摘要

胆总管囊肿是一种先天性胆道疾病，儿童常感兴趣。据最近的报道，他们在成年人中比较少见，但在过去的几十年里发病率越来越高。在亚洲人群和妇女中发病率较高。在成人中，临床表现包括疾病的特定症状和并发症，如黄疸、胆管炎、胰腺炎和胆道恶性肿瘤。为了避免这些严重的并发症，需要明确的治疗，包括手术切除囊肿和肝肠造口胆囊切除术。我们报告的情况下，一位年轻女性谁到达我们的注意腹部疼痛和黄疸为胆总管囊肿。

关键字

胆总管的囊肿;胆道囊肿;黄疸;囊肿切除术

介绍

胆道囊肿是一种罕见的胆道畸形，占良性胆道疾病的1%。报告的发病率可能因不同系列而异，亚洲人口(1:1.000 - 1:10.000)[2]高于东方国家，后者约为1:10.000 - 1:15 .000。女性更常感兴趣，比例为4:1[2,3]。

最早由Alonso-Lej在1959年分类，目前最被广泛接受的分类是Todani等人在1977年[4]提供的分类。这种分类将胆道囊肿分为五类:I型、II型和III型累及肝外胆管，形态和部位不同;IV型累及肝内和肝外胆管树;V型只累及肝内胆管(卡罗利病)。

胆总管囊肿多见于年轻人，但在最近的系列报道中，成人[5]的诊断增加。典型的临床表现包括右上象限腹痛、梗阻性黄疸、恶心呕吐和可触及腹部肿块，尤其是儿童。在其他病例中，并发症如胆囊炎、胆总管结石或胆管癌是该病的首次发病。胆管炎和胰腺炎也可发生。

胆道囊肿的早期诊断和治疗是非常重要的，因为它们具有随年龄增长而增加的恶性潜能。对于肝外胆管囊肿，尤其是I型和IVa型胆管囊肿，外科切除囊肿并随后肝肠吻合术是全世界公认的金标准，而肝切除术则适用于局限性Caroli病[6]。在这里我们报告的情况下，一位妇女来到我们的急诊科(ED)腹痛和黄疸。

病例报告

一位30岁的妇女来到费拉拉圣安娜医院的急诊科，主诉上腹疼痛和右疑病症，但没有发烧。她过去的记忆是阴性的，她没有假定家庭治疗。体格检查没有任何相关结果。病人接受腹部超声检查，发现胆总管内36毫米的囊性扩张，没有其他胆道扩张或其他急性病变的迹象。考虑到没有需要住院治疗的情况，该病人获准出院。三天后，这位女士回到我们的急诊科，说是症状持续。血清总胆红素为4mg /dL，直接胆红素为2.5 mg/dL。病人腹部CT扫描显示提交内部扩张和extra-hepatic胆管,最大直径5厘米的胆总管(CBD),初结束的胰腺部分CBD(图1)。病人承认为了尝试内镜逆行胰胆管造影(ERCP)。入院次日ERCP显示肝外CBD扩张伴乳头状狭窄，置入10fr塑料支架。患者还进行了磁共振胰胆管造影(MRCP)，证实了22毫米的CBD扩张(图2)。此外，对过去影像学的修正显示，2004年进行的腹部超声显示CBD扩张。 and re-evaluation of the patient anamnesis revealed a personal history of recurrent abdominal pain in paediatric age. These anamnestic findings, together with the clinical presentation and imaging, suggested Todani’s Type I CBD cyst diagnosis. In order to exclude other causes of biliary tract dilatation, the woman underwent endoscopic ultrasounds that confirmed the previous radiological investigations in absence of findings suspected for neoplasms. The patient was discharged few days later, after complete resolution of symptoms. One month later, the patient come back to ED complaining abdominal pain in right upper quadrant, nausea and vomit, and hyperpyrexia (38.5 ºC). Her blood tests showed neutrophilic leucocytosis, elevate C-reactive Protein and Procalcitonin plasma levels and a serum total bilirubin of 3 mg/dL. After admission, antimicrobial therapy started. Another ERCP found a partial dislocation of the biliary prosthesis, which was removed and substituted with a metallic one. She was discharged two days after the procedure.

图1:CT扫描显示胆总管扩张(白色星号)，胆囊扩张(黑色星号)，肝内胆管扩张(红色箭头)。

图2:RM证实胆总管引流后扩张(白色星号)。

在接下来的几个月里，患者因腹痛三次来我们的急诊科，但她没有要求住院。患者被送往Forlì的Morgagni-Pierantoni医院的普通和肿瘤外科，该医院是肝胰腺胆道疾病转诊中心，她在那里接受术前评估。确诊6个月后，患者行囊肿切除、胆囊切除及Roux-en-Y肝空肠造口术(图3)。术后患者未出现任何相关并发症，完全康复。

图3:胆总管囊肿及胆囊切除。

讨论

胆道囊肿的病因尚不清楚，许多理论已提出解释其发病机制。最广泛接受的一种观点认为，异常胰胆连接处(APBJ)，包含在十二指肠壁外的胰管和胰管的结合中，形成一条长总管，可导致胰胆反流并随后激活胆管中的胰酶。活化的胰酶破坏导管壁，导致囊肿形成[7]。其他理论认为，先天性导管板畸形或获得性远端胆道梗阻导致胆道囊肿[8]的发展。尽管约80%的胆道囊肿是在儿童时期诊断出来的，但其在成年期的外翻并不少见。近年来，由于现代影像技术[9]的普及，成人的诊断正在增加。由间歇性黄疸、右腹部肿块和疼痛构成的典型三联征很少被发现，多见于儿童。成人的症状没有特异性，取决于囊肿[10]的并发症。包括反复腹痛、恶心呕吐、消化不良和黄疸。囊肿也可导致复发性胆管炎的感染并发症，如肝脓肿或败血症和感染性休克。 Moreover, patient can reveal a personal history of recurrent abdominal pain, so attention on collecting anamnesis should be payed. Assesment of patients with symptomatic CC often starts from abdominal ultrasounds that demonstrate a cystic mass in right upper quadrant in continuity with biliary tree. CT scan can easily demonstrate if cystic lesions are in continuity with biliary tract and is more accurate than ultrasonography in diagnosis of biliary tract cysts, especially for intrahepatic and distal portions [10]. MRCP is considered the gold standard for diagnosis of CBD cysts, with report sensitivity of 90-100% [11]. It can also be performed with use of hepatospecific contrast agents, that can allow better visualization of biliary anatomy. In some cases, more invasive imaging techniques such as ERCP and Percutaneous Transhepatic Cholangiography should be performed. The advantage of these procedures is that they could be both diagnostic and therapeutic procedures. Biliary tract cysts can lead to severe complications related to chronic bile stasis and inflammation characterized by important mortality and morbidity. Moreover, association between CC and cholangiocarcinoma is well known. The risk of development of malignancies of the biliary tract increase with age, with reported incidence of 10-30%. Todani’s type I and type IV cysts are mostly associated with development of cholangio carcinoma. In order to avoid complications, definitive treatment of choledochal cysts is required [5]. The correct treatment depends on the cyst type. Many surgical strategies have been proposed for type I cysts. Internal drainage of the cyst through creation of a cystenterostomy was the first treatment that gained wide diffusion, but it was burdened by many long-term complications [8]. Moreover, incomplete excision of the cyst can lead to the development of malignancies. Nowadays, complete resection of the cyst with cholecistectomy and reconstruction with hepaticojejunostomy is considered the treatment of choice for Biliary Cysts [6,12]. Early postoperative complications include pancreatitis, cholangitis and anastomotic leakage, while bile ducts and anastomotic stenosis, recurrent cholangitis or pancreatitis, biliary stones and malignancies are reported as long term complications [12]. Therefore, lifelong follow up, especially for cancer surveillance, is mandatory. When required, it is advisable to send patient to a referral centre for treatment of biliary pathologies in order to concentrate resources and expertise, to reduce the risk of surgical complications, and to guarantee better outcomes to patients.

结论

胆总管囊肿在西方国家很少见，通常发生于年轻人，但成人也可诊断。手术治疗是必要的，以避免并发症的发展，包括切除囊肿。应将治疗委托给转诊中心，以确保患者得到最佳护理。

参考文献

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4. Todani T, Watanabe Y, Narusue M, Tabuchi K, Okajima K(1977)先天性胆管囊肿:分型、手术方法及37例包括胆管囊肿癌变的病例回顾。Am J Surg 134: 263-269。［Ref。］
5. Kelly-R SM, Soares KC, Ejaz A, Pawlik TM(2016)胆总管囊肿的治疗。Curr Opin Gastroenterol 32: 225-231。［Ref。］
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7. Babbitt DP(1969)先天性胆总管囊肿:基于胆总管和胰球异常关系的新病因学概念。安·拉多尔(巴黎)12:231 -240。［Ref。］
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9. Lewis VA, Adam SZ, Nikolaidis P, Wood C, Wu JG，等(2015)胆总管囊肿的影像学研究。腹部成像40:1567-1580。［Ref。］
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11. Park DH, Kim MH, Lee SK, Lee SS, Choi JS，等(2005)MRCP能否取代ERCP对胆总管囊肿的诊断作用?Gastrointest Endosc 62: 360-366。［Ref。］
12. edl BH, Olino K, Cameron JL(2009)胆总管囊肿的治疗现状。Adv Surg 43: 221-232。［Ref。］

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条信息

文章类型:病例报告

引用:Romeo L, Anania G, Rubino S, Bombardini C, Petrarulo F, et al.(2018)一名年轻女性的胆总管囊肿:西方成年人黄疸的一种罕见原因。临床资料开放存取4(2):d .doi。org/10.16966/2469 - 6714.135

版权:©2018 Romeo L，等。这是一篇开放获取的文章，在知识共享署名许可协议的条款下发布，该协议允许在任何媒体上无限制地使用、发布和复制，前提是注明原作者和来源。

出版的历史:

收到日期:2018年2月21日(

接受日期:02年3月2018

发表日期:07年3月2018