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Efficacy of ERCP via the accessory papilla in children with choledochal cysts.

BACKGROUND: ERCP via the major duodenal papilla in children with choledochal cyst may not clearly visualize the entire pancreatic duct and the junction of the pancreatic and biliary ducts. This may be caused by obstruction of the pancreatic duct by a dilated common bile duct. METHODS: Patients with choledochal cysts who underwent ERCP with injection of contrast medium at the major duodenal papilla were classified as either belonging to a MP group, in which the entire pancreatic duct and junction of the pancreatic and biliary ducts were visualized, or to a M/AP group, in which these structures were either partially visualized or not visualized. ERCP via the accessory papilla subsequently was performed in the M/AP group by using 0.6- or 0.8-mm metal-tip catheters. The efficacy of ERCP via the accessory papilla in children with choledochal cysts was evaluated. OBSERVATIONS: ERCP was performed in 13 patients. Seven were assigned to the MP group and 6 to the M/AP group. ERCP via the accessory papilla in the M/AP group was successful in 5 of the 6 patients; pancreas divisum was demonstrated in one, a protein plug at the main pancreatic duct in another, and the entire pancreatic duct and junction of the pancreatic and biliary ducts in the remaining 3 patients. No patient developed pancreatitis as a result of ERCP, including ERCP via the accessory papilla. CONCLUSIONS: In children with choledochal cyst, ERCP via the accessory papilla is an effective method for visualization of the detailed structure of the entire pancreatic ductal system and junction of the pancreatic and biliary ducts when ERCP via the major duodenal papilla is unsuccessful.

Child, Preschool↗

Spontaneous rupture of choledochal cyst: an unusual cause of acute abdomen in children.

Rupture is one of the rare complications of choledochal cysts. The cause of rupture is unknown in many cases and is considered to be spontaneous. Two patients admitted to the hospital with acute abdominal picture were initially thought to have postoperative abscess and appendicitis. However, the presence of bilelike fluid suggested a bile tract pathology, and evaluation showed rupture choledochal cysts. Although spontaneous rupture of a choledochal cyst is a rare complication, and can sometimes be the initial manifestation of a choledochal cyst, it should be considered in the presence of bilelike fluid and differentiated from spontaneous perforation of the bile tract. In spontaneous rupture of choledochal cyst, cystectomy, cholecystectomy, and Roux-Y hepaticoportoenterostomy is the treatment of choice.

Abdomen, Acute↗

Large choledochal cyst present through 2 pregnancies. A case report.

BACKGROUND: Choledochal cyst is rarely diagnosed during pregnancy, and it is very difficult to make a diagnosis of it clinically or radiologically. CASE: A woman was diagnosed as having an ovarian cyst and gallbladder mucocele on ultrasonography in both her first and second pregnancies. She was asymptomatic, and conservative management was adopted. Three days after delivery of her second child, the patient had a sudden onset of right upper quadrant pain associated with deranged liver function. Emergency laparotomy revealed a type I choledochal cyst with evidence of infection. Excision of the cyst, cholecystectomy and bilateral hepatojejunostomy in the Roux-en-Y fashion were performed. CONCLUSION: A choledochal cyst in pregnancy is difficult to diagnose and poses a threat to mother and fetus.

Adolescent↗

The current management of choledochal cyst.

The diagnosis and treatment of choledochal cysts have changed dramatically in the past decade. Although history and physical exam remain paramount in arousing clinical suspicions, the classic triad of abdominal pain, jaundice, and a palpable mass is present infrequently. Ultrasonography can readily identify and precisely locate upper abdominal masses and distinguish between intrahepatic, extrahepatic, renal, and gastrointestinal cystic and solid masses. Use of the new iminodiacetic acid tracers then allows visualization of these cysts even in the presence of significant jaundice or abnormal liver function tests. For many years choledochal cystenterostomy was considered the treatment of choice because of the high morbidity and mortality originally associated with attempts at total excision. Over the years, evaluation of cystenterostomy has revealed an unacceptable rate of recurrent jaundice, ascending cholangitis, stone formation, and anastamotic stricture. In addition, a disturbing incidence of highly malignant biliary duct carcinoma that increases with the passage of time has been reported. Total excision of choledochal cysts has been reported in the English and Japanese literature with minimal morbidity and mortality and has become the treatment of choice. The authors' recent experience of two patients with this relatively rare condition confirms these changing trends and will be reported here.

Adolescent↗

Pre-operative management of congenital choledochal cyst with ultrasound-guided percutaneous choledochalcystostomy.

Choledochal cyst is a rare congenital anomaly of the biliary system, presenting in infancy. Two cases of choledochal cyst (one infected) with grossly deranged liver function tests and associated biliary atresia, which were successfully managed pre-operatively by sonoguided percutaneous choledochalcystostomy, are reported here. The use of this technique in the treatment of patients such as these is proposed.

Choledochal Cyst↗

Congenital choledochal cyst with pancreatitis.

OBJECTIVE: To understand the relationship among congenital choledochal cyst, anomalous junction of pancreaticobiliary duct (AJPBD) and pancreatitis. METHODS: 25 children with choledochal cyst treated in our hospital were included in this study. Their ages ranged from 2 months to 14 years. Intraoperative cholangiography was performed in 24 children. Pancreatic samples obtained from the head and body of the pancreas at operation were observed under light and electron microscope. Serum amylase was analyzed one week before and after operation respectively. Bile amylase in the cyst and gallbladder was measured at operation. RESULTS: The incidence of AJPBD was 70%. Inflammatory cell infiltration and fibrous hyperplasia were seen in the pancreatic specimens of 5 children, of whom 4 had AJPBD. No ultrastructural changes were found in 2 children without AJPBD, while different changes were seen in 6 with AJPBD, including those showing no pathological changes under light microscope. All the changes became more severe as the age of the patient increased. The bile and serum amylase levels were higher in children with AJPBD than those without AJPBD (P < 0.05). CONCLUSION: Congenital choledochal cyst has a close relationship with AJPBD and pancreatitis. Pancreatic pathological changes have a long and chronic course from ultrastructural changes to macroscopic changes for the developing of pancreatitis.

Adolescent↗

Choledochal cyst.

The most commonly encountered variant of choledochal cyst (type I) is characterized by fusiform dilatation of the bile duct. Patients usually come to medical attention within the first decade of life. The diagnosis is readily established by ultrasound or computed tomography. Choledochal cyst has been diagnosed in the fetus by maternal ultrasound. Treatment is by surgical excision.

Child↗

Choledochal cyst. A case report.

Choledochal cyst is an unusual entity that has increasingly been encountered by ultrasound and computed tomography (CT) scan. The symptoms are variable and the recommended treatment is surgical excision.

Adult↗

Jejunal interposition hepaticoduodenostomy for choledochal cyst.

Four cases of choledochal cyst with interesting modes of presentation were managed by excision of the cyst and biliary drainage by a new physiological and antireflux technique of jejunal loop interposition hepaticoduodenostomy. The advantages of this new technique over earlier practiced procedures are discussed.

Adolescent↗

Prenatal diagnosis of choledochal cyst using magnetic resonance imaging: a case report.

Choledochal cysts are congenital anomalies of the biliary ducts, characterized by cystic dilatation of the ducts. Prenatal diagnosis of this anomaly using ultrasonography (US) has been well documented. Magnetic resonance imaging (MRI) has recently become an important complement to US in prenatal diagnosis of fetal anomalies. We herein report a patient in whom at 24 wk' gestation US suggested a right upper quadrant abdominal cyst and in whom at 26 wk' gestation MRI more clearly delineated the cyst and its surrounding structures and suggested a choledochal cyst, which was confirmed at postnatal surgery and histopathology.

Adult↗

Choledochal cyst complicating pregnancy: antepartum diagnosis with MRI.

Choledochal cyst presenting during pregnancy is a rare condition, associated with potentially life-threatening complications for both the mother and developing fetus. Clinical symptoms are nonspecific, and radiographic evaluation is complicated by the presence of the gravid uterus. The authors describe a 34-year-old pregnant female presenting in the third trimester with obstructive jaundice and a right upper quadrant mass. Multiplanar and multisequence imaging with MR established the antepartum diagnosis of choledochal cyst, avoiding the use of ionizing radiation during pregnancy.

Adult↗

Spontaneous rupture of choledochal cyst presenting in childhood.

Spontaneous rupture of choledochal cysts is one of the rare complications, and can sometimes be the initial manifestation. It should be considered in the presence of bile-like fluid. A 10-year-old girl had acute onset of abdominal pain, vomiting, and elevated bilirubin, alkaline phosphatase, glutamic oxaloacetic transaminase and glutamic-pyruvic transaminase levels. There was no trauma in her history. In ultrasonography and computed tomography, dilated common bile duct, cystic mass of 10 x 6 cm, and free intraperitoneal fluid in abdominal cavity were demonstrated. Radiological methods, especially intraoperative cholangiography, should be performed for evaluation. We report a case of spontaneous rupture of the choledochal cyst with clinical and radiological findings.

Child↗

Traumatic rupture of choledochal cyst in a child.

Traumatic rupture of choledochal cyst is an extremely rare disorder. The current patient is a 4-year-old boy who fell in a bathroom and suffered a blow to the abdomen. Percutaneous transhepatic cholangiography revealed pancreaticobiliary maljunction. Inflammation of the peritoneal cavity was moderate. At first look, the choledochal cyst was excised and hepaticojejunostomy was performed. At this time, a rupture approximately 2 mm in diameter was recognized at the rear surface of the inferior part of the common bile duct.

Abdominal Injuries↗

[Choledochus carcinoma in choledochal cyst--a rarity. Presentation of the disease with a case report].

Around 1800 choledochal cysts are described in the literature, of which around 6% have turned malignant. The incidence of malignant degeneration increases with age and amounts to as much as 28%. As the prognosis for malignant choledochal cysts is poor, the only therapy is an early cystectomy before the appearance of malignant changes. The diagnosis of the cyst is made using sonography, ERCP and abdominal CT. Histology alone provides conclusive diagnosis of malignant degeneration. The case of a 29-year-old-woman who entered our clinic with non-specific right-side abdominal pains is presented. A choledochal cyst was diagnosed by means of sonography, ERCP and abdominal CT. Intraoperatively, a choledochal carcinoma was found in the choledochal cyst (type Ia according to Todani), with infiltration to the surrounding area as well as liver metastasis. A cystectomy and Roux-Y-hepatico-jejunostomy were performed. The patient died 8 months after the diagnosis.

Adult↗

Clinical and operative findings of choledochal cysts in neonates and infants differ from those in older children.

PURPOSE: Significant differences in the clinical and pathological presentation of choledochal cysts between infants and older children have been noted. We developed a new management strategy according to these differences. METHODS: Between 1990 and 2001, we managed 34 cases of type I choledochal cyst. Nineteen patients were more than 1 year old. Fifteen patients underwent surgery before the age of 1 year, and eight before the age of 2 months. One patient was diagnosed prenatally. All patients underwent choledochal cyst excision and hepaticojejunostomy. Symptoms and signs, and findings from physical examination, laboratory tests, and pathology were recorded and analysed. Follow-up periods ranged from 6 months to 11 years. RESULTS: Eight cases who were less than 1 year old suffered from painless jaundice, and choledochal cysts in this group usually ended as a blind pouch. Their cystic amylase and lipase concentrations were within normal limits. Biliary cirrhosis was diagnosed by liver biopsy in three of these patients. In contrast, 17 of the 19 patients who were more than 1 year old had abdominal pain, and 15 of their cysts were connected to the pancreatic duct. Cystic amylase and lipase concentrations were often elevated. All patients had an excellent outcome. CONCLUSION: Diseases with different pathogeneses may cause different clinical manifestations and pathology of choledochal cysts in infants and older children. Some patients develop biliary cirrhosis in the early months of life. Our findings show that it is safe and imperative to treat neonates and infants with choledochal cysts as soon as possible, as delayed surgery may cause severe complications.

Age Factors↗

[Perforation of choledochal cyst in a 12-year-old boy].

Choledochal cyst is a rare congenital malformation that presents in children and young adults and is more frequent in women. Early diagnosis is essential to avoid possible complications. The method of choice for the initial diagnosis is ultrasound. Treatment consists of complete resection of the cyst. We describe a case of perforation of a choledochal cyst in a 12-year-old boy.

Child↗

Development of intrahepatic biliary stones after excision of choledochal cysts.

BACKGROUND: The incidence of intrahepatic cholelithiasis and cholangitis has not yet been well studied postoperatively in patients with choledochal cysts. METHODS: One hundred three patients with choledochal cysts had operative cholangiography, underwent standard excision of a choledochal cyst with Roux-en-Y hepatico-jejunal anastomosis, and were at a mean follow-up of 12 1/2 years. The incidence of intrahepatic bile duct stones was analyzed according to the 3 morphologic types of intrahepatic bile duct observed at initial operative cholangiography: type 1, no dilatation of the intrahepatic bile ducts; type 2, dilatation of the intrahepatic bile ducts but without any downstream stenosis; and type 3, dilatation of the intrahepatic bile ducts associated with downstream stenosis. Initially, there was no evidence of intrahepatic bile duct stones in any of the 103 patients. RESULTS: Among 50 type 1 patients, intrahepatic cholelithiasis developed in only 1 patient (2%). Among 43 type 2 patients, 1 patient (2%) had intrahepatic cholelithiasis, and 2 (5%) had postoperative cholangitis. Among 10 type 3 patients, 4 (40%) had intrahepatic cholelithiasis (P <.01), and 3 (30%) had postoperative cholangitis. Time intervals between the initial surgery and the first identification of intrahepatic stones ranged from 3 to 22 years. CONCLUSIONS: One of the major causes of formation of intrahepatic cholelithiasis has been clarified; patients with intrahepatic biliary dilatation with downstream stenosis can get intrahepatic bile duct stones long after excision of a choledochal cyst.

Bile Ducts, Intrahepatic↗

Surgical removal of protein plugs complicating choledochal cysts: primary repair after adequate opening of the pancreatic duct.

BACKGROUND/PURPOSE: Recently, pancreaticoduodenectomies have been performed for choledochal cysts complicated by protein plugs. The authors describe a new surgical procedure for removal of protein plugs or pancreatic stones that does not involve pancreatectomy. METHODS: Protein plugs were detected in 43 (34.1%) of 126 pediatric patients with choledochal cysts. The plugs disappeared spontaneously in 26 patients and were removed using nonsurgical methods in seven. The 10 patients treated surgically are considered here. RESULTS: Protein plugs were removed with the residual cyst in one patient. The plugs in the common channel were flushed into the duodenum by irrigation with saline through the narrow segment beneath choledochal cyst in another. The plugs were removed through the narrow segment using a blunt spoon in four. In the other four patients, the plugs that were too large or too hard to be removed through the narrow segment were taken away through an incision in the main pancreatic duct. This was followed by primary closure of the main pancreatic duct using the narrow segment as a flap to prevent stricture formation. All of the patients have done well. CONCLUSIONS: Protein plugs complicating choledochal cysts can be removed without pancreatectomy. Particularly, primary repair of the pancreatic duct using a flap made of the narrow segment beneath the choledochal cyst is a safe and feasible method to remove large or hard protein plugs.

Adolescent↗