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Choledochal cyst is a congenital or acquired anomaly affecting the biliary tree in which exists a dilatation of the bile duct, not only the choledochus is affected but also the intrahepatic and extrahepatic ducts might be affected. The clinical presentation is not specific, even the classic triad with abdominal pain, mass, and jaundice is not common as suspected, found only in 10% of cases. Clinicians must rely on imaging studies for diagnosis and classification. The treatment is cyst excision with hepaticoenterostomy in most of the cases, but in some others, a liver transplant would be necessary. These patients require lifelong follow-up due to its rate of recurrence compared with general population.
Pediatric Surgery Department, Hospital de Pediatría CMNO IMSS, Guadalajara, México
Mitzi Becerra
Pediatric Surgery Department, Hospital de Pediatría CMNO IMSS, Guadalajara, México
Beatriz Calderón
Pediatric Surgery Department, Hospital de Pediatría CMNO IMSS, Guadalajara, México
Iván Salinas
Pediatric Surgery Department, Hospital de Pediatría CMNO IMSS, Guadalajara, México
María Ruiz
Pediatric Surgery Department, Hospital de Pediatría CMNO IMSS, Guadalajara, México
Jorge Ventura
Pediatric Surgery Department, Hospital de Pediatría CMNO IMSS, Guadalajara, México
*Address all correspondence to: magtorresa@gmail.com
1. Introduction
Choledochal cyst (CC) is an entity where there is a dilatation at any level of the bile duct, more common in the choledochus, hence its name. Although in almost all the literature, it is referred to as a “choledochal cyst,” the most appropriate way to refer to this pathology would be dilation of the bile duct, since it does not necessarily present as a cyst, and it is not necessary to appear in the choledochus either.
Therefore, to refer to a dilated duct, it is necessary to know the normal diameter of the common bile duct (choledochus). A study by Pina and colleagues reports an average of 5–6 mm in diameter of the common bile duct in adult patients; however the diameter varies according to age and measurement method (Table 1) [1, 2]. Another study where measurements were made on 173 children aged between 1 day and 13 years reported an average diameter of the common bile duct of 1.27 mm (± 3.3 mm) and < 1.2 mm in newborn and children up to 3 months [3]. Consequently, any measurement greater than reported can be considered abnormal.
Age (years)
Range (mm)
≤ 4
2–4
4–6
2–4
6–10
2–6
10–12
3–6
12–14
3–7
Adapted from Witcombe JB, Cremin BJ. The width of the common bile duct in childhood. Pediatr Radiol. 1978;7:147–149.
Table 1.
Mean common bile duct diameter and range according to patient age.
Diverse classifications have been proposed to categorize the CC. Currently, Todani’s classification has been the most extensively accepted (Figure 1). It was described in 1977, based on evidence of the existence of various anatomical forms of biliary cystic dilatation that occur not only in the choledochus, but also in any part of the bile duct between the liver and the duodenum, previously the classifications for this entity include only choledochus dilations [4].
Figure 1.
Types of choledochal cysts according to Todani’s classification. Type I (a = choledochal cyst in a narrow sense; b = segmental choledochal dilatation; c = diffuse or cylindrical dilatation.). Type II supraduodenal diverticulum. Type III (a = choledochocele; b = diverticular choledochocele). Type IV multiple dilatations [a = involving the intrahepatic biliary tract; b = sparing the intrahepatic biliary tract. Type V (or Caroli’s disease): Corresponds to multiple intrahepatic dilatations.
According to Todani’s classification, CCs are classified into five types:
Type I: Common type, represents 50–80% of CCs. It is characterized by cystic dilation of the common bile duct. It is further divided into three subgroups: a. choledochal cyst in a narrow sense; b. segmental choledochal dilatation; and c. diffuse or cylindrical dilatation.
Type II: Diverticulum type in the whole extrahepatic duct, represents 2%.
Type III: Choledochocele, represents 1.4%–4.5% of CCs. It is an intraduodenal cystic dilation of the distal common bile duct.
Type IV: Represents 15–35% of CCs. It is further divided into two subgroups. a. Multiple cysts at the intra- and extrahepatic ducts; b. multiple cysts at the extrahepatic duct only.
Type V: Intrahepatic bile duct cyst (single or multiple). Also known as Caroli’s disease, represents 20% of CCs [5].
Furthermore, there exists a special variant of CC named “forme fruste,” where the patients present with typical symptoms of CCs and are associated with abnormal pancreaticobiliary duct junction but little or no dilation of biliary ducts. It could be considered as an incomplete or atypical pathology [6].
There is an outstanding regional tendency, which affects predominantly Asian population with an incidence of 1 in 1000 live births, and two-thirds of the reported cases occur in Japan, compared with an incidence of 1 in 100,000–150,000 live births in the Western population [7, 8].
CCs type I and IV are more common and have a female-to-male ratio of 4:1 or 3:1. The cause for the Asian and female predominance remains under study, recent research studies settle that congenic bile duct dilatation has genetic basis, not only genetically heterogeneous but also non-monogenic, requiring mutations in more than one gene for the disease to develop. That is consistent with the low frequency and sporadic presentation of CC [9, 10, 11].
The etiology of a choledochal cyst remains unknown at present time, leading to the postulation of multiple theories. “Long common channel” theory by Babbit has been the most accepted explanation for the origin of the choledochal cyst. This theory states that the pancreaticobiliary junction is located outside the duodenal wall [12, 13].
In normal conditions, the common bile duct and the pancreatic duct enter into the duodenal muscle layer (sphincter of Oddi) and join in the submucosal layer just before opening into the duodenal lumen. This junction helps to regulate the output of digestive enzymes into the intestinal lumen but in a pancreaticobiliary maljunction (PBM), there is a dysfunction of the sphincter because the duct is external of the duodenal muscle layer, forming an extended common channel, 1–2 cm proximal to the sphincter of Oddi [14].
The maljunction of both ducts occurs during the fifth week of gestation. In normal embryological development, the pancreatic ventral bud fuses side by side with the dorsal bud, and in the sixth week, 180-degree rotation (clockwise) of ventral and choledochal, bud of the pancreas occurs around the duodenum to reach their finale positions [15]. PBM appears to be associated to the malrotation of the ventral bud, as the proximal portion of the hepatic diverticulum extends and the ventral primordium has been displaced away from the duodenum by elongation of the proximal part of the diverticulum.
Histological and immunohistochemical studies have demonstrated that the union of these two buds might occur in an oblique position, producing the formation of a long common channel [14, 16].
PBM was classified by Komi in 1992 into three types, based on the fusion pattern (Figure 2):
Figure 2.
Pancreaticobiliary maljunction classification. Komi classification and Japanese study group on Pancreaticobiliary Maljunction (JSPBM).
Type I: The bile duct joins with the pancreatic duct forming a right angle. It is presented in 35.3% of the cases.
Type II: It seems that the pancreatic duct connects to the biliary duct in an acute angle. It was seen in 21.6% of the cases.
Type III in 43.1% of the cases. It is a complicated junction of both ducts [17].
In 2015, the Committee on Diagnostic Criteria of the Japanese Study Group on Pancreaticobiliary Maljunction (JSGPM) proposed a classification into four types [18]:
Type A (stenotic type): The narrow segment of the distal common bile duct joins the common channel and shows dilatation of the common bile duct.
Type B (non-stenotic type): The distal common bile duct without any narrow segment joins the common channel. Without dilatation of the common channel.
Type C (dilated type): The narrow segment of the distal common bile duct joins the common channel, and abrupt dilatation of the common channel is seen.
Type D (complex type): PBM associated with annular pancreas, pancreas divisum, or other complicated duct systems.
In addition to PBM and a higher pressure of the pancreatic duct, the developed long channel allows reflux of pancreatic juice into the common bile duct. However, it has been found that not all abnormal pancreaticobiliary junctions present dilatation of the bile duct, which could explain only a part of its pathophysiology.
Pancreatic proenzymes also play an important role in the origin of the choledochal cyst, inasmuch as they come into contact with the bile and activate before reaching the duodenum, they generate a state of inflammation, obstruction, increased pressure in the choledochus, and consequently, greater dilation [19]. Furthermore, trypsinogen, when activated into trypsin, modifies a protein called lithostatin in its insoluble form, aggregating and forming protein plugs. These plugs are compacted in the common channel or in the narrow distal part of the cyst, causing pancreatitis or increasing the pressure of the bile duct so much that it sometimes generates biliary perforation.
Pancreaticobiliary reflux and activation of proenzymes generate intermittent symptoms such as abdominal pain, vomiting, jaundice, and increased aminotransferases in children. This fact is confirmed by a retrospective study of 80 patients where biliary amylase measurements were performed, finding a relationship between the presence of biliary amylase and clinical manifestations, with the presence of jaundice as the most common symptom in those with amylase <200 U/L, and with the presence of abdominal pain in those with amylase >200 U/L [20].
It has been observed in adulthood that pancreaticobiliary reflux presents as dilatation of the bile duct and malignancy. This is secondary to the fact that biliary stasis, which was previously mixed with the refluxing pancreatic enzymes, creates damage to the biliary epithelium. Chronic inflammation activates the point mutation of KRAS, overexpresses COX2, and inactivates TP53, generating greater cell proliferation and consequently epithelial hyperplasia, which appears benign in childhood, but in adulthood presents as dysplasia and subsequent carcinogenesis. The site of malignant occurrence is generally within the cyst, but it can be anywhere within the biliary tree [21].
On the other hand, Babbitt’s theory is confronted by authors who state that PBM is present in only 50–80% of cases and that in choledochal cyst diagnosed prenatally, there wasn’t the presence of reflux, and suggest that neonatal pancreatic acini are not able to produce enough pancreatic enzymes [22]. For unknown reason, despite the presence of PBM, bile duct dilatation may not occur and frequently does not generate symptoms. Therefore, in these patients, PBM tends to be diagnosed at a later stage [23].
This previous theory applies to choledochal cyst types I and IV. Regarding type II (true CBD diverticulum) and type III (choledochocele), it is suspected that the cause is related to biliary duplications cysts for type II and biliary or duodenal duplications cysts for type III [24].
Type V CC and fibrocystic liver disease are related with both being a spectrum of the same congenital disease; ductal dysgenesis affects the biliary tree at multiple levels from the small intrahepatic bile ducts (congenital hepatic fibrosis) to the larger bile ducts (Caroli disease). The etiology type V CC is accepted to be a halt in the remodeling of the ductal plates, and it is associated with biliary atresia [25].
Alternatively, suboptimal number of ganglion cells has been demonstrated in the narrow portion in the distal common bile duct of patients with choledochal cyst when compared with controls. This might lead to the dilation of the proximal segment of the common bile duct, describing a pathogenesis similar to achalasia and Hirschsprung’s disease [26].
The bile duct cyst is scarcely detected in the prenatal stage, the cases reported in the literature range between 20 and 30sdg, being the earliest case described in the 16th week of gestation [27, 28]. The multi-slice high-resolution ultrasound visualizes several planes simultaneously, including sagittal, coronal, transverse, and oblique views being the main tool in the prenatal approach allowing an early diagnosis [27, 28, 29].
Among the suggestive findings is the presence of an anechoic cystic image in the right upper quadrant without central vascularity to the application of the Doppler and which is in relation to the contiguity with the gallbladder, in addition to allowing to evaluate the position of the cyst, the state of the proximal ducts, vascular anatomy, and the hepatic echotexture [27, 29].
The advantage of prenatal diagnosis is that it allows the multidisciplinary team adequate neonatal support and prompt surgical planning. During pregnancy, the behavior should be expectant with follow-up ultrasounds, prioritizing childbirth. After birth, the diagnosis can be confirmed with magnetic resonance cholangiopancreatography. There are no international publications so far about perinatal management [30, 31].
Differential diagnosis should include retroperitoneal cysts (hydronephrosis, polycystic kidney, cystic neuroblastoma, or adrenal hematoma) and intraperitoneal cysts (ovarian cysts, epiploic and mesenteric cysts, intestinal duplication, intestinal atresia, biliary atresia, pancreatic and hepatic cysts) [31, 32].
In a series of 13 patients with biliary disease and abnormal prenatal examinations, the correct diagnosis was made prenatally only in 15% of cases. The difficulty in differential diagnosis in the newborn lies between the bile duct cyst and cystic biliary atresia. Given the difficulty that exists of differential between these two entities by prenatal ultrasound, a child with presumed bile duct cyst should undergo an early examination to rule out biliary atresia. So far there are no unequivocal differential parameters that are accepted [30, 31, 32].
Clinical presentation patterns differ according to the age group at onset of symptoms and the type of cyst. The classic triad of abdominal pain, abdominal mass in the right upper quadrant, and jaundice, although predominant in children, is only reported in 5–10% [33, 34].
Abdominal pain is the most frequent symptom (61–94%) with a slight predominance in older children and adults, which has an intermittent course with a variable time interval from days to years [35, 36]. Abdominal mass and jaundice are usually a manifestation of newborns and infants. Cholangitis, pancreatitis, and liver function test abnormalities are common and are thought to be secondary to a PBM or choledocholithiasis [36, 37, 38].
The infantile presentation is characterized by obstructive cholestatic syndrome. Jaundice follows an intermittent pattern since the obstruction of the biliary tree is incomplete; unlike cystic biliary atresia, the main differential diagnosis in this age group.
The presentation of acute abdomen secondary to biliary peritonitis due to rupture of the cyst is rare, predominantly in infants (1–2%). Older children and adults with choledochal cyst present biliary or pancreatic symptoms, mainly associated with abdominal pain. The clinic in this age range is given by entities secondary to chronic biliary stasis, manifesting as complications: cholelithiasis (49%), cholangitis (32%), acute pancreatitis (10%), hepatolithiasis (7%), biliary carcinoma (3%), portal hypertension (2%), and chronic pancreatitis (2%) [39, 40, 41, 42, 43]. Fifteen percent of patients with CC may be asymptomatic [36].
On the one hand, some specialized centers in Asia reported direct comparison studies about the clinicopathological differences between children and adults with choledochal cyst. Pediatric patients were more likely to have abdominal mass (52.4% vs. 21.2%) and jaundice (33.3 vs. 0%) compared with adults. Children are more frequently associated with PBM (85.7% vs. 59.6%) and sudden severe stenosis of the terminal common bile duct (76.2% vs. 42.3%). Adults were more likely to have abdominal pain (98% vs. 76.2%), frequently stone disease, and they are more associated with neoplasms (21.2% vs. 21.0%). Malignancy rates are widely reported to be 14–18% [44, 45, 46].
On the other hand, a multi-institutional analysis from eight centers in North America and Europe reported clinical characteristics among children and adults, with mean age at diagnosis of 5 years and 45 years, respectively. Adults had more abdominal pain than children (71.8% vs. 40.7%), and children had more jaundice compared with adults (31.9% vs. 11.6%) [36].
Regarding the symptoms according to the type of cyst, jaundice is observed mainly in type I (56%) and IV cysts. Hepatomegaly, palpable mass and episodes of biliary pancreatitis are more prevalent in type I. Exclusively intrahepatic cysts (type V) present mainly with cholangitis and gallstones [47].
There is no specific marker for choledochal cyst in blood tests, what commonly occur are variations in serum concentrations of amylase, bilirubin, and hepatobiliary enzymes when the patient becomes symptomatic; however, in asymptomatic patients, it can occur or not some variation in blood tests.
Ultrasonography (US) is the initial study when pathology of the bile duct is suspected, the advantage of this study is that it is noninvasive, and in expert hands, it can give a very approximate diagnosis. The main finding in US is dilation of the biliary tract, although it is also useful for evaluating the position of the cyst, the proximal ducts, vascularity, whether there are stones in the bile duct and the characteristics of the liver parenchyma.
A technectium-99 HIDA scan may provide more information if a choledochal cyst is suspected by US, being helpful to distinguish a cyst for biliary atresia.
The abdominal computed tomography (CT) scan can show the bile duct along with intrahepatic and intrapancreatic ducts; it is especially useful to rule out tumors at this level with the disadvantage of exposing the patient to radiation and the difficulty to observe the common channel and the biliopancreatic junction clearly. Therefore, CT cholangiography is more sensitive to assess the biliary tree, it identifies the presence of stones and diagnose the choledochal cyst; with a sensitivity above 90%, but with the risk of generating hepatotoxicity or nephrotoxicity due to the contrast medium [48].
For these reasons, the most recommended study is magnetic resonance cholangiopancreatography (MRCP), which has a sensitivity of 90–100% for diagnosis, with the advantage of not being invasive and not exposing radiation (Figure 3). Some centers even have the possibility of generating 3D images for better visualization. This study allows evaluation of the anatomy of the intrahepatic and extrahepatic bile ducts, the pancreaticobiliary junction, as well as the measurement of the length of the common channel, which is generally >15 mm [49, 50, 51].
Figure 3.
Coronal T2 MRCP shows a cystic dilation of the common bile duct (arrow) with preserved intrahepatic bile ducts. The gallbladder (asterisk) and the duodenum (arrowhead) are visualized as well.
Endoscopic retrograde cholangiopancreatography (ERCP) is also a diagnostic option; however, it is not the most common to perform due to its invasive method, although it is very useful, especially in those patients whose clinical and imaging studies are inconclusive. With this study, the path of the common channel, the common bile duct, and the position of the cyst can be assessed. However, ERCP relies on experienced personnel to perform the procedure, with a 10% rate of complications such as pancreatitis, infection, or bleeding. [52].
Intraoperative cholangiography (IOCG), like ERCP, is not indicated as a routine diagnostic method, it is only suggested in exceptional cases where previous studies have not been conclusive.
Medical treatment limits to the administration of antimicrobial therapy in case of cholangitis or supportive therapy in pancreatitis, trying to stabilize the patient prior to operative approach. If rupture of the cyst manifests, a drainage must be placed, pointing out the weirdness of these presentation [53].
Historically, drainage or cystenterostomy was the surgical management, but the high risk of malignant transformation and the recurrence of the symptoms demands aggressive surgical management as the overriding treatment [53, 54].
The prenatal diagnosis of congenital biliary dilatation (CBD) has helped to follow these patients after birth, establishing the ideal time to treat them, if the conditions of the patient allow it, the surgical procedure is recommended at the age of 6 months old. About half of the patients with prenatally diagnosis of CBD are asymptomatic; but if obstructive jaundice, cholangitis, pancreatitis, liver dysfunction, vomiting due to compression of the gastric outlet, or even rupture of the cyst development may be a mandatory earlier surgical exploration, even in the neonatal period [54].
Surgical treatment depends on the CC type. Type I, which is the most common, requires complete cyst excision followed by restoration of the biliary-enteric continuity.
A transverse or oblique incision is the traditional way to reach the hepatic hilum, but the laparoscopic approach (LA) has been increasingly adopted since 1995 when the first successful laparoscopic cyst excision with hepaticojejunostomy (HJ) was performed. A systematic review and update meta-analysis where 1767 patients were enrolled (853 laparoscopic group, 914 open group) reported that the operative time was longer in laparoscopic group, but also less intraoperative bleeding and less intraoperative blood transfusion as well as less time to initial feeding. Length of hospital stay was longer in open group. Short- and long-term postoperative complications were similar in both groups, but the total postoperative morbidity was lower in the laparoscopic group [55].
Minimal invasive robotic-assisted (RA) cyst excision and Roux-en-Y HJ were first reported by Woo in 2006 in a Type I cyst; since then, this practice has gained supporters. In a systematic review and meta-analysis, six studies with 484 patients (307 LA and 177 RA) were analyzed, the results and total complications showed no significant difference between the two groups. [56, 57]. Examination of the data regarding robotic CC excision is needed to determine the utility of this approach in children [53].
The CC transection should be at level of the common hepatic duct to assure a wide anastomosis, a complete excision of the distal portion into the duodenum just above of the pancreatic duct to avoid damage. It is not mandatory to perform IOCG, unless there are no images prior surgery through MRCP; although it is reported that in infants the biliopancreatic junction is very small and can easily go unnoticed during the CC excision. Therefore, if any doubts exist about the situation of the pancreaticobiliary junction, it is recommended to perform IOCG for reducing the risk of injury to the main pancreatic duct.
Some authors practice intraoperative antegrade cholangiography to evaluate also the common channel in all cases because endoscopic retrograde cholangiopancreatography is difficult to perform in early infancy due to the skill required and the potential for complications.
During the transection of the cyst, an adequate view of the hepatic ducts near the dilation is essential to discard stenosis or hypoplasia, which is related to bad prognosis. If protein plugs are present, wash them out by irrigating with saline solution or remove them with a blunt instrument or a pediatric cystoscope (Figure 4) [21, 58].
Figure 4.
Intraoperative cystoscopy of the distal portion of the cyst with plug proteins. Level 1 likelihood of leaving residual cyst. Level 2 adequate to perform the transection. Level 3 likelihood of injuring the pancreatic duct.
The biliary reconstruction can be performed through either Roux-en-Y HJ or hepaticoduodenostomy (HD). A systematic review and meta-analysis reported 715 patients, of which 403 (56.3%) were performed a HJ and 312 (43.6%) a HD. Operative time, operative bleeding and length of stay favoring HD, similar rates of complications, including cholangitis. Some disadvantages of the HD are the bile reflux with an estimated incidence of ~5% and require more research and long-term studies [59]. The latest findings in patients followed up by Takada endoscopically demonstrate mild to moderate gastric erosion, but in his study, the development of metaplasia in the stomach [60] is unknown.
A wide Kocher maneuver and a further distal anastomosis in the duodenum have been described to decrease the bile reflux [61]. HD has the advantage to follow through with endoscopic revisions if a late complication is presented, like stenosis of the anastomosis or intrahepatic lithiasis. It is worth to mention that the HD is easier to perform laparoscopically in contrast to the technically challenging HJ. To prevent stenosis of the anastomosis, it is recommended to avoid excessive dissection when dissecting the anterior wall and completely excise ulcerative lesions from the inner wall of the cyst.
A prospective randomized controlled trial describes that a shorter loop Roux-en-Y HJ reconstruction for choledochal cyst is equally effective individualizing the length of the loop based on the distance between the hepatic hilum and the umbilicus, compared with the traditional 40 cm length loop; with no episodes of cholangitis in either group in the following 6 months [62].
8.1 Management for the other choledochal cysts
Type II is a diverticulum of the bile duct, it is the most infrequent presentation of a CC. Surgical removal through laparoscopic approach with excellent results in the reports, but it is known that long-term follow-up and more clinical reports are needed [63].
Type III is a cystic dilatation of the distal common bile duct within the ampulla of Vater protruding into the duodenum, also called choledochocele. The treatment depends on the type of choledochocele, needing endoscopic transduodenal drainage of the lesion in pure choledochocele and with sphincterotomy and complete excision in diverticular choledochocele. Multiple endoscopic technics have been referred such as balloon dilation after incision of the cyst or stent placement, unroofing by partial snare excision of the cyst wall, or complete resection with a polypectomy snare [64, 65].
Type IVa or IVb CC involves dilatation of both intrahepatic and extrahepatic biliary trees. The standard procedure remains to be a complete excision of the dilated common bile duct and a hepaticoenterostomy. Depending on imaging studies, a hepatic segmentectomy or lobectomy might be needed in case of obstruction or to eradicate the segment with the most dilated intrahepatic cysts.
Major late complications such as pancreatic duct stones, intrahepatic calculi, stenosis might need other interventions such as pylorus-preserving pancreatoduodenectomy or duodenum-preserving pancreatic head resection. And sometimes a liver transplant is the only way to preserve a high quality of life [66].
Type V CC benefits from liver transplantation, a related living donor should be considered in time before the onset of life-threatening complications [66, 67].
The advancement of surgical treatment to include cyst excision has resulted in minimal morbidity and mortality and reduced the number of late complications, compared with past operative treatment of cystenterostomy. The most common short-term outcome in hepaticoenterostomy is anastomotic leakage and as a late complication continues to be anastomotic stricture (2.5–17%) and cholangitis (23–40%) [68, 69, 70, 71].
Perioperative morbidity is higher in adults than children (35.1% vs. 16.3%), requiring more surgical procedures following resection of the common bile duct cyst. However, adults were more likely to have wound, hepatobiliary, or gastrointestinal complications: seromas (3.1%), wound infections (9.7%), and perihepatic abscesses (7.7%). Instead, children had more anastomotic leaks (3%) and gastrointestinal tract perforations (3%) [36].
Early diagnosis and cyst excision result in low complication rates in most experienced centers. The technique of Roux-en-Y HJ is favored by most, although comparable results can be achieved by HD. Anyway, either procedure can be performed laparoscopically as well [68, 69]. In most reports comparing laparoscopic cyst excision with open cyst excision in children, operative time was found to be longer and overall costs higher when laparoscopy was used, but there was significantly less blood loss, and the duration of hospitalization was shorter. There were no significant differences in the incidence of bile leakage or wound infection rates. Although technically challenging and time-consuming, laparoscopic excision imparts less surgical stress on patients than open excision, and parents are generally more satisfied with the smaller scars.
Despite late complications being reduced with current surgical management, studies suggest that long-term follow-up is indicated due to the potential for problems such as anastomotic stricture, cholangitis, intrahepatic stone formation, and malignancy [69, 72]. Malignant degeneration occurs more often in type I and IV cysts and rarely in type II and III. This is particularly important in incompletely resected cystic hepatic ducts or recurrent cysts [46, 73].
Biliary cancer is reported in 5–10% of patients, increasing the incidence greater than 50% in patients over 50 years. The risk of malignancy is greatly reduced after cyst excision but is still elevated as compared with the general population, with an incidence ranging between 2.5 and 28% in adults. Nevertheless, it is reduced between 0.7 and 5.4% after complete surgical excision, with a 95.5% 5-year survival (adults 94.6%; children, 97.2%) [36, 74]. Therefore, lifelong follow-up with ultrasound, liver profile, and CA19–9 levels are recommended annually. Some authors perform biochemical follow with aspartate transaminase and alkaline phosphatase, every 4 months for 2 years; then every 6 months for 5 years [46].
A technectium-99 HIDA scan at 6 and 18 months after surgery can reveal mild episodes of cholangitis in asymptomatic patients or in those with occasional symptoms (less than two episodes per year), requiring only conservative therapy. If cholangitis is recurrent, a reoperation is advocated. In addition, technectium-99 HIDA scan can suggest the presence of anastomotic stricture with delayed flow greater than 60 minutes. However, percutaneous transhepatic cholangiography has still been considered to achieve definitive diagnosis [75].
Anastomotic stricture could occur as a consequence of small, tensive anastomosis, inflammation, or infection. Among the most used options are endoscopic retrograde cholangiopancreatography, percutaneous transhepatic biliary drainage, balloon dilation, and stenting with good results. Some authors pointed out that balloon dilation should be the first step as successful rate was 81%, whereas reoperation was considered as the final choice in all circumstances [69, 76].
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 anywhere from 3 to 22 years postoperatively. However, if the duct is patent and there is no stenosis of the hepaticojejunostomy, stones are likely to pass spontaneously [77]. Intrahepatic stones usually present in cases of stenosis that initially cause bile stasis and lead to stone formation.
Todani and colleagues 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 [78].
Choledochal cyst is a biliary anomaly that is often diagnosed at early age due to the imaging studies that are currently used. A careful and meticulous search can help establish early treatment, before serious sequelae arise.
Although the management of CC is established with acceptable results, a high rate of complications related to surgical procedures is still reported, so it is necessary to acquire an extensive knowledge of the different variants and provide greater technical skills that are acquired with practice to have better results and minimize the number of complications. In the same way, follow-up is necessary to detect the presence of malignancy in time and offer timely treatment.
Conflict of interest
The authors declare no conflict of interest.
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Written By
Magaly Torres, Mitzi Becerra, Beatriz Calderón, Iván Salinas, María Ruiz and Jorge Ventura
Submitted: 19 June 2022Reviewed: 11 July 2022Published: 09 August 2022
Open access peer-reviewed chapter
