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Purpose: Laparoscopic resection of choledochal cyst (CC) has become a popular approach. As the discussion about optimal treatment and technical strategies continues, we aimed to investigate perspectives of IPEG members., Methods: An online survey was conducted in 2023 on behalf of the IPEG Research Committee. IPEG members were asked to complete an anonymous questionnaire that included 36 items on the management of CC., Result: 148 members responded to the survey (North America:49/Asia:44/Europe:23/South America:21/Others:11) and 116 completed all questions. Most surgeons (92.5%) operate on less than 5 cases annually. Diagnostic tools of choice were Magnetic Resonance Imaging (MRI, 95.9%) and ultrasonography (US, 74.5%). Regarding fusiform-type CC, operative indications were cyst size greater than 10 mm (68.9%), typical symptoms (78.5%), or anomalous pancreatico-biliary junction (63.8%). In unilateral intrahepatic biliary cysts (type IVa) cases, 81.3% of respondents do not perform a simultaneous liver resection with the initial cyst resection. While 22.0% resect the CC at diagnosis, even if asymptomatic, a larger group of surgeons (41%; 49/118) wait until the infant reaches six months. Intraoperative cholangiography and choledochoscopy are performed routinely by 38.9% and 13.7%, respectively. The majority (52.5%) ligates the common bile duct stump just below the CC. Laparoscopic reconstructions are performed by retrocolic hepatico-jejunostomy (48.3%) or hepatico-duodenostomy (45.8%) at similar rates, but when done open, 71.2% of respondents prefer retrocolic hepatico-jejunostomy. For the laparoscopic anastomosis, interrupted sutures with intracorporeal knot tying were most often utilized (48.3%)., Conclusion: Inidividual pediatric surgeons treat a small number of patients with CC each year. Laparosopic and open reconstruction techniques vary, likely due to technical challenges., Level of Evidence: III., Competing Interests: Conflict of interest The authors declare no conflict of interest associated with this study., (Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved.)

Introduction: An increasing proportion of congenital choledochal malformation (CCM) are being detected on antenatal ultrasound. However, the actual timing of its surgical correction remains controversial with some series showing an excess of complications the earlier the operation. The aim of this study was to characterize the pathophysiological aspects of this cohort from the perspective of age at surgery in order to inform a more rational basis for clinical decision-making., Methods: We analysed a prospective database of CCM acquired over a 26-year period (Jan. 1997 to Dec. 2022) for patient demography; details of pre- and post-natal imaging; age at surgical intervention; operative complications (Clavien-Dindo classification) and outcome. Data are quoted as median (IQR). All comparisons are non-parametric. A P value of 0.05 was accepted as significant., Results: There were 58 (72% female) children with an antenatally-detected CCM from a total of 265 (21.8%) in the series. These were classified as Type 1C (n = 47; 81%), type 4C (n = 3; 5%) and Type 5 (n = 8; 14%). There were no Type 1F lesions in this cohort. Median age at surgery was 113 (IQR 57-198) days. Postnatal cyst (US) size varied from 12 to 130 mm but there was little evidence of a relationship between this and post-natal liver biochemistry (e.g. bilirubin r S  = 0.01, P = 0.44; AST r S  = 0.14, P = 0.19). Choledochal pressure measurements (n = 46) showed resting pressures of 12 (9-21) mmHg with no significant correlation with age (P = 0.4) or aspartate aminotransferase (P = 0.2) or γ-glutamyl transferase (P = 0.06). The cohort was divided into 2 groups (Early and Late) based on the median age at surgery (all open) (113 days). Biliary obstruction was more common in the Early group (10 vs. 2; P = 0.01). Two perforations occurred, both in the Early group. With a deliberate policy of regular ultrasound-based follow-up we have seen no anastomotic complications (leak, stenosis, persistent intrahepatic biliary dilatation or stones) or post-operative cholangitis in any child [median follow-up 3.42 (1.30-8.05) years]., Conclusions: This is one of the largest series documenting the outcomes of antenatally-detected CCMs, certainly in Europe and North America. Such lesions are invariably cystic in nature, and either Types 1C, 4C or 5. The absence of complications using a policy of early intervention (where possible) in experienced hepatobiliary units was shown., Evidence Level: II., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Background: This study aimed to develop a postnatal treatment strategy for infants with prenatally diagnosed congenital biliary dilatation., Methods: We performed a retrospective study of patients with prenatal diagnosed congenital biliary dilatation (CBD), aged <1 year who underwent surgery at our hospital between 2013 and 2023. We classified the patients into two groups, the "early group," consisting of patients who could not wait for growth, and required early surgery, and the "scheduled group," consisting of patients who were asymptomatic and could undergo scheduled surgery, and compared them. The parameters for early surgical prediction were AST, ALT, TB, DB, and CRP levels at birth, 1 week, 2 weeks, 1 month, 2 months, and 3 months after birth, and immediately before surgery, as well as the cyst diameter, presence of intrahepatic bile duct dilation, and presence of debris in the common bile duct., Results: During the study period, 15 patients were diagnosed prenatally. The cyst diameter was significantly larger at all points in the early group. Patients with a cyst diameter of >30 mm at birth, intrahepatic bile duct dilatation at birth, and postnatal enlargement of the common bile duct to >30 mm are more likely to develop symptoms early. Blood biochemistry tests showed no significant differences between the two groups., Conclusions: Patients with a cyst diameter >30 mm in the early postnatal period require careful postnatal management and parents should be counseled regarding the high likelihood of their child needing surgery within the first 3 months of life., Level of Evidence: Level IV., Competing Interests: Declarations of interest None., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Aim of the Study: The aim of the study is to clarify the clinicopathological and biliary morphological characteristics in reported cases of diverticular congenital biliary dilatation (CBD)., Method: Using PubMed and the Japan Medical Abstracts Society, articles on possible diverticular CBD were extracted and the clinical pictures examined. We also sought evidence for definitions of diverticular CBD and the associated condition of pancreaticobiliary maljunction (PBM) using the original articles by Alonso-Lej and Todani. The characteristic biliary morphologies of cases with images were also investigated., Results: Analyses of 211 possible cases superficially demonstrated multiple diverticula in 12 (12%) and single diverticulum in 89 (88%), with diverticula located in the upper (n = 38, 38%), middle (n = 32, 32%), or lower (n = 26, 26%) biliary tract in and presence of intra-diverticular stones, PBM, and biliary carcinoma in 23% (n = 18), 39% (n = 25), and 11% (n = 14), respectively. However, evidence defining diverticular CBD or justifying the lack of associated PBM was not demonstrated even in the original articles. Scrutiny of the biliary anatomy in 59 cases with images showed incorrect inclusions of types I or IV-A with an irregular biliary duct wall or dilated cystic duct, periampullary choledochal diverticula, or even solitary biliary cysts. Authentic diverticular CBD, representing the diverticulum connected to the middle of the common bile duct via a thin, patent stalk was seen in only 6 cases., Conclusion: Real diverticular CBD appears extremely rare. The lack of an objective definition allows wide interpretations of clinical pictures, creating inconsistencies in the diagnosis and treatment of CBD and raising questions regarding the utility of conventional classifications., Level of Evidence: Level III., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Aim: The safety and success of laparoscopic choledochal cyst surgery (LapCC) depends upon two critical elements during the hepaticojejunostomy anastomosis (HJA). These may be termed the Difficulty of Differentiation (DOD) and the Difficulty of Suturing (DOS). The type of imaging system (2 or 4 K) used may influence either of these. We compared outcomes of LapCC using 2 or 4 K imaging systems., Methods: LapCC were performed at a single institution by the same team using a 2 K system (2009-2018; n = 26) and a 4 K system (2018-2019; n = 11) were compared. 4 K cases were chosen to match 2 K cases to minimize bias. Five independent senior pediatric surgeons scored DOD and DOS blindly from intraoperative video recordings of LapCC using a subjective 5-point scale (5: impossible, 4: difficult, 3: tedious, 2: slow, and 1: easy) and rated their over all impression as +1 if 4 K was better, 0 if they were the same, and -1 if 4 K was worse. Total HJA anastomosis time (TAT) and TAT/suture were also calculated., Results: LapCC was performed in 37 age/weight/HJA diameter matched children. Scores for DOD (p<0.001) were lower with 4 K with less variance, although there was no difference in DOS (p = 0.08). Operative time (p = 0.03) and duration of hospitalization (p < 0.001) were significantly shorter with 4 K. 4 K was rated +1 unanimously. There was no difference in TAT (p = 0.17) and TAT/suture (p = 0.22). There was one HJA leak with 2 K (3.8%) and no complications with 4 K., Conclusions: Improved resolution with 4 K improved the progress of surgery as reflected by shorter operative time and duration of hospitalization, enhancing the performance of LapCC in children., Level of Evidence: III., (Copyright © 2021 Elsevier Ltd. All rights reserved.)

Introduction: Type 4 choledochal malformations (CMs) can be defined as extra- and intrahepatic biliary dilatation. They are of uncertain etiology but make up about 20% of most series. The aim of this study was to investigate the pathophysiology and their natural history following surgical intervention., Methods: Ambispective review of a single-center series of type 4 CM between 1996 and 2017. Perioperative imaging, intraoperative pressure monitoring, bile amylase, and long-term follow-up data were analyzed based on extrahepatic morphology [(cystic (C), fusiform (F)]. Data were expressed as median (range). Statistical analysis was performed with nonparametric tests. P < 0.05 was considered significant., Results: 37 children had type 4 CM in the study period [age at surgery 4 (0.2-16) years] and could be further subdivided into cystic (4C) (n = 22) or fusiform (4F) (n = 15). There was significantly greater dilatation of the extrahepatic component in the cystic group [30 (11-94) versus 15 (8-90) mm; P = 0.0002] though there was no difference in left duct diameter [cystic 7.5 (0-17) mm versus fusiform 7.5 (3-16) mm; P = 0.86]. There was a trend to higher choledochal pressure in the cystic group [19 (4-40) versus 9 (6-25) mmHg; P = 0.09] and those in the fusiform group had higher bile amylase [8650 (3-890,000) versus 592 (1-123,000) IU/L; P = 0.01] and were older [4.1 (0.92-16.43) versus 2.4 (0.15-15.48) years; P = 0.03]. Children with type 4 CM were then separated simply on the basis of bile amylase into LOW (characterized by high pressure, cystic morphology and young age at surgery) and HIGH bile amylase (low pressure, fusiform morphology and older at time of surgery)., Conclusions: We propose further division of type 4 CM into 4C and 4F on clinical and pathophysiological grounds., Level of Evidence: II (prospective cohort)., (Copyright © 2020 Elsevier Inc. All rights reserved.)

A 3 year old girl presented initially with acute severe hemorrhagic pancreatitis diagnosed by clinical features including a positive Cullen's sign and raised serum amylase. A contrast enhanced computed tomography (CECT) scan corroborated the same and also reported a type I choledochal cyst. Magnetic resonance cholangiopancreatography (MRCP) 2 months later reported a type II choledochal cyst and a persistent pseudo pancreatic cyst. Intraoperative features and close perusal of the CECT and MRCP films showed the presence of mixed type I and II choledochal cyst. The case is reported for the presence of Cullen's sign, CECT and MRCP features of a mixed type I and II choledochal cyst and successful surgical outcome. It appears to be only the fifth case of mixed type I and II choledochal cyst in world literature in the pediatric age group, following the 4 previous cases in children reported from Japan., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Introduction: Choledochal malformations (CMs) are increasingly diagnosed antenatally. There is a dilemma between early surgery to prevent CM-related symptoms and postponing surgery to reduce complications. We aimed to identify the optimal timing of surgery in asymptomatic neonates with antenatally diagnosed CM and to identify predictors for development of symptoms., Methods: Using the Netherlands Study group on CHoledochal Cyst/malformation (NeSCHoc) we retrospectively collected demographic, biochemical and surgical data from all Dutch patients with an antenatally detected CM., Results: Between 1989 and 2014, antenatally suspected CM was confirmed in 17 patients at a median age of 10days (1day-2months). Four patients developed symptoms directly after birth (24%). Thirteen patients (76%) remained asymptomatic. Two of these progressed to symptoms before surgical intervention at 0.7 and 2.1months resp. Postoperatively, four patients developed short-term complications and three developed long-term complications. Patients <5.6kg (the series median) showed more short-term complications (66%) when compared to patients >5.6kg (0%, p=0.02)., Conclusion: When not symptomatic within the first days of life, the majority of children with antenatally detected CM remains asymptomatic. Surgery might safely be delayed to the age of 6months or a weight of 6kg. Postponing surgery in the clinically and biochemical asymptomatic patient might decrease the complication rate., Levels of Evidence: Level III., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Background: Choledochoceles may cause biliary obstruction and harbor malignancy. We conducted a 40-year systematic review of the literature for this rare anomaly., Methods: PubMed and Cochrane databases were accessed 1975-2015 using terms "choledochocele" or "choledochal cyst". Studies reviewed that met the following criteria: English language, published 1975-2015 with human subjects., Results: 325 patients with a choledochocele were identified, including 71 case reports and 254 cases within institutional reviews. 13 pediatric case reports of choledochocele exist, with abdominal pain being the most common symptom (n=11). The most frequent diagnostic and treatment modalities were ultrasound (n=10), and endoscopic sphincterotomy (n=5). No malignancies were reported. 58 adult case reports exist, with the most common presenting symptom being abdominal pain (n=54). Ultrasound was the frequently employed diagnostic modality (n=32). Open procedures were performed more often (n=30). Malignant lesions were identified in 5. In 42 institutional reviews, the frequency of choledochocele was 0.7%. Of those for whom treatment was reported, 69% underwent endoscopic sphincterotomy., Conclusion: Choledochocele is a rare malformation. Similarities exist between pediatric and adult patients, but malignancy has only been reported in adults. An algorithm based on patient age, cyst size, lining and amenability to endoscopic resection may be considered as a treatment strategy for this uncommon condition., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Purpose: Recent advances in ultrasonography have increased prenatal diagnosis of biliary atresia (BA) and choledochal cyst (CC). These conditions are not easy to distinguish before or just after birth. This study investigated diagnostic and therapeutic problems in prenatal diagnosis of BA and CC., Methods: We retrospectively studied clinical characteristics and progression of hepatobiliary cysts in 10 patients (4 cases of BA, 6 cases of CC) from the time of diagnosis. Chronological changes in cyst size and gallbladder morphology were assessed and measured sequentially by ultrasonography., Results: Three cases of BA were type I cyst and 1 case was type III-d. All cases of CC were type Ia. Cyst size decreased between birth and surgery in BA but increased in CC. The gallbladder appeared atrophic in BA. There was no significant difference in gestational age or cyst size at prenatal diagnosis, changes in cyst size between birth and surgery, and degree of liver fibrosis., Conclusions: BA should be suspected if cyst size decreases before and after birth and the gallbladder atrophies after birth. Cholangiography is the only reliable method to differentiate BA from CC. Neonatal surgery is indicated for CC with icterus and liver dysfunction., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Background: Strictures of the extra-hepatic biliary tree are rare in children and have a benign non-traumatic inflammatory origin or are related to idiopathic fibrosing pancreatitis. Primary sclerosing cholangitis (PSC) can manifest as multiple biliary strictures or as a single dominant stricture. We describe the presentation, treatment, and outcome of six cases of isolated benign choledochal stricture (IBCS)., Methods: All patients underwent magnetic resonance cholangiography (MRC). Five patients underwent diagnostic and therapeutic ERCP, and 4 patients underwent intra-choledochal mini-probe EUS and biopsy. Colonoscopy was performed in suspected ulcerative colitis (UC)., Results: We report 6 patients (mean age at diagnosis: four males, 12.1 years; two females, 14.2 years) with IBCS. Clinical onset included 3 cases of acute biliary pancreatitis and obstructive jaundice, one obstructive jaundice, one cholestasis, and one pancreatitis. At diagnosis, MRC confirmed IBCS in all patients. Biliary sphincterotomy, stricture dilation, and stenting were performed in 4 patients. One child underwent hepaticojejunostomy for a type I choledocal cyst. During follow-up (mean: 21 months; range: 1-3 years), all patients were asymptomatic. Four patients developed UC (three pancolitis, one descending colitis). One child developed PSC., Conclusion: IBCS can be successfully treated by therapeutic ERCP. The occurrence of UC could suggest that IBCS is a form of PSC., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Spontaneous perforation of a choledochal cyst with ensuing pseudocyst formation is a very rare complication. We report the development of a pseudocyst adjacent to a choledochal cyst in a very low-birth-weight infant at 2 months of age. Elective excision of the choledochal cyst and biliary tract reconstruction were successfully performed 2 months later when the infant weighed 3 kg. Delayed primary repair may be a viable alternative treatment for low-birth-weight infants with choledochal cysts., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Biliary tract duplication cysts with heterotopic gastric mucosa are rare congenital anomalies, with our case representing only the fourth reported case in the literature. An 8-year-old girl with several months of abdominal pain was found to have a complex cystic mass communicating with the biliary system via the common hepatic duct. Intraoperatively, inflammation caused by the cystic mass was found to have resulted in a Mirizzi-like syndrome, with a nearly complete obstruction at the confluence of the left and right hepatic ducts. Histopathologic examination of the biliary mass revealed it to be a duplication cyst lined by heterotopic gastric mucosa with secondary ulceration and fibrosis. Biliary duplication cysts are a rare but important process that should be considered in a child with a mass in the portal triad and biliary obstruction., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Background/purpose: Management of choledochal cysts consists of surgical excision and hepaticojejunal anastomosis. Endoscopic retrograde cholangiopancreatography (ERCP) can be used to resolve complications and to evaluate the biliary tract and pancreatobiliary duct junction. Our aim was to underline the importance of ERCP for optimal management., Methods: From 2005 to 2011, 28 patients were reviewed (21 female, 7 male; mean age, 5.71 years; range, 2-16 years). After imaging, all patients underwent elective ERCP and were referred for surgery., Results: Choledochal cyst was diagnosed at ultrasound and magnetic resonance cholangiopancreatography in all examined patients; common biliopancreatic duct was diagnosed in 3 (20%) of 15 patients at magnetic resonance cholangiopancreatography and in none at ultrasound. Endoscopic retrograde cholangiopancreatography showed choledochal cyst in all patients and common biliopancreatic duct in 19 (68%) of 28 patients. Twelve patients underwent sphincterotomy. All patients underwent surgical extrahepatic biliary tree resection and hepaticojejunal anastomosis. Mean period of hospitalization was 9.5 days (range, 6-13 days). No major complications related to ERCP were observed. Two patients needed postoperative ERCP for complications (pancreatitis during follow-up)., Conclusions: In our pediatric experience, ERCP is feasible and safe. It can rule out other possible biliary tract anomalies and help plan the timing and choice of the appropriate surgical procedure., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Background/purpose: Choledochal cysts (CDCs) are increasingly being diagnosed antenatally. The timing of surgery in this group of patients is unclear. We undertook a prospective randomized trial to establish the optimal timing of surgery for prenatally diagnosed asymptomatic CDCs., Methods: Between 2003 and 2011, 68 fetuses with ultrasound-detected subhepatic cysts (ie, potential CDC) were randomized into 2 groups: (1) early (≤1 month) operation group (n = 34) and (2) late (>1 month) operation group (LO; n = 34). Operative time, postoperative hospital stay, drainage duration, postoperative complications, and perioperative ultrasonographic and laboratory results were analyzed., Results: During the first month of life, 32 infants became symptomatic (eg, became jaundiced) and were excluded from the trial. This left 36 neonates who were asymptomatic (early operation, n = 16; LO, n = 20) and constituted the study group. It is noteworthy that a further 9 infants in the LO group then became symptomatic after 1 month of age. Grades III and IV hepatic fibroses were significantly more common in the LO group and in patients who were diagnosed during early pregnancy. Interestingly, liver functions in patients diagnosed at early fetal stage were only moderately deranged. Median follow-up period was 36 months. Neither mortality nor complications of bile leak or anastomotic stricture were encountered. However, complete liver function normalization was significantly delayed in the LO group and in patients with high-grade hepatic fibrosis., Conclusion: Prenatally diagnosed CDC is a distinct group with a tendency of developing liver fibrosis immediately after birth. Early surgical intervention is warranted in the neonatal period., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Background/purpose: The aim of this study was to investigate the diagnostic potential of computed tomography cholangiography (CTC) and magnetic resonance cholangiopancreatography (MRCP) in children with pancreaticobiliary maljunction (PBM)., Methods: Fifty-three children with PBM were consecutively treated between 1997 and 2009. Among them, the patients who underwent CTC and/or MRCP preoperatively were enrolled in this study. Computed tomography cholangiography was examined after infusion of meglumine iodoxamate with subsequent 3-dimensional rendering. The visualization of the biliary and pancreatic duct systems was evaluated and compared with that visualized with MRCP. The findings of direct cholangiography were used as the standard of reference., Results: Of the 53 cases with PBM, 17 cases were examined by CTC, 10 cases by MRCP, and 17 with both. The extrahepatic bile tract was visualized in 32 (94.1%) of 34 patients in CTC and in all 27 patients in MRCP. The intrahepatic bile duct was more frequently demonstrated by MRCP than by CTC (96.3% vs 70.6%, P = .02). Pancreaticobiliary maljunction was noted in 13 (38.2%) of 34 with CTC and in 12 (44.4%) of 27 with MRCP. The minimum age for visualization of PBM was at 10 months in CTC and at 1 year and 11 months in MRCP, respectively. The main pancreatic duct was more frequently visualized by MRCP than by CTC (81.5% vs 8.8%, P < .001)., Conclusions: Magnetic resonance cholangiopancreatography provides superior visualization of the intrahepatic duct and the pancreatic system when compared with CTC. However, it is still challenging to perform a good-quality examination in young infant. The great advantage of CTC is its ability to produce high-quality images without respiratory artifacts and that it allows accurate assessment of the presence of PBM equivalent to MRCP., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Purpose: Robotic technology allows surgeons to perform complex procedures which may be difficult with standard laparoscopic instruments. We believe that complex hepatobiliary procedures are ideally suited for robotic surgery in children and present our experience with Kasai portoenterostomy and excision of choledochal cysts., Methods: We performed 4 complex hepatobiliary procedures in children using the Da Vinci surgical robot (Intuitive Surgical, Sunnyvale, CA): 2 Kasai portoenterostomies and 2 choledochal cyst resections. Both Kasais had the Roux-en-Y jejunojejunostomy performed extracorporeally through the 12 mm umbilical trocar site. Both choledochal cysts had the Roux-En-Y jejunojejunostomy performed intracorporeally. All patients had their hepatobiliary to enteric anastomosis performed intracorporeally., Results: Total average time was 6 hours and 12 minutes for the Kasai and 7 hours and 38 minutes for the choledochal cysts. The average robotic console time for all cases was 6 hours. No intraoperative or perioperative complications occurred. Average length of hospital stay was 4 days. Both choledochal cyst patients were doing well after 9 and 12 months with no complications. One Kasai patient is doing well 14 months after Kasai with a normal bilirubin. The other Kasai patient did well for a year with a normal bilirubin. However, the patient slowly developed intrahepatic bile lakes despite a normal bilirubin and a well draining Kasai as demonstrated by hepatobiliary iminodiacetic acid (HDA) scan. He began having recurrent episodes of cholangitis and we referred him for liver transplantation., Conclusion: Minimally invasive robotic complex hepatobiliary surgery is safe and effective in children. The 3-dimensional imaging and improved articulations make these procedures particularly suited for robotics over standard laparoscopy.

Background/purpose: Refluxes through pancreaticobiliary maljunctions play an important role in the pathophysiology of choledochal cysts. Dynamic studies of the pancreaticobiliary tract were performed using secretin-stimulated magnetic resonance cholangiopancreatography., Methods: Six patients with choledochal dilation were recruited for this study. Four patients exhibited cystic and 2 exhibited fusiform dilatation of the common bile duct (CBD). Magnetic resonance cholangiopancreatography images were obtained every minute during the 15-minute period after secretin stimulation. The sequential morphological changes in the biliary trees, pancreas, and duodenum were assessed, and the total pixel values of these organs were measured for each image, then plotted as a ratio against the baseline image., Results: In 2 cases involving cystic dilatation, the intensity of bile duct images continued to rise after secretin stimulation. In a case involving fusiform dilatation, a transitory elevation in CBD intensity was observed. In 3 cases involving fusiform or cystic dilatation, the intensity of CBD did not change notably. In all cases, the duodenum was filled well after secretin stimulation., Conclusions: The sustained elevation in bile duct intensity after secretin stimulation indicates reflux and bile stasis. Transitory elevation may indicate reflux without stasis. This method allows assessment of the dynamics of pancreatic and bile fluid under more physiologic condition.

Purpose: A new generation of magnetic resonance imaging scanner offers a 3-dimensional magnetic resonance cholangiopancreatography (MRCP) with very high spatial resolution using a non-breath-hold technique with prospective navigator gating. The study aimed to investigate the new technology of the MRCP in infants with suspected pancreaticobiliary diseases., Methods: Seven patients (4 girls, 3 boys; mean age, 2.8 years; range, 3 months to 5.6 years) were examined. All patients underwent magnetic resonance imaging examination using a 1.5-T whole-body scanner (Magnetom Avanto, Siemens Medical, Erlangen, Germany). For MRCP, a heavily T2-weighted (time to echo, 678 ms) 3-dimensional turbo spin echo in coronal plane was performed with the voxel size of 1 x 1 x 1.5 mm. To enable non-breath-holding imaging, a diaphragm navigator sequence was simultaneously carried out. The MRCP findings were compared prospectively with the endoscopic retrograde cholangiopancreatography and/or with the surgical findings in 6 of 7 cases. In 1 patient, the follow-up was considered reference standard., Results: The high spatial resolution of the images without breathing artifacts allowed an excellent demonstration of all relevant pancreaticobiliary ducts and of the pathologic findings. All patients (n = 4) with choledocholithiasis were identified through MRCP. The finding of a high pancreaticobiliary junction (n = 3) was confirmed by endoscopic retrograde cholangiopancreatography in all cases. The length of the common channel ranged from 16 to 24 mm. In 1 patient, the long common channel was associated with a choledochal cyst. In a 1.7-year-old boy with recurrent abdominal pain and mild pancreatitis, the rare case of a gastric duplication with connection to the main pancreatic duct was diagnosed. A regular gastric wall with normal mucosa and parts of the duodenal wall with a circular layer of muscle (pylorus-like) were found in histology after surgical resection of the mass., Conclusion: The newly developed technique may be a reliable tool in the workup of pancreaticobiliary diseases in infants.

A 29-year-old woman with congenital biliary atresia underwent liver resection for intrahepatic biliary cyst and recurrent cholangitis long after Kasai procedure. The patient is alive with normal liver function and no episode of cholangitis after 12 months of follow-up.

Purpose: The aim of this study was to determine an appropriate postnatal management plan for prenatally diagnosed congenital biliary dilatation (CBD)., Methods: Between 1962 and 2002, 5 (5.9 %) of 85 patients had CBD diagnosed prenatally and were examined clinically. Of these 5 patients, 2 (group A) underwent delayed primary definitive surgery after percutaneous transhepatic cholangiodrainage (PTCD), 1 (group B) underwent early definitive surgery in the neonatal period, and 2 (group C) underwent delayed primary definitive surgery without PTCD in early infancy (within 6 months after birth). The clinical data, operative findings, intra- and postoperative complications, and follow-up were evaluated in these 3 groups., Results: There were no postoperative complications, such as catheter-related complications, in group A. However, there was adhesion around the choledochal cyst, and the operation was therefore difficult in group A. The diameter of the anastomosis in the hepaticojejunostomy was small, and the cyst wall was thin in group B. Consequently, anastomotic leakage of the hepaticojejunostomy occurred in group B. Neither operative nor postoperative complications such as anastomotic leakage or stenosis occurred in group C. Slight fibrosis of Glisson's sheath was seen in 2 patients of groups A and C. No liver cirrhosis was seen in any group., Conclusions: The authors propose that asymptomatic patients should undergo elective definitive surgery by 6 months of age. For symptomatic patients, especially when a differential diagnosis of type I cystic biliary atresia is doubtful, early definitive surgery is needed before 2 months of age. PTCD appears to be indicated only under certain circumstances, and delayed primary definitive surgery should be performed as early as possible thereafter.

Background/purpose: Biliary tree cystic malformations (BCM) occur rarely. Current progress in ultrasonography makes it possible to identify them prenatally. The aim of this study was to analyze retrospectively prenatal ultrasound patterns of 5 consecutive subhepatic cysts in an effort to differentiate the BCM before birth and onset of symptoms., Methods: The prenatal ultrasound patterns of 5 consecutive BCM were correlated with the postnatal diagnosis., Results: Case 1 showed a hyperechogenic cyst that underwent enlargement during fetal life; the diagnosis at birth was an obstructed choledochal cyst (CC). In cases 2 and 3, an anechoic cyst with small, stable dimensions during pregnancy was identified; a biliary atresia (BA) type III-C was confirmed at birth. A slightly hyperechogenic cyst with no increase in size at repeated prenatal scans, was observed in cases 4 and 5; a CC and a cystic lesion at the confluence of the hepatic ducts (without obstruction) were diagnosed postnatally, respectively., Conclusions: The ultrasound scan pattern and size allow to make a prenatal differential diagnosis of BCM. Anechoic, small cyst in the hepatic hilum is highly suspicious for BA. Large, echoic or enlarging cyst is strongly suggestive of "obstructed" CC. Echoic, small and stable cyst is more suggestive of "unobstructed" CC., (Copyright 2002, Elsevier Science (USA). All rights reserved.)

Background: The incidence of intrahepatic cholelithiasis and cholangitis has not yet been well studied postoperatively in patients with choledochal cysts., Methods: One hundred three patients with choledochal cysts had operative cholangiography, underwent standard excision of a choledochal cyst with Roux-en-Y hepatico-jejunal anastomosis, and were at a mean follow-up of 12 1/2 years. The incidence of intrahepatic bile duct stones was analyzed according to the 3 morphologic types of intrahepatic bile duct observed at initial operative cholangiography: type 1, no dilatation of the intrahepatic bile ducts; type 2, dilatation of the intrahepatic bile ducts but without any downstream stenosis; and type 3, dilatation of the intrahepatic bile ducts associated with downstream stenosis. Initially, there was no evidence of intrahepatic bile duct stones in any of the 103 patients., Results: Among 50 type 1 patients, intrahepatic cholelithiasis developed in only 1 patient (2%). Among 43 type 2 patients, 1 patient (2%) had intrahepatic cholelithiasis, and 2 (5%) had postoperative cholangitis. Among 10 type 3 patients, 4 (40%) had intrahepatic cholelithiasis (P <.01), and 3 (30%) had postoperative cholangitis. Time intervals between the initial surgery and the first identification of intrahepatic stones ranged from 3 to 22 years., Conclusions: One of the major causes of formation of intrahepatic cholelithiasis has been clarified; patients with intrahepatic biliary dilatation with downstream stenosis can get intrahepatic bile duct stones long after excision of a choledochal cyst.

Background/purpose: This study investigates the relationship between the location of the papilla of Vater and the length of the common channel in patients with congenital biliary dilatation (CBD)., Methods: Cholangiograms from 121 CBD patients and 13 normal controls were the subjects for this study. A length index defined as the length of the common channel divided by the height of the second lumbar vertebra was used for standardization., Results: In the controls, the papilla of Vater was located in the middle of the descending or second part of the duodenum in all cases. In 39 (32.2%) of the 121 CBD patients papilla of Vater was located in the descending duodenum (group I), and in 82 (67.8%) it was distal to the descending duodenum (group II). The average length index of the common channel in group II was significantly longer than in group I (1.123 +/- 0.374 v 0.660 +/- 0.246; P <.001). Findings for the common bile duct were similar., Conclusions: There is a significantly higher incidence of ectopic distal location of the papilla of Vater in CBD patients than in controls. The more distal the location of the papilla of Vater, the longer the common bile duct and the common channel., (Copyright 2001 by W.B. Saunders Company.)

This report describes the surgical management of 2 children with fusiform choledochal cysts who had accessory hepatic ducts (AHD) identified during excisional surgery for fusiform choledochal cysts (CC). Two children presenting with a triad of recurrent jaundice, fever, and abdominal pain were investigated and found to have type 1 choledochal cyst. Preoperative imaging and intraoperative cholangiography missed the AHD in both cases. In one of the patients, the main and the accessory ducts were separated by the right hepatic artery. In both the patients the accessory ducts were reconstructed successfully into a Roux loop along with the main common hepatic duct. Follow-up studies showed no evidence of biliary tract obstruction or atrophic changes in the liver. There was satisfactory uptake and drainage on hepatic scintigraphy. During excision of CC, AHD may be encountered. These may be missed on preoperative imaging. AHD may have a close relationship with neighboring vascular structures in the porta. Accessory hepatic ducts should be anticipated, identified, and reimplanted into the Roux loop during excisional surgery., (Copyright 2001 by W.B. Saunders Company.)

The authors report on an infant who had a multiloculated cystic lesion located in segment IV of the liver, consistent with Caroli's disease diagnosed, by routine prenatal ultrasound at 25 weeks' gestation, and confirmed by hepatobiliary HIDA scan and computed tomography soon after birth. Because there was no sign of biliary obstruction, the patient was observed initially, with gradual regression of the cysts noted by serial sonograms. Caroli's disease in older children and adults often is associated with recurrent cholangitis and cirrhosis, mandating resection when the disease is unilobar. However, the natural history of Caroli's disease diagnosed in utero is unclear, and a period of observation appears warranted in the asymptomatic patient.

One to two percent of patients who have choledochal cysts present with cyst rupture and bile peritonitis. Reported cases have been managed with external drainage of the cyst followed by a second procedure to excise the cyst and reconstruct the biliary tract. The authors report two cases of ruptured choledochal cyst treated with primary cyst excision and biliary drainage. The satisfactory outcome of these patients suggests that this is the preferred management.

The incidence of subhepatic cysts later confirmed as choledochal cyst has changed with the use of prenatal ultrasonography. A new group has emerged: the neonatal patient with an antenatal diagnosis. Optimal timing for cyst excision depends on variables such as gestational age, weight, associated conditions, biochemical liver alterations, development of complications, and the sonographic surveillance of size. The author's experience (1992) with an antenatally diagnosed choledochal cyst prompted this literature review. The present case showed an abnormal choledochopancreatic ductal junction, high amylase content, and a linear pattern of growth over time (2 mm/wk). Management consisted of cyst excision and bilio-enteric reconstruction. Fourteen cases (including the present one) have been reported in the world literature. All were females. Seventy-two percent of the ultrasound examinations were performed for dating purposes. Subhepatic cysts were identified at a mean gestational age of 26.9 weeks (range, 15 to 37 weeks). Excision and bilio-enteric reconstruction were performed at a mean age of 45 days (range, 9 hours to 6 months). Clinically, 50% of the babies were anicteric, 43% were jaundiced, and 7% had a palpable mass. Evidence of cyst growth was present in 56% of cases, and 60% had liver fibrosis that reverted to normal. The indications for surgery were jaundice (43%), cyst growth (21%), delayed HIDA excretion (7%), and elective (29%). Recommendations for managing the asymptomatic, anicteric neonate are discussed, entailing a rational approach based on growth potential, biochemical liver alterations, and the development of obstructive jaundice.

The authors report the first case of antenatally diagnosed choledochal cyst having evidence of early fetal bile drainage and complete obliteration of the distal bile duct. This disputes the traditional hypothesis of reflux of pancreatic enzymes and supports the recent theory of primary obstruction as the etiologic cause of choledochal cyst. Coexisting congenital hypothyroidism and pulmonary stenosis had not been reported previously.

The authors report the findings of endoscopic retrograde cholangiopancreatography in a 1-year-old girl with choledochal cyst; contrast dye injected through the common channel was excreted solely through the duct of Santorini. The obstruction to the bile and pancreatic juice caused by marked bending of the common channel was responsible for the abnormal drainage of bile and pancreatic juice.

A pair of monozygotic twins, 2-year-old Japanese girls, discordant for both anomalous pancreaticobiliary ductal junction and congenital biliary dilatation, are reported. The pathogenesis of the disease is discussed in relation to the occurrence of discordant monozygotic twins.