A nomogram for predicting choledochal cyst with perforation.

BACKGROUND: Choledochal cyst with perforation (CC with perforation) rarely occurs, early diagnosis and timely treatment plan are crucial for the treatment of CC with perforation. This study aims to forecast the occurrence of CC with perforation. METHODS: All 1111 patients were conducted, who underwent surgery for choledochal cyst at our hospital from January 2011 to October 2022. We conducted univariate and multivariate logistic regression analysis to screen for independent predictive factors for predicting CC with perforation, upon which established a nomogram. The predictive performance of the nomogram was evaluated using receiver operating characteristic (ROC) curves, calibration plots, and decision curve analysis (DCA) curves. RESULTS: The age of children with choledochal cyst perforation is mainly concentrated between 1 and 3 years old. Logistic regression analysis indicates that age, alanine aminotransferase, glutamyl transpeptidase, C-reactive protein, vomiting, jaundice, abdominal distension, and diarrhea are associated with predicting the occurrence of choledochal cyst perforation. ROC curves, calibration plots, and DCA curve analysis curves demonstrate that the nomogram has great discriminative ability and calibration, as well as significant clinical utility. CONCLUSION: The age of CC with perforation is mainly concentrated between 1 and 3 years old. A nomogram for predicting the perforation of choledochal cyst was established.

Cholangiocarcinoma identified in perforated choledochal cyst in a 3-year-old boy.

Cholangiocarcinoma in patients with Choledochal cysts is rare in childhood; however, it seriously affects the prognosis of the disease. The key to addressing this situation lies in completely removing the extrahepatic cyst. We herein present a case report of a 3-year-old boy with cholangiocarcinoma associated with a choledochal cyst (CDC). Preoperative 3D simulation, based on CT data, played an important role in the treatment of this patient.

An observational study on the prevalence of choledochal cyst with pancreatitis: Geographical implications and management.

The objective of this study was to analyse the prevalence, risk factors and need for intervention in a sample of Indian children with choledochal cyst (CDC) complicated by pancreatitis with a special focus on chronic pancreatitis. A retrospective review of medical records of children admitted with CDC over 11 years was done and pancreatitis identified using INSPPIRE guidelines. Children were divided into two groups-one having choledochal cyst alone and the other choledochal cyst along with pancreatitis to determine associated risk factors. 40.2% of children with CDC had pancreatitis based on elevation of enzymes or radiological imaging. Age, total bilirubin and indirect bilirubin, requirement of intervention was significantly higher in the group with pancreatitis. 47% of those with radiological features of pancreatitis had imaging features of chronic pancreatitis. Chronic pancreatitis has not been reported previously in children with CDC and maybe peculiar to the Indian subcontinent.

Choledochocele with hyperplastic epithelium in a patient who developed severe acute pancreatitis and underwent subtotal stomach-preserving pancreatoduodenectomy: a case report.

Choledochocele is defined as a congenital dilatation of the distal intramural part of the common bile duct protruding into the wall of the descending duodenum, typically without pancreaticobiliary maljunction. However, some cases present with a similar pathophysiology to pancreaticobiliary maljunction, including reciprocal reflux of pancreatic juices and bile, leading to protein plugs, pancreatitis, and biliary tract carcinogenesis. Choledochocele is relatively rare and its anatomy, physiology, pathology, and clinical features are thus not well known. We describe a patient with choledochocele who suffered from repeated severe acute pancreatitis and underwent subtotal stomach-preserving pancreatoduodenectomy, in whom the pathological findings of choledochocele showed hyperplasia.

An interesting and rare cause of post-operative intussusception: Roux limb intussusception.

Intussusception is a common disease in children. Most intussusception is idiopathic and approximately 1% develops postoperatively. We present a case of a 6-year-old male patient who underwent surgery for a type 1 common bile duct cyst. In his post-operative 2nd month, the patient presented with a complaint of abdominal pain after eating, but his pain was not accompanied by nausea or vomiting. No pathological findings were evident in the examination or an abdominal X-ray of the patient. Intussusception was detected by ultrasonography and computed tomography. We wanted to introduce this interesting intussusception that originated from the Roux limb that was not seen before.

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.

Characteristics, management and outcomes of choledochal malformations in Finnish adult patients.

CONCLUSIONS: Nearly half of operated patients developed long-term postoperative complications. A novel association between CMs and IBD was observed. Although no hepatobiliary malignancies regardless of treatment modality were encountered, the number of patients and length of follow-up remained limited.

Surgical outcomes after re-operation for excision of choledochal cyst with delayed biliary complications: A retrospective study on 40 patients.

PURPOSE: The purpose of this study is to review our experience in patients who underwent re-operation for delayed biliary complications after choledochal cyst (CDC) excision. METHODS: All the patients who underwent re-operation. for delayed biliary complications after CDC excision between August 2007 and July 2020 were included in this retrospective study. The outcomes of these patients were compared with those who underwent primary surgery (CDC excision) at our institution. RESULTS: Of the total 40 patients with delayed biliary complications, 25 (62.5%) were female. Thirty-seven (92.5%) patients had a history of cholangitis. The median interval between CDC excision and the reoperation was 70 (4-216) months. The median duration of symptoms before reoperation was 12 (2.5-84) months. Re-do hepaticojejunostomy and direct hepaticojejunostomy were performed in 34 and in 6 patients respectively. Median operative time and blood losses were 219 min and 150 ml respectively. The median postoperative stay was 9 days. Postoperative complications developed in 10 (25%) patients. There was no operative mortality. Over a median follow-up of 71 months, a satisfactory outcome was achieved in 86% of patients. Restricture and intrahepatic stones developed in three and two patients respectively. Incidence of type IV cyst, cholangitis before operation, and operative blood loss were significantly more in the re-operative group. Clinical outcomes like the incidence of recurrent cholangitis, re-stricture, and postoperative hospital stay were comparable between the two groups. CONCLUSION: Surgery affords excellent results for majority of the patients with delayed biliary complications after CDC excision. Type IV cysts are more commonly associated with the development of delayed biliary complications.

Usefulness of hepatobiliary scintigraphy for predicting late complications in patients with choledochal cysts.

PURPOSE: Hepatobiliary scintigraphy is a minimally invasive imaging method that evaluates bile flow dynamics. At our hospital, it has been performed for postoperative evaluation of patients with choledochal cysts (CC). This study evaluated the usefulness of biliary scintigraphy for predicting late complications in patients with CCs. METHODS: The study included pediatric patients with CC who underwent surgery at Chiba University Hospital from 1978 to 2020, followed by postoperative biliary scintigraphy and subsequent radiologic evaluation. The patients were divided into two groups according to the presence or absence of "biliary cholestasis" on biliary scintigraphy. RESULTS: The study included 108 patients, with a median age at surgery of 2 years and 11 months. The median follow-up period was 5203 days, with 11 hepatolithiasis cases and 8 cholangitis cases. No patients had cholangiocarcinoma. Twelve patients were considered to have "cholestasis" following biliary scintigraphy evaluation. There was no significant difference in the occurrence of hepatolithiasis between the cholestasis and non-cholestasis groups (p = 0.47), but cholangitis was significantly more common in the cholestasis group (p = 0.016). CONCLUSION: Biliary cholestasis on postoperative hepatobiliary scintigraphy was a risk factor for cholangitis in patients with CCs. These particular patients should be monitored carefully.

Endoscopic view of the intrahepatic biliary ducts by direct per-oral cholangioscopy in a patient with a choledochal cyst.

Sixty year old female with hypertension and crampy abdominal pain episodes. Admitted to hospital (September-2020) by obstructive jaundice. MRCP: biliary dilation due to Todani Ic (fusiform) choledocal cyst (CC), distal sludge. ERCP: normal mucosa prominent papilla; biliary dilation compatible with CC; choledocholithiasis; 8-mm CHD filling defect. Sphincterotomy, removal of stones/sludge, brush-cytology of the filling defect (pathology: atypias). US: dilation resolution (CBD: 6.5 mm).

Comparison of Clinical and Radiologic Findings Between Perforated and Non-Perforated Choledochal Cysts in Children.

OBJECTIVE: To compare the clinical and radiologic findings between perforated and non-perforated choledochal cysts in children. MATERIALS AND METHODS: Fourteen patients (mean age ± standard deviation, 1.7 ± 1.2 years) with perforated choledochal cysts (perforated group) and 204 patients (3.6 ± 3.8 years) with non-perforated choledochal cysts (non-perforated group) were included between 2000 and 2019. All patients underwent choledochal cyst excision after ultrasound, CT, or MR cholangiopancreatography. Relevant data including demographics, clinical symptoms, laboratory findings, imaging findings, and outcomes were analyzed. Statistical differences were compared using the Mann-Whitney U test and Fisher's exact test. RESULTS: Choledochal cyst perforation occurred only in children under the age of 4 years. Acute symptoms, including fever (p < 0.001), were more common in the perforated group than in the non-perforated group. High levels of white blood cells (p = 0.004), C-reactive protein (p < 0.001), and serum amylase (p = 0.002), and low levels of albumin (p < 0.001) were significantly associated with the perforated group. All 14 patients with perforated choledochal cysts had ascites, whereas only 16% (33/204) of patients in the non-perforated group had ascites (p < 0.001). In the subgroup of patients who had ascites, a large amount of ascites (p = 0.001), increase in the amount of ascites in a short time (p < 0.001), complex ascites (p < 0.001), and perihepatic pseudocysts (p < 0.001) were more common in the perforated group than in the non-perforated group. CONCLUSION: Children with perforated choledochal cysts have characteristic clinical and radiologic findings compared to those with non-perforated choledochal cysts. In young children with choledochal cysts, perforation should be differentiated in cases with acute symptoms, laboratory abnormalities, and characteristic ascites findings.

Triple gallbladder with heterotopic gastric mucosa: a case report.

BACKGROUND: Triple gallbladder is a rare congenital anomaly of the biliary tract that can be associated with heterotopic tissue. Gallbladder triplication results from the failure of rudimentary bile ducts to regress during embryological development, and can be difficult to distinguish from Todani type II choledochal cysts and biliary duplication cysts. CASE PRESENTATION: A 2-year-old patient presented to our institution with intermittent abdominal pain for 1 year. She had elevated transaminases with imaging concerning for a choledochal cyst. After assessment with magnetic resonance cholangiopancreatography and endoscopic retrograde cholangiopancreatography, she was diagnosed with a gallbladder multiplication and a common bile duct stricture. She underwent laparoscopic cholecystectomy, which confirmed the diagnosis of triple gallbladder. One of the three gallbladders demonstrated heterotopic gastric mucosa on final pathology, including at the cystic duct margin. Follow up testing with a technetium 99 m scan demonstrated a subtle focus of increased activity in the right upper abdomen at the expected location of the common bile duct, concerning for the presence of residual gastric mucosa. The patient remains well without abdominal pain. CONCLUSIONS: We describe the first case of heterotopic gastric mucosa in a triple gallbladder in a young patient presenting with chronic abdominal pain. We also demonstrate the safety and feasibility of laparoscopic cholecystectomy in young children with triple gallbladder. Finally, we propose an interdisciplinary approach to the management of common bile duct strictures in the setting of ectopic acid secretion, involving a combination of medical management, endoscopic intervention, and possible salvage laparoscopic Roux-en-Y hepaticojejunostomy.

Short- and intermediate-term evaluation of the initial definitive operation for perforated choledochal cysts compared to two-stage management.

BACKGROUND: Recently, single definitive hepaticojejunostomy has been proposed to replace two-stage procedures, including initial urgent biliary drainage and final biliary reconstruction, with a reduced hospitalization time and corresponding overall hospitalization cost. We aimed to investigate the safety and efficacy of the initial definitive operation compared with the two-stage approach. METHODS: The medical records of patients with perforated CDCs managed between 2010 and 2017 were retrospectively reviewed. The qualified samples were divided according to a single definitive operation or two-stage approach. We conducted a comparison of the clinical characteristics, including surgical parameters, length of stay, and short- and intermediate-term complications. RESULTS: A total of 117 patients with perforated CDCs were reviewed, with 48 cases of single-stage management and 69 cases of two-stage management. No differences in the baseline characteristics between the two groups were found, such as age, sex distribution, ultrasound presentation, or laboratory findings. The initial definitive operation was associated with a lower total duration of drainage, including abdominal drainage and biliary drainage (p < 0.001), and rate of unplanned readmission (OR, 0.056; 95% CI 0.007-0.435; p < 0.001) than those who underwent two-stage management. Furthermore, the initial definitive operation significantly reduced the postoperative hospital stay (p < 0.001), and the overall hospitalization cost was accordingly reduced compared with two-stage management (p < 0.001). CONCLUSIONS: The initial single definitive operation was safe and effective for most of the patients with perforated CDCs when expertise was available. Therefore, a single definitive operation should be considered the treatment of choice for perforated CDCs.

Risk of carcinogenesis in the biliary epithelium of children with congenital biliary dilatation through epigenetic and genetic regulation.

PURPOSES: Congenital biliary dilatation (CBD), defined as pancreaticobiliary maljunction (PBM) with biliary dilatation, is a high risk factor for biliary tract cancer (BTC). KRAS and p53 mutations reportedly affect this process, but the mechanisms are unclear, as is the likelihood of BTC later in life in children with CBD. We investigated potential carcinogenetic pathways in children with CBD compared with adults. METHODS: The subjects of this study were nine children with CBD and 13 adults with PBM (10 dilated, 3 non-dilated) without BTC who underwent extrahepatic bile duct resections, as well as four control patients who underwent pancreaticoduodenectomy for non-biliary cancer. We evaluated expressions of Ki-67, KRAS, p53, histone deacetylase (HDAC) and activation-induced cytidine deaminase (AID) in the biliary tract epithelium immunohistochemically. RESULTS: The Ki-67 labeling index (LI) and expressions of KRAS, p53, HDAC, and AID in the gallbladder epithelium were significantly higher or tended to be higher in both the children with CBD and the adults with PBM than in the controls. CONCLUSIONS: BTC may develop later in children with CBD and in adults with PBM, via HDAC and AID expression and through epigenetic and genetic regulation.

Quiste de colédoco: nunca bajar la guardia / Choledocal cyst: never let your guard down

El quiste del colédoco es una dilatación congénita de la vía biliar. La presentación clínica es inespecífica y a menudo supone un dilema diagnóstico. Se debe tener un elevado índice de sospecha ante casos de ictericia, dolor abdominal y masa abdominal palpable. Para su diagnóstico es fundamental un estudio de imagen, siendo el indicado de forma inicial la ecografía. Pueden presentar múltiples complicaciones, incluyendo colangitis, pancreatitis, colangiocarcinoma, cirrosis biliar y hepática. Para su manejo se recomienda la resección del quiste, para evitar complicaciones y disminuir el riesgo de malignización. Se recomienda un seguimiento a largo plazo, con ecografías y análisis anuales, dado que el riesgo de malignización se mantiene elevado incluso tras la escisión del quiste. El abordaje óptimo de esta patología requiere un enfoque multidisciplinar, desde la sospecha diagnóstica que a menudo procederá de los servicios de Atención Primaria, incluyendo también gastroenterólogos, cirujanos, patólogos y radiólogos (AU)

Bile duct cyst is a congenital dilation of the bile duct. Clinical presentation is non-specific and often poses a diagnostic dilemma. It should be highly suspected in cases of jaundice, abdominal pain and a palpable abdominal mass. An imaging study is essential for diagnosis, an ultrasound being the most suitable initially. It can present multiple complications, including cholangitis, pancreatitis, cholangiocarcinoma, biliary and hepatic cirrhosis. For its management, resection of the cyst is recommended to avoid complications and reduce the risk of malignancy. Long-term follow-up with annual ultrasound scans and tests is recommended, as the risk of malignancy remains high even after excision of the cyst. The optimal approach to this pathology requires a multidisciplinary perspective, starting from the diagnostic suspicion that will often come from Primary Care services, and including also gastroenterologists, surgeons, pathologists and radiologists (AU)

Laparoscopic management of type VI choledochal cyst with common bile duct stone: report of a case and review of literature.

A young adult male presented with biliary colic and intermittent jaundice for 1 year. Abdomen findings were unremarkable. Routine investigations revealed a raised total bilirubin. On abdominal ultrasonography, common bile duct (CBD) dilatation with multiple stones was noted. On further imaging with magnetic resonance cholangiopancreatography, type I choledochal cyst (CDC) was suspected. A laparoscopic approach was planned. Intraoperatively, dilatation of cystic duct was noted which constitute type VI CDC. Partial malrotation of the gut and accessory right hepatic artery were also noted as incidental finding. Laparoscopic cholecystectomy with CBD exploration and removal of stones, biliary stent placement, cystic duct cyst excision and primary repair of CBD was done. Postoperatively, the patient improved symptomatically with a fall in bilirubin to normal range. We are describing the laparoscopic management of a rare case of type IV CDC which was diagnosed intraoperatively.

Choledochal cyst with secondary cholangitis, choledochitis, duodenal papillitis, and pancreatitis in a young domestic shorthair cat.

Choledochal cysts, congenital segmental dilations of the common bile duct, have been reported in few cats, and histologic characterization is lacking. A 20-mo-old spayed female domestic shorthair cat was presented because of vomiting and weight loss. There was progressive elevation of liver enzyme activity (ALT > ALP, GGT) and hyperbilirubinemia. Diagnostic imaging identified focal cystic dilation of the common bile duct, dilation and tortuosity of adjacent hepatic ducts, and a prominent duodenal papilla. A choledochal cyst was suspected, and the animal was euthanized. On postmortem examination, there was a 2-cm, firm, thickened, cystic dilation of the common bile duct, patent with adjacent ducts. Histologically, the cyst wall was expanded by fibroblasts, collagen, and lymphoplasmacytic inflammation. Adjacent bile ducts were markedly dilated and tortuous, with lymphoplasmacytic inflammation and papillary mucosal hyperplasia that extended to the major duodenal papilla. There was chronic neutrophilic cholangitis, suggesting bacterial infection and/or disturbed bile drainage, extrahepatic obstruction, and lymphoplasmacytic pancreatitis with ductular metaplasia. Prominent lymphoid follicles within biliary ducts and duodenum suggested chronic antigenic stimulation. Choledochal cysts can be associated with chronic neutrophilic cholangitis, extrahepatic obstruction, choledochitis, duodenal papillitis, and pancreatitis, and should be a differential for increased hepatic enzymes and hyperbilirubinemia in young cats.