Project description:BACKGROUND:Choledochal cysts are divided into 5 types. Physicians believe that Caroli disease (which refers to type V biliary cysts) is a special type of biliary cyst caused by a mutation in the PKHD1 gene and is associated with autosomal recessive polycystic kidney disease (ARPKD). There is currently no clear association between other types of choledochal cysts and polycystic kidney disease. CASE PRESENTATION:We report a 65-year-old male patient with jaundice, decreased appetite, and itchy skin. His biochemistry test results indicated obstructive jaundice disease. Cross-sectional imaging showed a type IVA choledochal cyst accompanied by autosomal dominant polycystic kidney disease (ADPKD). Due to economic difficulties, the patient achieved percutaneous transhepatic cholangial drainage (PTCD) instead of surgery. CONCLUSION:To our knowledge, this is the second case report of the coexistence of type IVA choledochal cysts and ADPKD. We conclude that it is vital to be aware that the above condition is a possibility. This case report will aid earlier diagnosis and management and possibly prevent further damage to liver and kidney function.
Project description:Complete resection of Todani type IV choledochal cyst (CC) is not possible, because the intrahepatic portion is not resectable. We present a case of intrahepatic cholangiocarcinoma that arose from the remnant CC portion that was located within the liver 10 years after resection. A 59-year-old female patient had undergone resection of type IV CC 10 years ago, leaving large remnant portions of CC at the liver and pancreas. Two and four years after resection of the extrahepatic CC, cholangitis with intrahepatic stones developed hence these episodes were treated with percutaneous transhepatic cholangioscopy. Ten years after the first operation, intrahepatic stones and a new mass were identified in follow-up imaging studies. Because the mass was identified as adenocarcinoma on biopsy, we performed left hepatectomy with redo hepaticojejunostomy. Pathologic examination showed a 4.5-cm-sized moderately differentiated adenocarcinoma arising from the remnant CC with lymph node metastasis. The patient recovered uneventfully and is currently undergoing adjuvant chemotherapy. Our case indicates that the remnant intrahepatic CC can undergo malignant transformation long after resection of CC. Since the intrahepatic CC portion in type IV CC is usually unresectable, wide hepaticojejunostomy and life-long observation with regular imaging study follow-up are highly recommended for prevention and early detection of malignant transformation.
Project description:Congenital choledochal cysts are rare in adults. Due to the risk of developing cholangiocarcinoma, the current standard of care is complete excision of the cyst and reconstruction with hepaticojejunostomy. So far, more than 200 laparoscopic resections have been reported in adults, the majority being from Far Eastern countries over the last five years. Herein, the technique of laparoscopic type I choledochal cyst excision and hepaticojejunostomy is presented in a 37-year-old male with an accompanying video. The advantages of laparoscopic surgery are applicable for choledochal cyst excision as well. We believe that teamwork, expertise on intracorporeal suturing and hepatobiliary surgery are central issues for this operation.
Project description:The usual etiologies of giant abdominal cystic masses in infants are mesenteric cyst, enteric duplication cyst, ovarian cyst in females, cystic lymphangioma, however, the presentation of a choledochal cyst in a gigantic form, is unusual. The primary modality for diagnosis of this entity is ultrasound, followed by MRI. The characteristic ultrasound features of a choledochal cyst are a well-defined cystic lesion which may be found to replace any segment of the biliary tree and is distinctly separate from the gallbladder. The associated anomalies are biliary atresia, gallbladder atresia, hepatic fibrosis and those of the pancreatico-biliary ductal system. MRI with MRCP has a conclusive role in confirming the ultrasound diagnosis. Choledochal cysts are currently classified as proposed by Todani et al, into five types. Herein, we report the case study of a 4-month-old male infant afflicted with a gigantic, Type1 Choledochal cyst, complicated by perforation, which was diagnosed by us at the first instance itself, using ultrasound examination and confirmed by MRI. The diagnosis was further confirmed at surgery and histopathology. The recommended treatment of cyst resection accompanied by a hepatico-jejunostomy bypass procedure, was successfully performed in the reported infant.
Project description:IntroductionCholedochal cyst (CC) is a morphological malformation characterized by dilatations of the biliary tree that might present later with clinical symptoms, including jaundice, abdominal pain or pancreatitis.Presentation of caseHere, we reported a 10-month-old female infant with CC presenting with jaundice and a right upper quadrant mass and who was malnourished following a surgical excision of retroperitoneal teratoma one month ago. Laboratory findings were total bilirubin of 14.17 mg/dL, direct bilirubin of 12.24 mg/dL, gamma glutamyl transferase of 1157 U/L, and alkaline phosphatase 187 U/L. Abdominal computed tomography scan showed a CC that caused dilatation of the proximal common bile duct (CBD), common hepatic duct, and intrahepatic bile duct. We decided to perform an explorative laparotomy and found a CC with diameter of 5 cm. Then, we conducted a Roux-en-Y hepaticojejunostomy. Due to hepaticojejunostomy anastomosis leakage, relaparotomies were done. The patient was uneventfully discharged 17 days after the third surgery.DiscussionOur findings are unique because the patient had a normal biliary tree previously and underwent intraabdominal tumor surgery. Notably, besides being an acquired CC, our case might be due to inadvertent bile duct ligation during the first operation or bile duct obstruction as a complication of the first operation.ConclusionsCC should be considered as a potential complication of intraabdominal tumor excision, especially if its location is near the CBD. Roux-en-Y hepaticojejunostomy is still the best choice for CC management.
Project description:Choledochal cyst (CC) is a disease with a strong Asian preponderance. As laparoscopic surgery has become mainstay in its treatment, the experience in these countries has been phenomenal. However, there are many contentious issues related with the laparoscopic management of CC. In this review article, we will try to answer the contentious questions related to the laparoscopic management of CC. The issues related to aetiology, classification, surgical technique, type of biliary anastomosis, intrahepatic stones and malignancy are discussed. We also discuss the current and future considerations of laparoscopic management with reference to it becoming a gold standard. This article describes the standard surgical approach and will discuss its technical nuances. This article will also discuss the outcome of treatment in different settings of low- and middle-income countries based on lessons learnt by the authors from their experience and research.
Project description:Background and study aims After stone removal in endoscopic retrograde cholangiopancreatography (ERCP), an occlusion cholangiogram (OC) is performed to confirm bile duct clearance. OC can miss residual stones that can lead to recurrent biliary symptoms. The aim of this study was to assess if digital peroral cholangioscopy (POC) increased the diagnostic yield of residual biliary stones that are missed with OC. Patients and methods Patients having ERCP performed for choledocholithiasis were enrolled into the study only if they had one of the following criteria: dilated bile duct ≥ 12 mm and/or if lithotripsy was being performed. An OC was performed to confirm duct clearance after removal of stones followed by POC, based on inclusion criteria. The incremental yield of biliary stones missed by OC but confirmed by POC was then measured. A total of 96 POC procedures were performed on 93 patients in two tertiary care centers. Results Residual biliary stones were found in 34 % of cases. The average bile duct size in cases with residual stones was 15.1 mm ± 0.7 mm. One- to three-mm stones were found in 41 % of cases, 4- to 7-mm stones in 45 % of cases, and ≥ 8-mm stones in 14 % of cases. Lithotripsy was performed in 13 % of cases and was significantly associated with residual stones (30 % vs. 3 %, P < 0.001). Conclusions Occlusion cholangiogram can miss residual stones in patients with dilated bile ducts and those receiving lithotripsy. Digital POC can increase the yield of residual stone detection in these patients and should be considered to confirm clearance of stones. (ClinicalTrials.gov-NCT03482375).