Type IVa Choledochal Cyst Research Articles

Liver Transplantation From a Donor With Asymptomatic Type IV-A Choledochal Cyst

Liver Transplantation From a Donor With Asymptomatic Type IV-A Choledochal Cyst

Hepatic lobectomy and mucosectomy of intrahepatic cyst for type IV-A choledochal cyst

ObjectiveExcision of extrahepatic cyst with wide biliary-enteric anastomosis is the treatment of choice in choledochal cyst. The diseased mucosa of the residual cyst may be the cause for postoperative complications. Mucosectomy of the cyst wall may prevent such complications in type IV-A choledochal cyst. Patients and MethodsFive cases (male:female, 3:2) of type IV-A choledochal cyst, aged between 15 and 120 months, are presented. The intrahepatic cyst was confined to only the left lobe in 1 patient and the left and part of the right lobe in 4 patients. Excision of the extrahepatic cyst, left hemihepatectomy, mucosectomy of the residual intrahepatic cyst wall, and wide hepaticojejunostomy were done. ResultsMedian hospital stay was 20 days. Postoperative biliary leak in 1 patient stopped spontaneously after 3 weeks. Postoperative MRCP (magnetic resonance cholangiopancreatography) scan in 1 patient showed shrinkage of intrahepatic residual cyst and good biliary drainage. Liver function tests at 3 months, 6 months, and at yearly intervals were within reference range in all patients. Cholangitis, hepatolithiasis, or malignancy was not noted in any of the patients. Median follow-up period was 36 months. ConclusionExcision of the diseased mucosa from residual intrahepatic cyst wall may prevent long-term complications in patients with type IV-A choledochal cyst.

MRI manifestations of adult choledochal cysts associated with biliary malignancy: a report of ten cases

To retrospectively review the MRI imaging features of adult choledochal cysts associated with biliary malignancy. Ten out of 72 cases of adult choledochal cysts were found to be associated with biliary malignancy between January 1, 2003 and April 1, 2011 in our hospital database. The following MRI findings of these ten patients were retrospectively reviewed: the type of choledochal cysts, the presence of anomalous union of the pancreaticobiliary duct (AUPBD), manifestations of biliary malignancy, and concomitant findings. Among the ten patients, there were five type I and five type IVA choledochal cysts. AUPBD was noted in four cases. The biliary malignancy was diagnosed as cholangiocarcinoma in seven cases (70.0%) and as gallbladder cancer in three cases. Cholangiocarcinoma manifested with irregularly thickened cyst wall (n = 2), mass with irregularly thickened cyst wall (n = 4), or multiple papillary nodules without thickened cyst wall (n = 1). Most of them showed mark enhancement (n = 4) after contrast administration. Gallbladder cancer appeared as mass with irregular thickening of the gallbladder wall with inhomogeneous enhancement. Concomitant findings included liver invasion or metastases in five cases, lymph node metastases in two cases, cholangitis and/or hepatic abscess in two cases, biliary stones in three cases. The type of choledochal cysts and the extent of malignant tumor invasion revealed by MRI were consistent with the surgical findings. Most malignancies associated with choledochal cysts are cholangiocarcinoma and gallbladder cancer. MRI is a reliable method for the detection of choledochal cysts with biliary malignant changes. MR features such as irregular thickening of the gallbladder wall or cyst wall, mass or papillary nodules are suggestive of biliary malignant changes.

Outcomes of choledochal cysts with or without intrahepatic involvement in children after extrahepatic cyst excision and Roux-en-Y hepaticojejunostomy

Background. Type I and type IV-A choledochal cysts (CC) in Todani’s classification are the most frequent types of CC. Unlike type I cyst, in which the dilatation is confined to the extrahepatic bile duct, type IV-A affects both extra and intrahepatic ducts.Aim. To review our experience of complete cyst excision with Roux-en-Y hepaticojejunostomy for the treatment of type I and type IV-A CC in childhood, in order to better characterize these entities.Material and methods. Data was collected retrospectively from a cohort of children who underwent cyst resection for CC from 1989 to 2011 in our institution.Results. Twelve patients were submitted to surgical excision of extrahepatic cyst and hepaticojejunostomy for treatment of type I (n = 6) and type IV-A (n = 6) cysts, with a complication rate of 25% (n = 3) and no mortality. Long term follow-up was available in 92% of patients, with a median of 10 years (2-22 years). Morbidities consisted of bile leak (2 patients) and late-onset cholestasis (1 patient); two of these required anastomotic revision. The results did not reveal any significant differences between the groups regarding postoperative outcomes (P > 0.05). Preoperative intrahepatic dilatation was found to permanently vanish in 83% of patients diagnosed with type IV-A cyst after operative repair.Conclusions. Intrahepatic dilatation of type IV-A cyst in children did not adversely affect the postoperative outcome after conventional surgical repair. This operative approach was effective in the management of type I and type IV-A cysts.

Computed tomography virtual endoscopy with angiographic imaging for the treatment of type IV-A choledochal cyst

Type IV-A choledochal cysts (CCs) are a congenital biliary anomaly which involve dilatation of the extrahepatic and intrahepatic bile ducts. We present the case of a 30-year-old woman with type IV-A CC, on whom three-dimensional computed tomography (3D CT) and virtual endoscopy were performed. 3D CT revealed partial dilatation in the posterior branch of the intrahepatic bile duct and a relative stricture between it and the extrahepatic bile duct. Virtual endoscopy showed that this stricture was membrane-like and separated from the surrounding blood vessels. Based on these image findings, complete cyst resection, bile duct plasty for the stricture, and hepaticojejunostomy were safely performed. To the best of our knowledge, there are no reports of imaging by virtual endoscopy of the biliary tract which show the surrounding blood vessels running along the bile duct.

Assessment of a postoperative anastomotic stricture following correction surgery of a type IVa choledochal cyst using Gd-EOB-DTPA-enhanced magnetic resonance cholangiography.

Choledochal cyst is a relatively uncommon disease which is characterized by congenital dilatation of the intra and/or extrahepatic part of the biliary tree. Type IVa choledochal cysts are managed surgically through total excision of the entire extrahepatic part of the abnormal bile ducts and a simultaneous hepaticoenterostomy. Postoperative anastomotic stricture after excision of choledochal cysts and hepaticojejunostomy is a well-known late complication. We report a case of a 17-year-old female in whom gadoxetic acid-enhanced magnetic resonance cholangiography assisted in the evaluation of a biliary stricture following bile duct procedures after choledochal cyst correction surgery.

Totally Laparoscopic Management of Choledochal Cyst: Roux-en-Y Jejunojejunostomy and Wide Hepaticojejunostomy with Hilar Ductoplasty

Cyst excision with hepaticojejunostomy is the treatment of choice for choledochal cyst. However, late complications after definitive surgery develop occasionally, including intrahepatic stones and cholangitis, because of bile stasis resulting from anastomotic stricture, intrahepatic bile duct stricture, and remnants of intrahepatic ductal dilatation. In type IV-A choledochal cysts in particular, biliary stricture is frequently observed around the hepatic hilum, and ductoplasty for stricture is necessary. In this article, we present our experiences with totally laparoscopic surgery comprising excision of the extrahepatic bile duct, Roux-en-Y jejunojejunostomy, and wide hepaticojejunostomy combined with hilar ductoplasty for choledochal cyst. We performed totally laparoscopic surgery on 8 children with choledochal cyst between June 2009 and October 2010. One of them had undergone bile drainage through gallbladder laparoscopically for biliary perforation. Four patients (1 Ic and 3 IV-A cysts) had hepatic duct stricture around the hepatic hilum. Laparoscopic surgery comprising excision of the extrahepatic bile duct and wide Roux-en-Y hepaticojejunostomy with ductoplasty was performed by using four trocars. The operation was completed laparoscopically for all patients. The mean operation time was 390 minutes (range, 310-460). The mean postoperative stay was 8.4 days (range, 7-14). After surgery, the dilatation of the intrahepatic bile duct was remarkably reduced in size, and all patients are doing well. Laparoscopic surgery comprising excision of the extrahepatic bile duct, Roux-en-Y limb formation, and wide hepaticojejunostomy with hilar ductoplasty appears to be feasible for children with choledochal cyst. When there is a stricture near the confluence of the hepatic ducts, laparoscopic ductoplasty appears to be feasible for the surgeon with an advanced laparoscopic skill set.

Type IVa choledochal cyst: Results following haemihepatectomy and mucosectomy of intrahepatic cyst

Choledochal cyst is a rare condition characterised by congenital dilatation of the biliary tree. Commonly seen in the oriental countries, patients usually present with a varying combination of abdominal pain, jaundice, lump or cholangitis. Untreated patients or incomplete removal of the cysts usually leads to portal hypertension and cholangiocarcinoma. Almost one-third of the cases have intrahepatic cyst, making complete cyst excision often impossible. We are reporting a 9-year-old girl with type IVa choledochal cyst presented to us with recurrent pain abdomen and intermittent jaundice for 1 year. Excision of extrahepatic cyst, left hepatectomy, mucosectomy of the residual cyst wall of right lobe of the liver and a wide bilioenteric anastomosis was done. Patients followed up with an magnetic resonance cholangiopancreatography (MRCP) 2 months later showed shrinkage of the residual cyst and good bile drainage. Mucosectomy of intrahepatic cyst may prevent recurrent cholangitis, calculus formation and cholangiocarcinoma in the long run.

Type IV-A Choledochal Cyst with Intrahepatic Bile Duct Stricture

Choledochal cysts are rare congenital anomalies of the bile ducts. They consist of cystic dilatations of the extrahepatic biliary tree, intrahepatic radicles, or both. The standard treatment of choledochal cyst is complete excision of cyst and drainage procedure. I report a case of 48-year-old women who was diagnosed with type IV-A choledochal cyst with intrahepatic bile duct stricture and large stones in extrahepatic choledochal cyst. Sixteen years ago, she received a cholecystectomy and Reux-en Y cystojejunostomy due to choledochal cyst. Because of the intrahepatic bile duct stricture I performed a left hepatectomy, complete cyst excision and hepaticojejunostomy. I report this case with a review of the literature.

Partial Hepatectomy and Total Cyst Excision is Curative for Localized Type IV-A Biliary Duct Cysts – Report of Four Cases and Review of Management

With the increasing use of newer modalities such as CT cholangiography and MRCP offering an accurate delineation of the ductal anatomy of choledochal cysts, the incidence of type IV-A biliary duct cysts has increased. Although the management of the more common type I cysts is well established, that of type IV-A cysts is still controversial. The localized/unilobar variety of type IV-A cysts is a unique entity amenable to curative surgical management. Between Jan 2000 and Jan 2005, 10 of 25 cases with choledochal cysts were diagnosed as having type IV- cysts. Four of these were localized or unilobar variants affecting only one lobe of the liver. Three cases had a left ductal involvement and one had a right ductal involvement of their intrahepatic component. We describe the presentation and curative surgical management of these patients with a unilobar variety of type IV-A cysts and discuss the management options for type IV-A cysts in the literature. One child with previous surgical treatment for presumed type 1 cyst, diagnosed as having a type IV-A right ductal cyst complicated with liver abscess, underwent right hepatectomy, two children had a left hepatectomy and one child had a left lateral lobectomy. All four children had uncomplicated intraoperative and postoperative courses. After 4-8 years' follow-up, all are symptom-free. There were no biliary tract or hepatic abnormalities on radiological surveillance with MRCP. In some series, the incidence of type IV-A choledochal cysts is equal to or higher than that of type 1 cysts. MRCP and intraoperative cholangiogram can help to confirm the anatomical varieties and associated ductal strictures. In the unilobar/uniductal varieties, complete cyst excision can be safely achieved in children through an additional hepatectomy/lobectomy. This can result in a curative status, leaving the child free from complications such as cholangitis, abscess, hepatolithiasis and the risk of biliary duct malignancy.

Laparoscopically Assisted Extrahepatic Cyst Excision and Left Hemihepatectomy for a Type IV-A Choledochal Cyst

Some studies have reported on laparoscopic excision for treating the choledochal cyst, yet there are no reports on laparoscopic surgery for treating type IV-A choledochal cysts that require a liver resection. In this paper, we report on a case of laparoscopic cyst excision combined with left hemihepatectomy and laparoscopy-assisted Roux-en-Y hepaticojejunostomy for treating a type IV-A choledochal cyst. A 51-year-old female was admitted with symptoms of jaundice and cholangitis. Percutaneous transhepatic biliary drainage (PTBD) was done preoperatively for controlling the cholangitis. The imaging studies revealed a type IV-A choledochal cyst with an associated stricture of the left main intrahepatic duct. After the resolution of the cholangitis, total laparoscopic cyst excision and left hemihepatectomy were performed by using the four-port technique, and then a Roux-en-Y hepaticojejunostomy was done by a laparoscopy-assisted method. The total operation time was 420 minutes. The estimated blood loss was 300 mL, and no perioperative transfusion was needed. The tubogram, which was performed through the PTBD on postoperative day 5, showed good patency of the bilioenteric anastomosis and no biliary leakage. The patient was discharged at postoperative day 7 without any complications. This case shows the feasibility of performing laparoscopic surgery for treating a type IV-A choledochal cyst that requires a liver resection. We believe that laparoscopic cyst excision with a liver resection can be one of the treatment options for selected patients with type IV-A choledochal cysts.

Traumatic rupture of a choledochal cyst masking as a duodenal hematoma

Choledochal cysts are rare, congenital malformations of the intra and/or extrahepatic biliary tree. We describe a case in which a patient was transferred to our hospital with a reported duodenal hematoma. The patient ultimately required exploration when his condition deteriorated. Laparotomy resulted in the discovery and successful treatment of a ruptured type IV-A choledochal cyst.

Diagnóstico y tratamiento de los quistes de colédoco: Presentación de 10 nuevos casos

the aim of this study was to analyze the diagnostic and therapeutic options for the various types of this rare disease. 10 patients with choledochal cysts (CC) were diagnosed in our hospital since 1991. Type of cyst was established according to the Alonso-Lej classification. we report 7 type-I, 1 type-III, 1 type-IVa, and 1 type-V CC cases. Clinical manifestations were abdominal pain in all cases with biliary or pancreatic features. The diagnosis was established using abdominal ultrasonography, computed tomography, and endoscopic retrograde cholangiopancreatography (ERCP). All 7 patients with type-I CC underwent total cyst excision with Roux-en-Y hepatojejunostomy. For type-III CC an endoscopic sphincterotomy (ES) was performed, and in type-IVa CC a transductal sphincterotomy and cholecistectomy was made. The patient with Caroli s disease (type V) underwent liver transplantation. We have followed up all patients for several years without significant complications. CC is more frequent in childhood, but is not exceptional in the adult. Imaging techniques and ERCP play an important role in the diagnosis, and also in the treatment of type-III cysts. Therapeutic options depend on cyst type, but due to the potential malignancy of this disease total cyst excision is recommended for types I, II and IV. In type-III CC endoscopic sphyncterothomy is recommended, while liver transplantation is sometimes necessary for type V. Long-term follow-up is crucial to prevent malignant transformation except for type-III CC where this complication is very unusual.

Risk Factors of Postoperative Anastomotic Stricture After Excision of Choledochal Cysts with Hepaticojejunostomy

The aim of this study was to investigate the risk factors of postoperative anastomotic stricture after excision of choledochal cysts and hepaticojejunostomy. Among 65 patients who underwent surgery for choledochal cyst between March 1995 and June 2005, we selected 34 adult patients who were diagnosed as having choledochal cyst. We divided patients into two groups, depending on postoperative anastomotic stricture developed or not. Medical records and radiological findings of each patient were reviewed retrospectively. H&E stain and Masson–Trichrome stain of each specimen of the resected cyst were performed, and thickness of cyst wall, the grade of fibrosis, loss of smooth muscle layer, loss of mucosa, and infiltration of inflammatory cells were measured. Of the 34 patients, excision of choledochal cyst and hepaticojejunostomy were done in 33 patients, and 1 patient with chronic pancreatitis underwent pylorus-preserving pancreaticoduodenectomy. Anastomotic stricture and intrahepatic duct stones postoperatively developed in eight patients; one patient of 19 type I cyst and seven patients of 15 type IVa, developing significantly more in the type IVa choledochal cyst (P < 0.05). The size of choledochal cyst in the stricture group was 7.0 cm, and that of the non-stricture group, 4.2 cm, showing significant difference between the two groups (P < 0.05). The stricture group presented shorter duration of symptoms (27.63±61.72 days; ranged, 1 ∼ 180 days) than the non-stricture group (483.33±916.41 days; ranged, 1∼3,560 days), and it was statistically significant (P < 0.05). Pathologically, significant difference was found between anastomotic stricture and infiltration of inflammatory cells (P < 0.05). The results indicate that anastomotic stricture is influenced by the type IVa choledochal cyst, size of cyst, duration of symptoms, and the grade of infiltration of inflammatory cells. Therefore, closed careful follow-up is important in patients who underwent cyst excision with hepaticojejunostomy for type IVa choledochal cyst. If the anastomotic stricture develops, nonoperative management should be recommended, rather than operation, as much as possible.

Management of choledochal cyst

BACKGROUND: Choledochal cysts are rare diseases in adults. The risk of malignant transformation is well documented to be age-related and early complete cyst excision is currently advocated. Type IVa cyst management is more controversial. Extensive intrahepatic involvement of the biliary system may preclude complete excision. AIM: The aim of this study was to present a case of type IVa choledochal cyst in a young female patient. CASE REPORT: An 18-year-old female, complaining of right upper quadrant pain, nausea, vomiting, fever, and jaundice was admitted. The diagnosis of type IVa choledochal cyst was made by computerized tomography. The patient was underwent cholecystectomy, excision of the anterior wall of the cyst, and Roux-en-Y hepaticojejunostomy. No postoperative complication was noted and the patient was discharged five days after operation. CONCLUSION: Total cyst excision is the ideal treatment of choledochal cysts in adults. However, in IVa type, due to the extensive intrahepatic involvement of the biliary system, the anatomy of the cyst, and the surgeon's judgment will determine the surgical approach taken for the patient.

Management of a ruptured bile duct cyst

A case of a ruptured bile duct cyst in a 25-year-old male patient is presented. The initial management of the clinical presentation of acute abdomen consisted of an exploratory laparotomy and a T-tube cystostomy of a choledochal cyst. Two months later, he was admitted to our surgical department. Preoperative evaluation showed a type IV-A choledochal cyst. The patient underwent excision of the choledochal cyst, cholecystectomy, and the construction of a Roux-en-Y end-to-side hepaticojejunostomy.

Type IV-a choledocal cyst—a rare adolescent presentation as acute abdomen

A 17-year-old adolescent girl from El Salvador presented to the emergency room (ER) with severe abdominal pain associated with one episode of nausea and vomiting. The pain that started 5 days earlier was sharp in nature and epigastric in location with radiation to back and was relieved by half a tablet of Vicodin. The patient has a history of intermittent epigastric pain for the past 2 years and was treated for Helicobacter pylori for 1 year. In the ER, the serum chemistry demonstrated elevated amylase. Further workup with abdominal ultrasonography (US), computed tomography (CT), magnetic resonance cholangiopancreatography (MRCP), and hepatobiliary scintigraphy confirmed a type IV-a choledochal cyst with intra- and extrahepatic dilation of bile ducts. We report an unusual acute abdomen presentation of type IV-a choledochal cyst in a 17-year-old young adult from El Salvador.

An unusual case of Type IVA choledochal cyst with subaortic ventricular septal defect

An unusual case of Type IVA choledochal cyst with subaortic ventricular septal defect

Type IV-A choledochal cysts: a challenge

Type IV-A choledochal cysts are characterized by congenital cystic dilatation of the biliary tree extending to involve the intrahepatic biliary channels also. A single-center experience of the management of type IV-A choledochal cysts is presented. Thirty-five out of 105 (33%) patients with choledochal cysts, who underwent surgery at a tertiary care center in northern India from January 1989 to December 2002, were found to have a type IV-A (Todani's classification) cyst. The mean age of the patients was 24 years (range, 3 months to 60 years); 17 patients in the group were adults and 22 were females. Presenting features were abdominal pain, jaundice, cholangitis, and abdominal lump, in various combinations. Excision of the extrahepatic part of the cyst and a wide bilio-enteric anastomosis was achieved in 32 (91%) patients, while internal drainage of the cyst was necessitated in 3 patients, for technical reasons: collaterals due to portal hypertension (1 patient) and dense adhesions (2 patients). Six (17%) patients developed postoperative complications: 3 had bilio-enteric anastomosis leaks, with 2 requiring a percutaneous proximal biliary diversion; 2 had intraabdominal bleeds requiring re-exploration, and 1 had external pancreatic fistula that closed spontaneously. Follow-up information was available for 28 (80%) patients. Mean duration of follow up was 25 months (median, 12 months; range, 6 months to 9 years). Three patients required re-operation, for anastomotic stricture (n = 2) and hepatolithiasis and recurrent cholangitis (n = 1) during follow up. Excision of the extrahepatic part of the cyst and drainage of the intrahepatic part by a wide hilar or subhilar anastomosis gave satisfactory results in the majority of patients with type IV-A choledochal cysts. Close long-term follow up of these patients is essential, because they are likely to present with complications related to the residual intrahepatic part of the disease.

A Case of Type IVa Choledochal Cyst Presented with Acute Pancreatitis

A Case of Type IVa Choledochal Cyst Presented with Acute Pancreatitis