Giant choledochal cyst and infantile polycystic kidneys as prenatal sonographic features of Caroli syndrome.

Caroli syndrome is a developmental disorder caused by complete or partial arrest of ductal plate remodeling, leading to dilated bile ducts along with fibrosis surrounding the portal tracts. It is most commonly associated with autosomal recessive polycystic kidney (ARPKD). We report a unique case of Caroli syndrome, diagnosed prenatally at 24 weeks of gestation in a 29-year-old Thai woman. Ultrasound findings revealed the association of a fetal giant choledochal cyst with ARPKD. Autopsy findings showed ductal plate malformation, typical of Caroli syndrome, associated with giant choledocal cyst and ARPKD.

Separate pancreatic ducts draining into a type IV choledochal cyst: simultaneous anatomical variants in a child suffering from cholelithiasis and acute pancreatitis.

The biliary tree and the pancreatic ducts originate from different buds of the primitive foregut. A plethora of anatomical variants and congenital anomalies have been described for both the biliary and the pancreatic duct systems, although they are usually asymptomatic. However, in some cases, these abnormalities can lead to serious clinical scenarios, especially when involving the biliopancreatic junction, with consequent mixing and reflux of the two different juices. To the best of our knowledge, we present the first case of pancreas divisum ducts draining into a choledochal cyst, with the lower third of the choledochus configuring a common biliopancreatic duct, in a child suffering from cholelithiasis and acute pancreatitis.

Treatment and outcomes of adults with remnant intrapancreatic choledochal cysts.

BACKGROUND: The purpose of this study is to report our experience with the treatment of remnant intrapancreatic choledochal cysts (CCs) in adults. METHODS: We reviewed retrospectively the records of patients with intrapancreatic remnant CCs who underwent reoperation by our surgical team from January 2000 to December 2012. Postoperative complications, surgical outcomes, and rate of malignancy were noted. RESULTS: A total of 41 patients were included: 35 without malignant transformation after the primary operation and 6 with malignant transformation. After the primary operation, all patients experienced cholangitis, and more than one-half had cholangiolithiasis (66%), pancreatitis (51%), and vomiting (51%). The mean age of the 35 patients (9 males and 26 females) was 49 years (range, 27-69), and the majority had originally a Todani type I cyst (74%). Most patients (74%) had undergone previously subtotal extrahepatic cyst excision and Roux-en-Y hepaticochojejunostomy. After excision of the remnant cyst, the rate of an excellent or good outcome was obtained in 91% of patients. The mean age of the patients with malignant transformation was 51 years (range, 35-70), and the rate of malignancy was 15% (6/41). The average time between the original incomplete CC excision and the diagnosis of malignancy was 140 months (range, 52-265). Four patients underwent pancreatoduodenectomy, and 2 underwent palliative surgery because the lesions were invading adjacent tissues. Five patients died of their disease within 37 months after reoperation. CONCLUSION: Complications seem to be common after incomplete resection of a CC with remnant intrapancreatic CCs, and the malignancy rate is relatively high. To improve outcomes, all attempts at complete resection of the intrapancreatic portion of CCs should be made at the time of the primary operation.

Quiste de colédoco mixto (tipo I y II) asociado a malformación de la unión pancreatobiliar. Descripción de un caso y revisión de la literatura / Mixed choledochal cyst (type I and II) associated with a malformation of the pancreatobiliary junction. A case report and review of the literatura

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Adult choledochal cysts: current update on classification, pathogenesis, and cross-sectional imaging findings.

Approximately 20% of choledochal cysts (CC) present in adult patients and they are commonly associated with a high risk of complications, including malignancy. Additionally, children who underwent internal drainage procedures for CCs can develop complications during adulthood despite treatment. Concepts regarding classification and pathogenesis of the CCs have been evolving. While new subtypes are being added to the widely accepted Todani classification system, simplified classification schemes have also been proposed to guide appropriate management. The exact etiology of CCs is currently unknown. The two leading theories involve either the presence of an anomalous pancreatico-biliary junction with associated reflux of pancreatic juice into the biliary system or, more recently, some form of antenatal biliary obstruction with resulting proximal bile duct dilation. Imaging studies play an important role in the initial diagnosis, surgical planning, and long-term surveillance of CCs.

Biliary cysts: a review and simplified classification scheme.

Biliary cystic disease has been known of for centuries. It has traditionally been classified as 5 major types of disease, each with different clinical profiles and attributes. In this article, the basis for the existing classification schemes is reviewed and a simplified classification scheme and treatment regimen are suggested.

Double common bile duct with choledochal cyst and cholelithiasis: report of a case.

Double common bile duct (DCBD) is a rare congenital anomaly of the biliary system, often associated with biliary lithiasis, choledochal cyst, pancreaticobiliary maljunction (PBM), and upper gastrointestinal tract malignancies. We report a case of type I DCBD with choledochal cyst and cholelithiasis in a 52-year-old Chinese man. We also reviewed 24 cases of DCBD reported in the Chinese literature between 1965 and 2012. Most (58.3%) of these cases were classified as type I DCBD, with accompanying choledocholithiasis in 79.2%, cholecystolithiasis in 37.5%, choledochal cyst in 33.3%, and PBM in 8.3%. There was no case of concomitant cancer. The type and coexistence of PBM with DCBD are clinically important because of its close implications with concomitant pathology. Most Chinese people with DCBD have type I. Moreover, the high incidences of choledochal cyst and biliary lithiasis and the extremely low incidences of PBM and biliary cancer are the major clinical characteristics of DCBD in China.

Role of laparoscopy during surgery at the porta hepatis.

Minimally invasive surgery in children has evolved to the extent that complex procedures can be performed with safety, with comparable outcomes to open surgery and with the advantages of minimal scarring and less pain. In this article, we describe the latest laparoscopic techniques used at Juntendo University Hospital in Japan, for treating conditions affecting the porta hepatis, focusing on biliary atresia and choledochal cysts. We also summarise our postoperative management protocols and discuss preliminary outcomes.

Delayed presentation of complete pancreatic ductal transection in children: management of two cases without resection.

Pancreatic ductal injuries in children are rare, and ductal transections presenting in a delayed or subacute fashion are seldom reported. We describe two cases of traumatic pancreatic ductal transection secondary to physical abuse, both of which presented late to medical care. Both were managed successfully without pancreatic resection. Judicious application of non-resectional management can yield favorable outcomes in this subset of pediatric patients.

Solving difficult hepatobiliary problems in children.

Most difficult hepatobiliary (HPB) problems in infancy and childhood result from pathological anatomical/mechanical derangements; therefore, surgery on the liver and bile ducts depends on a detailed understanding of liver structure, function and repair response to injury or disease. The surgeon must be aware of the very diverse range of anatomical variations. Perhaps key to improving the outcome of paediatric HPB surgery is centralised management and associating this with a paediatric liver transplant programme, which adds expertise and, frequently, the added benefit of adult HPB surgical input to paediatric surgical care. In the United Kingdom, this has resulted in excellent measurable benefit, particularly in the management of biliary atresia, but also of choledochal cysts, portal hypertension and liver tumours. These conditions are briefly discussed here, with focus on the technical aspects of operative management.

Biliary cysts: etiology, diagnosis and management.

Biliary cysts (BC) are rare dilatations of different parts of a biliary tract. They account for approximately 1% of all benign biliary diseases. BC occur the most frequently in Asian and female populations. They are an important problem for pediatricians, gastroenterologists, radiologists and surgeons. Clinical presentation and management depend on the BC type. Cholangiocarcinoma is the most serious and dangerous BC complication. The other complications associated with BC involve cholelithiasis and hepatolithiasis, cholangitis, acute and chronic pancreatitis, portal hypertension, liver fibrosis and secondary liver cirrhosis and spontaneous cyst perforation. Different BC classifications have been described in the literature. Todani classification dividing BC into five types is the most useful in clinical practice. The early diagnosis and proper treatment are very important, because BC are associated with a risk of carcinogenesis. A malignancy risk increases with the age. Radiological investigations (ultrasonography, computed tomography, endoscopic retrograde cholangiopancreatography and magnetic resonance cholangiopancreatography) play an important role in BC diagnostics. Currently, prenatal diagnosis using ultrasonography is possible. It allows to differentiate biliary disorders in fetals and to perform the early surgical treatment that improves results. In most patients, total cyst excision with Roux-Y hepaticojejunostomy is the treatment of choice. Surgical treatment of BC is associated with high success rate and low morbidity and mortality. The early treatment is associated with a lower number of complications. Patients following BC surgery require permanent and careful postoperative observation using laboratory and imaging investigations because of possibility of biliary anastomosis stricture and biliary cancer in tissue remnant.

Choledochal cysts : a review of literature.

Choledochal cysts are cystic dilation of extrahepatic duct, intrahepatic duct, or both that may result in significant morbidity and mortality, unless identified early and managed appropriately. The incidence is common in Asian population compared with western counterpart with more than two third of the cases in Asia being reported from Japan. The traditional anatomic classification system is under debate with more focus on etiopathogenesis and other aspects of choledochal cysts. Even though categorized under the same roof, choledochal cysts vary with respect to their natural course, complications, and management. In this review, with the available literature on choledochal cysts, we discuss different views about the etiopathogenesis along with the natural course, complications, diagnosis, and surgical approach for choledochal cysts, which also explains why the traditional classification is questioned by some authors.

Duodenal duplication cyst: a potentially malignant disease.

BACKGROUND: Duodenal duplication cysts constitute a rare congenital anomaly of the gastrointestinal tract. A recent meta-analysis of the literature between 1999 and 2009 reported a total of 47 cases of duodenal duplication cysts.1 These abnormalities are mostly diagnosed in infancy and childhood. In rare cases, they can remain asymptomatic until adulthood, and 38 % of patients are diagnosed after age 20 years.1 (,) 2 Duodenal duplication cysts are generally benign lesions; nevertheless, three cases of malignant tumours arising inside have been reported.3 (-) 5 METHODS: In this multimedia article, we illustrated the case of an 18 year-old female patient presenting with recurrent episodes of mild pancreatitis. MRI revealed a cystic structure measuring 2.5 cm in diameter located in the duodenal wall next to the papilla of Vater. Endoscopic ultrasound showed a cystic lesion cephalad to the papilla, protruding into the duodenal lumen. Endoscopic retrograde cholangiopancreatography was not feasible due to the dislocation of the papilla, whose macroscopic aspect was normal. To further elucidate the anatomical relations, 3D reconstruction of the MRI images was performed. There was neither dilatation of the biliary tract nor a visible communication between the common bile duct and the cystic structure. The pancreatic duct also was at distance. Those findings were suggestive of a duodenal duplication. Nevertheless, the differential diagnosis6 of a choledochocele (Todani III) could not be formally excluded. Indication for surgical resection was symptomatic disease in a context of potential malignancy. RESULTS: By right subcostal incision (video), surgical exploration revealed a soft tissue mass palpable at the second portion of the duodenum. Following duodenotomy, the mucosa was incised cephalad to the papilla of Vater, which could previously be localized by methylene blue injection by a catheter inserted into the cystic duct. The cystic structure was dissected and no communication between the cyst and the biliary tract was individualized. The final diagnosis was made by histological examination showing duodenal duplication. There was neither heterotopic gastric mucosa nor excreto-biliary epithelial layer. There were no signs of malignancy. The postoperative course was marked by hematemesis externalised by the nasogastric tube. We reintervened at postoperative day 2 to ensure hemostasis. A clot was removed from the area of duodenal mucosa without any visible active bleeding. Further recovery was uneventful; the patient was discharged at postoperative day 10 and is actually asymptomatic. DISCUSSION: The ideal treatment of duodenal duplication cysts is complete surgical resection.7 Due to proximity to the bilio-pancreatic duct, total resection sometimes requires pancreaticoduodenectomy. This major surgical procedure entails the disadvantages of high morbidity and mortality with poor quality of life. In our opinion, this procedure should remain an ultimate option. Less invasive approaches have been proposed, including partial resection or internal derivation.7 Marsupialization is a surgical approach that has been accomplished even endoscopically.1 Nevertheless, these techniques do not provide total resection and leave the risk of degenerescence. As cases of malignancy are reported, we decided to realize a complete surgical excision of the lesion. Three-dimensional reconstruction of the biliary anatomy is an innovative procedure, which allowed us to show the absence of any communication between the cyst and either the common bile duct or the pancreatic duct.8 So, the surgical approach could be specified preoperatively ensuring the integrity of the common bile duct. Duplication cysts could be connected to the pancreaticobiliary ducts in about 29 %.1 Subsequent realization of a total surgical excision combined the advantages of complete resection with minimal invasiveness. CONCLUSIONS: For relieving symptoms and preventing further complications, such as pancreatitis or malignant transformation, surgical resection of duodenal duplication cysts is indicated. In cases of difficulties to individualize the neighboring anatomical structures preoperatively, 3D reconstruction is a helpful approach to determine the surgical strategy. Enucleation allows a total excision while minimizing the adverse effects and therefore it is our treatment of choice for duodenal duplication cysts without communication.

Quistes de colédoco: un diagnóstico inadvertido. Serie de casos / Choledochal cysts: an inadvertent diagnosis. Case series

Las lesiones quísticas de vías biliares, constituyen una entidad, que rara vez se observa o diagnostica en el adulto. El motivo de este trabajo es mostrar la experiencia de un centro de referencia biliopancreática en el diagnóstico y tratamiento de quistes de colédoco. Usamos la clasificación de Todani para la descripción de los mismos. Fue un estudio Descriptivo y Retrospectivo con 502 colangiopancreatografías retrógradas endoscópicas y de ellas 20 casos de quistes de colédoco identificados mediante colangiografías de alta calidad.

Cystic lesions of the bile ducts constitute an entity that is rarely seen or diagnosed in adults. The purpose of this study is to show the experience of a biliopancreatic referral center in the diagnosis and treatment of choledochus cysts. The Todani classification was used to describe them. It was a retrospective, descriptive study of 502 endoscopic retrograde cholangiopancreatographies and of these 20 cases of choledochal cysts were identified by high-quality cholangiographies.

Biliary disease in children.

Biliary diseases in children are infrequent; however, they can be associated with high morbidity and mortality if an accurate diagnosis is not made and adequate treatment provided in a timely fashion. Biliary atresia, choledochal cysts, gallbladder disease, and Alagille syndrome can be associated with similar clinical symptoms, laboratory findings, and radiographic findings, which makes accurate diagnosis difficult. The correct treatment for each of these clinical entities is different and can significantly reduce morbidity and mortality from these diseases. In this article, we discuss the epidemiology, approach to diagnosis, prognosis, and treatment modalities for these four disease processes.