CHAPTER 106  第 106 章

Choledochal cyst is a congenital anomaly involving cystic dilation of various ducts of the biliary tree. They are relatively rare in the Western world but more common in Asia. Several hypotheses exist as to the etiology of choledochal cysts including pancreaticobiliary maljunction. Three patterns of presentation have emerged since Alonso-Lei and colleagues published their landmark paper describing their classification of ductal dilation. The first is a cystic mass in the abdomen identified prenatally, the second is jaundice presenting in infancy, and the third is ascending cholangitis, obstructive jaundice, or pancreatitis presenting later in childhood or in adulthood. Regardless of the time of presentation, surgical intervention is indicated to mitigate potential damage to the liver, prevent jaundice and pancreatitis, and prevent cancer.
先天性胆道扩张是指胆管树的各种导管囊性扩张的先天性畸形。在西方世界相对罕见，但在亚洲更为常见。关于胆道囊肿的病因存在多种假说，包括胰胆管连接不良。自 Alonso-Lei 及其同事发表其标志性论文描述其对导管扩张的分类以来，出现了三种表现模式。第一种是产前发现的腹部囊性肿块，第二种是婴儿期出现的黄疸，第三种是儿童晚期或成人期出现的升高性胆管炎、梗阻性黄疸或胰腺炎。无论何时出现，均需进行手术干预，以减轻对肝脏的潜在损害，预防黄疸和胰腺炎，并预防癌症。

The incidence of choledochal cyst ranges from 1 in 100,000 to 150,000 live births in Western populations with the incidence in the United States reportedly as high as 1 in 13,500. Choledochal cysts are significantly more common in Asia, with rates as high as 1 in 1000 in Japan. There is a well-documented female dominance ( 3 to $4:1$$4:1$4:14: 1 ) that contributes to the belief that choledochal cyst is sex-linked. ${}^{1-3}$${}^{1-3}$^(1-3){ }^{1-3} Familial cases have been recognized, but no genetic basis has been evident. The majority ( $\approx 60\mathrm{\%}$$\approx 60\mathrm{\%}$~~60%\approx 60 \% ) of choledochal cysts are diagnosed in the first
胆道囊肿的发病率在西方人群中为 1/100,000 至 150,000 活产，据报道，美国的发病率高达 1/13,500。胆道囊肿在亚洲更为常见，在日本的发病率高达 1/1000。女性占主导地位（3 比 $4:1$$4:1$4:14: 1 ）这一事实为胆道囊肿与性别有关的观点提供了支持。 ${}^{1-3}$${}^{1-3}$^(1-3){ }^{1-3} 已发现家族性病例，但尚未发现遗传基础。大多数（ $\approx 60\mathrm{\%}$$\approx 60\mathrm{\%}$~~60%\approx 60 \% ）胆道囊肿在
decade of life. Roughly 20% remain undiagnosed until later in childhood or adulthood, and the remaining 20% to $25\mathrm{\%}$$25\mathrm{\%}$25%25 \% of cases are diagnosed prenatally. Prenatal diagnosis of choledochal cysts is increasing in frequency, ${}^2$${}^2$^(2){ }^{2} as is the number of cases diagnosed in adulthood in the United States and Europe, perhaps due to higher index of suspicion or improved imaging techniques.
生命中的十年。大约 20% 的患者在儿童期或成年期才被诊断出来，其余 20% 至 $25\mathrm{\%}$$25\mathrm{\%}$25%25 \% 的病例在产前被诊断出来。先天性胆总管囊肿的产前诊断越来越频繁， ${}^2$${}^2$^(2){ }^{2} 在美国和欧洲成年患者的诊断数量也在增加，这可能是由于更高的怀疑指数或改进的影像技术。

The etiology of choledochal cyst remains unknown but is commonly accepted to be congenital in nature. Distal obstruction, weakness of the duct wall, or a combination of the two are the predominant hypotheses. ${}^{4-6}$${}^{4-6}$^(4-6){ }^{4-6} The etiologic basis for choledochal cyst is likely multifactorial. In 1852 Douglas reported that there is potential congenital weakness of the common bile duct in the common fusiform type. ${}^5$${}^5$^(5){ }^{5} In 1936 Yotsuyanagi reported that the defect is due to failed formation in the early embryologic development of the biliary system from an overproduction of epithelial cells, leading to a dilated common bile duct. ${}^7$${}^7$^(7){ }^{7} This hypothesis is now largely regarded as unlikely, however.
胆总管囊肿的确切病因仍不明，但通常认为是先天性疾病。主要的假说为远端梗阻、胆管壁薄弱或两者兼而有之。 胆总管囊肿的病因基础可能是多因素的。1852 年 Douglas 报道了常见的梭形型胆总管可能存在先天性薄弱点。 1936 年由 Yotsuyanagi 报道该缺陷是由上皮细胞过度增生导致的胆道系统早期胚胎发育形成失败，从而导致胆总管扩张。然而，现在这个假说很大程度上被认为不可能成立。

In 1916 Kozumi and Kodama recognized an anomalous junction between the bile and pancreatic ducts during an autopsy case with choledochal cyst. ${}^8$${}^8$^(8){ }^{8} In 1969 Babbitt described the “long common channel” theory, also known as pancreaticobiliary maljunction (PBM), which is commonly seen in association with choledochal cyst. ${}^{9,10}$${}^{9,10}$^(9,10){ }^{9,10} PBM is a rare congenital anomaly described as a proximal insertion of the pancreatic duct into the common bile duct as a result of incomplete migration of the choledochopancreatic junction into the duodenal wall, creating a “long common channel.” In 1984 Todani and colleagues were able to show PBM through analysis of endoscopic retrograde cholangiopancreatography (ERCP). ${}^{11}$${}^{11}$^(11){ }^{11} In fetal development, PBM creates a nidus for reflux of pancreatic enzymes into the common duct that causes damage to the ductal wall and leads to cyst formation. Due to the reflux, PBM is also commonly associated with carcinoma of the bile duct and gallbladder. Distal obstruction at the level of the duodenum is an additional factor leading to damage of the ductal wall and causing formation of a saccular dilation. ${}^{11-15}$${}^{11-15}$^(11-15){ }^{11-15} Investigators have proposed various mechanisms related to distal ductal stenosis such as an abnormal ductal insertion into the duodenum, distal common bile duct stenosis, persistent epithelial web, valve dysfunction, or neuromuscular irregularity of the sphincter. ${}^{13}$${}^{13}$^(13){ }^{13} PBM has a reported $3\mathrm{\%}$$3\mathrm{\%}$3%3 \% incidence. ${}^{16}$${}^{16}$^(16){ }^{16} PBM is commonly associated with choledochal cysts and carcinoma of the biliary duct and gallbladder. The majority of choledochal cysts are associated with PBM; however, PBM can be seen without an associated choledochal cyst in $20\mathrm{\%}$$20\mathrm{\%}$20%20 \% to $30\mathrm{\%}$$30\mathrm{\%}$30%30 \% of cases. Carcinoma of the gallbladder and, less frequently, the bile duct remains an association with PBM even without ductal dilation. ${}^{16}$${}^{16}$^(16){ }^{16}
1916 年，Kozumi 和 Kodama 在一次胆总管囊肿的尸检病例中，观察到胆管和胰管之间存在异常连接。 ${}^8$${}^8$^(8){ }^{8} 1969 年，Babbitt 提出了“长共同通道”理论，也称为胰胆管汇合异常（PBM），通常与胆总管囊肿有关。 ${}^{9,10}$${}^{9,10}$^(9,10){ }^{9,10} PBM 是一种罕见的先天性畸形，其特征是胰管的近端插入胆总管，这是由于胆胰管连接未完全移行到十二指肠壁，从而形成“长共同通道”。1984 年，Todani 和同事通过内镜逆行胰胆管造影（ERCP）分析证实了 PBM。 ${}^{11}$${}^{11}$^(11){ }^{11} 在胎儿发育过程中，PBM 为胰酶反流回胆总管提供了条件，从而导致胆管壁的损伤并形成囊肿。由于反流，PBM 还常与胆管癌和胆囊癌有关。十二指肠水平的远端阻塞是导致胆管壁损伤并形成囊袋状扩张的另一个因素。 ${}^{16}$${}^{16}$^(16){ }^{16} $20\mathrm{\%}$$20\mathrm{\%}$20%20 \% $30\mathrm{\%}$$30\mathrm{\%}$30%30 \%

Other types of choledochal cysts, specifically choledochocele and Caroli disease, have different proposed etiologies. Choledochocele has two main variations as described by Manning and colleagues. ${}^{17}$${}^{17}$^(17){ }^{17} In the most common variation, the common bile duct and pancreatic duct enter the duodenum separately. In the second variation, a common bile duct diverticulum arises at the level of the ampulla of Vater with the pancreatic duct entering the distal common bile duct in its normal anatomic variation. The formation of the diverticulum is thought to be due to either an obstruction of the ampulla and a low insertion or a congenital duodenal duplication. Caroli disease is believed to arise from an arrest in the twelfth
其他类型的胆总管囊肿，特别是胆总管膨出和卡罗利病，具有不同的发病机制。曼宁及其同事描述了胆总管膨出的两种主要变异方式。在最常见的变异中，胆总管和胰管分别进入十二指肠。在第二种变异中，胆总管憩室发生在 Vater 壶腹部平面，而胰管则在解剖变异的正常情况下进入胆总管远端。憩室的形成被认为是由于壶腹部梗阻和低位插入或先天性十二指肠重复所致。卡罗利病被认为是由第十二周的胚胎发育停止引起的。
week of gestation in the ductal plate at the level of the large ducts of the hilum. This is distinct from Caroli syndrome, in which there is arrest of the small ductules of the periphery. ${}^{18,19}$${}^{18,19}$^(18,19){ }^{18,19} Caroli disease has been associated with polycystic kidney disease. ${}^{1,20-22}$${}^{1,20-22}$^(1,20-22){ }^{1,20-22} Overall, the etiologic background of choledochal cyst is congenital in nature, resulting from reflux of pancreatic enzymes causing damage to the bile duct wall and complete or partial distal obstruction of the bile duct.
妊娠期乳腺导管板水平的巨大导管处的一周。这不同于卡罗里综合征，后者是小外周细小导管的停滞。卡罗里病与多囊肾病有关。总的来说，胆总管囊肿的病因背景是先天性的，由胰酶反流引起的胆管壁损伤和胆管完全或部分远端阻塞导致。

In normal development the fourth week of gestation marks the formation of a hepatic diverticulum extending from the ventral aspect of the foregut. Thus the biliary structures are endodermal in nature. The diverticulum progresses to cranial and caudal buds. The liver and extrahepatic biliary tree form from the cranial bud and the caudal bud becomes the superior and inferior buds. The gallbladder and cystic duct are derived from the superior bud, and the inferior bud gives rise to the right and left ventral pancreas. There is some controversy regarding whether there is a solid phase to the biliary tree early in gestation and eventual recanalization or whether the biliary tree has a continuous lumen. ${}^{23}$${}^{23}$^(23){ }^{23} At the sixth week, the ventral pancreatic bud and common bile duct rotate around the duodenum clockwise by 180 degrees. The common bile duct at this point enters the duodenum at the left posterior surface. In the seventh week the main pancreatic duct (Wirsung duct) and common bile duct junction ends in the developing duodenum as closed cavities through elongation to form the ampulla of Vater. The junction retracts in the eighth week of gestation to reside in the submucosa of the duodenal wall. A concentric ring of mesenchyme forms around the junction of the pancreatic and biliary ducts, beginning the formation of the sphincter of Oddi. In the twelfth week of gestation, the main pancreatic and common bile ducts are obliquely arranged in the duodenum. PBM is believed to arise from a misarrangement of the pancreatic and common bile duct and not due to the
在正常发育过程中，妊娠第四周标志着肝憩室从前肠腹侧延伸形成，因此胆道结构在本质上是内胚层的。憩室进展为头尾芽。肝脏和肝外胆道树源于头部芽，尾部芽成为上下芽。胆囊和胆总管源于上芽，下芽形成左右腹侧胰腺。妊娠早期胆道树是否存在固相阶段并最终再管化，或是否存在胆道树连续腔的问题尚存在争议。\ 第六周，腹侧胰芽和胆总管顺时针围绕十二指肠旋转 180 度。此时胆总管在左后表面进入十二指肠。第七周，主胰管(Wirsung 管)和胆总管汇合处在发育的十二指肠内以闭塞腔的形式终止，通过伸长形成 Vater ampulla。 第八周，十二指肠壁粘膜下出现胰管和胆管的连接。围绕胰管和胆管连接处形成一圈同心间充质，开始形成 Oddi 括约肌。第十二周，主胰管和胆总管在十二指肠内呈斜行排列。由于胰腺和胆总管排列不当，而不是由于
pancreaticobiliary system or termination of the junction migration as once thought. ${}^{24}$${}^{24}$^(24){ }^{24}
胰腺胆管系统或移行处的终止，正如曾经认为的那样。 ${}^{24}$${}^{24}$^(24){ }^{24}

The original anatomic classification of choledochal cysts included types I, II, and III. ${}^4$${}^4$^(4){ }^{4} After review of cholangiograms, Todani and colleagues broadened the classification into five types with some subtypes (Fig. 106-1). ${}^{25,26}$${}^{25,26}$^(25,26){ }^{25,26} Type I is by far the most common, accounting for $90\mathrm{\%}$$90\mathrm{\%}$90%90 \% to $95\mathrm{\%}$$95\mathrm{\%}$95%95 \% of cases, ${}^4$${}^4$^(4){ }^{4} and constitutes the cystic/saccular or fusiform dilation of the common bile duct. ${}^{27}$${}^{27}$^(27){ }^{27} Type I is divided into three subtypes (types Ia, Ib, and Ic). Type Ia consists of cystic dilation of the entire common bile duct. Type Ib is cystic dilation of a segment of the common bile duct, and type Ic is fusiform dilation of the common bile duct. Type II is a diverticulum of the common bile duct with no dilation of the common bile, extrahepatic, or intrahepatic ducts. Type III, also referred to as a choledochocele (Fig. 106-2), usually has a normal common bile duct and main pancreatic duct with cystic dilation of the distal common bile duct that is either intraduodenal or intrapancreatic in location. The ducts may either enter the choledochocele separately or in union at the wall of the duodenum, but are usually stenotic at their openings due to chronic inflammation. Type IV is composed of multiple cysts located intrahepatically, extrahepatically, or in both locations. Type V comprises single or multiple intrahepatic cysts without extrahepatic duct dilation. Type V cysts in conjunction with hepatic fibrosis are commonly referred to as Caroli disease.
原有的先天性胆总管囊肿的解剖分类包括 I、II 和 III 型。 ${}^4$${}^4$^(4){ }^{4} 复习了胆管造影后，Todani 及其同事将分类扩展为五种类型，并带有一些亚型（图 106-1）。 ${}^{25,26}$${}^{25,26}$^(25,26){ }^{25,26} I 型是最常见的，约占 $90\mathrm{\%}$$90\mathrm{\%}$90%90 \% 到 $95\mathrm{\%}$$95\mathrm{\%}$95%95 \% 的病例， ${}^4$${}^4$^(4){ }^{4} 构成胆总管的囊状/囊状扩张或梭形扩张。 ${}^{27}$${}^{27}$^(27){ }^{27} I 型分为三种亚型（Ia、Ib 和 Ic 型）。Ia 型包括整个胆总管的囊状扩张。Ib 型是胆总管某一部分的囊状扩张，Ic 型是胆总管的梭形扩张。II 型是胆总管的憩室，无胆汁、肝外或肝内管道的扩张。III 型也称为胆总管囊肿（图 106-2），通常胆总管和主胰管正常，而胆总管远端囊状扩张，位于十二指肠内或胰内。 导管可能分别或联合进入十二指肠壁的胆总管憩室，但由于慢性炎症，其开口通常狭窄。IV 型由位于肝内、肝外或两者位置的多个囊肿组成。V 型包括单个或多个肝内囊肿，无肝外导管扩张。伴有肝纤维化的 V 型囊肿通常被称为卡罗利病。

The gross pathology of choledochal cysts is largely described in the anatomic classification section earlier. As described, choledochal cysts can occur in the intrahepatic ducts, the common bile duct, intraduodenally, or in a combination of
解剖分类部分已经详细描述了先天性胆囊囊肿的总病理学改变。如前所述，先天性胆囊囊肿可发生于肝内胆管、总胆管、十二指肠内或上述部位的组合。

FIGURE 106-1 Classification of the five major choledochal cyst forms and some subtypes.
图 106-1 5 种主要胆总管囊肿形式和一些亚型的分类。

FIGURE 106-2 Two types of choledochoceles. (A from O’Neill JA: Choledochal cyst. Curr Probl Surg 1992;29:374. Used with permission.)
図 106-2 两种类型的胆总管囊肿。(A 引自 O'Neill JA：胆总管囊肿。《当代外科问题》1992；29：374。经许可使用。)
locations. The dilation is primarily fusiform. Pancreaticobiliary maljunction is common, as is stricture of the common bile duct. Sludge, cholelithiasis, or choledocholithiasis is commonly found with choledochal cysts.
原位。扩张主要为梭形。胰胆管汇合异常很常见，胆总管狭窄也常见。胆管囊肿常伴有泥沙、胆结石或胆总管结石。

The histopathology depends on patient age and severity of symptoms. The extrahepatic ducts are thickened with dense connective tissue intermixed with smooth muscle strands. Inflammatory reaction is usually present but may be minimal in the infantile stage. Generally, the inflammatory reaction increases with age and may lead to ulceration in the mucosa and submucosa. The inflammation can extend through the cyst leading to possible adherence to adjacent structures. The most marked inflammation is noted in the intrahepatic cysts. The lining of the cyst is not composed of typical biliary mucosa but rather is relatively acellular and may lack a typical mucosal lining. In the wall of the cyst, mucin-secreting cells are seen in intramural glands. A gastrointestinal mucosa-type composition of immunoreactive gastrin- and somatostatin-containing cells may also be found in the cyst wall due to repeated destruction and regrowth causing epithelial metaplasia. Small areas of columnar epithelium and bile ducts can be noted in the cystic wall of young patients. ${}^{28}$${}^{28}$^(28){ }^{28} The degree of epithelial metaplasia generally increases with age, as does the risk for adenocarcinoma. ${}^{1,29-31}$${}^{1,29-31}$^(1,29-31){ }^{1,29-31} These histologic changes are commonly seen in pancreaticobiliary maljunction as well. As mentioned, the distal common bile duct has various degrees of stricture, with a high-grade stricture commonly seen in the infantile stage. Older patients have stenosis of the distal common bile duct but not complete obstruction.
组织病理学取决于患者年龄和症状的严重程度。肝外胆管增厚，间杂有密集的结缔组织和平滑肌束。通常存在炎症反应，但在婴儿期可能很轻微。一般来说，炎症反应会随着年龄的增长而加剧，并可能导致粘膜和粘膜下层的溃疡。炎症可以延伸穿过囊肿，可能导致与邻近结构粘连。最明显的炎症见于肝内囊肿。囊肿内壁并非由典型的胆管粘膜组成，而是相对无细胞的，可能缺乏典型的粘膜内壁。在囊肿壁上，可見黏液分泌細胞位於壁內腺體中。由于反复破坏和再生導致上皮化生，囊肿壁中也可能存在免疫反應性胃泌素和生长抑素阳性细胞的胃肠粘膜样成分。在年轻患者的囊肿壁中，可以观察到小面积的柱状上皮和胆管。 ${}^{28}$${}^{28}$^(28){ }^{28} 上皮化生的程度通常随着年龄的增长而增加，腺癌的风险也随之增加。 ${}^{1,29-31}$${}^{1,29-31}$^(1,29-31){ }^{1,29-31} 这些组织学改变在胰胆管异常连接中也常见。如前所述，远端胆总管狭窄程度不同，婴儿期常出现高度狭窄。老年患者远端胆总管狭窄但无完全阻塞。

The histologic changes of choledochocele differ from those of the other forms of choledochal cysts that were described earlier. As these ducts are primarily intraduodenal, the cystic lining takes on a duodenal lining rather than biliary. The common bile duct, although normal on gross pathology, reveals inflammatory change on histologic examination. Sludge and stones may also be identified in the common bile duct extending into the extrahepatic and intrahepatic ducts, especially in older patients.
胆总管憩室的组织学变化不同于先前描述的其他类型的胆总管囊肿。由于这些导管主要位于十二指肠内，囊性内膜呈现十二指肠内膜而非胆道内膜。虽然在肉眼病理学上胆总管是正常的，但在组织学检查中显示有炎症改变。尤其在老年患者中，胆总管内也可能存在泥沙和结石，延伸至肝外和肝内胆管。

Liver pathology in a newborn is usually normal or reveals mild proliferation of intrahepatic ducts. Mild periportal fibrosis is often noted on liver histology of older patients. Congenital hepatic fibrosis has also been described in some patients. ${}^{32}$${}^{32}$^(32){ }^{32}
新生儿的肝脏病变通常正常或显示轻微的肝内导管增生。在较年长患者的肝脏组织学检查中，通常可见轻度门周纤维化。一些患者还曾被描述为先天性肝纤维化。 ${}^{32}$${}^{32}$^(32){ }^{32}

Chronic inflammation is the likely cause of carcinoma in the choledochal cyst wall found in older patients. PBM with or without the presence of choledochal cysts increases the risk factor for carcinoma of the biliary tract. ${}^{28,33,34}$${}^{28,33,34}$^(28,33,34){ }^{28,33,34} Adenosquamous carcinoma is the most common type of carcinoma found in the wall, although small-cell carcinoma has also been reported. The choledochal cyst wall is the primary location of carcinomas, but the gallbladder and pancreatic head are other common sites. The location of carcinoma in the head of the pancreas is usually associated with the presence of PBM. ${}^{2,10,35}$${}^{2,10,35}$^(2,10,35){ }^{2,10,35}
慢性炎症可能是老年患者胆总管囊肿壁癌的病因。 伴或不伴有胆总管囊肿的 PBM 会增加胆道癌的风险因素。 ${}^{28,33,34}$${}^{28,33,34}$^(28,33,34){ }^{28,33,34} 腺鳞癌是囊壁最常见的癌类型，但也曾报道过小细胞癌。 胆总管囊肿壁是癌的主要部位，但胆囊和胰头也是常见的部位。 胰头癌通常与 PBM 存在相关。 ${}^{2,10,35}$${}^{2,10,35}$^(2,10,35){ }^{2,10,35}

Choledochal cysts are being diagnosed with increasing frequency on prenatal anatomic ultrasounds in the second and third trimesters. ${}^{36-42}$${}^{36-42}$^(36-42){ }^{36-42} The typical finding on high-resolution ultrasound is that of a cyst in the porta hepatis. The differential diagnosis includes hepatic cysts, duodenal atresia, mesenteric or omental cysts, intestinal duplication, gallbladder duplication, and ovarian cysts. ${}^{43}$${}^{43}$^(43){ }^{43} Because choledochal cysts are rare anomalies, prenatal diagnosis can be challenging. ${}^{44}$${}^{44}$^(44){ }^{44} Having a skilled and experienced sonologist performing the prenatal ultrasound has, in our experience, been critical in increasing both the sensitivity and specificity of prenatal ultrasound for choledochal cysts.
先天性胆总管囊肿在产前解剖超声检查中，尤其是在妊娠的第二和第三个三个月，被诊断的频率越来越高。胎儿解剖超声的高分辨率图像通常会显示肝门处的囊肿。鉴别诊断包括肝囊肿、十二指肠闭锁、肠系膜或网膜囊肿、肠道重复、胆囊重复和卵巢囊肿。先天性胆总管囊肿是罕见的畸形，因此产前诊断可能具有挑战性。根据我们的经验，在进行产前超声检查时有经验的声学家能够提高产前超声对 先天性胆总管囊肿的敏感性和特异性。

Fetal magnetic resonance imaging (MRI) has also been performed to aid in the diagnosis. ${}^{45}$${}^{45}$^(45){ }^{45} Our group has not found that MRI adds significantly to the information found on ultrasound, but as both imaging techniques evolve, they may increase diagnostic yield in a complementary fashion.
胎儿磁共振成像 (MRI) 也已被用于辅助诊断。 ${}^{45}$${}^{45}$^(45){ }^{45} 我们的小组没有发现 MRI 对超声检查发现的信息有显著的补充，但随着两种影像技术的发展，它们可能会以互补的方式提高诊断率。

As discussed in further detail later in this chapter, choledochal cysts historically presented in two broad categories: (1) the infantile form indicated by obstructive jaundice and (2) the adult form generally presenting with obstructive jaundice, pancreatitis, or ascending cholangitis. ${}^{46}$${}^{46}$^(46){ }^{46} Prenatally diagnosed choledochal cysts could fall into either of these categories. The true natural history of choledochal cysts diagnosed prenatally is impossible to accurately delineate due to widespread support for early surgical management to avoid the high risk of potential complications from untreated choledochal cysts. Clearly, those with an infantile form need surgical excision and reconstruction within the first several weeks of life. Many of these neonates may have choledochal cysts in conjunction with complete or nearly complete biliary obstruction. Some categorize these patients as having biliary atresia in association with choledochal cyst, whereas others may term
如本章稍后将更详细讨论的那样，先天性胆总管囊肿在历史上可分为两大类: (1) 婴儿型，表现为阻塞性黄疸; (2) 成人型，通常表现为阻塞性黄疸、胰腺炎或升高性胆管炎。 ${}^{46}$${}^{46}$^(46){ }^{46} 产前诊断的先天性胆总管囊肿可能属于这两类中的任何一类。 由于普遍支持早期手术治疗以避免未治疗先天性胆总管囊肿的潜在并发症的高风险，因此无法准确地描绘产前诊断先天性胆总管囊肿的真正自然史。 显然，婴儿型患者需要在出生后几周内进行手术切除和重建。 许多这些新生儿可能伴有完全或几乎完全的胆道梗阻的先天性胆总管囊肿。 有些学者将这些患者归为伴有先天性胆总管囊肿的胆道闭锁，而有些学者可能将其称为
these patients as having what was historically called surgically correctable biliary atresia. Some overlap between the two diseases is likely.
这些患者被诊断为历史上称为外科可矫正的胆道闭锁。这两种疾病之间可能存在一些重叠。

In those patients with prenatally diagnosed choledochal cysts that are asymptomatic, opinions differ in management. Some have advocated for repair within the first several months of life to avoid potential complications of cholangitis and hepatopathy. ${}^{39,47}$${}^{39,47}$^(39,47){ }^{39,47} These papers compare infants diagnosed prenatally who underwent surgery within the first 6 months of life to children diagnosed later who underwent surgery at an average age of 4 to 6 years. In one series, children in the older group had greater hepatic fibrosis, as well as a higher incidence of preoperative clinical manifestations of choledochal cysts. However, no long-term follow-up was conducted to provide data on long-term liver function and quality of life in order to compare these two different cohorts. In our center, our general recommendation has been to perform laparoscopic operation for asymptomatic choledochal cysts diagnosed prenatally within the first year of life.
对于那些产前诊断为先天性胆总管囊肿的无症状患者，其治疗方案存在分歧。一些学者主张在生命的前几个月内进行修复，以避免胆管炎和肝病等潜在并发症。这些文献将产前诊断并于生命的前 6 个月内接受手术的婴儿与稍后诊断并在平均年龄为 4 至 6 岁时接受手术的儿童进行了比较。在一项系列研究中，年龄较大组的儿童肝纤维化程度更高，术前发生先天性胆总管囊肿临床表现的发生率也更高。然而，没有进行长期随访以提供关于这两种不同队列的长期肝功能和生活质量的数据。在我们中心，我们的一般建议是在生命的第一年内对产前诊断的无症状先天性胆总管囊肿进行腹腔镜手术。

Patients with choledochal cysts can manifest clinical symptoms at any time during their life, with $80\mathrm{\%}$$80\mathrm{\%}$80%80 \% of patients being symptomatic before the age of 10 years. ${}^{48}$${}^{48}$^(48){ }^{48} Abdominal pain, jaundice, and a palpable right upper quadrant abdominal mass is the classic triad described for patients with choledochal cyst. The triad is only reported in about $20\mathrm{\%}$$20\mathrm{\%}$20%20 \% of patients diagnosed. Two of the three symptoms are seen in two thirds of patients at the time of diagnosis. ${}^{48}$${}^{48}$^(48){ }^{48} Some have applied a classification according to age at presentation. The infantile form occurs before 12 months of age, and these patients tend to present with obstructive jaundice, acholic stools, and hepatomegaly similar to biliary atresia. ${}^{49}$${}^{49}$^(49){ }^{49} The adult form occurs anytime after 12 months of age and usually has a greater number of symptoms including fever, nausea, vomiting, and jaundice. ${}^4$${}^4$^(4){ }^{4} As previously mentioned, choledochal cysts diagnosed prenatally may fall into either category.
胆道囊肿患者在任何年龄阶段均可出现临床症状，其中 $80\mathrm{\%}$$80\mathrm{\%}$80%80 \% 的患者在 10 岁之前出现症状。 ${}^{48}$${}^{48}$^(48){ }^{48} 腹部疼痛、黄疸和可触及的右上腹部肿块是胆道囊肿患者的典型三联征，仅在约 $20\mathrm{\%}$$20\mathrm{\%}$20%20 \% 的诊断患者中出现。诊断时，三分之二的患者出现三联征中的两种症状。 ${}^{48}$${}^{48}$^(48){ }^{48} 一些学者根据发病年龄进行了分类。婴儿型发生在 12 个月之前，这些患者往往表现为梗阻性黄疸、陶土样大便和类似胆道闭锁的肝肿大。 ${}^{49}$${}^{49}$^(49){ }^{49} 成人型发生在 12 个月之后，通常包括发热、恶心、呕吐和黄疸等更多症状。 ${}^4$${}^4$^(4){ }^{4} 如前所述，产前诊断的胆道囊肿可能属于这两类中的任何一类。

Signs of hepatic fibrosis may be present in the infantile form, and these patients benefit the most from early treatment. Delayed treatment of asymptomatic infants is advocated by some; however, the risk of progressive hepatic fibrosis exists. In the infantile form, patients with the fusiform type of choledochal cyst tend to present with transient jaundice. Perforation of the choledochal cyst is rare ( $1\mathrm{\%}$$1\mathrm{\%}$1%1 \% to $12\mathrm{\%}$$12\mathrm{\%}$12%12 \% ) and thought to be due to a fragile cystic wall from inflammation, increased ductal pressure, or increased intraabdominal pressure. The site of rupture is at the low-flow region of the junction of the cystic and common bile ducts. These patients present with abdominal pain, sepsis, and peritonitis. ${}^{50}$${}^{50}$^(50){ }^{50}
婴儿型肝纤维化迹象可能存在，这些患者最受益于早期治疗。一些人主张延迟治疗无症状婴儿；然而，进行性肝纤维化的风险依然存在。在婴儿型中，壶腹型胆总管囊肿患者往往表现为一过性黄疸。胆总管囊肿穿孔很少见（ $1\mathrm{\%}$$1\mathrm{\%}$1%1 \% 至 $12\mathrm{\%}$$12\mathrm{\%}$12%12 \% ），据认为是由于炎症、胆管压力升高或腹腔压力升高导致的脆弱囊壁。破裂部位位于胆囊和胆总管汇合处的低流量区域。这些患者表现为腹痛、脓毒症和腹膜炎。 ${}^{50}$${}^{50}$^(50){ }^{50}

The adult form usually presents after 2 years of age. These patients tend to have a fusiform dilation of the common bile duct without complete obstruction of the distal common bile duct and present with intermittent symptoms from a mucous plug or biliary sludge. Patients with the adult form are more likely to present with the classic triad of abdominal pain, jaundice, and a palpable right upper quadrant abdominal mass compared with the infantile form.
成年型通常在 2 岁以后出现。这些患者往往存在胆总管梭形扩张，但远端胆总管没有完全梗阻，表现为粘液栓或胆汁淤积引起的间歇性症状。与婴儿型相比，成年型患者更容易出现腹痛、黄疸和右上腹部可触及肿块的经典三联征。

Symptoms arise from biliary obstruction leading to ascending cholangitis and complications of pancreatitis. ${}^{3,27,46,48,51,52}$${}^{3,27,46,48,51,52}$^(3,27,46,48,51,52){ }^{3,27,46,48,51,52}
症状源于胆道梗阻，导致继发性胆管炎和胰腺炎并发症。 ${}^{3,27,46,48,51,52}$${}^{3,27,46,48,51,52}$^(3,27,46,48,51,52){ }^{3,27,46,48,51,52}

Bile stasis, sludge, stone formation, inflammation, and recurrent superinfection have all been identified as complications leading to intermittent jaundice and abdominal pain in the right upper quadrant. Mucous or protein plugging is likely due to chronic inflammation leading to formation of albuminrich exudates or dysplastic epithelium with hypersecretion of mucin. ${}^{53}$${}^{53}$^(53){ }^{53} Persistent bacterial colonization of intrahepatic ducts in type IV and V cysts is exacerbated by bile stasis, sludge, and stone formation and is one theory for recurrent cholangitis. Secondary biliary cirrhosis is noted in $40\mathrm{\%}$$40\mathrm{\%}$40%40 \% to $50\mathrm{\%}$$50\mathrm{\%}$50%50 \% of patients due to obstruction and inflammation and leads to portal hypertension in some patients. ${}^{48}$${}^{48}$^(48){ }^{48} In cases of choledochal cyst not diagnosed until adulthood, patients may present with cholelithiasis and symptoms mimicking biliary colic or cholecystitis. If imaging has been inadequate, the diagnosis of choledochal cyst may only be made intraoperatively by visual inspection or cholangiography. In some, cholecystectomy may be performed without diagnosis of the choledochal cyst. Pain similar to recurrent pancreatitis has been described by some patients, which has led to a misdiagnosis of abnormal amylase clearance; however, after further work-up, an elevated serum amylase is noted. ${}^{54,55}$${}^{54,55}$^(54,55){ }^{54,55} However, these patients may truly be having pancreatitis as a result of mucous plugging in the PBM.
胆汁淤积、淤泥、结石形成、炎症和反复继发感染都被认为是导致右上腹间歇性黄疸和腹痛的并发症。黏液或蛋白质栓塞可能是由于慢性炎症导致形成富含白蛋白的渗出物或伴有粘液过度分泌的异型上皮。 ${}^{53}$${}^{53}$^(53){ }^{53} IV 和 V 型囊肿中持续存在的细菌定植因胆汁淤积、淤泥和结石形成而加剧，是复发性胆管炎的一种理论。由于梗阻和炎症，次发性胆汁性肝硬化在 $40\mathrm{\%}$$40\mathrm{\%}$40%40 \% 到 $50\mathrm{\%}$$50\mathrm{\%}$50%50 \% 的患者中出现，并导致部分患者门静脉高压。 ${}^{48}$${}^{48}$^(48){ }^{48} 在直到成年才诊断出胆总管囊肿的病例中，患者可能表现为胆石症和类似胆绞痛或胆囊炎的症状。如果影像学检查不足，可能只能通过术中目视检查或胆管造影确诊胆总管囊肿。在某些情况下，可能在未诊断出胆总管囊肿的情况下进行胆囊切除术。 一些患者描述了类似复发性胰腺炎的疼痛，这导致了淀粉酶清除异常的误诊；然而，经过进一步检查，发现血清淀粉酶升高。然而，这些患者实际上可能是由于 PBM 中的黏液栓塞而导致的胰腺炎。

Laboratory data are usually obtained in patients with choledochal cysts in the diagnostic workup but are reserved primarily for evaluating the clinical condition of the patient and not diagnosis. Serum markers of obstructive jaundice (e.g., conjugated hyperbilirubinemia and increased serum alkaline phosphatase) are usually present. An elevated conjugated bilirubin is typical in the infantile form but may or may not be present in the adult form due to intermittent or incomplete biliary obstruction. In chronic cases an abnormal coagulation profile may be evident due to hepatic injury.
实验室数据通常在诊断工作中从胆道囊肿患者身上获取，但主要用于评估患者的临床状况，而非诊断。梗阻性黄疸的指标（例如结合高胆红素血症和血清碱性磷酸酶升高）通常存在。婴儿型通常会出现结合高胆红素血症，但成人型可能出现也可能不出现，因为胆道梗阻是间歇性或不完全的。在慢性病例中，由于肝损伤，可能出现异常的凝血功能检查。

Diagnosis of choledochal cysts is made by imaging studies. An abdominal ultrasound is the first imaging modality used because it is noninvasive, inexpensive, and provides excellent detail of the portal structures. Choledochal cyst on ultrasound is commonly identified as a cystic mass that is not part of the gallbladder in the right upper quadrant and is usually in the porta hepatis. The cyst needs to be in continuity with the biliary tree to be diagnosed as a choledochal cyst because other cystic abnormalities such as pancreatic pseudocyst, biliary cystadenomas, or echinococcal cysts can exist in this area. ${}^{18}$${}^{18}$^(18){ }^{18} The diagnosis of choledochal cyst on ultrasound carries a $71\mathrm{\%}$$71\mathrm{\%}$71%71 \% to $97\mathrm{\%}$$97\mathrm{\%}$97%97 \% sensitivity. ${}^{56}$${}^{56}$^(56){ }^{56} Ultrasonography is an excellent screening tool and is used by some as the only imaging study in infants.
胆道囊肿的诊断通过影像学检查。腹部超声是首选的影像学检查方式，因为它无创、价格低廉，并且对门静脉系统提供极佳的细节。 超声检查显示的胆道囊肿通常是位于右上腹、非胆囊的囊性肿块，通常位于肝门。该囊肿需要与胆道树相连才能被诊断为胆道囊肿，因为该区域可能存在其他囊性异常，例如胰腺假性囊肿、胆管腺瘤或棘球蚴病囊肿。 ${}^{18}$${}^{18}$^(18){ }^{18} 超声检查诊断胆道囊肿的敏感性为 $71\mathrm{\%}$$71\mathrm{\%}$71%71 \% 至 $97\mathrm{\%}$$97\mathrm{\%}$97%97 \% 。 ${}^{56}$${}^{56}$^(56){ }^{56} 超声检查是一种极好的筛查工具，一些人仅在婴儿中使用超声检查作为唯一的影像学检查。

In the event that choledochal cyst is suspected on ultrasound, a technectium-99 HIDA scan may provide more definitive data. Photopenia is initially evident at the cyst followed by filling and delayed emptying. The HIDA scan has varying sensitivities, with 100% for type I cysts and 67% for type IV. ${}^{56}$${}^{56}$^(56){ }^{56}
超声检查疑似胆总管囊肿时，锝 99m-HIDA 扫描可提供更明确的资料。光缺损最初见于囊肿，随后充盈，排空延迟。HIDA 扫描的敏感性不同，I 型囊肿为 100%，IV 型为 67%。 ${}^{56}$${}^{56}$^(56){ }^{56}

A HIDA scan has decreased sensitivity for intrahepatic cysts. A HIDA scan may also be helpful for distinguishing between choledochal cyst and biliary atresia. Biliary atresia on a HIDA scan is characterized by lack of contrast emptying into the duodenum, whereas a choledochal cyst will have contrast entering the duodenum. A HIDA scan may also be useful in the diagnosis of cystic rupture, in which case contrast would empty into the peritoneal cavity. ${}^{48}$${}^{48}$^(48){ }^{48}
肝脏闪烁扫描对肝内囊肿的敏感性降低。肝脏闪烁扫描还有助于鉴别先天性胆管扩张和胆道闭锁。肝脏闪烁扫描显示先天性胆道闭锁的特点是造影剂未排空入十二指肠，而先天性胆管扩张则会有造影剂进入十二指肠。肝脏闪烁扫描还有助于诊断囊肿破裂，在这种情况下，造影剂会排空入腹腔。 ${}^{48}$${}^{48}$^(48){ }^{48}

An abdominal computed tomography (CT) scan may be another useful modality. A CT can show the intrahepatic ducts, distal common bile ducts, and the pancreatic duct, features that are not reliably identified on ultrasound, making CT scan highly useful in identifying type IV and type V cysts. The full anatomy of the biliary tree can be delineated using CT cholangiography, a valuable modality in preoperative planning. The sensitivity of CT cholangiography is $93\mathrm{\%}$$93\mathrm{\%}$93%93 \% for biliary tree visualization and diagnosing lithiasis, $90\mathrm{\%}$$90\mathrm{\%}$90%90 \% for diagnosing choledochal cysts, and only 64% for characterizing the pancreatic duct. ${}^{57}$${}^{57}$^(57){ }^{57} The risk of using CT or CT cholangiography is contrast toxicity that can cause nephrotoxicity or hepatotoxicity and radiation exposure.
腹部计算机断层扫描 (CT) 可能是另一种有用的方式。CT 可以显示肝内胆管、远端胆总管和胰管，这些超声无法可靠识别的特征，这让 CT 扫描在识别 IV 型和 V 型囊肿方面非常有用。使用 CT 胆道造影可以描绘出胆管树的完整解剖结构，这在术前计划中是一种有价值的方式。CT 胆道造影在胆管树可视化和诊断结石方面的灵敏度低于 $93\mathrm{\%}$$93\mathrm{\%}$93%93 \% ，在诊断胆管囊肿方面的灵敏度为 $90\mathrm{\%}$$90\mathrm{\%}$90%90 \% ，在描述胰管方面的灵敏度仅为 64%。 ${}^{57}$${}^{57}$^(57){ }^{57} 使用 CT 或 CT 胆道造影的风险是造影剂毒性，可能会造成肾毒性或肝毒性以及辐射暴露。

Invasive imaging studies also play a role in diagnosis and evaluation of the biliary anatomy. These modalities include cholangiography through endoscopic retrograde cholangiopancreatography (ERCP), percutaneous transhepatic cholangiography (PTC), and intraoperative cholangiogram. Cholangiography will delineate the anatomy and locate abnormalities such as PBM and filling defects caused by stones, stenosis, or carcinomas. ${}^{58,59}$${}^{58,59}$^(58,59){ }^{58,59} The risk of cholangitis and pancreatitis after invasive cholangiography is higher than in the general population, with reported incidences as high as $87.5\mathrm{\%}{}^{48}$$87.5\mathrm{\%}{}^{48}$87.5%^(48)87.5 \%{ }^{48} due to the frequency of PBM, dilated ducts, and dysfunctional sphincters in patients with choledochal cyst. Due to recurrent inflammation and scarring, ERCP can be challenging with respect to cannulization of the ampulla. A high volume of dye load is often required for adequate visualization, especially in the setting of large cysts. ${}^{48}$${}^{48}$^(48){ }^{48}
侵入性影像检查在胆道解剖的诊断和评估中也起着作用。这些方式包括经内镜逆行胰胆管造影 (ERCP)、经皮肝穿刺胆管造影 (PTC) 和术中胆管造影。胆管造影将描绘解剖结构并定位异常，例如 PBM 和由结石、狭窄或癌引起的充盈缺损。 ${}^{58,59}$${}^{58,59}$^(58,59){ }^{58,59} 侵入性胆管造影后发生胆管炎和胰腺炎的风险高于一般人群，据报道发生率高达 $87.5\mathrm{\%}{}^{48}$$87.5\mathrm{\%}{}^{48}$87.5%^(48)87.5 \%{ }^{48} ，这是因为胆道囊肿患者存在 PBM、扩张的导管和功能障碍的括约肌的频率较高。由于反复的炎症和瘢痕形成，ERCP 在壶腹的插管方面可能具有挑战性。为了充分显影，通常需要大量造影剂，尤其是在大型囊肿的情况下。 ${}^{48}$${}^{48}$^(48){ }^{48}

Magnetic resonance cholangiopancreatography (MRCP) is now considered the gold standard for imaging choledochal cyst, especially given the complications and concerns with invasive cholangiography (Fig. 106-3). The diagnostic sensitivity of MRCP is $90\mathrm{\%}$$90\mathrm{\%}$90%90 \% to $100\mathrm{\%}.{}^{60}$$100\mathrm{\%}.{}^{60}$100%.^(60)100 \% .{ }^{60} Administration of secretin increases pancreatic secretion and dilates the pancreatic duct. Thus some centers administer secretin before MRCP to increase diagnostic yield. ${}^{61}$${}^{61}$^(61){ }^{61} As the technology for MRCP has improved, smaller ductal structures are being visualized with finer detail. This has allowed for increased diagnosis of PBM and pancreatic divisum and usually gives accurate information as to the location of insertion of the pancreatic duct. The type and extent of the choledochal cyst are well visualized, and images can be reconstructed in three dimensions (Fig.106-4). MRCP avoids radiation associated with CT scan and ERCP and avoids the potential post-ERCP complications of cholangitis and pancreatitis.
磁共振胰胆管造影 (MRCP) 目前被认为是胆总管囊肿影像检查的金标准，尤其是考虑到侵入性胆道造影的并发症和安全性问题（图 106-3）。MRCP 的诊断灵敏度为 $90\mathrm{\%}$$90\mathrm{\%}$90%90 \% 至 $100\mathrm{\%}.{}^{60}$$100\mathrm{\%}.{}^{60}$100%.^(60)100 \% .{ }^{60} 。给予促胰液素可增加胰腺分泌并扩张胰管。因此，一些中心在 MRCP 前给予促胰液素以提高诊断率。 ${}^{61}$${}^{61}$^(61){ }^{61} 随着 MRCP 技术的进步，更小的导管结构得以更精细地显示。这使得 PBM 和胰腺分隔的诊断率提高，通常可以准确显示胰管的插入位置。胆总管囊肿的类型和范围可清晰显示，图像可进行三维重建（图 106-4）。MRCP 避免了 CT 扫描和 ERCP 相关的辐射，也避免了 ERCP 后可能的胆管炎和胰腺炎并发症。

Choledochoceles (type III cysts) can be diagnosed with various imaging modalities such as upper gastrointestinal series (UGIS), endoscopy, ERCP, MRCP, and CT cholangiography. On UGIS for choledochoceles, a filling defect is noted at the level of the cyst entering the duodenum. The papilla will have a smooth bulging on endoscopy and ERCP. The advantage of ERCP over MRCP and CT cholangiography for choledochoceles is the ability to perform a therapeutic procedure such
食管十二指肠造影 (UGIS)、内镜、ERCP、MRCP 和 CT 胆管造影等多种影像学方式均可诊断胆道囊肿（III 型囊肿）。UGIS 检查胆道囊肿时，可在囊肿进入十二指肠处发现充盈缺损。内镜和 ERCP 检查时，乳头会呈现平滑的膨出。与 MRCP 和 CT 胆管造影相比，ERCP 用于诊断胆道囊肿的优势在于可以进行治疗性操作，例如

FIGURE 106-3 A magnetic resonance cholangiopancreatography of a patient with type I choledochal cyst and an accessory pancreatic duct entering the duodenum.
图 106-3 一位 I 型胆总管囊肿患者的磁共振胰胆管造影，伴有进入十二指肠的副胰管。

FIGURE 106-4 Three-dimensional reconstruction of a magnetic resonance cholangiopancreatography image of a choledochal cyst with mild dilation ( 6 mm ) of the right and left extrahepatic ducts. Pancreatic duct is seen entering the normal caliber distal common bile duct.
图 106-4 磁共振胰胆管造影图像的三维重建，显示胆总管囊肿，右、左肝外胆管轻度扩张（6 毫米）。可见胰管进入正常口径的远端胆总管。
as a sphincterotomy. Endoscopic ultrasound is also wellsuited to diagnosing choledochoceles because this modality can reach close to the cyst for appropriate visualization. The best imaging techniques for Caroli disease (type V cysts) are ultrasound, CT, and MRI. ${}^{48}$${}^{48}$^(48){ }^{48} The “central dot sign” has been described for Caroli disease but is not pathognomic. ${}^{62}$${}^{62}$^(62){ }^{62} On HIDA scan a beaded appearance due to intraductal bridging may be indicative of Caroli disease.
作为括约肌切开术。内镜超声也非常适合诊断胆总管囊肿，因为这种方式可以靠近囊肿进行适当的可视化。Caroli 病（V 型囊肿）最佳的影像学检查方法是超声、CT 和 MRI。 ${}^{48}$${}^{48}$^(48){ }^{48} Caroli 病的“中心点征”已被描述，但并非特异性。 ${}^{62}$${}^{62}$^(62){ }^{62} 在 HIDA 扫描中，由于导管内桥接导致的串珠样外观可能提示 Caroli 病。

Our preference for imaging for all types of choledochal cysts is ultrasound followed by MRCP. If the MRCP does not give sufficient anatomic detail to guide therapy, we perform either an ERCP before operation or an intraoperative cholangiogram at the time of cyst resection.
首选所有类型的胆总管囊肿影像检查为超声，其次为 MRCP。如果 MRCP 提供的解剖细节不足以指导治疗，我们会在手术前进行 ERCP 或在囊肿切除时进行术中胆道造影。

The management of types I and IV choledochal cysts has evolved from either internal or external drainage to cyst excision and hepaticojejunostomy to a Roux-en-Y limb (Fig. 106-5). Due to the relatively complex nature of cyst excision, early attempts at treatment focused on external drainage to avoid complications of biliary tract obstruction. However, an external biliary fistula proved to have high morbidity and mortality. ${}^4$${}^4$^(4){ }^{4} As surgical techniques advanced, subsequent attempts focused on cyst duodenostomy or cyst jejunostomy to a Roux-en-Y limb to internalize biliary drainage. ${}^{15,27,63,64}$${}^{15,27,63,64}$^(15,27,63,64){ }^{15,27,63,64} However, complications of cholangitis and progressive liver damage and the high risk of cancer in the choledochal cyst led to the current surgical strategy of cyst excision with Roux-en-Y jejunostomy as advocated by Kasai and colleagues ${}^{65}$${}^{65}$^(65){ }^{65} and Ishida and colleagues. ${}^{66-68}$${}^{66-68}$^(66-68){ }^{66-68} This operative approach eliminates the potentially premalignant epithelial cyst lining and also separates the pancreatic drainage from the biliary drainage. This strategy is summarized in Figure 106-6. This technique is essentially identical to our laparoscopic technique for treatment for type I and type IV cysts and is described in detail later in the laparoscopic section. Some surgeons prefer cyst resection with hepaticoduodenostomy, which yields similar outcomes to that of drainage with a Roux-en-Y limb. ${}^{69}$${}^{69}$^(69){ }^{69}
随着外科技术的进步，随后的尝试集中在将胆囊十二指肠造口术或胆囊空肠造口术改为 Roux-en-Y 肢体来将胆汁引流内化。 ${}^{15,27,63,64}$${}^{15,27,63,64}$^(15,27,63,64){ }^{15,27,63,64} 然而，胆管炎、进行性肝损伤和胆管囊肿高风险的并发症导致了目前开刀切除胆囊并进行 Roux-en-Y 空肠造口的策略，如 Kasai 及其同事 ${}^{65}$${}^{65}$^(65){ }^{65} 和 Ishida 及其同事 ${}^{66-68}$${}^{66-68}$^(66-68){ }^{66-68} 所提倡的。这种手术方法消除了潜在的癌前上皮囊肿内壁，并将胰腺引流与胆汁引流分开。该策略总结于图 106-6。 该技术与我们腹腔镜治疗 I 型和 IV 型囊肿的技术基本相同，详细信息将在后面的腹腔镜部分中介绍。一些外科医生更喜欢肝空肠吻合术切除囊肿，这与 Roux-en-Y 吻合术引流的结果相似。 ${}^{69}$${}^{69}$^(69){ }^{69}

In previous series of choledochal cysts, diagnosis was often delayed, leading to patient presentation with repeated episodes of cholangitis. Delayed diagnosis led to inflammation of the cyst and adherence to the periportal structures, particularly the portal vein. In patients such as these, for whom excision of the full thickness of the cyst may carry an unacceptably high complication rate, the technique described by Lilly can be performed ${}^{70,71}$${}^{70,71}$^(70,71){ }^{70,71} : The cyst can be incised anteriorly away from the hepatic artery and portal vein, allowing the inner epithelial lining of the entire choledochal cyst to be excised while avoiding the difficult and potentially dangerous plane of dissection between the outer cyst wall and the portal vein. This removes the potentially premalignant aspects of the cyst while minimizing potential operative complications.
此前的一系列胆管囊肿病例中，诊断常常延误，导致患者反复出现胆管炎。诊断延误会导致囊肿发炎并粘连到门静脉周围结构，特别是门静脉。对于这类患者，切除囊肿的全层可能会带来不可接受的高并发症率，利利描述的技术可以进行 ${}^{70,71}$${}^{70,71}$^(70,71){ }^{70,71} ：可以在远离肝动脉和门静脉的前方切开囊肿，从而切除整个胆管囊肿的内上皮衬里，同时避免在囊肿外壁和门静脉之间进行困难且可能危险的解剖平面。这可以去除囊肿的潜在癌前病变，同时最大限度地减少潜在的手术并发症。

Type II choledochal cysts are rare but appear to have a low malignant potential. This type requires simple cyst resection and is easily amenable to a laparoscopic approach because complicated dissection and reconstruction are not required. In fact, the first laparoscopic treatment for a choledochal cyst in the pediatric population was reported in a child with a type II cyst. ${}^{72}$${}^{72}$^(72){ }^{72}
II 型胆总管囊肿较为罕见，但似乎具有较低的恶性潜能。这种类型的囊肿只需要进行简单的囊肿切除术，且由于不需要进行复杂的解剖和重建，因此很容易采用腹腔镜的方法进行治疗。事实上，在小儿人群中，针对 II 型胆总管囊肿进行腹腔镜治疗的首例报道是一名患有 II 型囊肿的儿童。

Type III choledochal cysts, or choledochoceles, are intraduodenal or intrapancreatic dilations of the distal common bile duct, as outlined previously. Management has traditionally been operative marsupialization of the cyst, usually through a transduodenal approach. ${}^{73}$${}^{73}$^(73){ }^{73} Increasingly, however, choledochoceles are being treated by sphincterotomy or cyst marsupialization during an ERCP. ${}^{74-76}$${}^{74-76}$^(74-76){ }^{74-76} The incidence of cancer is unclear due to the rarity of this type of cyst but is likely significantly elevated. ${}^{77}$${}^{77}$^(77){ }^{77} It is unclear to what extent drainage with either an operative approach or via ERCP minimizes the malignant potential for choledochoceles.
Ⅲ型胆总管囊肿，或胆总管憩室，是指远端胆总管的十二指肠内或胰内扩张，如前所述。传统上，其治疗方法一直是通过经十二指肠入路进行手术缝合术。 ${}^{73}$${}^{73}$^(73){ }^{73} 然而，越来越多的胆总管憩室正通过 ERCP 期间的括约肌切开术或囊肿缝合术治疗。 ${}^{74-76}$${}^{74-76}$^(74-76){ }^{74-76} 由于这种类型的囊肿较为罕见，因此其致癌率尚不明确，但很可能显著升高。 ${}^{77}$${}^{77}$^(77){ }^{77} 目前尚不清楚以手术方式或经 ERCP 引流在多大程度上可以最大程度地降低胆总管憩室的恶性潜能。

FIGURE 106-5 Surgical operative management of choledochal cyst and the types of anastomoses that can be created.
图 106-5 胆总管囊肿的外科手术治疗和可建立的吻合类型。

FIGURE 106-6 Diagram reveals preoperative anatomy $(\mathbf{A})$$(\mathbf{A})$(A)(\mathbf{A}) and postoperative anatomy (B) after cyst excision and Roux-en-Y reconstruction.
그림 106-6 囊肿切除和 Roux-en-Y 重建术后术后解剖图 (B)。

For patients with type IV disease, most surgeons recommend cyst resection to the hepatic ducts, leaving in place the dilated intrahepatic ducts because they may decrease in size without distal obstruction. However, careful long-term follow-up is required to monitor the relative size of the intrahepatic ducts and for the possibility of symptomatic lithiasis and cholangitis. Surgical treatment of type V (Caroli disease) is challenging. Segmental resections can be performed if the disease is localized to a portion of the liver. Liver transplantation has also been performed. ${}^{78,79}$${}^{78,79}$^(78,79){ }^{78,79}
对于 IV 型疾病患者，大多数外科医生建议对肝管进行囊肿切除，保留扩张的肝内胆管，因为它们在没有远端梗阻的情况下可能会缩小。但是，需要仔细进行长期随访，以监测肝内胆管的相对大小，以及有症状的结石和胆管炎的可能性。V 型（Caroli 病）的手术治疗具有挑战性。如果疾病局限于肝脏的一部分，可以进行分段切除。也已经进行了肝移植。 ${}^{78,79}$${}^{78,79}$^(78,79){ }^{78,79}

We and others have reported laparoscopic excision of choledochal cysts with either Roux-en-Y reconstruction or hepatico-duodenostomy. ${}^{80-83}$${}^{80-83}$^(80-83){ }^{80-83} The largest series, from Tang and colleagues, ${}^{83}$${}^{83}$^(83){ }^{83} was a retrospective review of 62 children undergoing laparoscopic resection of choledochal cysts. They reported outcomes comparable with those of published open series with a low rate of complications including only one case of a biliary leak. Long-term outcome for laparoscopic surgery for choledochal cyst in the pediatric population has not been well-reported, and data are similarly scarce for outcomes of open surgery. A recent series from the long-term follow-up data reported by Ono and colleagues is discussed in “Outcome and Complications” later and provides the best overall longterm data from open cases. ${}^{84}$${}^{84}$^(84){ }^{84}
我们和其他研究者已经根据文献报告了胆总管囊肿腹腔镜切除术，伴有 Roux-en-Y 再建或肝十二指肠吻合术。来自唐及同事的 ${}^{83}$${}^{83}$^(83){ }^{83} 最大病列是 62 名接受胆总管囊肿腹腔镜切除术的儿童的回顾性研究。 他们报道的结果与已发布的开放式病例系列结果相当，并发症发生率低，包括只有一例胆漏。 儿童人群胆总管囊肿腹腔镜手术的远期结果尚未得到充分的报道，而开放手术结果的数据同样匮乏。 后面“结果和并发症” 中将讨论 Ono 及其同事报告的长期随访数据最近的一系列数据，该数据提供了开放病例最佳的总体远期数据。 ${}^{84}$${}^{84}$^(84){ }^{84}

Techniques for laparoscopic repair including ours are all similar. This next section describes in detail our technique for laparoscopic excision of type I choledochal cysts with Roux-en-Y reconstruction, as previously reported. ${}^{81}$${}^{81}$^(81){ }^{81}
腹腔镜修复技术，包括我们的技术，都十分相似。下一节将详细描述我们之前报道的用于 I 型胆总管囊肿腹腔镜切除术的 Roux-en-Y 改道技术，如 ${}^{81}$${}^{81}$^(81){ }^{81} 所示。

Detailed preoperative workup including an MRCP is essential in determining the type of cyst and the insertion of the pancreatic duct(s) for safe resection. Particularly in instances in which the pancreatic duct inserts near the distal extent of the choledochal cyst, care must be taken to avoid the pancreatic duct in the distal dissection. Intraoperative cholangiography may also be performed by placing a catheter in the gallbladder if MRCP is inadequate or not available. The placement of four ports for the procedure is illustrated in Figure 106-7. A $5-\mathrm{mm}$$5-\mathrm{mm}$5-mm5-\mathrm{mm} port is placed through the umbilicus for the camera. Typically, we prefer a $4-\mathrm{mm}$$4-\mathrm{mm}$4-mm4-\mathrm{mm} or $5-\mathrm{mm}$$5-\mathrm{mm}$5-mm5-\mathrm{mm} 30-degree endoscope due to the improved optics as compared with smaller endoscopes. A 3-mm port is placed in the right abdomen between the midaxillary line and the midclavicular line just inferior to the level of the umbilicus. This is the
切除囊肿和安全分流胰管，术前详细评估（包括 MRCP）至关重要。尤其是当胰管开口接近胆总管囊肿末端时，在远端解剖中必须注意避免损伤胰管。如果 MRCP 不充分或不可行，也可以通过在胆囊中放置导管进行术中胆道造影。图 106-7 展示了该手术的四个端口放置方式。将一个 $5-\mathrm{mm}$$5-\mathrm{mm}$5-mm5-\mathrm{mm} 端口置于脐部用于放置摄像头。通常，我们更喜欢 $4-\mathrm{mm}$$4-\mathrm{mm}$4-mm4-\mathrm{mm} 或 $5-\mathrm{mm}$$5-\mathrm{mm}$5-mm5-\mathrm{mm} 的 30 度内镜，因为与更小的内镜相比，它们的图像质量更好。在中腋线和锁骨中线之间的右侧腹部，将一个 3 毫米的端口置于略低于脐部的高度。这是一个

FIGURE 106-7 Port placements for laparoscopic choledochal cyst excision and Roux-en-Y hepaticojejunostomy.
图 106-7 腹腔镜胆总管囊肿切除术和 Roux-en-Y 肝空肠吻合术的端口放置。
left-handed working port for the primary surgeon. A 5-mm port is placed in the left midclavicular line just superior to the level of the umbilicus for the right-handed working port for the primary surgeon. The fourth port is located in the left anterior axillary line just below the costal margin. This port is used by the assistant to retract the gallbladder and liver superiorly. The location of this port, although it may seem counterintuitive, has proven to be the optimal location for retraction and affords adequate distance from the other instruments. Placing two ports in the right abdomen, as one would typically do for a cholecystectomy, results in inadequate distance between instruments placed through these ports. After laparoscopically confirming the diagnosis of a choledochal cyst, a cholangiogram is performed, if necessary, by placing an angiocatheter into the gallbladder and installing water soluble contrast (Fig. 106-8).
左旋助手工作孔用于主刀医生。对于主刀医生的右手工作孔而言，在脐水平线上方，左侧胸骨中线处放置一个 5 毫米的端口。第四个端口位于左前腋线，靠近肋缘下方。此端口由助手用于向上牵拉胆囊和肝脏。虽然似乎违反直觉，但该端口的位置已被证明是牵拉的最佳位置，并与其他器械保持足够的距离。在右腹部放置两个端口，如胆囊切除术通常所做的那样，会导致通过这些端口放置的器械之间的距离不足。腹腔镜确诊胆总管囊肿后，可通过将血管导管插入胆囊并注入水溶性造影剂来进行胆道造影（图 106-8）。

The gallbladder is then divided at the cystic duct junction with clips or ties (Fig. 106-9). This allows the assistant to retract the gallbladder superiorly through the left subcostal port and expose the porta hepatis. If the cyst is large enough that the hepatic hilum is obscured, it may need to be decompressed. Next, the hepatic duct is divided just superior to the
胆囊在胆囊管与胆囊管汇合处用夹子或结扎线切开（图 106-9）。这使助手能够通过左侧肋下切口向上牵拉胆囊，暴露肝门部。如果囊肿大到遮挡肝门，可能需要进行减压。接下来，肝总管在

FIGURE 106-8 Intraoperative cholangiogram during laparoscopic cyst excision and Roux-en-Y hepaticojejunostomy revealing the gallbladder $(GB)$$(GB)$(GB)(G B) and the choledochal cyst (CY).
图 106-8 腹腔镜囊肿切除和 Roux-en-Y 肝空肠吻合术期间的术中胆管造影，显示胆囊 $(GB)$$(GB)$(GB)(G B) 和胆总管囊肿 (CY)。

FIGURE 106-9 Intraoperative laparoscopic view of the gallbladder (GB) and cystic duct in relation to the choledochal cyst.
图 106-9 腹腔镜下胆囊 (GB) 和胆囊管与胆总管囊肿的关系。
most cephalic extent of the choledochal cyst. Care should be taken to identify the right and left branches. In some instances, separate anastomoses may be required from the right and left ducts to the Roux-en-Y limb. Dividing the hepatic duct early in the operation allows the surgeon to manipulate both the superior and right lateral aspects of the choledochal cyst to aid in the dissection. The cyst is then carefully dissected away from the hepatic artery and the portal vein. At this point, the cyst is attached only to the distal common bile duct within the pancreas. Dissection continues distally, dissecting closely on the cyst wall to avoid damage to the duodenum or pancreas until the cyst begins tapering in size to become a normal-sized common bile duct. Again, the MRCP can be valuable in guiding the distal dissection with respect to the location of the
最头侧的胆总管囊肿，需要注意识别左右分支。在某些情况下，可能需要从右肝管和左肝管到 Roux-en-Y 肢体分别进行吻合。手术早期切开肝管可以让外科医生更好地操作胆总管囊肿的上下和右侧，以帮助进行分离。然后，小心地将囊肿从肝动脉和门静脉分离。在这一点上，囊肿仅附着于胰腺内的远端总胆管。继续向下分离，紧贴囊肿壁进行，以避免损伤十二指肠或胰腺，直到囊肿开始呈锥形缩小，变成正常大小的总胆管。同样，MRCP 有助于指导远端分离，以了解以下结构的位置：
pancreatic duct insertion. The common bile duct is excised, and the distal common bile duct is ligated with a tie or occluded with a clip. If the cyst is not leaking bile, we prefer to place the cyst on the lateral aspect of the liver and remove it subsequently along with the gallbladder.
胰腺导管插入。切除胆总管，用结扎线结扎或用夹子闭塞远端胆总管。如果囊肿没有渗漏胆汁，我们倾向于将囊肿置于肝脏外侧，随后与胆囊一起切除。

We then begin the creation of the Roux-en-Y limb. The ligament of Treitz is identified by elevating the transverse colon. The area of the jejunum that will be divided for the Roux-en-Y limb is identified approximately 15 cm from the ligament. We prefer to mark this area by placing vessel loops of different colors separated by 1 to 2 cm . Marking the jejunum in this manner allows easy identification of the proximal and distal aspects of the jejunum when the bowel is exteriorized. Next, the umbilical incision is extended to approximately 1.5 cm and the marked jejunum is exteriorized. A 30- to 40-cm Roux-en-Y limb is then created in the standard fashion, the bowel is then returned intracorporeally, and the umbilical port is replaced. The Roux-en-Y limb is brought to the porta hepatic in a retrocolic fashion, and then an end-to-side anastomosis is performed from the hepatic duct to the Roux-en-Y limb. We use fine absorbable monofilament suture material. We have found that using a continuous suture on the posterior aspect of the anastomosis and interrupted sutures on the anterior and lateral aspects is simplest. All knots are tied on the outside of the anastomosis to prevent the potential for a focus of formation or collection of biliary sludge on the knots. Finally, a cholecystectomy is performed and the gallbladder and the choledochal cyst are removed through the umbilical port site.
然后我们开始创建 Roux-en-Y 肢。通过抬起横结肠确定 Treitz 韧带。Roux-en-Y 肢将被切开的空肠区域在韧带远端约 15 厘米处识别。我们更喜欢通过放置间隔 1 到 2 厘米的不同颜色的血管环来标记该区域。以这种方式标记空肠可以轻松识别肠道外翻时空肠的近端和远端。接下来，将脐切口延长至约 1.5 厘米，并将标记的空肠外翻。然后以标准方式创建 30 至 40 厘米的 Roux-en-Y 肢，然后将肠道送回腹腔内，并更换脐孔。Roux-en-Y 肢以乙状结肠后方式带到肝门，然后从肝管到 Roux-en-Y 肢进行端侧吻合。我们使用细的可吸收单丝缝合线材料。我们发现，在吻合的后面使用连续缝合线，在前面和侧面使用间断缝合线是最简单的。 所有结扎线均结扎于吻合口外侧，以防止结扎线处形成胆泥沉积或聚集。最后，切除胆囊并通过脐部切口取出胆囊和胆总管囊肿。

In past operative treatment of cystenterostomy, there was a high rate of complications such as anastomotic stricture, recurrent ascending cholangitis, bowel obstruction, portal hypertension, and malignancy. However, the advancement of surgical treatment to include cyst excision resulted in minimal morbidity and mortality and reduced the number of late complications. The most common late complication continues to be anastomotic stricture. Early diagnosis and cyst excision results in low complication rates in most experienced centers. The type of internal drainage after cyst excision has not been shown to affect postoperative complications. The technique of Roux-en-Y hepaticojejunostomy is favored by most, although comparable results can be achieved by hepaticoduodenostomy. Either procedure can be performed laparoscopically as well.
过去经手术治疗的肠吻合术存在并发症发生率较高的问题，例如吻合口狭窄、复发性升结性胆管炎、肠梗阻、门静脉高压和恶性肿瘤。然而，手术治疗的进步包括囊肿切除术导致了最小的发病率和死亡率，并减少了晚期并发症的数量。最常见的后发症仍然是吻合口狭窄。早期诊断和囊肿切除术在大多数经验丰富的中心会导致并发症发生率低。囊肿切除术后的内引流类型尚未显示出影响术后并发症。大多数人喜欢 Roux-en-Y 肝空肠吻合术，尽管肝十二指肠吻合术也能获得可比的结果。两种手术都可以腹腔镜下进行。

Although late complications are reduced with current surgical management, studies suggest that long-term follow-up to 17 years or beyond is indicated due to the potential for problems such as anastomotic stricture, cholangitis, intrahepatic stone formation, and malignancy. ${}^{85}$${}^{85}$^(85){ }^{85} This is particularly important for patients with type IV disease because malignancy can occur in incompletely resected cystic hepatic ducts or recurrent cysts. ${}^{85}$${}^{85}$^(85){ }^{85} The risk of malignancy is greatly reduced after cyst excision but is still elevated as compared with the general population. Intrahepatic stone formation in the intraoperative setting has been evaluated and reported in the literature and seen in patients who showed no stone formation in the preoperative course. Stone formation has been reported to occur
尽管目前的外科手术治疗减少了晚期并发症，但研究表明，由于可能出现吻合口狭窄、胆管炎、肝内结石形成和恶性肿瘤等问题，因此需要进行长达 17 年或更长时间的长期随访。 ${}^{85}$${}^{85}$^(85){ }^{85} 这对于 IV 型疾病患者尤其重要，因为恶性肿瘤可能发生在切除不完全的囊性肝管或复发性囊肿中。 ${}^{85}$${}^{85}$^(85){ }^{85} 虽然囊肿切除术后恶性肿瘤的风险大大降低，但与普通人群相比，仍然较高。 术中肝内结石形成已在文献中进行了评估和报道，并见于术前无结石形成的患者。据报道，结石形成
anywhere from 3 to 22 years postoperatively. ${}^{86}$${}^{86}$^(86){ }^{86} However, if the duct is patent and there is no stenosis of the hepaticojejunostomy, stones are likely to pass spontaneously. Intrahepatic stones usually present in cases of stenosis that initially cause bile stasis and lead to stone formation. ${}^{85}$${}^{85}$^(85){ }^{85} Todani and colleagues ${}^{87}$${}^{87}$^(87){ }^{87} reported a 25 -year review with the identification of biliary complications primarily associated with either anastomotic stricture or primary ductal stricture and recommended a wide hepatic hilum anastomosis to prevent biliary complications.
术后 3 至 22 年的任何时间。 ${}^{86}$${}^{86}$^(86){ }^{86} 但是，如果管道通畅，肝空肠吻合口无狭窄，则结石很可能会自行排出。肝内结石通常发生于狭窄的情况下，狭窄最初会导致胆汁淤积并导致结石形成。 ${}^{85}$${}^{85}$^(85){ }^{85} Todani 和同事 ${}^{87}$${}^{87}$^(87){ }^{87} 进行了一项为期 25 年的回顾研究，确定了主要与吻合口狭窄或原发性胆管狭窄相关的胆道并发症，并推荐进行广泛的肝门吻合术以预防胆道并发症。

The long-term complication of malignancy can be avoided with cyst excision. In addition, any cause of chronic inflammation and stasis, as seen with PBM, has to be treated appropriately. ${}^{34}$${}^{34}$^(34){ }^{34} The incidence of malignancy of the gallbladder and bile ducts remains high in patients with PBM compared with the general population and occurs at a younger age. Further studies reviewing the genetic changes of PBM have revealed KRAS gene mutations early in epithelial hyperplasia and metaplasia. In the late progression of adenocarcinoma of the biliary tract, inactivation of DPC4 gene occurs. ${}^{28,44,88}$${}^{28,44,88}$^(28,44,88){ }^{28,44,88}
恶性肿瘤的长期并发症可以通过囊肿切除来避免。此外，任何慢性炎症和瘀滞的病因，如 PBM 所示，都必须得到适当的治疗。 ${}^{34}$${}^{34}$^(34){ }^{34} 与普通人群相比，PBM 患者的胆囊和胆管恶性肿瘤发病率仍然很高，而且发生在更年轻的年龄。进一步研究 PBM 的基因变化发现，上皮增生和化生早期存在 KRAS 基因突变。在胆道腺癌的晚期进展中，DPC4 基因失活。 ${}^{28,44,88}$${}^{28,44,88}$^(28,44,88){ }^{28,44,88}

The management of choledochal cysts is performed predominantly by pediatric surgeons. Generally considered a congenital abnormality, most cases are diagnosed in infancy with many now being diagnosed prenatally, though presentation may be delayed until adulthood. Because of the high risk of serious sequelae including malignancy, early surgical excision is warranted even in asymptomatic patients. Adequate imaging with MRCP helps map the extent of the dilated biliary tree, as well as the insertion of the pancreatic duct(s), ensuring safe resection. Increasingly, surgery is being performed with minimally invasive techniques with good outcomes. Because of the rare nature of this disease, extensive knowledge of the different variants and experience with advanced biliary tract surgery is critical in attaining good outcomes.
胆总管囊肿的治疗主要由小儿外科医生进行。通常被认为是一种先天性异常，大多数病例在婴儿期被诊断出来，现在许多病例在产前就被诊断出来，尽管症状可能会延迟到成年。由于包括恶性肿瘤在内的严重并发症的高风险，即使在无症状患者中也需要进行早期手术切除。使用 MRCP 进行充分的成像有助于绘制扩张的胆管树的范围以及胰管的插入，确保安全切除。手术越来越多地采用微创技术进行，取得了良好效果。由于这种疾病的罕见性，对不同变异的广泛了解和先进胆道手术的经验对于获得良好的结果至关重要。

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