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 4:14: 1 ) that contributes to the belief that choledochal cyst is sex-linked. ^(1-3){ }^{1-3} Familial cases have been recognized, but no genetic basis has been evident. The majority ( ~~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 4:14: 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} 已发现家族性病例，但尚未发现遗传基础。大多数（ ~~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%25 \% of cases are diagnosed prenatally. Prenatal diagnosis of choledochal cysts is increasing in frequency, ^(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%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} 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} 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} 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} 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} 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} 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} 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} 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} PBM has a reported 3%3 \% incidence. ^(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%20 \% to 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} 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} 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} 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} 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}20%20 \%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} 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} Caroli disease has been associated with polycystic kidney disease. ^(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。
Embryology 胚胎学
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} 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} 胰腺胆管系统或移行处的终止，正如曾经认为的那样。 ^(24){ }^{24}
Anatomic Classification 解剖分类
The original anatomic classification of choledochal cysts included types I, II, and III. ^(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} Type I is by far the most common, accounting for 90%90 \% to 95%95 \% of cases, ^(4){ }^{4} and constitutes the cystic/saccular or fusiform dilation of the common bile duct. ^(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} 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} I 型是最常见的，约占 90%90 \% 到 95%95 \% 的病例， ^(4){ }^{4} 构成胆总管的囊状/囊状扩张或梭形扩张。 ^(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。
Pathology 病理学
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} The degree of epithelial metaplasia generally increases with age, as does the risk for adenocarcinoma. ^(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} 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} 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} 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}
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} 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} 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} 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}
Prenatal Diagnosis 产前诊断
Choledochal cysts are being diagnosed with increasing frequency on prenatal anatomic ultrasounds in the second and third trimesters. ^(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} Because choledochal cysts are rare anomalies, prenatal diagnosis can be challenging. ^(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} 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} 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} 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} 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} 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。
Clinical Presentation 临床表现
Patients with choledochal cysts can manifest clinical symptoms at any time during their life, with 80%80 \% of patients being symptomatic before the age of 10 years. ^(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%20 \% of patients diagnosed. Two of the three symptoms are seen in two thirds of patients at the time of diagnosis. ^(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.
