳的治疗选择。应重视肝脏EHE移植术后复发的防治,以期进一步提高该病的疗效。     

再次背驮式肝移植并肾移植治疗肝移植术后慢性排斥并肾功能不全(附一例报告)

目的 探讨用原位背驮式肝移植并肾移植术治疗先天性多囊病肝移植术后慢性排斥并肾功能不全。方法 供肝取自同血型脑死亡供体;顺利切除原病肝,行原位再次背驮式新肝植入及右髂窝肾移植。结果 再次背驮式肝移植并肾移植手术成功,供肝,供肾功能良好,术后１８ｄ死于曲霉菌性腹膜炎所致的多器官功能衰竭。结论 背驮式技术适用于再次肝移植;肝移植术后慢性排斥反应并肾功能不全是联合肝肾移植的适应证。     

改良背驮式肝移植治疗wilson's病

目的: 探讨改良背驮式肝移植术治疗肝豆状核变性的意义.方法: 2例患者采用改良背驮式原位肝移植术.结果: 手术成功,2例存活至今(分别为术后22个月和11个月),且生存质量明显改善.结论: 改良背驮式肝移植术是治疗肝豆状核变性的一种优选方法.     

肝移植治疗原发性肝癌切除术后肝内复发七例

目的 探讨对原发性肝癌切除术后肝内复发患者进行肝移植手术的适应证和围手术期的治疗经验.方法 回顾性分析2000年9月至2005年9月间7例原发性肝癌切除术后肝内复发的患者接受原位肝移植治疗的临床资料,其中男性6例,女性1例,平均年龄43.7岁,肝移植术前均经病理学检查确诊为原发性肝癌,肿瘤组织学分级为高、中分化,肝癌切除术后无瘤期为6～31个月,均未发生肿瘤细胞侵犯大血管和肝外转移.所有患者均采用改良背驮式肝移植术.术后采用他克莫司(或西罗莫司)+霉酚酸酯+激素的三联免疫抑制方案.观察肝移植术后受者并发症及存活率情况.总结肝移植治疗原发性肝癌切除术后肝内复发的经验.结果 所有受者肝移植手术过程顺利,围手术期无死亡.1例术后22 h发生腹腔出血,1例术后13 d发生腹腔感染,1例术后4个月发生门静脉血栓,其余未发生严重并发症,7例受者均顺利出院.有3例受者分别于移植术后9、13及19个月时,因肿瘤复发而死亡,其余4例均长期无瘤存活,最长已达52个月.受者的1、2年存活率分别为85.7%和57.1%.结论 肝移植能有效治疗原发性肝癌切除术后肝内复发,受者适应证的选择和围手术期的辅助治疗非常关键.     

Highly symptomatic adult polycystic liver disease: options and results of surgical management

BACKGROUND: The majority of patients afflicted with adult polycystic liver disease (APLD) are asymptomatic. For those who are symptomatic, there are a variety of treatment procedures that have been proposed but these lack verification through long-term studies with respect to safety and long-term effectiveness. Choice of surgical procedure is related to the severity of APLD and morphology of the cysts within the liver. The aim of the present study was to analyse the immediate and long-term results of fenestration and combined resection-fenestration at Singapore General Hospital. METHODS: A retrospective analysis of clinical, operative, imaging and follow-up data was carried out for 12 patients (10 women and two men) with symptomatic APLD who underwent surgery from January 1992 to December 2000. The primary outcome measures assessed were postoperative alleviation of symptoms, performance status, complications, mortality and long-term recurrence of symptoms. RESULTS: Nine patients underwent 12 fenestration procedures and three patients had combined resection-fenestration. Fenestration was carried out for eight of nine patients with a dominant cyst morphology and combination resection-fenestration was carried out for those three patients with diffuse cyst morphology. There was no operative mortality and all patients were discharged from hospital free of their preoperative symptoms. Overall morbidity rate was 58%. The mean follow up for the present cohort was 29.3 months. Only two patients had recurrence of symptoms. One patient with dominant cyst morphology who underwent laparoscopic fenestration had recurrence at 26 and 43 months but this was successfully treated finally with open fenestration. The other patient had diffuse cyst morphology and was treated with fenestration for recurrent cyst infection that recurred 1 month postoperatively. This required subsequent intravenous antibiotics and percutaneous drainage for resolution of symptoms. CONCLUSION: Treatment for symptomatic APLD should be based on the morphology of the liver cysts. Fenestration is a safe and acceptable procedure for patients with a dominant cyst pattern where liver size can be reduced after the cysts collapse. A combination of resection-fenestration is suitable for those with a diffuse cyst pattern where grossly affected segments are resected in combination with fenestration to allow for reduction in liver size.     

Discussion

OBJECTIVE: The aim of this study was to evaluate the immediate and long-term results in a retrospective series of patients with highly symptomatic adult polycystic liver disease (APLD) treated by extensive fenestration techniques. A classification of APLD was developed as a stratification scheme to help surgeons conceptualize which operation to offer to patients with APLD. SUMMARY BACKGROUND DATA: Treatment options for APLD remain controversial, with partisans of fenestration techniques or combined liver resection-fenestration. METHODS: Clinical symptoms, performance status, liver volume measurement by computed tomography (CT), and morbidity were recorded before surgery and after surgery. Adult polycystic liver disease was classified according to the number, size, and location of liver cysts and the amount of remaining liver parenchyma. Follow-up was obtained by clinical and CT examinations in all patients. RESULTS: Ten patients with highly symptomatic APLD were operated on using an extensive fenestration technique (by laparotomy in 8 patients and by laparoscopy in 2 patients, 1 of whom conversion to laparotomy was required). The mean preoperative liver volume was 7761 cm3. There was no mortality. Postoperative morbidity occurred in 50%, mainly from biliary complications, requiring reintervention in two cases. Massive intraoperative hemorrhage occurred in one patient. During a mean follow-up time of 71 months (range, 17 to 239 months), all patients were improved clinically according to their estimated performance status. The mean postoperative liver volume was 4596 cm3, which represents a mean liver volume reduction rate of 43%. However, in type III APLD, despite absence of clinical symptoms, a significant increase in liver volume was observed in 40% of the patients. CONCLUSIONS: Extensive fenestration is effective in relieving symptoms in patients with APLD. Hemorrhage and biliary complications are possible consequences of such an aggressive attempt to reduce liver volume. The procedure can be performed laparoscopically in type I APLD. A longer follow-up period is mandatory in type II APLD, to confirm the usefulness of the fenestration procedure. In type III APLD, significant disease progression was observed in 40% of the patients during long-term follow-up. Fenestration may not be the most appropriate operation for long-term management of all types of APLD.     

Laparoscopic fenestration for the treatment of patients with severe adult polycystic liver disease

BACKGROUND: The purpose of this study was to present our experience in laparoscopic fenestration for patients with severe symptomatic adult polycystic liver disease (APLD), analyze its feasibility, and evaluate its immediate and mid-term outcome. METHODS: Between January 2000 and January 2002, 9 patients underwent laparoscopic fenestration for symptomatic APLD in our laparoendoscopic unit. All patients had both liver lobes affected with multiple cysts, whereas type II disease (present in 8 patients) was not a contraindication for the procedure. The results were retrospectively evaluated. RESULTS: Conversion to laparotomy was required in 1 patient who was submitted to a second laparoscopic procedure (2 years postoperatively) after being admitted to our department with sepsis. Complete regression of symptoms was achieved in 7 of our patients (77.8%). One death occurred because of acute renal failure established 5 weeks after the patient was discharged. During a mean follow-up of 25.8 months, 2 patients presented with recurrence of their symptoms (22.2%). One of them was reoperated on; both of them remain symptom free 14 months postoperatively. CONCLUSIONS: Laparoscopic fenestration appears to be a useful and effective approach for severe APLD. It is associated with short hospital stay and a significant symptom-free period. Despite the reported morbidity, aggressive and meticulous deroofing of as many cysts as possible can be successfully applied for carefully selected patients with type II disease.     

Resection, including transplantation, for hepatoblastoma and hepatocellular carcinoma: impact on survival.

Long-term survival in children with primary hepatic malignancies can not be expected without complete tumor resection. In the last ten years we have treated 21 children with hepatocellular carcinoma (HCC) and 21 children with hepatoblastoma (HEP), with tumor extirpation our surgical goal. Operative treatment included partial hepatectomy ([PH] 20), either primary (10) or delayed (following chemotherapy) (10), total hepatectomy and orthotopic liver transplantation ([OLT] 13), or upper abdominal exenteration and multiple organ transplantation (2). Two patients had both PH and subsequent total hepatectomy and OLT. Overall survival was 48% (20/42), with 9 patients dying of progressive disease prior to removal of their tumor. HEP patient survival was 67% (14/21), including 2 of 6 who underwent primary PH, 7 of 8 who had delayed PH, and 5 of 6 who underwent OLT. Survival for the children with HCC was 29% (6/21), including 1 of 4 after primary PH, 1 of 2 following delayed PH, 3 of 7 following OLT, and 1 of 2 after exenteration and multiple organ transplantation. Preoperative chemotherapy facilitated removal of 10 initially unresectable tumors (8 HEP, 2 HCC) at a second-look procedure. Total hepatectomy and OLT markedly improved survival in patients with disease unresectable by standard methods. Partial hepatectomy, either primary or delayed, should be attempted in all children with hepatic malignancies. Total hepatectomy and OLT appears to be a viable adjunct in the treatment of childhood malignancies, and should be used for otherwise unresectable tumors as part of a carefully planned protocol.     

Choice of transplantation techniques and indications for liver transplantation in polycystic liver disease in patients with no signs of end-stage liver disease

OBJECTIVE: Since the initiation of the Liver Transplant Program, 500 liver procedures have been performed. Polycystic liver disease (PLD) and polycystic kidney-liver disease (PKLD) have been rare indications for orthotopic liver transplantation (OLT). Only 7 patients (1.4%) underwent transplantation due to PLD and PKLD. MATERIALS AND METHODS: The group consisted of 4 patients who underwent OLT (0.8%) and 3 patients who received simultaneous liver kidney transplantation (LKT; 0.6%). Our objective was to analyze the indications for either OLT or combined LKT as well as indications for surgical techniques during OLT among patients with PLD or PKLD. RESULTS: The main indication for OLT was massive hepatomegaly causing severe physical handicaps, fatigue, and clinically advanced malnutrition. All 3 patients with indications for combined LKT were dialysis-dependent. None of the patients had symptoms of end-stage liver disease and/or hepatic failure. In 4 cases, a portal bypass was applied, and the piggy-back method used in the other 3 cases. The hepatectomy caused no uncommon difficulty. In cases of simultaneous transplantations, the kidney was implanted separately after OLT. All patients are alive following the transplantation; major surgical complications have occurred. CONCLUSIONS: Patients with PLD can undergo OLT safely with good results. They benefit from the relief of abdominal distension and anorexia. Patients with PKLD who are dialysis-dependent should undergo simultaneous LKT. The surgical technique was solely dependent on the intraoperative conditions determined during the dissection phase.     

大鼠部分肝移植的实验研究

目的 建立稳定的大鼠部分肝移植动物模型,以对移植肝的功能、移植肝的增殖活性进行研究。方法 采用雄性SD大鼠,用两袖套法行活体供肝移植,设部分肝移植组、全肝移植组及部分肝切除组。分别于术后不同时间段取血检测总胆红素、丙氨酸转氨酶、天冬氨酸转氨酶及谷氨酰转移酶;取肝组织行组织学检查。结果 部分肝移植组和全肝移植组术后受者存活时间的差异无显著性;各组术后1周肝功能酶学指标增高,后逐步降至正常;组织学检查可见大量单核细胞浸润,特别是在门静脉周围汇管区,术后1个月细胞浸润和坏死减少,可见胆管增殖;肝切除和部分肝移植组的肝组织可见线粒体肥大以及二倍体和多倍体的肝细胞,中央小静脉、肝窦和叶间静脉轻度扩张;术后300d,各组的肝组织结构基本正常。结论 本模型是研究活体供肝部分肝移植的较好模型。     

Bridging a patient with acute liver failure to liver transplantation by the AMC-bioartificial liver

Recently a phase I clinical trial has been started in Italy to bridge patients with acute liver failure (ALF) to orthotopic liver transplantation (OLT) by the AMC-bioartificial liver (AMC-BAL). The AMC-BAL is charged with 10 x 10(9) viable primary porcine hepatocytes isolated from a specified pathogen-free (SPF) pig. Here we report a patient with ALF due to acute HBV infection. This patient was treated for 35 h by two AMC-BAL treatments and was bridged to OLT. There was improvement of biochemical and clinical parameters during the treatment. No severe adverse events were observed during treatment and follow-up of 15 months after hospital discharge. Possible porcine endogenous retrovirus (PERV) activity could not be detected in the patient's blood or blood cells up to 12 months after treatment.     

Adult polycystic disease of the liver

Adult polycystic liver disease (APLD) is a rare disorder of liver parenchyma occasionally requiring surgical treatment. Its association with adult polycystic kidney disease has meant that as renal dialysis has become widely available there is an increased number of patients surviving with cystic liver changes. Although usually asymptomatic, patients with APLD may present with abdominal pain or swelling. Liver function is not usually compromised and computed tomography or abdominal ultrasonography are the most useful investigations. The complications of cyst rupture, infection, cholangiocarcinoma and compression of surrounding structures are discussed. Surgical treatment remains controversial, and the options of cyst puncture, fenestration with or without hepatic resection, and liver transplantation are reviewed.     

Emergency subtotal hepatectomy: a new concept for acetaminophen-induced acute liver failure: temporary hepatic support by auxiliary orthotopic liver transplantation enables long-term success

INTRODUCTION: Acetaminophen (paracetamol) overdose (AOD) has recently emerged as the leading cause of acute liver failure (ALF) in the United States, with an incidence approaching that seen in the United Kingdom. We describe a new way to treat AOD ALF patients fulfilling King's College criteria for "super-urgent" liver transplantation. METHODS: Beginning in June 1998, we have been piloting a clinical program of subtotal hepatectomy and auxiliary orthotopic liver transplantation (ALT) for AOD ALF. Our technique is based on the following principles: (1) subtotal hepatectomy; (2) auxiliary transplantation of a whole liver graft; (3) gradual withdrawal of immunosuppression after recovery. Results were compared with patients who had undergone an orthotopic liver transplantation (OLT) for AOD ALF in the same period. Quality of life comparisons were made using the SF36 questionnaire. RESULTS: Thirteen patients underwent this procedure between June 1998 and March 2005. Median survival is 68 months (range, 0-102 m). Actual survival data show that 9 of 13 patients are alive (69%) compared with 7 of 13 OLT patients (54%). One ALT patient required a retransplantation with an OLT due to hepatic vein thrombosis, and immunosuppression is therefore maintained. The other 8 surviving ALT patients are off immunosuppression. These 8 ALT patients have normal liver function and have a better quality of life compared with the 7 surviving OLT patients. CONCLUSION: Our results with this new technique are encouraging: 69% actual survival, no long-term immunosuppression requirement, and improved quality of life in the 62% successful cases.     

Liver Resection Associated With Mini Porto-Caval Shunt as Salvage Treatment in Patients With Progression of Hepatocellular Carcinoma Before Liver Transplantation: A Case Report

Tumor progression before orthotopic liver transplantation (OLT) is the main cause of dropouts from waiting lists among patients with hepatocellular carcinoma (HCC). Performing a porto-caval shunt (PCS) before parenchymal liver transection has the potential to allow an extended hepatectomy in patients with decompensated liver cirrhosis, reducing portal hyperflow and therefore the sinusoidal shear-stress on the remnant liver. We report the case of a 59-year-old man affected by hepatitis C virus (HCV)-related decompensated liver cirrhosis (Child Pugh score presentation, C-10; Model for End Stage Liver Disease score, 18) and HCC (2 lesions of 2 and 2.8 cm). The patient began the evaluation to join the OLT waiting list, but, in the 3 months required to complete the evaluation, he developed tumor progression: 3 HCC lesions, the largest 1 with a diameter of about 4.4 cm. These findings excluded transplantation criteria and the patient was referred to our center. After appropriate preoperative studies, the patient underwent a major liver resection (trisegmentectomy) after side-to-side PCS by interposition of an iliac vein graft from a cadaveric donor. The patient overcame the worsened severity of cirrhosis. After 6 months of follow-up, he developed 2 other HCC nodules. He was then included on the waiting list at our center, undergoing OLT from a cadaveric donor at 8 months after salvage treatment. At 36 months after OLT, he is alive and free from HCC recurrence. Associating a partial side-to-side PCS with hepatic resection may represent a potential salvage therapy for patients with decompensated cirrhosis and HCC progression beyond listing criteria for OLT.