Online Exclusives
A very low-birth-weight infant with spontaneous perforation of a choledochal cyst and adjacent pseudocyst formation
Spontaneous perforation of a choledochal cyst with ensuing pseudocyst formation is a very rare complication. We report the development of a pseudocyst adjacent to a choledochal cyst in a very low-birth-weight infant at 2 months of age. Elective excision of the choledochal cyst and biliary tract reconstruction were successfully performed 2 months later when the infant weighed 3 kg. Delayed primary repair may be a viable alternative treatment for low-birth-weight infants with choledochal cysts.Histopathologic improvement in biliary cirrhosis after definitive surgery for choledochal cyst
Choledochal cyst causes liver fibrosis, the extent of which varies with each case. Liver damage seldom progresses to cirrhosis, but when it does, it is generally irreversible. We report an infantile case of liver cirrhosis associated with choledochal cyst in which complete clinical resolution was achieved by surgery. Pancytopenia caused by splenomegaly, massive ascites, hypoalbuminemia, and coagulation abnormality that were observed during the early postoperative period had disappeared within 4 months after surgery.Neuroendocrine tumor of the common hepatic duct, mimicking a choledochal cyst in a 6-year-old child
We report a rare case of a neuroendocrine tumor of the extrahepatic biliary tree in a child. A 6-year-old girl who presented with jaundice and pruritus was found to have elevated liver enzymes along with a cystic dilatation of the common hepatic duct. After further diagnostic testing, a working diagnosis of a type I choledochal cyst was established. Laparotomy revealed hydrops of the gall bladder, an elongated and dilated cystic duct and a cystic dilatation of the common hepatic duct. An unusually thickened common bile duct was also noted at the time.Chylous ascites after excision of a choledochal cyst in a child
We report a case of chylous ascites developing 2 weeks after excision of a choledochal cyst with a Roux-en-Y hepaticojejunostomy. Despite the failure of the initial attempts to resolve the chylous ascites by fasting, subsequently, we successfully treated the patient's chylous ascites with intravenous somatostatin. No obvious adverse side effects occurred during the use of somatostatin. To the best of our knowledge, this is the first report of chylous ascites after choledochal cyst excision in children, which was successfully treated with somatostatin.Association of gastroschisis and choledochal cyst
Two infants are described who presented in the neonatal period with a direct hyperbilirubinemia. This was initially presumed to be because of the diagnosis of gastroschisis and the prolonged use of parenteral nutrition. However, both infants were eventually found to have an associated choledochal cyst. The cases are a novel association not previously reported and should heighten the awareness that anatomical causes of a direct hyperbilirubinemia need to be ruled out in all neonates.Development of bile duct cancer in a 26-year-old man after resection of infantile choledochal cyst
Anomalous arrangement of the pancreaticobiliary duct is considered to be a high-risk factor for biliary tract malignancy. We report a case of intrahepatic cholangiocarcinoma in a 26-year-old man after total resection of choledochal cyst with anomalous arrangement of the pancreaticobiliary duct at the age of 5 months. He had been doing well after total resection of the choledochal cyst; however, he suddenly presented with a spiky fever and abdominal pain in the right upper quadrant at the age of 26 years.Mature cystic teratoma arising at the porta hepatis: a diagnostic dilemma
Mature cystic teratoma (MCT) is a tumor of embryological origin, which contains tissue derived from any or all germ cell lines found in an abnormal location. We present 2 cases of MCT both arising from the porta hepatis that were incorrectly thought perioperatively to be a choledochal cyst, which subsequently were demonstrated on histology to be a MCT.Duodenojejunal atresia with absent dorsal mesentery, choledochal cyst, and malrotation in a premature newborn—a case report
We present the case of a premature baby who was born with the following conditions: an extensive atresia from the first part of the duodenum to the mid small bowel; malrotation of the distal part, in volvulus and in an “apple peel” configuration; no connection of the bile ducts to the bowel; and presence of a type II choledochal cyst. To our knowledge, this is the first case in which a combination of these anomalies is reported. A brief review of the relevant literature is also presented.Spontaneous rupture of choledochal cyst with pseudocyst formation—report on 2 cases and literature review
Spontaneous rupture and subsequent bile peritonitis are rare complications of choledochal cysts. Of these complications, the formation of a biliary pseudocyst is an unusual form, and its preoperative diagnosis is difficult. In this report, we describe 2 cases showing spontaneous rupture with biliary pseudocyst formation. Inflammatory tissue surrounded those pseudocysts, one of which was adjacent to the perforation and the other formed in the transverse mesocolon apart from the biliary tract. These pseudocysts were removed by careful dissection, and single-stage cyst excision with biliary reconstruction was successfully performed in both cases.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.
