Biliary Disorders, Anomalies, and Malignancies in Children.

Biliary abnormalities in children are uncommon, and the spectrum of biliary disorders is broader than in adult patients. Unlike in adults, biliary disorders in children are rarely neoplastic and are more commonly rhabdomyosarcoma rather than cholangiocarcinoma. Pediatric biliary disorders may be embryologic or congenital, such as anatomic gallbladder anomalies, anomalous pancreaticobiliary tracts, various cholestatic processes, congenital cystic lesions, or genetic conditions. They may also be benign, such as biliary filling anomalies, biliary motility disorders, and biliary inflammatory and infectious disorders. Distinguishing these entities with a single imaging modality is challenging. US is the primary imaging modality for initial evaluation of biliary abnormalities in children, due to its wide availability, lack of ionizing radiation, and low cost and because it requires no sedation. Other examinations such as MRI, CT, and nuclear medicine examinations may provide anatomic and functional information to narrow the diagnosis further. Hepatobiliary-specific contrast material with MRI can provide better assessment of biliary anatomy on delayed images than can traditional MRI contrast material. MR cholangiopancreatography (MRCP) allows visualization of the intra- and extrahepatic biliary ducts, which may not be possible with endoscopic retrograde cholangiopancreatography (ERCP). Suspected biliary atresia requires multiple modalities for diagnosis and timely treatment. Determining the type of choledochal cyst calls for a combination of initial US and MRCP. Many benign and malignant biliary masses require biopsy for definitive diagnosis. Knowledge of the imaging appearances of different pediatric biliary abnormalities is necessary for appropriate imaging workup, providing a diagnosis or differential diagnosis, and guiding appropriate management. ©RSNA, 2024 Test Your Knowledge questions for this article are available in the supplemental material.

Surgical aspects of choledochal cyst in children and adults: an experience of 106 cases.

PURPOSE: To describe clinical features of choledochal cyst (CC) patients in terms of demographic data, clinical presentation, investigations, treatment, and outcomes among children and adults. METHODS: The medical records of patients undergoing choledochal cyst (CC) surgery from 2002 to 2021 at a university hospital were retrospectively reviewed. The patients were divided into two groups: children (< 15 years) and adults (≥ 15 years). Descriptive statistics were used. RESULTS: There were 106 cases of CC (Female/male = 88/18, children/adult = 53/53). Abdominal pain was the predominant presenting symptom, followed by jaundice in both groups. Adults were significantly more prone to present with abdominal pain compared to children (86.8% vs. 52.8%; p < 0.001), while children were more likely to experience acholic stool than adults (22.6% vs. 3.8%; p = 0.004). Ultrasound was the preferred investigation screening modality (75.5%). Most patients were presented with type I CC (71.7%). Laparoscopic-assisted approach was performed in 8.5%. CC excision with roux-en-y hepatico-jejunostomy was the main procedure (88.7%). Adults had a higher incidence of post-op complications, including stones, anastomosis stricture, abdominal collection, and cholangitis. Adults were significantly more likely to require intervention after surgery, compared to children (26.4% vs. 5.7%; p = 0.04). CONCLUSIONS: Ultrasound was the most common screening tool for diagnosis. Postoperatively, adults with CC experience more serious post-op complications compared to children. This could be attributed to long-standing cystic inflammation. Therefore, prompt definitive surgery is recommended for CC patients.

STAT3 promotes migration and invasion of cholangiocarcinoma arising from choledochal cyst by transcriptionally inhibiting miR200c through the c-myb/MEK/ERK signaling pathway.

Signal transducer and activator of transcription 3 (STAT3) have been highlighted in cancer regulation. Its roles in Cholangiocarcinoma (CCA) arising from the choledochal cyst (CC) were unclear. Here, we attempted to elucidate the roles of STAT3 in CCA-CC and explore its mechanism. A total of 20 patients with CCA arising from CC, that underwent CC excision in the infant stage were included. The expressions of STAT3, miR200c and c-Myb in clinical samples were assessed by RT-qPCR and/or western blot. Their expression correlations in tumor tissues were evaluated by Pearson correlation analysis. Their roles in CCA cell migration and invasion were investigated by gene silence using siRNA or miRNA inhibitor mediated approach and MEK activator. The expression levels of EMT, metastasis and MEK/ERK pathway-related proteins were checked by western blot. The high expressions of STAT3 and c-Myb, and low expression of miR200c were detected in CCA samples. We defined the transcription inhibition of STAT3 in miR200c expression and the negative correlation between miR200c and c-Myb expression. Silence of STAT3 increased miR200c expression and retarded the migration and invasion of CCA cells, accompanied by decreased levels of Vimentin, N-cadherin, MMP2 and MMP9, and elevated expression of E-cadherin, resulting in inactivating MEK/ERK pathway. MiR200c inhibitor reversed the changes induced by STAT3 silence, which was restored by si-c-Myb. MEK activator significantly reversed the inactivation of the MEK/ERK pathway induced by si-STAT3+miR200c inhibitor+si-c-Myb. In summary, the silence of STAT3 suppressed metastasis and progression of CCA cells by regulating miR200c through the c-Myb mediated MEK/ERK pathway, suggesting STAT3 is the effective target for CCA arising from CC.

An audit of Complicated Choledochal Cysts- 15-years' experience at a tertiary care center.

PURPOSE: Complicated choledochal cysts (CDC) have a variable presentation, and their management differs from an uncomplicated CDC. They are infrequently reported. We present our 15 years of experience in the management of complicated CDC. METHODOLOGY: We reviewed the data of patients with CDCs managed at a tertiary level center from 2005 to 2020 from a prospectively maintained database. RESULTS: Of 215 patients with CDC, 123 patients presented with complicated CDC. The median age of complicated CDC was 31 years with a female preponderance (62.6%). The most common type of CDC associated with complications was type I (69.1%), followed by type IVA (29.3%). The Complicated CDC was presented as cholangitis with or without cystolithiasis (n = 45), cystolithiasis and hepatolithiasis(n = 44), malignancy(n = 10), complications associated with incomplete cyst excision (n = 10), acute pancreatitis (n = 8), chronic pancreatitis(n = 8), portal hypertension (n = 6), spontaneous rupture (n = 4), gastric outlet obstruction (n = 1). These patients were managed as a one-stage approach (52.03%) and a two-stage approach (47.96%). On univariate and multivariate analysis, increasing age, prolonged duration of symptoms, and presence of abnormal pancreaticobiliary ductal junction (APBDJ) were significantly associated with complicated CDC. CONCLUSION: The management of complicated CDC varied depending on the associated pathology, many of them required a staged approach. Increasing age, prolonged duration of symptoms, and presence of APBDJ were significantly associated with complicated CDC.

Expression of CD56 is Not Limited to Biliary Atresia and Correlates with the Degree of Fibrosis in Pediatric Cholestatic Diseases.

BACKGROUNDS AND AIMS: CD56 immunostain is used as an adjunct to aid in the preoperative diagnosis of biliary atresia (BA) by liver biopsy. We aimed to study the expression of CD56 in different pediatric cholestatic diseases thereby evaluating its utility in the diagnosis of BA. METHODS: We performed immunohistochemistry for CD56 on 35 cases of pediatric cholestatic diseases and five age-matched controls. CD56 expression was assessed by a multiplication score (percentage positivity x intensity) in the biliary epithelium. RESULTS: The multiplication score between BA and choledochal cyst was not significantly different. High scores were also encountered in other cholestatic disorders. The score showed a significant negative association with serum albumin and a significant positive correlation with the serum ALT level. Very significant positive correlation between the score and portal fibrosis was obtained. CONCLUSION: CD56 expression is an infidel marker for the histological diagnosis of BA and rather provides a clue to the disease status in pediatric cholestatic diseases.

Short-term and long-term outcomes after Roux-en-Y hepaticojejunostomy versus hepaticoduodenostomy following laparoscopic excision of choledochal cyst in children.

BACKGROUND: Choledochal cysts are congenital dilations of the biliary tree. Complete cyst excision and biliary-enteric reconstruction have been the standard operations. In our center, more than 95% of choledochal cyst excision is now performed laparoscopically. Majority of current studies describe laparoscopic-assisted reconstruction using Roux-en-Y hepaticojejunostomy (HJ). However, only a few have studied laparoscopic hepaticoduodenostomy (HD) as an alternative method of biliary-enteric reconstruction. In this study, we focused on comparing longer-term outcomes between laparoscopic HJ and HD reconstruction following choledochal cyst excision. METHODS: We performed retrospective analysis of 54 children who had undergone laparoscopic choledochal cyst excision and biliary-enteric reconstruction between October 2004 and April 2018. Short-term outcomes including operative time, complications such as anastomotic leakage and bleeding, and hospital stays were included. Long-term outcomes including contrast reflux into biliary tree, cholangitis, anastomotic strictures, and need of reoperation were analyzed. RESULTS: Of the 54 patients, 21 of them underwent laparoscopic HD and 33 underwent laparoscopic Roux-en-Y HJ anastomosis reconstruction. There were no significant differences in gestation, gender, age at operation, antenatal diagnosis, and Todani type of choledochal cyst between HD and HJ group. Operative time was significantly shortened in HD group (p = 0.001). Median time to enteral feeding was 3 days in both groups. Median intensive care unit (p = 0.001) and hospital stay (p = 0.019) were significantly shorter in HD group. There was no perioperative mortality. There was no significant difference in anastomotic leakage requiring reoperation (p = 0.743). There were no significant differences in long-term outcomes including anastomotic stricture (p = 0.097), cholangitis (p = 0.061), symptoms of recurrent abdominal pain or gastritis (p = 0.071), or need of reoperation (p = 0.326). All patients had normal postoperative serum bilirubin level. CONCLUSIONS: Laparoscopic excision of choledochal cyst with HD reconstruction is safe and feasible with better short-term outcomes and comparable long-term outcomes compared to Roux-en-Y HJ reconstruction.

The Presence of Pericholedochal Hyaline Cartilage in Biliary Atresia: A Report and A Review.

Background: The presence of cartilage in extra hepatic biliary tree is an unusual finding. An isolated presence of the cartilage is possibly heterotopic or occurs as a metaplastic response to the inflammatory insult.Material and methods: We had examined the liver biopsy and the resected specimen of a biliary atresia (BA) after Kasai procedure.Results: There was hyaline cartilage around the common hepatic and common bile duct in a 3-months-old male infant with distal obstructive cholangiopathy on liver biopsy and had positive serum IgM for cytomegalovirus (CMV). Similar findings could not be documented in the pericholedochal tissue of any of the 25 other pediatric cases operated for BA or choledochal cyst and three neonatal autopsies performed for liver-related deaths.Conclusion: Peri-bile duct cartilage is a unique finding and could represent an unusual form of heterotopia or connective tissue metaplasia.

Laparoscopic-assisted cyst excision and ductoplasty plus widened portoenterostomy for choledochal cysts with a narrow portal bile duct.

BACKGROUND: Complete cyst excision with Roux-en-Y hepaticojejunostomy is the standard procedure for choledochal cysts (CCs). In recent years, neonates have been increasingly diagnosed with CCs prenatally. Earlier treatment has been recommended to avoid complications. For type IVa malformation without extensive intrahepatic bile duct dilatation, laparoscopic hepaticojejunostomy is technically challenging, and anastomotic stricture is a concern. Therefore, we propose laparoscopic synthetical techniques-laparoscopic excision of cyst and ductoplasty plus widened portoenterostomy to avoid stricture in CCs with a narrow hilar duct. METHODS: An anastomosis was created around the transected end of the common bile duct in 12 minipigs (Group A), and another 12 minipigs (Group B) received conventional cholangiojejunostomy. Anastomotic diameter measurements and cholangiography were conducted at different times. Histological findings of inflammation and scarring were compared. The expression levels of TGF-ß1 and type I collagen were detected by real-time quantitative PCR. Between January 2012 and January 2016, laparoscopic excision of cyst and ductoplasty plus widened portoenterostomy were performed on 29 children with confirmed CCs with a narrow portal bile duct who were followed up for 12-48 months. RESULTS: Group A survived well without obstruction. Slight inflammation and fibrotic tissue were confined to the bile duct periphery. In Group B, five pigs developed stricture. Severe inflammation and diffuse fibrosis affected the whole layer of the anastomosis. Fibrotic biomarkers were significantly higher postoperatively in Group B. Clinically, 29 patients exhibited satisfactory outcomes. No anastomotic stricture has been observed to date. CONCLUSIONS: Laparoscopic synthetical techniques may be a superior option to prevent anastomotic stricture in treating CCs with a narrow portal bile duct.

A degenerate Todani Ia choledochal cyst in a patient with pancreas divisum.

Bile duct cysts represent congenital abnormalities associated with biliopancreatic maljunction that may undergo malignant degeneration. We report herein the case of a 72-year-old male patient with cholangitis. MR-cholangiography and abdominal CT revealed a mass at the biliary-pancreatic-duodenal crossroads, extrahepatic biliary dilation up to 38 mm, and pancreas divisum. Gastroscopy found an infiltrative bulbar mucosa with adenocarcinoma in biopsy samples, and extrinsic bulging of the second duodenal portion. Endoscopic ultrasound showed a choledochal cystic dilation with solid contents, and FNA findings were nonspecific. ERCP confirmed an adenomatous papilla at the lower portion of the extrinsic formation, and a large cystic, saccular dilation of extrahepatic bile ducts (Todani Ia). Fistulotomy was required for deep cannulation of the proximal biliary tract, and attention was drawn to extruding polypoid lesions originating in the biliary epithelium, identified in biopsies as adenoma with dysplasia. Finally, a diagnosis was made of advanced adenocarcinoma in choledochal cyst.

Cystic biliary atresia or atretic choledochal cyst: A continuum in infantile obstructive cholangiopathy.

Introduction: Both cystic biliary atresia and choledochal cyst present as infantile obstructive cholangiopathy. Methods: We detail an infant with congenital biliary dilatation and obstructive cholangiopathy where clinicoradiological features (antenatally imaged subhepatic cyst, early onset jaundice, intrahepatic dilated biliary radicals) suggested a choledochal cyst but operative findings (lack of a distal communication of cyst with duodenum) and histomorphological features (cicatricial collagen and myofibroblastic hyperplasia in the cyst wall; ductal plate malformation, ductular cholestasis and strong expression of CD56 in the liver) were those associated with biliary atresia. Conclusion: The observations support the contention that BA and CC may be interim entities in a continuum of manifestation of the same pathology.

A systematic review of the anatomical findings of multiple gallbladders.

BACKGROUND: Multiple gallbladders (MG) are a rare malformation, with no clear data on its clinical impact, therapeutic indications or risk for malignancy. METHODS: A systematic review of all published literature between 1990 and 2017 was performed using the PRISMA guidelines. RESULTS: Data of 181 patients extracted from 153 studies were reviewed. MG were diagnosed during the treatment of a gallstone-related disease in 83% of patients, of which 13% had previous cholecystectomy and had a recurrence of biliary stone disease. The sensitivity of ultrasound scan was 66%, and that of magnetic resonance imaging cholangio-pancreatography, 97%. The cystic duct was common to both gallbladders (type1) in 43% and separated (type 2) in 50% of patients. In the latter case, there was no way to differentiate preoperatively an accessory gallbladder from a Todani II bile duct cyst. Cholecystectomy was performed in 129 patients by laparotomy (43%) or laparoscopy (56%). MG was undiagnosed before surgery in 24% of the patients. The postoperative biliary leakage rate was 0.7%. In two patients, gallbladder cancers were detected. CONCLUSION: MG are difficult to diagnose and share a common natural history with single gallbladders, without evidence of increased risk for malignancy. Excision of both gallbladders is indicated in symptomatic stone disease. However, prophylactic cholecystectomy must be considered for type 2 MG, since it cannot be preoperatively differentiated from a Todani II bile duct cyst, which is associated with a risk of malignant transformation.

Per-oral video cholangiopancreatoscopy with narrow-band imaging for the evaluation of indeterminate pancreaticobiliary disease.

BACKGROUND AND AIMS: Cholangiopancreatoscopy for evaluating pancreaticobiliary pathology is currently limited by suboptimal optics. The aim of this study was to characterize the operating characteristics of per-oral video cholangiopancreatoscopy with narrow-band imaging (POVCP) findings in indeterminate pancreaticobiliary disease and to describe their association with neoplasia. METHODS: Data from consecutive patients undergoing POVCP for the evaluation of indeterminate pancreaticobiliary disease at a single tertiary care center were analyzed. Two experienced investigators had previously agreed on POVCP findings and terminology that were documented in endoscopy reports. Endoscopic procedural data from POVCPs performed between January 2006 and April 2015 and clinical data were abstracted from the endoscopic database and electronic medical records. Study endpoints included tissue-proven neoplasia or benign disease with ≥1 year of follow-up. RESULTS: A total of 109 patients were identified; 13 were excluded because of the presence of stone disease, known pancreaticobiliary malignancy, or presumed benign disease with ≤1 year of follow-up. Most patients (85%) underwent POVCP for biliary disease and 15% underwent POVCP for a pancreatic cause. Tortuous and dilated vessels (P < .001), infiltrative stricture (P < .001), polypoid mass (P = .003), and the presence of fish-egg lesions (P = .04) were found to be significantly associated with neoplasia. The overall POVCP impression had a high sensitivity (85%) and negative predictive value (89%) in assessing for the presence of neoplasia. CONCLUSIONS: Per-oral video cholangiopancreatoscopy is effective in the evaluation of indeterminate pancreaticobiliary disease. Tortuous and dilated vessels, infiltrative stricture, polypoid mass, and the presence of fish-egg lesions are significantly associated with neoplasia.

Use of LigaSure™ on bile duct in rats: an experimental study.

BACKGROUND: The closure of a cystic duct during cholecystectomy by means of radiofrequency is still controversial. We report our preliminary experimental results with the use of LigaSure™ on common bile duct in rats. METHODS: Thirty Wistar rats weighing 70 to 120 g were employed for this study. The animals were all anesthetized with intraperitoneal ketamine and then divided into three groups. The first group (10 rats, Group C) underwent only laparotomy and isolation of the common bile duct. The second (10 rats, Group M) underwent laparotomy and closure of the common bile duct (CBD) with monopolar coagulation. The third group (10 rats, Group L) underwent laparotomy and sealing of the common bile duct with two application of LigaSureTM. Afterwards, all rats were kept in comfortable cages and were administered dibenzamine for five days. They were all sacrificed on day 20. Through a laparotomy, the liver and bile duct were removed for histological examination. Blood samples were obtained to dose bilirubin, amylase and transaminase levels. RESULTS: Mortality rate was 0 in the control group (C), 3/10 rats in group M and 0 in group L. In group L, the macroscopic examination showed a large choledochocele (3-3.5 × 1.5 cm) with few adhesions. At the histological examination there was optimal sealing of the common bile duct in 9/10 rats. In group M, 2/10 rats had liver abscesses, 3/10 rats had choledochocele and 5/10 rats, biliary peritonitis. There was intense tissue inflammation and the dissection was difficult. Analyses of blood samples showed an increase in total bilirubin, aspartate aminotransferase (AST) and alanine aminotransferase (ALT) in groups M and L. CONCLUSIONS: The preliminary results of our study confirm that radiofrequency can be safely used for the closure of the common bile duct. The choledochocele obtained with this technique could represent a good experimental model. These results could be a further step for using the LigaSureTM in clipless cholecystectomy.

Cystic duct cyst lesions (type VI).

Todani proposed the most accepted classification for cystic lesions of the bile duct, including 5 types. Serena described another one, type VI, which includes cystic lesions of the isolated cystic duct, of which there are less than twenty reported cases. They differ from type II in terms of the distal cystic caliber, which is normal in type VI, and in the diagnosis which is performed intra-operatively in most of the cases. Neoplastic degeneration has an incidence of 10 to 30% in all these lesions, which is the reason why the most widely accepted treatment is cholecystectomy with resection of the cystic duct cyst dilatation and preservation of the main bile duct by a laparoscopic approach.

The Density of Interstitial Cells of Cajal Is Diminished in Choledochal Cysts.

BACKGROUND AND AIMS: Interstitial cells of Cajal (ICC) have been shown to be present in the extrahepatic biliary tract of animals and humans. However, ICC distribution in choledochal cysts (CC) has not been investigated. A study was conducted to investigate the distribution of ICC in the extrahepatic biliary tract, including CC, in pediatric human specimens. METHOD: The specimens were divided into two main groups as gallbladders and common bile ducts. Gallbladders were obtained from the cholelithiasis, CC operations and autopsies. Common bile ducts were obtained from autopsies. Tissues were stained using c-kit immunohistochemical staining. ICC were assessed semi-quantitatively by applying morphological criteria and were counted as the number of cells/0.24 mm(2) in each area under light microscopy. RESULTS: A total of 35 gallbladders and 14 CC were obtained from operations. Ten gallbladders plus common bile ducts were obtained from autopsies. The mean numbers of ICC in the gallbladders of cholelithiasis and the gallbladders of CC were 12.2 ± 4.9 and 5.3 ± 1.2, respectively (p = 0.003). The mean numbers of ICC in the common bile ducts and CC were 9.8 ± 2.9 and 3.4 ± 1.4, respectively (p = 0.001). CONCLUSION: The scarcity of ICC in the extrahepatic biliary tract may be responsible for the etiopathogenesis of the CC.

Intracholecystic papillary-tubular neoplasm in a patient with choledochal cyst: a link between choledochal cyst and gallbladder cancer?

BACKGROUND: Intracholecystic papillary-tubular neoplasms are rare precursor lesions of gallbladder cancer. They were proposed as a separate pathologic entity in 2012 by Adsay et al. for the unification of a variety of mass-forming precursor lesions including papillary adenomas, tubulopapillary adenomas, intestinal adenomas, and others. They are considered homologous to intrapapillary mucinous neoplasms of the pancreas and intrabiliary papillary neoplasms of the common bile duct. In contrast with the commoner flat-type precursor gallbladder cancer lesions, they follow a more indolent clinical course and probably different genetic pathways to carcinogenesis. They are largely uninvestigated with only a handful of studies providing biological and clinical information. Choledochal cysts are dilation of the common bile duct. Diagnosis is usually established during childhood, and only a minority of patients are diagnosed at adulthood. They are of major clinical importance as they are known predisposing factors for biliary carcinogenesis. CASE PRESENTATION: The current report describes a patient with a simultaneous diagnosis of choledochal cyst and intracholecystic papillary-tubular neoplasm. The patient underwent excision of the extrahepatic biliary tree for a Todani I choledochal cyst, and histological examination of the specimen revealed an intracholecystic papillary-tubular neoplasm of the gallbladder. Authors describe diagnostic and clinical course of the patient alongside clinical and biological characteristics of these rare lesions. CONCLUSIONS: To the best of our knowledge, this is the first report of a patient with a simultaneous diagnosis of choledochal cyst and intracholecystic papillary-tubular neoplasm. Those rare lesions shed light on different forms of gallbladder cancer carcinogenesis and its relationship with choledochal cysts and cholestasis.

Increased survivin expression and its association with clinical parameters of congenital choledochal cysts.

The aim of the current study was to investigate survivin expression in congenital choledochal cysts (CCCs), and its associations with clinical parameters of CCCs. In total, 121 children with CCCs were included in this study as the case group, and their cysts were staged according to the Todani classification system. Additionally, 49 normal gallbladder specimens from healthy children were included as the control group. Survivin detection was conducted using immunohistochemical staining. Associations between positive survivin expression and clinical parameters of CCCs were then analyzed. Positive survivin expression was observed in the cytoplasm, and was seen as granular with yellow or dark brown staining. In the case group, positive survivin expression was detected in most tissues. Specifically, compared to that of normal tissues, the cystic-shaped and fusiform-shaped CCC tissues had significantly higher positive survivin expression rates (all P < 0.05). Importantly, positive survivin expression was also shown to be significantly associated with gender and histological type (both P < 0.05). In conclusion, increased survivin expression was observed in CCC tissues, and was correlated with certain clinical parameters of CCCs, suggesting a possible prognostic value of survivin for CCC progression.

Choledochal Cyst: Hepaticoduodenostomy or hepaticojejunostomy?

Choledochal cysts are cystic dilatation of the common bile duct managed by excision and hepatico-intestinal reconstruction. The gold standard after choledochal cyst excision is reconstruction using the jejunum. With the advent of laparoscopy the pendulum has changed toward hepaticoduodenostomy reconstruction. We review the classification, diagnosis and recent management of choledochal cyst.

[Bile duct cysts; an unusual cause of jaundice in paediatrics. Presentation of a case series]. / Quistes de colédoco, una causa inusual de ictericia en pediatría. Presentación de serie de casos.

INTRODUCTION: Cysts of the bile duct or choledochal cysts are rare diseases in our area. The aetiology is unknown, with the most accepted hypothesis being a pancreatobiliary maljunction anomaly. OBJECTIVE: To analyse the clinical data, diagnosis and treatment of a number of patients with choledochal cyst, as well as presenting an update on this condition. METHOD: A retrospective descriptive study was performed on paediatric patients diagnosed with choledochal cyst in the last 20 years in a tertiary hospital. CASE REPORTS: A total of 4 choledochal cyst cases in childhood, predominantly female, are pre- sented. The most frequent reason for consultation was vomiting, and presenting with jaundice and choluria in all cases. Patients with choledochal cyst were classified as type I in 3 cases, and one case of type IVa. In all cases surgical treatment was performed; any patient had complications to date. CONCLUSIONS: Cysts of the bile ducts have a low prevalence. The treatment of choice is surgical, requiring close monitoring due to the risk of cholangiocarcinoma.

What is the incidence of biliary carcinoma in choledochal cysts, when do they develop, and how should it affect management?

The incidence of cancer in choledochal cysts (CCs) in adults was calculated to determine the timing and need for surgery. In 78 publications (1996-2010), 434 of 5780 reported CCs patients had cancer. Cholangiocarcinoma (70.4 %) and gallbladder cancer (23.5 %) were the most common malignancies. Only nine malignancies were reported before age 18 (0.42 %). In contrast, the incidence of malignancy in adults was 11.4 %. The median age for diagnosis of cancer was 42 years, and the incidence increased with each decade.