Incidental intraoperative finding of gallbladder duplication in a patient with a choledochal cyst.

Here, we report a case of coexistence of a duplicate gallbladder and choledochal cyst, which was overlooked preoperatively and confirmed via intraoperative inspection and pathological examination. We concluded that a high index of suspicion is required to diagnose a double gallbladder, and special attention should be paid to preoperative radiological imaging when treating patients with choledochal cysts because these patients present a higher incidence of biliary anomalies.

Meandering Main Pancreatic Duct in Association with Choledochal Cysts and Acute Pancreatitis in Pediatrics.

Meandering main pancreatic duct (MMPD) is an uncommon anomaly of the main pancreatic duct characterized by an abnormal curvature at the pancreatic head region. This variant can be diagnosed on imaging, particularly magnetic resonance cholangiopancreatography (MRCP). Although its clinical significance remains debatable, recent research suggests an association with recurrent acute pancreatitis. To our knowledge, no pediatric cases of acute or recurrent acute pancreatitis have been attributed to the presence of MMPD. In this article, we report on two patients. The first case is of a 15-year-old girl with MMPD discovered on investigation of idiopathic acute pancreatitis. The second case is of a 5-year-old boy who presented with his second episode of acute pancreatitis. In this patient, MRCP imaging revealed MMPD and type IVA choledochal cyst. With appropriate care, both patients experienced clinical improvement with resolution of abdominal pain. This article highlights MMPD as a distinct entity that should be considered in pediatric patients with recurrent attacks of acute pancreatitis. This report also describes the first association of MMPD with choledochal cysts. [Pediatr Ann. 2019;48(10):e412-e416.].

Polypoid Undifferentiated Carcinoma With Osteoclast-like Giant Cells Arising in the Distal Common Bile Duct: A Rare Case Report.

BACKGROUND: Undifferentiated carcinoma with osteoclast-like giant cells (UC-OGC) in distal common bile duct (CBD) is a rare entity. CASE REPORT: This case report describes a 45-year-old male with a history of a choledochal cyst status post partial excision and cholecystectomy who presented with a mass in the remaining distal/intrapancreatic common bile duct. It was initially mistaken for post-surgery hematoma; however, the rapid growth raised concern for malignancy, and prompted a pancreaticoduodenectomy (Whipple) procedure. Macroscopic examination revealed a 5.5 cm polypoid mass grossly confined in the lumen of the distal CBD. Histology was consistent with UC-OGC, with minimal invasion into the polyp stalk and adjacent CBD wall. Immunohistochemistry demonstrated co-expression of CK7 and p40, normal/wild-type p53, and retained SMAD4 expression in tumor cells. Next-generation sequencing detected mutations at p.Q61H (c.183A>C) of KRAS and p.E545K (c.1633G>A) of PIK3CA, keeping in line with similarity to conventional cholangiocarcinoma. The patient remained disease-free after two years of follow-up without chemotherapy. CONCLUSION: To our knowledge, this is the first case report of UC-OGC presented as a polypoid mass in the distal CBD. It highlights the complex dynamism and controversial pathogenesis of this unique entity, which should be made aware to avoid diagnostic pitfalls.

Proper bile duct flow, rather than radical excision, is the most critical factor determining treatment outcomes of bile duct cysts.

BACKGROUND: The purpose of this study was to compare the impact of the extent of excision and the patent bile duct flow on treatment outcomes of bile duct cysts (BDCs). METHODS: We retrospectively analyzed the records of 382 patients who received surgery for BDCs from January 2005 to December 2014. RESULTS: For Type Ia cysts, proper bile flow was associated with good long-term treatment outcomes with a greater level of significance (p < 0.001) than complete excision (p = 0.012). For Type IVa cysts, proper bile flow, but not complete excision, was associated with good long-term outcomes (p < 0.00001). In addition, 96.3% (104/108) of Type IVa patients with proper bile flow had no late complications and good biliary function, while no patient without patent bile flow had a good clinical outcome. For Type Ic cysts, 92 patients who received partial excisions had good outcomes when proper bile flow was restored. Regression analysis revealed that the absence of proper bile flow, in comparison to incomplete excision, is a greater risk factor for poor long-term treatment effects for Type Ia and Type IVa cysts. CONCLUSIONS: Compared to complete excision, the establishment of proper bile flow exerted a greater impact on improving long-term clinical outcomes after BDC surgery.

Different clinical presentations of choledochal cyst among infants and older children: A 10-year retrospective study.

Choledochal cyst is a rare and often benign congenital cystic dilation throughout the biliary tree. Due to the benign nature of choledochal cyst among early-diagnosed patients, the clinical assumption and diagnosis seem to be of utmost significance. Therefore, we sought to assess different clinical manifestations of choledochal cyst and relevant laboratory findings in infants and older children.Retrospectively, medical records of all patients with the diagnosis of choledochal cyst between 2005 and 2015 were reviewed. Demographic data, initial clinical presentation, positive findings on physical examination, history of any remarkable behavior such as persistent and unexplained crying and poor feeding, and diagnostic imaging modalities were listed. In addition, laboratory values for total and direct bilirubin, alkaline phosphatase, alanine transaminase, aspartate transaminase, prothrombin time, and partial thromboplastin time (PTT) were recorded for each patient. Patients were divided into 2 groups; younger than 1-year-old (infants), and 1 year to 18 years old (older children). Demographic data, clinical data, and laboratory values were compared between the infants and older children.Thirty-two patients with a diagnosis of choledochal cyst were included in the study: 9 patients (28.12%) were infants and 23 patients (71.87%) were older children. Abdominal pain was the most common presenting symptom (62.5%), followed by nausea/vomiting (59.4%) and jaundice (28.1%). None of the patients presented with the classic triad of abdominal pain, jaundice, and right upper quadrant mass. Seventeen older children (73.91%) presented with nausea and vomiting, while 2 subjects (22.22%) in the infantile group presented with this feature (P = .01). Similarly, abdominal pain was found in 20 older children (86.95%); however, none of the infants presented with abdominal pain at diagnosis (P < .001). By contrast, the abdominal mass was more detected in infants than the older children (33.33% vs. 0%, P = .01). In terms of laboratory values, the median PTT was 44 and 36 s in infants and older children, respectively (P = .04).Infants were more likely to present with abdominal mass and older children were more likely to have nausea, vomiting, and abdominal pain. Furthermore, infants had more prolonged PTT than older children, implying a potential bleeding tendency.

Acute pancreatitis complicating choledochal cysts in children.

AIM: To analyse the characteristics of patients with choledochal cysts presenting with acute pancreatitis. METHODS: Multicenter retrospective review of all paediatric patients (<18 years) with choledochal cysts managed over a 14-year period (2001-2014) at two tertiary paediatric surgical centres. Patient data were analysed for demographics, presentation, radiological classification of cyst type (Todani), operative interventions, complications and long-term follow-up. RESULTS: A total of 49 patients with choledochal cysts were identified with 15 (31%) being Type I fusiform, 18 (37%) Type I cystic and 16 (32%) Type IV-A. Seventeen (35%) patients presented with acute pancreatitis, one having had an ante-natally diagnosed choledochal cyst. Patients presenting with pancreatitis were older when compared to the non-pancreatitis group (5.1 vs. 1.2 years, P = 0.005). Nine out of 16 (53%) patients with Type IV-A cysts presented with pancreatitis compared to five (33%) of Type I fusiform and three (17%) of Type I cystic. There was however no statistically significant association between Todani types and the development of pancreatitis (Type I fusiform, P = 1.0; Type I cystic, P = 0.063; Type IV-A, P = 0.053). The rate of complications was similar in both groups. CONCLUSION: Pancreatitis was a common presentation in children with a choledochal cyst, however, there was no clear statistically significant association with Todani types and pancreatitis.

Laparoscopic treatment of congenital choledochal cyst and hepaticojejunostomy with extracorporeal Roux-en-Y anastomosis: technical aspects and early experience with three cases.

Choledochal cyst (CDC) is a congenital dilatation of the extra and/or intrahepatic bile ducts and it is a rare condition in western countries. Classical treatment consists of cyst excision and hepaticojejunostomy. The first case of a laparoscopic CDC excision was described in 1995 and since that time an increasing number of institutions have adopted this technique, with good success. We describe our early experience of 3 cases of CDC treated with laparoscopic approach. We used a 10 mm umbilical port for the camera, and four 3-5 mm operative ports. We performed the laparoscopic removal of the cyst and gallbladder, videoassisted preparation of the Roux-en-Y loop and laparoscopic hepaticjejunostomy. No post-operative complications occurred. Laparoscopic excision of CDCs has been supposed to give better observation, a better cosmetic result, potentially less postoperative pain, and a shorter recovery. The main argument for performing an extracorporeal anastomosis is that it decreases the operative time. We recommend caution to prevent injury to the pancreatic duct and biliary structures during dissection and anastomosis. Lifelong surveillance is mandatory, even after resection of the choledochal cyst.

Clinical differences between choledochal cysts in infancy and childhood: an analysis of 160 patients.

BACKGROUND: Choledochal cysts (CDC) can become symptomatic at any age. Clinical features of infants and older children are different and have been documented by many authors. OBJECTIVE: The aim of this report was to study the differences in clinical spectrum of CDC between the infants and children treated at our hospital during a 17-year period. MATERIAL AND METHOD: A retrospective study was conducted of 160 patients with CDC treated at Queen Sirikit National Institute of Child Health between 1996 and 2012. The patients were categorized into 2 groups based on age at clinical presentation: an infantile group (up to one year old) and a childhood group (over one year old). Clinical characteristics of patients in the infantile and childhood groups were compared with statistical analysis using the Chi-square and Fisher's exact test. RESULTS: Of the 160 patients with CDC, 48 cases (30%) were categorized in the infantile group and 112 cases (70%) in the childhood group. Over three-quarters ofthe patients in the infantile group presented with jaundice and acholic stool, and this incidence was significantly higher than in the childhood group (77% vs. 46.4%, p = 0.001 and 50% vs. 13.4%, p < 0.001). Abdominal pain was the most common symptom of patients in the childhood group, whereas it was noted in only a small number of those in the infantile group (82.1% vs. 8.3%, p < 0.001). Average amylase level in CDC content was markedly elevated in the childhood group but much lower in the infantile group (43,630.5±90,234.5 vs. 79±189.9, p < 0.001). Only type I and type IV CDC as defined by Todani's classification were found in our patients, and there were no statistical differences in incidences of type I and type IV in the two groups (79.2% vs. 67.8% and 20.8% vs. 32.2% p > 0.05). Neonates and infants with CDC had a significantly higher risk of liver cirrhosis than did the childhood group (25% vs. 8%, p < 0.001). However, surviving patients with cirrhosis in both groups were doing well at least 3 years after surgical CDC excision. CONCLUSION: Neonates and infants with CDC were more likely to present with jaundice and acholic stool, whereas older children were more likely to present with abdominal pain. Amylase level in CDC content was markedly elevated in the childhood group but at a much lower level in the infantile group. Neonates and infants with CDC tended to develop liver cirrhosis earlier and more often than older children.

Transcriptome profiling of biliary atresia from new born infants by deep sequencing.

Biliary atresia is the major kind of liver disease that mainly affects the new born infants. The pathological and biological mechanism of biliary atresia is still unclear to date. In this work, we attempt to identify biliary atresia relevant genes and to get the knowledge of the underlying genetic basis. We collected liver samples from new born infants with biliary atresia and congenital choledochocyst, and the RNA-seq technology was used to performed a transcriptome profiling in order to comprehensively study their expression signatures. We identified 877 differentially expressed genes between samples from biliary atresia and congenital choledochocyst patients in total. Several biological pathways related to the immunity and inflammation response were found to involve in the development of biliary atresia. Our results may helps to better investigate the molecular mechanisms of this disease.

Quiste de colédoco tipo II intrapancreático del adulto malignizado: duodenopancreatectomía / Type II intrapancreatic choledochal malignant cyst in adults: Duodenopancreatectomy

Presentamos el caso de una paciente de 62 años que ingresó de urgencias por pancreatitis aguda leve. En las pruebas de imagen se observó una lesión sólido-quística en la cabeza de páncreas comunicante con la vía biliar distal, y se diagnosticó quiste de colédoco tipo II de Todani con degeneración neoplásica en su interior confirmada tras una biopsia por punción en una ecoendoscopia. La paciente fue tratada con una duodenopancreatectomía cefálica con intención curativa

A 62-year-old female patient was admitted for abdominal pain and vomiting. Imaging tests revealed a solid-cystic lesion at the head of the pancreas communicating with the distal bile duct. A Todani type II choledochal cyst was diagnosed with neoplastic degeneration after cytological diagnosis with endoscopic ultrasound-guided puncture. The patient was treated with a cephalic duodenopancreatectomy with curative intention (AU)

Pancreatitis aguda recidivante como complicación a largo plazo del quiste congénito de colédoco operado / Recurrent acute pancreatitis as a long-term complication of congenital choledochal cyst surgery

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Choledochoceles: are they choledochal cysts?

The classification of choledochoceles as a type of choledochal cyst stems from the 1959 article by Alonso-Lej and colleagues describing 94 choledochal cysts, only 4 of which were choledochoceles. Even then, Alonso-Lej questioned the propriety of including the choledochocele, stating it was unclear "as to whether or not it originates from the same etiologic factors [as other choledochal cysts]". In 1971, Trout and Longmire also questioned the validity of classifying choledochoceles as choledochal cysts, noting the anatomic position article and variant mucosa of the choledochocele. Wearn and Wiot, in an article titled "Choledochocele: not a form of choledochal cyst", cite the differences in clinical presentation, demographics, and histology as reasons why choledochoceles represent separate entities from choledochal cysts. Over the ensuing decades, numerous investigators have questioned the legitimacy of classifying choledochoceles as choledochal cysts. In our recent series (the only one to our knowledge directly comparing patients with choledochocele and other [type I, II, IV, and V] choledochal cysts), patients with choledochoceles differed from patients with choledochal cysts in their age, gender, presenting symptoms, history of previous cholecystectomy, pancreatobiliary ductal anatomy, management, and most importantly, propensity to developing biliary malignancy. Based on the available cases of choledochoceles found in the literature, combined with the recent series from our institution, we conclude that choledochoceles seem to be distinct entities from choledochal cysts.

Choledochal cysts: differences between pediatric and adult patients.

Choledochal cysts in children and adults are believed to be different, but direct comparison between them is lacking in the literature. This study was aimed to identify the clinicopathological differences between 42 children and 59 adults with choledochal cyst treated by same surgeons at the Cathay General Hospital. The mean follow-up period was 8.9 years. The result showed that the female-to-male ratios were 1.5:1 in pediatric patients and 4.9:1 in adult patients. Compared with adults with choledochal cyst, the pediatric patients presented more abdominal mass (52.4% vs 21.2%, P = 0.002) and less abdominal pain (76.2% vs. 98.0%, P = 0.002), are more frequently associated with anomalous pancreaticobiliary ductal union (85.7% vs. 59.6%, P = 0.005) and sudden severe stenosis of terminal choledochus (76.2% vs. 42.3%, P = 0.001), are less commonly associated with choledocholithiasis, are not associated with malignant transformation (0% vs 21.2%), and have fewer perioperative and long-term complications. Nevertheless, patients who received total excision had fewer surgical complications in both groups. This result shows that choledochal cysts in pediatric and adult patients are different in clinicopathological manifestations, prognosis, and the underlying abnormalities of the pancreaticobiliary system, suggesting that patients with choledochal cyst should be managed according to these differences.

The perinatal transition of the hepatobiliary cyst size provides information about the condition of bile flow in biliary cystic malformation cases.

PURPOSE: The aim of this study was to understand the level of bile flow by perinatal transitions of the hepatobiliary cyst size in biliary cystic malformation (BCM) cases and to examine the association between the transitions of the cyst size, cholangiographic images of the intrahepatic bile ducts (IHBDs), and histological features of the liver specimens in BCM. METHODS: We measured perinatal size of the hepatobiliary cyst in 7 BCM cases, composed of 1 case with choledochal cyst and 6 cases with biliary atresia (BA). Bile excretion was inferred from the transition of cyst size and the postoperative course. The IHBD cholangiographic images were also examined. In addition, histology of liver specimens obtained at the time of initial surgery was evaluated for IHBD maturity and fibrosis. RESULTS: The size of the small cyst in 2 BA cases remained almost unchanged throughout gestation. These cases required longer postoperative period for the clearance of jaundice. Their IHBD images were indistinct, and histology showed severe fibrosis. The ratio of bile ducts to portal tracts was 0.5 in 1 of the cases. However, in other BCM cases where the cysts grew large perinatally, bile excretion was good postoperatively, and their jaundice cleared in shorter period. Their cholangiographic images demonstrated clearer periphery of the IHBDs, and their histology showed mild or moderate fibrosis, if any. CONCLUSIONS: The perinatal transition of the cyst size in BCM cases can be helpful in assessing the level of bile flow. BCM with a small cyst during gestation can include some BAs with poorer bile flow, vaguer IHBD images, and prominent liver fibrosis. For the treatment of BCM cases, especially patients with a small biliary cyst, prompt response may be required after birth.

Under pressure: choledochal malformation manometry.

PURPOSE: The cause of choledochal (cystic or fusiform) malformation is not known. A favoured hypothesis suggests that abnormal reflux of activated pancreatic secretions via a common pancreatobiliary channel may initiate mucosal injury and mural weakness leading to bile duct dilatation, at normal intraduct pressures. However, bile duct pressures in both normal or disease states are not known in such children. METHODS: Intraoperative choledochal pressure (CP) measurements were made before any other manipulation. Bile was cultured and its amylase content measured. Biochemical liver function (bilirubin, aspartate aminotransferase, gamma-glutamyl transpeptidase, and alkaline phosphatase) was measured. Data were quoted as median (interquartile range). Statistical tests were parametric, where appropriate, and P = .05 was regarded as significant. RESULTS: Twenty-five children (age 2.5 [1.25-5.91] years) with choledochal (cystic [n = 13] and fusiform [n = 12]) malformation coming to surgery were studied. Median CP was 13 (8.5-17) mm Hg. Median bile amylase was 6722 (241-18,000) IU/L. Choledochal pressure inversely correlated with bile amylase (r = -0.60, P = .001), serum aspartate aminotransferase (r = 0.46, P = .01), and log gamma-glutamyl transpeptidase (r = 0.4, P = .04) but not with bilirubin (P = .11), alkaline phosphatase (P = .20), or age (P = .11). No difference in CP, bile amylase, or liver biochemistry could be identified between the 2 biliary phenotypes. All bile cultures were sterile. CONCLUSIONS: Increased CP is inversely related to the level of bile amylase (and hence degree of the functional common channel). This suggests that obstructive stenosis at the level of the pancreatobiliary junction (but not the ampulla) may be a causal factor in a proportion of choledochal malformations.