Successful management of ruptured choledochal cyst by primary cyst excision and biliary reconstruction

One to two percent of patients who have choledochal cysts present with cyst rupture and bile peritonitis. Reported cases have been managed with external drainage of the cyst followed by a second procedure to excise the cyst and reconstruct the biliary tract. The authors report two cases of ruptured choledochal cyst treated with primary cyst excision and biliary drainage. The satisfactory outcome of these patients suggests that this is the preferred management.     

Is it possible to differentiate between choledochal cyst and congenital biliary atresia (type I cyst) by antenatal ultrasonography?

Both choledochal cyst and congenital biliary atresia (type I cyst) may share the same ultrasonographic pattern. We report 2 cases which were shown to have cystic structures on the upper abdomen by antenatal ultrasonography. The size of the choledochal cyst found at 24 weeks' gestation increased steadily as gestational age advanced. But the size of the cyst in congenital biliary atresia found at 29 weeks' gestation remained unchanged throughout the remaining pregnancy. Cyst enlargement, therefore, may suggest the possibility of a choledochal cyst.     

Choledochal cyst and neoplasm: a comprehensive review of 106 cases and presentation of two original cases

Choledochal cysts are prone to complications: cholangitis, biliary cirrhosis, portal hypertension, lithiasis, rupture, pancreatitis, and carcinoma. The coincidence of choledochal cysts and neoplasia ranges from 2.5 to 26 per cent. One hundred six cases of choledochal cysts with neoplasms have been collected from the literature. We have tabulated the results of 68 cases found to have a neoplasm at the initial laparotomy and of 38 patients with pristine choledochal cysts treated electively who subsequently developed a neoplasm. Two original cases are presented. The primary site of neoplasia was not confined only to choledochal cysts. There appears to be a propensity for malignancy to develop anywhere in the biliary tract or gallbladder or pancreas in conjunction with the choledochal cyst. Accompanying choledochal cysts is a high incidence of an anomalous relation at the pancreaticobiliary junction with subsequent malignancy formation. A pathogenetic basis is postulated.     

Magnetic resonance imaging of proximal aortopulmonary window

Of the 187 cases of infantile choledochal cyst treated at our hospitals, we encountered 13 with spontaneous perforation. All cases were under 4 years old. Eight cases were found to have biliary peritonitis and 5 had a sealed perforation. The shape of the extrahepatic bile duct was cystic in 8 and fusiform in 5. The cyst wall around the perforation was filmy and bile was found to be oozing through the thinned wall. Nine perforations were single while 4 cases had multiple perforations. Four of 17 perforations occurred in the posterior part of the cyst wall. Only 1 case of perforation was associated with protein plugs in a common channel, while 7 of the 10 cases of choledochal cyst requiring percutaneous biliary drainage due to signs of raised intrabiliary pressure were found to have protein plugs. We consider that spontaneous perforation of a choledochal cyst is not rare in infancy. The etiology of a perforation must be epithelial irritation of the biliary tract due to refluxed pancreatic juice caused by pancreatico-biliary malunion associated with mural immaturity due to infancy, rather than an abnormal rise in ductal pressure or congenital mural weakness at a certain point.     

Biliary atresia and biliary cysts

The authors present a review of the classification, aetiology, presentation, treatment and long-term outcome of children and adults with biliary atresia and choledochal cyst disease. Biliary atresia should be suspected in any infant with jaundice beyond the second week of life. Although the aetiology and pathogenesis remain unclear, early management with portoenterostomy has significantly improved the course of this disease. Recent advances in immunosuppression have made liver transplantation a valuable and necessary adjunct to biliary bypass. With choledochal cyst disease, adults, unlike children, often present with acute biliary tract symptoms or pancreatitis. The treatment of choice remains extrahepatic cyst excision and biliary bypass. This treatment has excellent long-term results that minimize the development of malignancy.     

Malignant change in the biliary tract after excision of choledochal cyst

BACKGROUND: Choledochal cyst is a rare congenital condition with a high risk of malignant change if untreated. The risk of malignancy after surgical excision of choledochal cyst is not known. METHODS: Forty-eight patients with choledochal cysts managed over a 21-year period were reviewed, to determine the risk of malignant change after cyst excision. Thirty-nine of 48 patients had no carcinoma at first admission; their mean(s.d.) age was 20(18) years. Thirty-seven of 39 patients underwent cyst excision and cholecystectomy followed by hepaticoenterostomy. RESULTS: Cyst excision was incomplete in 28 of the 37 patients because dilated portions of the biliary ducts remained proximally and/or distally. In these 37 patients, no carcinoma has developed in the remnant proximal hepatic duct or the terminal bile duct after mean(s.d.) follow-up of 9.1(6.4) years. In the remaining nine patients, biliary carcinoma was diagnosed at the first visit. Six patients died from recurrence with a mean(s.d.) survival time of 13(11) months, while three patients were alive and free from recurrence 2 months, 1 year and 7 years after operation. CONCLUSION: Malignant change has not been observed after total or subtotal excision of choledochal cysts in this series.     

Scanning electron microscopy studies of the human lens capsule after capsulorhexis

Choledochal cysts are malformations of the biliary ductal system, which rarely occur in infancy. In neonates and infants, choledochal cysts are congenital, may be associated with distal biliary atresia and typically presents with cholestatic jaundice and acholic stools characteristic of biliary obstruction. In older children and adults, it may be acquired in association with an anomalous pancreaticobiliary union and presents with any combination of intermittent jaundice, abdominal pain, and a palpable abdominal mass. Early detection of choledochal cysts is important in the prevention of the fatal complications of biliary obstruction. Here, we present two cases of congenital choledochal cyst in which the patients were 9 weeks old and 7 weeks old respectively. Both of them presented with jaundice, acholic stools, and hepatomegaly. Combined use of sonography, computed tomography and hepatobiliary scintigraphy demonstrated the presence of choledochal cysts. Thereafter, they underwent surgical excision of the cysts with Roux-en-Y reconstruction of the biliary tree; the results were successful.     

Value of MR cholangiopancreatography in evaluating choledochal cysts

OBJECTIVE: The aim of this retrospective study was to clarify whether MR cholangiopancreatography (MRCP) is a suitable replacement for ERCP in evaluation of the choledochal cyst. MATERIALS AND METHODS: Sixteen patients (six adult and 10 pediatric) with choledochal cysts underwent MRCP using a half-Fourier acquisition single-shot turbo spin-echo sequence. Extent of the cyst, defects within the biliary tree, and presence or absence of the anomalous junction of the pancreaticobiliary duct were evaluated. Findings were compared with those of ERCP. RESULTS: MRCP better defined the proximal biliary tree than did ERCP in two patients. Defects within the biliary tree were diagnosed correctly on MRCP in eight patients; however, two defects within the distal common bile duct were missed in pediatric patients. The presence of the anomalous junction of the pancreaticobiliary duct was revealed accurately by MRCP in all adult patients but was revealed accurately in only four of the 10 pediatric patients. CONCLUSION: MRCP appears to offer diagnostic information that is equivalent to that of ERCP for assessment of choledochal cysts in adults. In pediatric patients, MRCP should not replace ERCP; however, MRCP can play an important role as a noninvasive examination and should be considered a first-choice imaging technique for evaluation of choledochal cysts.     

Mitchell osteotomy for adolescent hallux valgus

BACKGROUND: Complications often follow if a choledochal cyst is treated simply by drainage, either internal or external. This study reviews 17 patients who had had previous cystoenterostomy (n = 9) or external drainage (n = 8) and who required reoperation and cyst excision. METHODS: The study was a retrospective review including ten women and seven men managed over 9 years. The indications for reoperation were stone formation (10 patients), pancreatitis (three), portal hypertension (two) and hepatic abscess (one); two patients were asymptomatic. RESULTS: Definitive surgery with cyst excision was possible in all patients who had previously had external cyst drainage and in seven of nine who had had previous cystoenterostomy. There were no deaths. Two postoperative biliary leaks and two duodenal fistulas resolved spontaneously. CONCLUSION: Excision of a choledochal cyst is possible and desirable even after a previous drainage operation. In severely ill patients with a complication of choledochal cyst, external drainage may be a preferable initial manoeuvre.     

Radiation and concurrent carboplatin administration in locally advanced head and neck cancer. A Hellenic Cooperative Oncology Group Study

We report our first experience with a laparoscopic treatment of congenital choledochal cysts involving the total cyst resection and the reconstruction of the biliary and gastrointestinal tracts through a transmesocolic hepatic-jejunal Roux-en-Y loop anastomosis. The procedure was carried out in a 14-kg 6-year-old girl with a congenital choledochal cyst of the first type, according to the Alonso-Lej classification. The cyst was divided using a Multifire EndoGIA 30 stapler. Hepatic-jejunal and jejunojejunal anastomoses were made with 4.0 chrome catgut interrupted sutures. Intestinal recanalization occurred on the 2nd postoperative day and the postoperative course was uneventful. The laparoscopic approach affords several advantages: excellent intraoperative visualization of tiny structures and, therefore, great surgical accuracy; early resumption of peristalsis; no postoperative pain; no laparocele; prevention of adhesions; excellent esthetics; and quicker resumption of school and sports activities.     

Three-dimensional magnetic resonance cholangiopancreatography for evaluation of obstructive jaundice

The purpose of this study was to assess the diagnostic value of magnetic resonance cholangiopancreatography (MRCP) in studying the anatomy, sites, and causes of obstructive jaundice. From September 1994 to May 1996 three-dimensional MRCP was performed on 31 patients with abdominal pain and obstructive jaundice with a fast spin-echo T2-weighted pulse sequence. The images were reconstructed using maximal intensity projection, AVERAGE and SURFACE algorithm processing techniques at a graphics workstation. All the reconstructed images were compared with those obtained using conventional cholangiographic techniques, such as endoscopic retrograde cholangiopancreatography, percutaneous transhepatic cholangiography and drainage, and intraoperative cholangiography. The patients' diagnoses included choledochal cyst (13), cholangiocarcinoma (five), choledocholithiasis (four), pancreatic head carcinoma (three), rhabdomyosarcoma (one), papillary Vater carcinoma (one), recurrent gastric carcinoma (one), ascaris (one), and biliary atresia (two). Extrahepatic biliary dilatation was present in all 13 patients with choledochal cyst; the pancreatic ducts and their entrance level to the common bile duct were observed in eight of these patients. The level of obstruction in patients with cholangiocarcinoma was well documented but the biliary tract of one patient with biliary atresia was not identified by MRCP. In one patient with biliary rhabdomyosarcoma, MRCP clearly delineated the extrabiliary extension of the tumor. In a patient with ascaris in the common bile duct an increase in signal intensity inside the digestive tract of the worm denoted fluid in its gut. Lithiasis was shown in all of the four patients with choledocholithiasis. Thus, MRCP is a useful tool in the assessment of biliary tract obstruction and its causes, and is a valuable addition to ultrasonography.     

The princess and the pea. An unusual complication after a laparoscopic cholecystectomy

A vena porta choledochal fistula caused by an adenocarcinoma arising from a type I choledochal cyst was detected in a 42-year-old woman. The diagnostic and therapeutic aspects of this malignancy are discussed.     

Anatomic anomalies in neonatal cholestatic jaundice

Disorders of the biliary tree are an important cause of cholestatic jaundice in infancy. For the most frequent diseases in this group, biliary atresia and choledochal cyst, prognosis is strongly dependent on timely diagnosis and treatment. In biliary atresia the bile flow is obstructed due to obliteration of the extrahepatic bile ducts. Construction of an hepatic portoenterostomy before 60 days of age will result in restoration of bile flow in the vast majority of patients. When failed, the disease is progressive and ultimately fatal, unless a liver transplantation is performed. For those patients in which restoration of the bile flow succeeds, the subsequent course is strongly dependent on the occurrence of cholangitis. For all patients fat-soluble vitamins should be supplemented and caloric intake should be carefully monitored. Presentation of a choledochal cyst can be either before or after the first year of life. It is mostly characterized by jaundice, with or without abdominal pain. Therapy consists of resection of the cyst, followed by a hepatico-jejunostomy. Paucity of bile ducts is an intrahepatic disorder, in which--almost--no bile ducts can be found in the portal tracts. This anomaly is frequently found in combination with a typical facies, a pulmonary stenosis and vertebral anomalies, a combination which is called Alagille syndrome. Prognosis is generally good.     

Choledochal cysts in adults: a report of two cases and review of the literature

A choledocal cyst is a dilation of some component of the biliary tract that may include both intra- and extra-hepatic sites. They are classified into six types, all of which are relatively rare. Previously, choledochal cysts were treated with biliary-enteric bypass procedures. The current recommendation is to attempt complete excision to minimize the known risk of malignancy and the development of recurrent cholangitis or pancreatitis that may occur in patients with these cystic lesions. Two cases are discussed in which type I choledochal cysts presented. One was removed from a 31-yr-old man who presented with vague abdominal complaints the other from a 32-yr-old man who presented with pancreatitis. The epidemiology, diagnosis, surgical treatment, and risk of cancer in choledochal cysts is described.     

Lithiasic, congenital, choledochal dilatation: problems of diagnosis and therapy

We report a case of a 30-year old female with congenital lithiasic choledochal dilatation which was not diagnosed at the ultrasonographic examination. Congenital biliary dilatation abnormalities are rare and may clinically present with episodic biliary colics or more rarely with recurrent pancreatis. Ultrasound, CT-scanning and ERCP usually make these anatomic alterations evident but in some cases there may be some doubt despite the vast range of radiological techniques available (PTC, Tc99m-Isida scinti-scan). The best results from a diagnostic point of view are obtained from the ERCP that may in fact visualize an anomalous pancreatico-biliary junction, rule out carcinoma, accurately define the cyst dimensions or show the intrahepatic ductal radicals. Furthermore, the extraction of intracystic stones or the treatment of choledochocele through a papillostomy may be performed. However the ERCP may cause traumatic pancreatitis, above all in youngster as was verified in our patient. During surgical exploration, the definitive diagnosis can be achieved via intraoperative cholangiography. We emphasize that in patients with congenital choledochal dilatation, the dilated choledochus should be excised even in young children to avoid the risk of malignancy which may occur also following cyso-duodeno- or cystojejunostomy treatment. In our patient an hepatiocojejunostomy on a Roux-en-Y limb was performed. This single case has been oresented along with a review of the literature to recall such anomalies in differential diagnosis of biliary colics and to stress that the choice treatment is surgical resection.     

Spiral computed tomography for bilioenteric anastomotic stricture

Spiral computed tomography scanning after intravenous cholangiography (IVC-SCT) was used to assess the anatomy of the biliary tree and anastomosis in a 7-year-old girl with recurrent cholangitis 5 years after surgical excision of a choledochal cyst. The technique clearly visualized the stenosis of the hepaticojejunostomy and hepatic duct and the dilated intrahepatic ducts. IVC-SCT provides a precise, prompt, and accurate image with minimal invasiveness and is especially suitable for the preoperative evaluation of pediatric patients.     

Cystic dilation of extrahepatic bile ducts in adulthood: diagnosis, surgical treatment and long-term results

To evaluate the long-term results of surgery for choledohal cyst in adulthood, a series of 13 patients over the age of 16 operated on for choledochal cyst during a period of six years and followed-up for a minimum of 3 years was analyzed. Patients with type I and IVa cysts underwent extrahepatic cyst resection and Roux-en-Y hepatico-jejunostomy. Choledochoceles (type III) were managed endoscopically. No operative mortality or morbidity occurred. Type I and III cysts showed almost ideal follow-up with no sign of stricture on HIDA scan. One type IVa cyst patients developed recurrent cholangitis due to anastomotic stricture, managed percutaneously. Whenever possible, complete cyst resection and Roux-en-Y reconstruction is the treatment of choice for all extrahepatic biliary cysts. Intra- and extrahepatic dilatations are adequately treated by extrahepatic resection and careful endoscopic or radiologic surveillance. Small choledochoceles can be safely managed by endoscopic sphincterotomy.     

Multiple respiratory gas monitoring causes changes of inspired oxygen concentration in closed anesthesia system

This case report describes an accessory hepatic duct (AHD) identified by intraoperative cholangiography during excisional surgery of a choledochal cyst (CC). The accessory duct was divided and reconstructed successfully to the Roux-en-Y jejunal loop. The postoperative course was uneventful, and follow-up abdominal sonography revealed neither evidence of biliary tract obstruction nor atrophic changes of the liver. It is advocated that an AHD should be meticulously reconstructed if it is divided during excisional surgery of a CC.     

Evaluation of mebrofenin hepatoscintigraphy in neonatal-onset jaundice

BACKGROUND: The prognosis of infants with prolonged neonatal jaundice is dependent on early diagnosis because of the need for prompt surgical management of biliary atresia. OBJECTIVE: To evaluate the usefulness of 99 mTcm-trimethylbromo-iminodiacetic acid (TBIDA, mebrofenin) in the investigation of infantile jaundice. MATERIALS AND METHODS: A retrospective study was undertaken of 58 patients with unexplained prolonged neonatal jaundice. Sixty-eight scans were reviewed. RESULTS: Mebrofenin scintigraphy confirmed the presence of a choledochal cyst in three of the four cases with that diagnosis. There were no false negative results in the nine patients with extrahepatic biliary atresia (EHBA). Three further infants had an incorrect histological diagnosis of EHBA. A gall bladder was identified by US in each case and in one of these, scintigraphy showed gut excretion. In the 16 patients with no gut excretion by 24 h, the final diagnoses were intrahepatic cholestasis (n = 7), Alagille's syndrome (n = 3), neonatal hepatitis (n = 3), alpha-1-antitrypsin deficiency (n = 2) and juvenile xanthogranuloma (n = 1). Seven infants had repeat scintigraphy after the administration of ursodeoxycholic acid (URSO). This changed five non-excretors with hepatitis into excretors. Two infants with hepatitis continued to show non-excretion after URSO, but a gallbladder was identified by US in both. CONCLUSIONS: Mebrofenin scintigraphy is accurate in confirming the presence of a choledochal cyst and in refuting the diagnosis of EHBA. While histology and scintigraphy are each 100 % sensitive for the diagnosis of EHBA, neither, individually, is accurate and the investigation of prolonged neonatal jaundice requires a multi-modality imaging strategy.