EUS diagnosis of asymptomatic type III choledochal cyst.
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ABSTRACT:
SUBMITTER: Abou Saleh M
PROVIDER: S-EPMC7859545 | biostudies-literature |
REPOSITORIES: biostudies-literature
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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.
| S-EPMC8649116 | biostudies-literature
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| S-EPMC7947370 | biostudies-literature
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| S-EPMC6831943 | biostudies-literature
Project description:BackgroundCholedochal 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 presentationWe 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.ConclusionTo 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.
| S-EPMC7507809 | biostudies-literature
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.
| S-EPMC7452807 | biostudies-literature
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.
| S-EPMC4942164 | biostudies-literature
Project description:A choledochal cyst is a rare condition that requires surgical treatment to prevent complications, such as obstructive jaundice, cyst rupture, cholangitis, and the risk of malignancy. Complete cyst excision is considered the best option, as it reduces the risk of inflammation and the development of cholangiocarcinoma. Therefore, cholecystectomy and complete cyst resection followed by reconstruction with a Roux-en-Y hepaticojejunostomy is the treatment of choice. We present a case (with video) that shows the complete resection of a type I choledochal cyst with Roux-en-Y reconstruction of two separate ducts since the right posterior duct reached the cyst independently. The laparoscopic approach offers all the advantages of mini-invasive surgery and better visualization of the structures; however, biliary reconstruction to fine ducts implies a surgical challenge that requires high training in mini-invasive surgery.
| S-EPMC11358056 | biostudies-literature
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.
| S-EPMC7452007 | biostudies-literature
Project description:Tyrosinemia type 3 (HT3) is a rare inborn error of tyrosine metabolism caused by mutations in the HPD gene encoding 4-hydroxyphenyl-pyruvate dioxygenase, which is transmitted in an autosomal recessive trait. The disorder is characterized by tyrosine accumulation in body fluids and massive excretion of tyrosine derivatives into urine (www.orpha.net). Since it is the least frequent form of tyrosinemia, only few cases with the variable but rather mild clinical features have been described so far. We report an 11 year old girl presenting with no clinical symptoms and with normal mental development who has been diagnosed with HT3 through metabolic screening on the basis of elevated serum level of tyrosine ranging from 425 to 535 μmol/L (normal values: 29-86 μmol/L), and elevated urinary excretion of p-hydroxyphenyl derivatives confirmed genetically with the homozygous c.479A > G (p.Tyr160Cys) missense change in the HPD gene. The girl has been only presenting with recurrent proteinuria of unknown etiology. A phenylalanine- and tyrosine-restricted diet has never been administered. Presented case may suggest that high tyrosine concentration itself does not participate directly in neuronal damage described in patients with tyrosinemia type 3.
| S-EPMC5471395 | biostudies-literature
Project description: Not available
| S-EPMC6669307 | biostudies-literature