Caroli's disease misdiagnosed as bile duct cystadenoma: A case report.

Caroli's disease is also known as Congenital intrahepatic bile duct dilatation, and previously known as a congenital intrahepatic bile duct cyst; it is characterized by single or multiple intrahepatic cystic dilatations. In this article, we report a case of Caroli's disease (CT size 21.2 × 16.9 × 19.8 cm). Preoperative abdominal ultrasound and enhanced CT were misdiagnosed as biliary cystadenoma or hepatic echinococcosis, and finally diagnosed as Caroli's disease by postoperative histopathological examinations. Most of the disease is single or multiple cystic dilatation of small bile duct. Giant Caroli disease, cystic dilations with diameter >20 cm is very rarely seen in the clinic. The lack of experience of diagnosing giant cystic dilatation makes it difficult to make accurate diagnosis. Therefore, we analyze the causes of imaging misdiagnosis through this case report, and summarize the imaging diagnostic skills of the disease combined with relevant imaging diagnosis experience. The purpose of this study is to deepen the understanding of giant Caroli disease among imaging doctors so as to reduce the misdiagnosis of the disease in the future.

A Unique Type or Variation of Bile Duct Cyst in a 9-Year-Old Girl:A Remarkable Case Study.

Patients with bile duct cysts require careful radiological assessment of the hepatobiliary system prior to surgical intervention. This clinical case is uncommon with an atypical clinical presentation and radiological findings. According to the most widely used classification of choledochal cysts, this case presents a combination of Type I and Type IV of choledochal cyst (CC) combining the form of extra, intrahepatic bile ducts and cystic duct dilations.

Resección laparoscópica de dilatación quística congénita del conducto cístico en un paciente pediátrico / Laparoscopic Resection of Congenital Cystic Dilatation of the Cystic Duct in a Pediatric Patient

Introducción: La dilatación quística congénita del conducto cístico o dilatación tipo VI de la clasificación de Todani, es una variante rara de dilatación congénita de la vía biliar. Objetivo: Explicar la metodología diagnóstica y terapéutica empleada en un caso pediátrico con esta entidad, y destacar la ventaja de su tratamiento oportuno por vía mínimamente invasiva. Presentación del caso: Paciente masculino de 6 años con sintomatología sugestiva de enfermedad vesicular, referido desde la atención secundaria por sospecha de quiste de colédoco. En el ultrasonido se detectaba una lesión ecolúcida adyacente a la vesícula biliar, sin dilatación de las vías biliares intrahepáticas. Se realizó colangiografía laparoscópica y se confirmó una dilatación quística aislada del conducto cístico, la cual se resecó por vía laparoscópica, igualmente. Conclusiones: La incidencia de la dilatación quística del conducto cístico es muy baja y se puede presentar en niños con sintomatología variable. El diagnóstico generalmente es tardío, puede sospecharse mediante la ecografía abdominal y confirmarse con la colangiografía laparoscópica, aun en ausencia de otros medios diagnósticos más modernos. Su reconocimiento y correcta clasificación permiten realizar el tratamiento quirúrgico definitivo exitosamente, de preferencia por vía laparoscópica(AU)

Introduction: Congenital cystic duct dilatation, or Todani classification type VI dilatation, is a rare variant of congenital bile duct dilatation. Objective: To explain the diagnostic and therapeutic methodology used in a pediatric case with this entity, and to highlight the advantage of its timely minimally invasive treatment. Case presentation: Six-year-old male patient with symptoms suggestive of gallbladder disease, referred from secondary care for suspicion of a common bile duct cyst. Ultrasound showed an echolucent lesion adjacent to the gallbladder, without dilatation of the intrahepatic bile ducts. Laparoscopic cholangiography was performed and confirmed an isolated cystic dilatation of the cystic duct, which was resected laparoscopically, likewise. Conclusions: The incidence of cystic dilatation of the cystic duct is very low and may present in children with variable symptomatology. Diagnosis is usually late, can be suspected by abdominal ultrasound and confirmed by laparoscopic cholangiography, even in the absence of other more modern diagnostic tools. Its recognition and correct classification permit a successful definitive surgical treatment, preferably laparoscopically(AU)

Tratamiento quirúrgico inmediato en niños con perforación espontánea de la vía biliar. Experiencias de 16 años

Introducción: La perforación espontánea de los conductos biliares es una rara enfermedad caracterizada por una disrupción no traumática de la vía biliar en pacientes aparentemente sanos. Se trata de una grave situación potencialmente letal, pero diagnosticada y tratada correctamente tiene un pronóstico excelente. Objetivo: Caracterizar los principales elementos clínico-quirúrgicos expresados en una serie de 5 pacientes operados en un servicio de referencia nacional. Presentación de casos: Se presenta la experiencia con una serie de casos en 16 años en una sola institución. La afección se observó en niñas recién nacidas y lactantes con una edad media de 4 meses, y se presentó desde la clínica como una colestasis acompañada de distensión abdominal, ascitis biliar, acolia, y signos de irritación peritoneal. El 80 % de los casos se intervinieron en el hospital "William Soler", y en un caso se ejecutó el procedimiento después de una laparotomía por una posible apendicitis aguda, en otro hospital. El diagnóstico se basó en el cuadro clínico descrito, la ecografía abdominal, la paracentesis con medición del índice bilirrubina líquido ascítico/bilirrubina sérica, y la colangiografía intraoperatoria. La cirugía definitiva se realizó inmediatamente, y consistió en: lavado peritoneal, colangiografía diagnóstica, reparación hepaticoyeyunostomía en Y de Roux y colocación de drenaje. Conclusiones: El tratamiento realizado resulta eficaz y seguro en todos los casos, con una excelente evolución, sin complicaciones importantes y con una total supervivencia posoperatoria. La colangiografía intraoperatoria permitió identificar el sitio de la perforación y diagnosticar malformaciones asociadas como dilataciones biliares congénitas y anomalías de la unión bilio-pancreática.

Introduction: Spontaneous bile duct perforation is a rare condition characterized by non-traumatic disruption of the bile duct in apparently healthy patients. It is a serious potentially lethal situation, but correctly diagnosed and treated its prognosis is excellent. Objetive: To characterize the main clinical-surgical elements expressed in a series of 5 patients operated in a national referral service. Case presentation: The experience with a series of cases during a period of 16 years in a single institution is presented. The condition was observed mainly in newborn girls and infants with an average age of 4 months, and presented clinically as cholestasis accompanied by abdominal distension, biliary ascites, acholia, and signs of peritoneal irritation. 80% of the cases were operated primarily in the "William Soler" hospital, and in one case the procedure was performed after a laparotomy for a possible acute appendicitis, in another hospital. The diagnosis was based on the clinical picture described, abdominal ultrasound, paracentesis with measurement of the ascitic liquid bilirubin/serum bilirubin index, and intraoperative cholangiography. Definitive surgery was performed immediately and consisted of: peritoneal lavage, diagnostic cholangiography, Roux-en-Y liver and jejunostomy repair and drainage placement. Conclusions: The treatment performed was effective and safe in all cases, with an excellent evolution, no major complications and total postoperative survival. Intraoperative cholangiography made it possible to identify the site of perforation and to diagnose associated malformations such as congenital biliary dilatations and anomalies of the biliary-pancreatic junction.

Spontaneous Rupture of a Choledochal Cyst.

Teaching point: Spontaneous perforation with biliary peritonitis is a rare complication of bile duct cysts which should be considered in a patient presenting with acute abdomen, ascites, and dilated biliary tree on imaging.

[Surgical treatment of common bile duct malformations in children]. / Khirurgicheskoe lechenie mal'formatsii obshchego zhelchnogo protoka u detei.

The review is devoted to the current classifications of common bile duct malformations, authors' opinion regarding surgical approach and reconstruction of the bile ducts. The authors describe the modern modifications of bile duct reconstructions and estimate their effectiveness. PubMed database, Google Scholar and National Scientific Electronic Library eLIBRARY.ru databases were used. We analyzed the complications in children depending on various reconstructions, anatomical form, timing of reconstructive surgery and early diagnosis of this pathology.

Epigastric hernia as a rare manifestation of a bile duct cyst and gallbladder cancer: A first case report.

INTRODUCTION: Elective repair epigastric hernia is a frequent minor surgical procedure. In most cases the hernial content is pre-peritoneal fat. PRESENTATION OF CASE: We report the case of a patient with epigastric hernia containing part of a bile duct cyst. DISCUSSION: Bile duct cysts are often asymptomatic, but when symptoms are present they may include intermittent, recurrent epigastric or right hypochondrial pain; abdominal tenderness; fever and mild jaundice. CONCLUSION: The presence of a bile duct cyst within a hernia is a very rare finding, especially making the diagnosis through an epigastric hernia. This case report is the first of a bile duct cyst within an epigastric hernia.

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.

Impact of previous cyst-enterostomy on patients' outcome following resection of bile duct cysts.

AIM: To analyze the impact of previous cyst-enterostomy of patients underwent congenital bile duct cysts (BDC) resection. METHODS: A multicenter European retrospective study between 1974 and 2011 were conducted by the French Surgical Association. Only Todani subtypes I and IVb were included. Diagnostic imaging studies and operative and pathology reports underwent central revision. Patients with and without a previous history of cyst-enterostomy (CE) were compared. RESULTS: Among 243 patients with Todani types I and IVb BDC, 16 had undergone previous CE (6.5%). Patients with a prior history of CE experienced a greater incidence of preoperative cholangitis (75% vs 22.9%, P < 0.0001), had more complicated presentations (75% vs 40.5%, P = 0.007), and were more likely to have synchronous biliary cancer (31.3% vs 6.2%, P = 0.004) than patients without a prior CE. Overall morbidity (75% vs 33.5%; P < 0.0008), severe complications (43.8% vs 11.9%; P = 0.0026) and reoperation rates (37.5% vs 8.8%; P = 0.0032) were also significantly greater in patients with previous CE, and their Mayo Risk Score, during a median follow-up of 37.5 mo (range: 4-372 mo) indicated significantly more patients with fair and poor results (46.1% vs 15.6%; P = 0.0136). CONCLUSION: This is the large series to show that previous CE is associated with poorer short- and long-term results after Todani types I and IVb BDC resection.

Bile duct cyst in adults: interventional treatment, resection, or transplantation?

Cystic dilatations of the bile ducts may be found along the extrahepatic biliary tree, within the liver, or in both of these locations simultaneously. Presentation in adults is often associated with complications. The therapeutic possibilities have changed considerably over the last few decades. If possible, complete resection of the cyst(s) can cure the symptoms and avoid the risk of malignancy. According to the type of bile duct cyst, surgical procedures include the Roux-en-Y hepaticojejunostomy and variable types of hepatic resection. However, the diffuse forms of Todani type V cysts (Caroli disease and Caroli syndrome) in particular remain a therapeutic problem, and liver transplantation has become an important option. The mainstay of interventional treatment for Todani type III bile duct cysts is via endoscopic retrograde cholangiopancreatography. The diagnostic term "bile duct cyst" comprises quite different pathological and clinical entities. Interventional therapy, hepatic resection, and liver transplantation all have their place in the treatment of this heterogeneous disease group. They should not be seen as competitive treatment modalities, but as complementary options. Each patient should receive individualized treatment after all of the clinical findings have been considered by an interdisciplinary team.

Choledochal cyst with ectopic distal location of the papilla of Vater.

In cholangiographic techniques, the close relationship between choledochal cyst and anomalous union of pancreaticobiliary duct has attracted medical attention. There have been rare cases in which the papilla of Vater was found in a position other than its normal position, and such cases have been reported sporadically. However, such cases are interesting in the anatomical context. In this review, we present our experience of choledochal cyst in a 30-month-old boy in whom the papilla of Vater was positioned in the third portion of the duodenum.

Choledochal cyst with ectopic distal location of the papilla of Vater / 대한외과학회지

In cholangiographic techniques, the close relationship between choledochal cyst and anomalous union of pancreaticobiliary duct has attracted medical attention. There have been rare cases in which the papilla of Vater was found in a position other than its normal position, and such cases have been reported sporadically. However, such cases are interesting in the anatomical context. In this review, we present our experience of choledochal cyst in a 30-month-old boy in whom the papilla of Vater was positioned in the third portion of the duodenum.

Bile duct cyst type V (Caroli's disease): surgical strategy and results.

BACKGROUND: Caroli's disease (CD) is a benign congenital disorder characterized by segmental cystic dilatation of the intrahepatic biliary ducts. Therapeutic strategy includes medical treatment, percutaneous, endoscopic or surgical drainage of the affected bile ducts, liver resection or transplantation. The aim of this study was to analyse the results and long-term follow-up of a consecutive series of patients who underwent surgical treatment for CD. PATIENTS AND METHODS: Between 1995 and 2005, 10 patients were surgically treated for CD. Variables evaluated were: age, gender, clinical presentation, diagnostic procedures, percutaneous and surgical treatments, histopathological analysis and outcome. RESULTS: The average age of the patients was 45.8 years. Recurrent cholangitis was the main clinical manifestation (70%). In unilateral CD a liver resection was performed in nine patients (left lateral sectionectomy in seven, left hepatectomy in one and right hepatectomy in one). In bilateral disease a cholecystectomy, duct exploration, hepaticojejunostomy and liver biopsy of both lobes were performed. Average follow-up was 60 months. All the patients are alive and free of symptoms without recurrence in the remnant liver. DISCUSSION: Liver resection is the preferred therapeutic option for unilateral CD, demonstrating good results in long-term follow-up. In bilateral disease, hepaticojejunostomy could be considered as an alternative or a previous step to liver transplantation, which still remains the ultimate option.

Cyst carcinoma after internal drainage operation for congenital choledocal cyst: a reports of 25 cases / 中国普通外科杂志

Objective To detective the carcinogenesis and operation principle of cyst canceration after internal drainage(ID) operation for congenital choledocal cyst (CCC).Methods The clinical data of 25 patients with cyst carcinoma after ID operation for CCC in the past 28 years were analysed retrospectively.Results The total canceration rate after internal drainage of CCC were 30.49%(25/82); after cystoduodenostomy was 35.29%(14/51),after cystojejunostomy was 22.58%(11/31), respectively. In the 25 cases, three of them were operated with Wipple operation , 4 with tumour resection plus biliary reconstrustion operation, 4 local resection with external drainage ,14 with external drainage only. Conclusions Internal drainage of CCC should be aborted becaus of the high canceration rate after the operation.

Choledochal cyst: a report studies of 3 cases of choledochal cyst among infants

1. Three new cases of choledochal cyst all occurring in female infants are reported, one undiagnosed and 2 suspected2. A brief review of some collected cases from the world literature was made to compare clinical manifestations with our patients3. The combination of jaundice and tumor mass with equivocal pain was noted in all three patients4. A suspicion of the condition in infants with the combination of symptoms especially when jaundice is intermittent may be the crux of the matter for the intended surgical procedure. Choledochal cyst must be always made a differential diagnosis in infants with such manifestations5. Morbidity and mortality continue to be affected by-a. preoperative recognition of the diseaseb. preoperative condition of the patientc. pre and postoperative care. (Summary)

Malignant changes in bile duct cysts in adults: a report of 24 cases / 中华肝胆外科杂志

Objective To investigate the surgical measures for relieving biliary malignancies that occur in bile duct cysts (BDCs). Methods Twenty four cases of biliary carcinoma occurring in BDCs treated in our Department from October 1965 to June 1999 were reviewed. Results Of the patients, 5 were male and 19 female. The average age at the time of being diagnosed was 38. Nine patients had undergone cystoenterostomy before. The mean duration between operation of drainage and detection of carcinoma was 10 years. Operations included excision of the extrahepatic bile duct plus pancreatoduodenectomy in 5, excision of extrahepatic bile duct in 6, and various palliative procedures because of extensive spread of tumor in 13. Follow up data were obtained in all but 2 patients who died in hospital. The mean survival time for the 16 death cases after discharge was 11 months. Four patients have been alive and free from disease for 12, 7, 6.5 and 0.5 years, respectively. Conclusions 1) Early diagnosis and curative resection for the biliary carcinoma in BDCs are the key points to achieve better outcomes. 2) Prophylactic excision of cyst in adults with BDCs, especially those previously treated with cystoenterostomy, can effectively reduce the risk of malignant changes.

Bile duct carcinomas in choledochal cysts in adults: a report of 11 cases / 中华肝胆外科杂志

Objective To study the diagnosis, therapy and prevention of bile duct carcinomas in choledochal cysts. Methods We retrospectively analysed clinico pathologic data, the ways of operation and living time of 11 patients with bile duct carcinomas in choledochal cysts in two hospitals from 1986 to 1997. Results There were complete excision of the choledochal cysts associated with choledochojejunostomy in 4 patients, exploration of bile duct and drainage with T shape tube in 3 patients. And there were also the excision of the wall of bile duct cysts and the tumor and cholangioplasty, choledochocystoduodenostomy, biopsy, pancreaticoduodenectomy and cholangiojejunostomy at the porta hepatis, resection of original rhynchostoma of choledochojejunostomy in one patient, respectively. One patient has lived for 3.6 years after radical resection. Others lived for 13.6 months after operation. Conclusions It is the key of improving and lengthening lives to find this disease in the early stage and to resect radically as early as possible.

Congenital bile duct cyst

No abstract available.