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PubMed1982

Intravenous cholescintigraphy using Tc-99m-labeled agents in the diagnosis of choledochal cyst.

Twelve patients with choledochal cyst have undergone intravenous radionuclide cholescintigraphy (IVRC) with Tc-99m-labeled HIDA or PG before surgery. The most characteristic findings are: (1) A round or ovoid photon-deficient area in the region of the gallbladder in the early images; (2) progressive accumulation of radioactivity in the same region in later images, especially at 2 hr after injection; (3) the long axis of this particular area directed downward and to the patient's left from the right midclavicular line, suggesting the direction of the common bile duct; and (4) persistent pooling of the tracer up to 24 hr, even after a fatty meal. By these criteria, correct diagnoses have been made preoperatively in ten of 12 cases (83.3%). We conclude that IVRC using Tc-99m HIDA or Tc-99m PG is an excellent first-line diagnostic tool for choledochal cyst.

Adolescent↗
PubMed2006

Totally laparoscopic management of choledochal cysts using a four-hole method.

BACKGROUND: Choledochal cyst is a rare benign disease of the biliary tract. However, once diagnosed, it must be excised with the gallbladder because of the risk for cancer developing in the biliary tree, including the gallbladder. This report introduces a new surgical technique for totally laparoscopic excision of choledochal cyst and hepaticojejunostomy using a four-hole method. METHODS: Between October 2003 and May 2005, the authors performed totally laparoscopic choledochal cyst excision for 12 patients. All the patients except one were women, and the mean age was 37.3 years (range, 17-62 years). According to the Todani classification, there were five type Ia cases, four type Ic cases, and three type IV cases. Choledochal cyst excision and Roux-en-Y hepaticojejunostomy were performed laparoscopically using the four-port technique. RESULTS: The mean operation time was 228 min (range, 150-330 min). No operative or postoperative transfusion was required. An oral diet was started on postoperative day 3. The average length of hospital stay was 5.8 days. There was no major complication associated with anastomosis leakage or obstruction. No patient had an adverse response, as determined by clinical or laboratory evaluation during a 2- to 19-month follow-up period. CONCLUSIONS: Considering that choledochal cyst is common among young women, who are especially interested in cosmetic results in addition to complete resolution of medical problems, the laparoscopic management of choledochal cyst may be an attractive treatment option.

Adolescent↗
PubMed1989

Hepatico-jejuno-duodenostomy reconstruction following excision of choledochal cysts in children.

Ten children with choledochal cysts were treated at Children's Memorial Hospital in Chicago between 1979 and 1980 and at Henrietta Egleston Hospital for Children or Grady Memorial Hospital in Atlanta between 1980 and 1987. Age at the time of operation ranged from one to 46 months. There were eight girls and two boys with Type I choledochal cysts. One child with a dilated but histologically normal common bile duct underwent a choledochoduodenostomy. The remaining nine patients had excision of the choledochal cyst. Continuity with the GI tract was then re-established with a valved isolated jejunal segment interposed between the common hepatic duct and the duodenum. The valve serves to prevent reflux of enteric contents up into the biliary tract. The short jejunal segment allows bile to drain into the duodenum with minimal stasis; this more closely approximates the natural physiologic state than does a Roux-en-Y reconstruction. There was no mortality, with follow up ranging from six months to over eight years. Two post-operative complications (one anastomotic stricture and one small bowel obstruction) occurred, both in the same patient. There were no anastomotic leaks and no episodes of post-operative cholangitis. All patients are currently free of jaundice and none have signs of portal hypertension. The treatment of choledochal cysts is excision. Biliary tract reconstruction with the valved common hepatico-jejuno-duodenostomy is safe, effective, and physiologically appealing.

Child, Preschool↗
PubMed1983

Antenatal diagnosis and early surgery for choledochal cyst.

The pathogenesis and optimal treatment of choledochal cyst have long remained questions of considerable speculation and dispute. Because the pregnancy of a 37-year-old woman was felt to be at risk, five antenatal ultrasound examinations were made. The fourth examination at 31.5 weeks of gestation demonstrated a choledochal cyst. Following birth, the child was studied with repeat ultrasound examinations and scintigraphy. The results of these studies plus the findings at surgery contributed new evidence regarding the possible pathogenesis and optimal treatment of choledochal cysts. Although the time at which the cyst originated is compatible with the concept of reflux of pancreatic juice into the common duct, no abnormal junction of the pancreatic and common duct was identified. No evidence of obstruction as part of the pathogenesis could be demonstrated. Progressive changes in the choledochal cyst in the first ten days of life suggested that delay in diagnosis and treatment of a choledochal cyst may contribute to early complications such as cholangitis. Early excision of the cyst in the newborn is considered to be the optimal treatment and may pose less risk to the patient than delayed surgical exploration.

Common Bile Duct Diseases↗
PubMed2004

Choledochal cyst in adults: aetiological considerations to intrahepatic involvement.

BACKGROUND: Choledochal cysts are rare anomalies of the biliary tree and their presentation in adults is infrequent. The high incidence of associated anomalous pancreaticobiliary duct junction (APBDJ) has been well documented. However, the prevalence of APBDJ in different anatomic distribution of cysts has yet to be clarified. METHODS: A total 39 adult patients with choledochal cysts were consecutively enrolled: 22 patients had Todani type I cysts, 16 patients had type IVa cysts, and one patient had a type IVb cyst. RESULTS: No statistical differences were found in age, sex or manifestations. A higher frequency of associated APBDJ was identified in patients with type IVa cyst (100%) than those with type I cyst (P < 0.05). With a mean follow-up of 7.2 years after cyst excision, three patients with type I choledochal cyst and four with type IVa cyst have had episodes of recurrent cholangitis, all accompanied with preoperatively diagnosed APBDJ. CONCLUSION: Anomalous pancreaticobiliary duct junction has a close relationship with adult choledochal cysts, particularly type IVa. We propose the hypothesis that intrahepatic involvement results from progressive enzymatic destruction. Adult choledochal cysts in association with APBDJ, with or without intrahepatic component, should be carefully monitored.

Adult↗
PubMed2001

Factors of biliary carcinogenesis in choledochal cysts.

The long-term complications of choledochal cysts include malignant degeneration of the epithelial lining of the biliary tract. Possible factors leading to malignant degeneration were examined by measuring amylase levels in the biliary tract, intraoperative biliary manometry and a histopathological study. 27 patients, 1 to 13 years of age, with choledochal cysts, were studied over a 13-year period. Amylase levels in the bile of choledochal cysts and the gallbladder were measured. The pressure profile of the biliary tract was recorded as the probe was withdrawn from the sphincter of Oddi (SO) to the distal end of the common bile duct. Hyperplasia of the gallbladder mucosa and malignancy of the biliary tract were investigated. Patients with high levels of biliary amylase had higher pressure differences between the SO and the duodenum than those with low levels of biliary amylase. The incidence of mucosal hyperplasia of the gallbladder mucosa was significantly higher in the fusiform type than in the cystic type. Adenocarcinoma in a cystic choledochal dilatation was found in a 12-year-old girl with high amylase levels. The cause of regurgitation of pancreatic juice into the biliary system might be due to a high pressure difference between the SO and the duodenum in addition to the lack of sphincter function at the abnormal junction of the pancreaticobiliary ductal system. Early diagnosis and surgical treatment to prevent the regurgitation leading to hyperplasia and malignancy of the biliary tract are important for children with choledochal cysts.

Adolescent↗
PubMed1997

Protein plugs cause symptoms in patients with choledochal cysts.

OBJECTIVES: Symptoms in patients with choledochal cysts are believed to be caused by pancreaticobiliary maljunction. However, this anomaly alone cannot explain the occurrence of symptoms. The aim of this study was to elucidate the etiology of the symptomatology in patients with choledochal cysts. METHODS: Clinical data and preoperative and operative cholangiopancreatography were reviewed in 55 consecutive patients with choledochal cysts seen between 1980 and 1996. RESULTS: The bile duct was significantly larger in the symptomatic phase than in the asymptomatic phase. External biliary drainage resulted in rapid resolution of symptoms in 11 patients. A radiolucent filling defect in the pancreaticobiliary duct was found in 22 patients (40.0%). The defects were in the common channel in 15 patients and near the common channel in 7 patients. Filling defects disappeared spontaneously or after irrigation in 19 patients. In three patients, the material in the common channel removed during surgery was fragile and consisted of more than 98% protein. CONCLUSION: The filling defects were protein plugs. The simultaneous occurrence of symptoms and signs may be explained by disturbances in bile and pancreatic secretory flow caused by a protein plug in the common channel.

Adolescent↗
PubMedDate not supplied

Secondary excision of choledochal cysts after previous cyst-enterostomies.

BACKGROUND/AIMS: Patients with a choledochal cyst previously treated by internal drainage should undergo secondary cyst excision. The results of such secondary excisions have not yet been reported. METHODOLOGY: Over a 27-year period, 121 patients underwent excision of a choledochal cyst at our hospitals. Of these, 14 patients underwent secondary cyst excision following internal drainage. These patients were compared retrospectively with the remaining 107 patients who underwent primary cyst excision. RESULTS: Blood loss and operative time were greater, and early and late post-operative complications were significantly more frequent in the secondary excision group. Wound infection (early complication), pancreatic stones (late complication), and hepatolithiasis (late complication) were significantly more common in the secondary excision group. Histologically, inflammation and biliary glands were more frequently seen in the resected bile ducts in the secondary excision group. CONCLUSIONS: The long-term results of secondary excision of choledochal cysts are worse than after primary excision. The frequent late complications may be related to the development of biliary glands as a consequence of cyst-enterostomy.

Adolescent↗
PubMed1979

The surgical treatment of choledochal cyst.

Eleven consecutive infants and children with choledochal cyst were evaluated for operative resection of the cyst as the primary surgical therapy. Total excision and biliary reconstruction by choledochojejunostomy was successfully performed in nine patients. Simple intestinal drainage of the cyst was used in two instances because of severe liver disease. Four patients had coexisting biliary malformations. In all five infants, biopsy of the liver was consistent with biliary atresia. In addition, the extrahepatic bile ducts proximal to the cyst were obliterated in two infants. The findings suggest that pathogenesis of choledochal cyst may be different in infants than in older children. The indications for excision of the cyst in four patients were due to complications from earlier internal drainage procedures. In most patients, including all those having had a cystoenterostomy earlier, resection was done from the inside of the cyst, thus minimizing the danger of injury to the neighboring vascular structures. There were no operative deaths, and morbidity was minor. The traditional operation for a choledochal cyst has been internal drainage of the cyst into the intestine. Because of the high incidence of late complications and the frequent association of major co-existing biliary malformations, the procedure should be reserved for highly specific indications. Total surgical excision is the procedure of choice for a choledochal cyst.

Adolescent↗
PubMed2004

Initial experience with complex laparoscopic biliary surgery in children: biliary atresia and choledochal cyst.

BACKGROUND: Laparoscopic approaches are possible for many pediatric surgical diseases, including complex biliary disorders. The authors describe their early experience with laparoscopic procedures for biliary atresia and choledochal cysts. METHODS: A retrospective review was performed with Institutional Review Board (IRB) approval of children undergoing laparoscopic Kasai portoenterostomy (n = 2) or laparoscopic excision of a choledochal cyst with Roux-en-y biliary reconstruction (n = 3). The authors report the operative technique for these procedures and present their initial results. RESULTS: All procedures were performed with 4 to 5 trocars using 3- and 5-mm ports. Excision of the fibrous biliary remnant (biliary atresia) or of the cyst (choledochal cysts) was performed laparoscopically in all cases. The Roux-en-y limb was created through a 1-cm extension of the umbilical port site. Laparoscopic biliary reconstruction was performed successfully in 4 of 5 patients. In one child, the proximal extent of the choledochal cyst was at the bifurcation of the hepatic duct, and the biliary reconstruction was performed via an open incision with separate anastomoses of the right and left ducts. The gallbladder was used as a handle for retraction of the liver for portal visualization, as in a cholecystectomy, and was resected at the end of the procedure. All children with choledochal cysts have normal bilirubin values. One of 2 children who underwent laparoscopic Kasai has a normal postoperative bilirubin level, whereas the other child did not drain bile and underwent a successful liver transplantation. Mean length of surgery was 338 minutes; mean length of stay was 5.5 days. There were no significant postoperative complications. Cosmesis was excellent in all cases. CONCLUSIONS: Laparoscopic approaches for children with biliary atresia and choledochal cysts are possible. Excellent visualization of the portal structures can be achieved laparoscopically with adequate retraction of the liver. Further follow-up is needed to determine broader application.

Anastomosis, Roux-en-Y↗
PubMed1995

Choledochal cyst mimicking a pancreatic pseudocyst.

Choledochal cyst mimicking a pancreatic pseudocyst radiographically has not yet been reported. We report such a patient with a choledochal cyst whose initial presentation was acute pancreatitis. Ultrasound and computed tomography scan showed images that resembled a pancreatic pseudocyst.

Acute Disease↗
PubMed1989

Ultrasonography and 99mTc-DISIDA cholescintigraphy in the diagnosis of choledochal cyst in children.

Eighteen children with choledochal cyst have underwent abdominal ultrasonography and 99mTc-DISIDA (di-isopropylphenylcarbamoyl-methylimidodiacetic acid) cholescintigraphy before surgery. All patients had typical sonographic findings of a large cystic mass in the porta hepatis with direct continuity to the biliary system. The gallbladder could be easily recognized and distinguished from the cystic mass in every except one case. Dilatation of the intrahepatic ducts was noted in 12 patients (67%). Coexisting biliary stones were found in 3 patients (17%). On the cholecintigrams, 14 patients (78%) had characteristic findings of a round or ovoid photon-deficient area in the porta hepatis with delayed filling and stasis of the tracer in the same area. Three infants (17%) showed a persistent round photon-defect over the porta hepatis without visualization of gallbladder or bowel radioactivity, which was thought to suggest a choledochal cyst with obstruction. A combination of ultrasonography and cholescintigraphy can provide a very accurate preoperative diagnosis of choledochal cyst.

Bile Ducts↗
PubMed2005

Type IV congenital choledochal cyst concurrent with acute pancreatitis: a case report.

BACKGROUND: Congenital choledochal cyst is a rare kind of bile duct deformity, resulting from cystic or shuttle-like dilation of part of the choledochal duct congenitally. We present a 20-year-old girl with a congenital choledochal cyst complicated by acute pancreatitis. METHOD: The clinical data of the woman with a congenital choledochal cyst concurrent with acute pancreatitis were retrospectively analyzed. RESULT: The congenital choledochal cyst of the woman was type IV complicated by acute pancreatitis. CONCLUSIONS: The diagnosis of congenital choledochal cyst mainly depends on CT, MRCP, and ERCP. Total excision of the choledochal cyst with Roux-en-Y hepaticojejunostomy is recommended as the treatment. For patients with type V cysts with frequently recurrent cholangitis resulting biliary liver cirrhosis, liver transplantation should be considered.

Acute Disease↗
PubMed2002

Congenital choledochal cysts in adults--comparative study.

Congenital choledochal cysts are rare anomalies of the biliary tree and their presentation in adults is infrequent. They are more common in Asia. Females are more commonly affected. Surgery remains the treatment of choice. Nine patients were operated for congenital choledochal cysts in the last fifteen years, i.e. from 1988 to 2002. The diagnosis was established by case history, clinical features and laboratory tests. The imaging methods proved to be the most informative among them. Classification of the choledochal cysts was based on modified Todani classification. All patients have undergone cyst excision with Roux-en-Y hepaticojejunostomy. The complications, like recurrent cholangitis or pancreatitis, were avoided.

Adult↗
PubMed2004

Some considerations for management of choledochal cysts.

BACKGROUND: There are five types of choledochal cysts, which are anomalies that involve intrahepatic or extrahepatic bile ducts, or both. These lesions are found most frequently in patients who are Asian, female, infants but are recognized with increasing frequency in adults. METHODS: We have managed 16 patients with this anomaly. One patient was Asian, and 1 was a child. There were 3 males and 13 females. The mean age was 29 years. There were 9 type I, 1 type II, 1 type III, 4 type IV, and 1 type V cysts. Resection of cysts and hepatico Roux-en-Y jejunostomy were performed in 9 patients for type I cysts. Pancreaticoduodenectomy was performed for a type I and a type IV cyst. The extrahepatic portion of a type IV cyst along with a segment of liver was resected in 1 patient. Operation was terminated on 1 patient with a type IV cyst because of extensive involvement of the intrahepatic ducts. She will undergo liver transplantation. The type II cyst was resected. No surgery was performed on a type III and type V cyst. Four of these patients were previously treated unsuccessfully by internal drainage procedures. RESULTS: There was no mortality. Morbidity was limited to a patient who previously underwent incomplete resection of a cyst and a cyst Roux-Y jejununostomy. No cholangiocarcinoma has been encountered in our patients after a mean follow-up of 5.5 years from the time of initial discovery of the choledochal cyst. CONCLUSIONS: Management of choledochal cysts is successful after their complete removal. Partial cyst resection and internal drainage is less satisfactory because of occasional pancreatitis, cholangitis, and cholangiocarcinoma. Resection of the intrahepatic and intrapancreatic portions of the cysts reduces the risk of cancer even though this risk is low after incomplete cyst excision. Biliary continuity after cyst resection is best established by Roux-Y hepaticojejunostomy.

Adult↗
PubMed2004

Some considerations for management of choledochal cysts.

BACKGROUND: There are five types of choledochal cysts, which are anomalies that involve intrahepatic or extrahepatic bile ducts, or both. These lesions are found most frequently in patients who are Asian, female, infants but are recognized with increasing frequency in adults. METHODS: We have managed 16 patients with this anomaly. One patient was Asian, and 1 was a child. There were 3 males and 13 females. The mean age was 29 years. There were 9 type I, 1 type II, 1 type III, 4 type IV, and 1 type V cysts. Resection of cysts and hepatico Roux-en-Y jejunostomy were performed in 9 patients for type I cysts. Pancreaticoduodenectomy was performed for a type I and a type IV cyst. The extrahepatic portion of a type IV cyst along with a segment of liver was resected in 1 patient. Operation was terminated on 1 patient with a type IV cyst because of extensive involvement of the intrahepatic ducts. She will undergo liver transplantation. The type II cyst was resected. No surgery was performed on a type III and type V cyst. Four of these patients were previously treated unsuccessfully by internal drainage procedures. RESULTS: There was no mortality. Morbidity was limited to a patient who previously underwent incomplete resection of a cyst and a cyst Roux-Y jejununostomy. No cholangiocarcinoma has been encountered in our patients after a mean follow-up of 5.5 years from the time of initial discovery of the choledochal cyst. CONCLUSIONS: Management of choledochal cysts is successful after their complete removal. Partial cyst resection and internal drainage is less satisfactory because of occasional pancreatitis, cholangitis, and cholangiocarcinoma. Resection of the intrahepatic and intrapancreatic portions of the cysts reduces the risk of cancer even though this risk is low after incomplete cyst excision. Biliary continuity after cyst resection is best established by Roux-Y hepaticojejunostomy.

Adolescent↗
PubMed1988

Adenocarcinoma in a choledochal cyst during pregnancy: a case report and guidelines for management.

Choledochal cysts, although more common in females than males, have only rarely been encountered in association with pregnancy. We report a patient with a large choledochal cyst that was excised at the time of cesarean section. Pathologically, the cyst wall was found to contain a focus of adenocarcinoma. The development of carcinoma is a recognized risk in patients with choledochal cysts that mandates cyst excision, rather than bypass, whenever possible. The literature regarding choledochal cysts in pregnancy is reviewed. Pregnant patients with choledochal cysts require careful management so as to avoid cyst rupture; and cesarean section is preferable to normal vaginal delivery in most cases. Although the preferred management of a choledochal cyst is excision and Roux-en-Y reconstruction, this may have to be deferred until after delivery, depending on gestation age, because of the risk of fetal mortality and maternal morbidity that is associated with this procedure.

Adenocarcinoma↗
PubMed1996

Choledochal cysts: a ten year experience.

Choledochal cyst (CC) is a rare disorder that usually presents in childhood. Prognosis depends on early diagnosis, complete excision of the cyst, and reconstruction by hepaticojejunostomy. This report details our 10-year experience and emphasizes innovations in our management. Sixteen patients presented with CC at a mean age of 3 years. (Range, newborn to 21 years, with a M:F ratio 1:4). Two groups could be identified on the basis of age at presentation. Group I (N = 7), presented in the neonatal period, three with obstructive jaundice and four without symptoms. In Group II (N = 9), all patients presented with ascending cholangitis at a mean age of 6 years. Thirteen patients had a type 1 CC, one patient had a type 3 CC, and two had type 4 CC. The patients with type 1 and type 4 CC underwent primary cyst excision with Roux-en-Y hepaticojejunostomy, whereas the patient with type 3 CC underwent cyst excision with sphincteroplasty of the ampulla. There was only one complication of postop cholangitis that cleared with antibiotic therapy. All patients have remained free from symptoms in the follow-up period (6 months-10 years). Our four most recent cases were diagnosed in utero by prenatal ultrasonography. This led to appropriate antenatal counseling and prospectively planned neonatal surgery. These infants were asymptomatic, with no clinical signs at birth, and antenatal ultrasonography prevented diagnostic delay. Excision of the choledochal cyst and primary hepatico-enteric anastomosis is confirmed as the therapy of choice. Antenatal sonography is a sensitive method in the diagnosis of CC and offers the opportunity for early diagnosis and planned surgery before the onset of complications.

Adolescent↗