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Caroli病是一种先天性的肝内胆管囊性扩张症。外科治疗的目的包括清除病灶,防止胆管癌变;通畅胆汁引流,缓解症状。肝切除是目前最常用的外科治疗方式,对局限性的Caroli病可达到根治的效果,病人术后胆管炎等症状可长期缓解。而对于少部分弥漫型病人,也可选择性清除肝内主要的扩张病灶,并进行密切的随访,如病人进一步进展,肝移植常成为治疗的选择。对于多数弥漫型病人,肝移植是最终的治疗选择,可获得满意的长期效果。     

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1997年肝移植首次应用于Caroli病的治疗,此后陆续出现肝移植成功治疗Caroli病的报道。临床研究发现Caroli病病人肝移植术后受体与移植物的5年存活率分别可以达到76%和71%。活体肝移植在Caroli病治疗方面的应用也逐渐增多,大量的临床观察发现Caroli病病人经活体肝移植术后受体2年存活率可达81.6%。因此,对于肝内胆管扩张弥漫全肝或反复出现胆管炎、肝内胆管结石的Caroli病病人来说,肝脏移植是其最终可行的治疗手段。     

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Caroli病是种常染色体隐性遗传疾病,表现为肝内胆管多发性囊性扩张。1958年由法国医生Caroli系统描述其特征而命名。Todani等将其归入先天性胆管扩张症的第Ⅴ型。如同时合并肝外胆管囊性扩张,属于Flanigan分型中的Ⅳ型,Todani分型中的Ⅳa型。同时合并肝纤维化、髓质海绵肾、肾皮质囊性变等称之为Caroli综合征。Caroli病是一种少见病,常合并肝内胆管结石,临床表现为反复发作的胆管炎,易被误诊为肝内胆管结石合并肝内胆管扩张引起的肝内胆管炎和肝脓肿。     

先天性胆总管囊肿的外科治疗

目的：探讨先天性胆总管囊肿的诊治方法。方法：分析我院近10年外科手术治疗的36例先天性胆总管囊肿的临床资料。结果：全组36例,行囊肿切除、肝管空肠吻合术32例,行囊肿切除、肝左叶切除、肝管空肠吻合2例,2例Caroli病行囊肿前壁切除及部分肝组织切除、囊肿空肠吻合术。全组病例1例发生胆瘘经保守治疗痊愈,2例成年病人发生恶变,全部痊愈。结论：针对先天性胆总管囊肿的分型,采用相应的手术方式是成功的关键。对Ⅱ型Caroli病人肝移植是最有效的方法。     

Caroli氏病八例报告

Caroli氏病也称先天性肝内胆管囊状扩张症,以前文献多认为它是一种罕见的肝内胆道疾病。近年来,由于对本病的认识和诊断技术的提高,临床发现日渐增多。本院近年收治并获病理证实的Caroli氏病8例,现报告如     

中国儿童肝移植麻醉技术操作规范(2019版)

儿童肝移植作为一项复杂的系统性工程,其成功离不开肝移植外科、麻醉科、ICU、儿科和肝病科等多个科室专家所组成的多学科团队。儿童肝移植麻醉管理有其特殊性,充分的术前评估、严密的术中监测和精细化的麻醉管理以及麻醉医师与其他多学科团队成员间充分的沟通与合作是确保手术成功的关键。为进一步规范儿童肝移植麻醉与围手术期管理,中华医学会器官移植学分会组织移植外科和麻醉专家,总结国内外相关研究最新进展,并结合国际指南和临床实践,从儿童肝移植麻醉前评估与准备、麻醉方法与用药、围手术期麻醉监测和管理以及麻醉恢复与术后管理等方面,制订《中国儿童肝移植麻醉技术操作规范(2019版)》。     

闪烁图和X线检查Caroli病

Caroli病为先天性肝内胆管囊状扩张,有时亦有肝外胆管扩张,并可伴肝纤维化或门脉高压。用侵入性内窥镜逆行胆管造影或经皮肝穿刺胆管造影可诊断此病。     

肝移植治疗Caroli病七例报道

目的 探讨肝移植治疗Caroli病的临床效果.方法 回顾性分析1999年9月至2008年2月期间7例弥漫型Caroli病患者行肝移植治疗后的临床资料.对其临床特征、手术方式、并发症类型以及随访情况进行分析.结果 7例患者术前均表现为复发性胆管炎症状并最终确诊为弥散型Caroli病.其中男性3例,女性4例;年龄10～31岁,平均16岁.6例患者肝移植术前接受保守治疗,1例曾行胆囊切除、T型管引流术.4例患者行劈离式肝移植,2例行全肝移植,1例行亲属活体供肝肝移植.其中2例患者术中采用静脉-静脉转流术.手术平均耗时9.1 h.术后并发症包括:肺部感染3例、急性排斥反应2例、胸腔积液2例、肝断面胆汁漏1例.经抗感染、调整免疫抑制方案及用量、胸腔穿刺抽吸及经皮穿刺引流等对症保守治疗后症状得到控制.1例肝移植受者术后19 d死于急性肾功能衰竭合并多器官功能衰竭,其余6例受者均存活.随访发现,存活的6例受者健康状况良好,其中最长存活已达7年.结论 应用肝移植治疗弥散型Caroli病具有良好的临床效果.     

Caroli''''s病——一种恶性疾病的前期状态

Caroli′s病是一种以肝内胆管呈多发性节段性囊性扩张,并与胆道系统相通为特点的肝内胆管先天性疾病.1958年Caroli首次描述了本病的特点:(1)肝内胆管呈节段性囊性扩张;(2)胆管结石、胆管炎、和肝脓肿的发生率增加;(3)无肝硬化和门静脉高压症;(4)同时有肾小管扩张或类似于肾的囊肿性疾病.1968年Schiewe等报告在本病的基础上合并发生胆管癌.世界文献已报告Caroli′s病138例,6例合并胆管癌.     

Caroli's病诊治现状

Caroli’s病是一种少见的先天性肝内胆管多发节段性囊性扩张性疾病,又称交通性海绵状胆管扩张症或先天性肝内胆管扩张症,由法国医生Caroli于1958年系统描述并报道其特征性表现而命名。本病属先天性胆管囊肿第Ⅴ型,病因尚不明确,国外多数学者认为该病是因胚胎发育过程中胆管发育异常所致先天性结构薄弱或交感神经缺如引起。发病机制与下列因素有关:①胆管发育不良,致部分胆管壁先天性薄弱,胆管由实心向空心演变时组织增殖快慢不均,部分节段发育慢,表现为狭窄,其远端因阻塞而扩张,阻塞愈重则扩张愈大,从而形成大小不一囊样病变。近来实验研究…     

Caroli Disease Revisited: A Case of a Kidney Transplant Patient With Autosomal Polycystic Kidney Disease and Recurrent Episodes of Cholangitis

Polycystic kidney disease (PKD) is a genetic disorder leading to end-stage renal disease more commonly in the fourth to sixth decades of life. Cyst formation in the kidneys and other organs such as the liver and pancreas is the main characteristic of this disease. A significant number of patients with PKD undergo kidney transplantation and receive significant immunosuppression, predisposing them to comorbidities such as infections and malignancies. The link between these cystic syndromes and Caroli disease (which is radiologically demonstrated as bile duct ectasia, segmental cystic dilation of intrahepatic bile ducts, with a normal common bile duct and absence of hepatic fibrosis or portal hypertension), is extremely important. Suspicion, screening, and timely diagnosis of the presence of Caroli disease in patients with PKD prior or post receiving a kidney transplant will reduce morbidity in these patients and possibly prolong both graft and patient survival. We describe a patient with autosomal dominant polycystic kidney disease who underwent recurrent admissions for presumed cholangitis and was eventually diagnosed with Caroli disease.     

Liver transplantation for severe hepatic graft-versus-host disease in two children after hematopoietic stem cell transplantation

Chronic graft-versus-host disease (GVHD) is a frequent complication of bone marrow transplantation (BMT). After the skin, the liver is the second, most frequent target of GVHD, which presenting with hyperbilirubinemia, elevated liver enzymes, and coagulopathy. Progressive destruction of small intrahepatic bile ducts causes vanishing bile duct syndrome and leads to end-stage liver disease. We report 2 successful cases of orthotopic liver transplantation performed in children with severe GVHD after hematopoietic stem cell transplantation from a matched unrelated donor (HSCT-MUD).     

Congenital intrahepatic bile duct dilatation is a potentially curable disease: long-term results of a multi-institutional study

OBJECTIVE: To report clinical presentation, perioperative outcome, and long-term results of surgical management of congenital intrahepatic bile duct (IHBD) dilatations (including Caroli disease) in a multi-institutional setting. SUMMARY BACKGROUND DATA: Congenital IHBD dilatations are a rare congenital disorder predisposing to intrahepatic stones, cholangitis, and cholangiocarcinoma. The management remains difficult and controversial for bilobar forms of the disease or when concurrent congenital hepatic fibrosis is associated. METHODS: From 1976 to 2004, 33 patients (range 11 to 79 years) were retrospectively enrolled. Disease extent into the liver was unilobar in 26 patients and bilobar in 7 patients (21%). Cholangiocarcinoma, congenital hepatic fibrosis, and intrahepatic stones were present in 2, 10, and 20 patients, respectively. Transplantations or liver resections were performed in 5 and 27 patients, respectively, whereas 1 asymptomatic patient was managed conservatively. RESULTS: Postoperative mortality was nil. Postoperative complications occurred in 16 of 32 operated patients (50%) and additional procedures for residual stones were required in 5 patients. During a median follow-up of 80 months (1 patient being lost for follow-up) no patient developed metachronous carcinoma. Six patients (30%) developed recurrent intrahepatic stones but satisfactory late outcome was achieved in 27 patients (87%). CONCLUSIONS: Partial or total liver resection achieves satisfactory late outcome in congenital IHBD dilatations, when the affection is treated at an early stage and when the extent of liver resection is tailored to intrahepatic disease extent and takes into consideration the presence and severity of underlying chronic liver and renal diseases.     

Caroli syndrome. Clinical aspects--diagnosis--therapy

The Caroli syndrome is a rare autosomal-recessively inherited disease with cystic dilatation of the intrahepatic bile ducts. Cystic bile duct dilatations are usually accompanied by cholelithiasis and subsequent cholangitis. This is the most substantial danger implied in the Caroli syndrome. It has often been left unconsidered as a cause in differential diagnosis of cholangitis, resulting in undetected protracted course of the disease. Once an unambiguous diagnosis has been established, the authors, by their own experience from three cases and with reference to the literature, recommend subtotal hepatectomy as optional therapy.     

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先天性胆管扩张症以Todani分型分为I～V型,且已为广大外科医师所接受。手术方式上对于I、Ⅱ和Ⅳ型大多选择胆总管囊肿的全切除+肝总管空肠Roux-en-Y吻合;基本废弃了内引流术式;外引流作为急诊时的过渡性手术。对于Caroli病则根据病变的部位而有所不同,单发局限型行肝叶或肝段切除术;对于肝内多发病灶或弥漫型,治疗上主要是针对反复发作的胆管炎及肝内胆管结石等并发症问题的处理,必要时进行肝脏移植;对于中央型的,尽量切除囊壁,选择最低位行空肠Roux-en-Y吻合。治疗上不可一概而论,对各种术式应有清晰的认识。     

Liver Transplantation Consequential to Caroli's Syndrome: A Case Report

Caroli's disease is a rare condition that includes fibrocystic malformations of the bile duct. It consists of multifocal congenital dilatations of the intrahepatic bile ducts, which may be diffuse or limited, presenting in sack form that produces cystic structures which communicate with the biliary tree. Herein we have presented the case of a 44-year-old woman with recurrent cholangitis consequential to Caroli's syndrome. The distinctive feature of this case was that it was the first and only liver transplantation performed to date for this cause at our center among 700 procedures that had been performed over 19 years. The hepatectomy sample from the liver transplantation showed large cystic dilatations at the level of segments VII and VIII. The pathological study reported congenital dilatation of the intrahepatic bile ducts, associated with congenital hepatic fibrosis (Caroli's syndrome). Caroli's syndrome is a complex association of conditions which usually presents together with polycystic kidney lesions. Orthotopic liver transplantation is still the only therapeutic option for diffuse, uncontrollable cases or those with significant portal hypertension, as well as being the final option in the other cases in the event of a lack of response to other therapeutic options or as an alternative to them.     

Caroli's disease and orthotopic liver transplantation.

Caroli's disease is a rare congenital hepatic disease, characterized by segmental dilatation of the biliary tree. Patients who have recurrent bouts of biliary infection, particularly those with complications related to portal hypertension, may require orthotopic liver transplantation (OLT). Few case reports have described the outcome of OLT in patients with Caroli's disease and to date there is no large series reported in the literature. We retrospectively analyzed the outcome of OLT in patients with Caroli's disease who underwent OLT between 1982 and 2002 at Starzl Transplantation Institute, University of Pittsburgh. Patients were identified and data was collected by computerized search of the electronic database system. All patients had confirmation of diagnosis by histopathology of explanted liver. A total of 33 patients with Caroli's disease were listed for liver transplantation, 3 of whom were excluded, as they were not transplanted. A total of 90% had signs of hepatic decompensation at the time of OLT. Median posttransplantation follow-up was 7.7 yr. Short-term graft and patient survival at 1 month was 83% and 86%, whereas overall long-term graft survival rates at 1, 5, and 10 yr were 73%, 62%, and 53%, respectively, and patient survival rates were 76%, 65%, and 56%, respectively. Long-term outcome in patients who survived the first year after transplantation was significantly better. Their survival rate at 5 and 10 yr was 90% and 78%. On univariable analysis, recipient age, donor male gender, coexistent congenital hepatic fibrosis, and re-OLT were associated with poor patient survival. Eight patients were retransplanted, 3 of whom had primary nonfunction. A total of 13 patients died; the most common cause of death being sepsis and cardiovascular complications. Patients who died of sepsis had cholangitis pre-OLT. In conclusion, OLT is a form of curative and life-saving therapy in patients with Caroli's disease, especially in those with decompensated liver disease. Overall survival is better with liver transplantation and is comparable with the survival of recipients who undergo OLT for other etiologies of chronic liver disease. Survival was poor in patients with congenital hepatic fibrosis (Caroli's syndrome) and in those who had cholangitis at the time OLT.