1/106. Extrahepatic biliary atresia associated with trisomy 18.A case of extrahepatic biliary atresia (EBA) associated with trisomy 18 is presented. A 1-month-old boy was suspected to have alagille syndrome with obstructive jaundice, a systolic heart murmur, growth retardation, and a small, pointed chin. However, surgery and chromosomal analysis revealed EBA associated with trisomy 18. Chromosomal examination must be performed in patients with jaundice and congenital anomalies. It is possible that EBA in trisomy 18 syndrome is due to a chromosomal disorder.- - - - - - - - - - ranking = 1keywords = extrahepatic (Clic here for more details about this article) |
2/106. Antenatal diagnosis of biliary atresia (noncorrectable cyst type): a case report.At 32 weeks of gestation a cystic mass was identified in the hepatic hilum of a fetus by maternal sonography. laparotomy was performed at 39 days of life after a diagnosis of correctable type of biliary atresia (Type I). A cystically dilated extrahepatic duct, in which the proximal and distal sides of the common bile duct were occluded (Type III with cyst, noncorrectable type), was identified by operative cholangiography. A standard Kasai operation was performed, and 1 year after operation the patient was doing well and was jaundice-free. From this experience in routine maternal sonography, a cystic mass in the hepatic hilum may also suggest Type III biliary atresia with a cyst.- - - - - - - - - - ranking = 1.0042713125052keywords = extrahepatic, bile duct, bile, duct (Clic here for more details about this article) |
3/106. Combined pure esophageal atresia, duodenal atresia, biliary atresia, and pancreatic ductal atresia: prenatal diagnostic features and review of the literature.The authors recently managed a case of combined pure esophageal, duodenal, biliary, and pancreatic ductal atresia in a fetus and newborn with trisomy 21. The authors present a case report and review of the literature, emphasizing the prenatal radiographic features of the combined lesions and the high incidence of associated anomalies including Down's syndrome. Prenatal suspicion of these anomalies warrants karyotype analysis as well as careful pre- and postnatal screening for other anomalies.- - - - - - - - - - ranking = 0.0010444677223015keywords = duct (Clic here for more details about this article) |
4/106. Unexpected life-threatening complications in Kabuki syndrome.Kabuki syndrome is a rare multiple congenital anomalies/mental retardation syndrome comprising a distinct facial appearance and fetal fingertip pads. We observed two patients with Kabuki syndrome and describe unusual life-threatening complications, including stenosis of the central airways (not previously reported), extrahepatic biliary atresia, and congenital diaphragmatic hernia.- - - - - - - - - - ranking = 1keywords = extrahepatic (Clic here for more details about this article) |
5/106. biliary atresia splenic malformation syndrome--is it a result of embryonically midline rotational defects? A case report.The authors present a case of biliary atresia splenic malformation (BASM) syndrome in a 34-week gestational age boy who died shortly after birth. autopsy results showed polysplenia, short pancreas, extrahepatic biliary atresia, heterotaxy of liver, tracheoesophageal and duodenal atresia, right lung hypoplasia, cardiac defects with tetralogy of fallot, axial skeletal defects, and minor genitourinary disorders. Additional histopathologic findings were congenital hepatic fibrosis and bronchopulmonary dysplasia. The authors consider that the abnormalities of this case are possibly a unique combination. Midline defects, which are the most notable features of the presented case, may be closely related to pathogenesis of BASM syndrome.- - - - - - - - - - ranking = 1keywords = extrahepatic (Clic here for more details about this article) |
6/106. A case of biliary atresia with cystic dilatation of the extrahepatic bile duct and polysplenia syndrome.A 79-day-old girl presented with jaundice and acholia. Laboratory findings disclosed elevated levels of bilirubin, transaminases, and gamma-glutamyl transferase. A chest x-ray film showed dextrocardia. A computed tomographic scan revealed a cystic mass at the porta hepatis, multiple spleens in the right side of the abdomen, and absence of the inferior vena cava. Under the diagnosis of biliary atresia (BA) (I-cyst) associated with polysplenia syndrome, a hepatic portojejunostomy was performed at 80 days of age. The postoperative course was uneventful, and the jaundice cleared. Although the occurrence of associated anomalies in BA is rare, polysplenia is most commonly seen, and its incidence is reported to be 2% to 10%. patients with BA and polysplenia usually have a poor prognosis, however, this patient may have a good prognosis due to uneventful recovery from the jaundice.- - - - - - - - - - ranking = 4.0162496758429keywords = extrahepatic, bile duct, bile, duct (Clic here for more details about this article) |
7/106. Effective percutaneous transhepatic biliary drainage for cystic dilatation of the intrahepatic biliary system in biliary atresia: a case report.We report on a 16-year old girl with postoperative biliary atresia developing cystic dilatation of the intrahepatic biliary system (CDIB) with abdominal pain, which was relieved after percutaneous transhepatic biliary drainage (PTBD). A cyst was detected in segment 4 of the liver at the age of 14 years. She developed severe right hypochondralgia, subfever and liver dysfunction. Percutaneous cholangiogram showed a solitary cyst without communication and a PTBD tube was placed within the cyst. After lavage of the cyst through PTBD, CDIB was re-communicated with the intestine and the pain was relieved with reduction of the cyst size. From this experience, PTBD may be a less invasive treatment as the first choice for non-communicating CDIB and may alleviate the symptoms when recommunication with the intestine is obtained.- - - - - - - - - - ranking = 0.0002088935444603keywords = duct (Clic here for more details about this article) |
8/106. A case of biliary atresia with duplication of the common bile duct.The authors report a case of biliary atresia (BA) with duplication of the common bile duct. A 1-month-old girl was referred for investigation of persistent jaundice. BA was suspected and confirmed with intraoperative cholangiography. A portoenterostomy was performed at 50 days of age. During dissection of the fibrous remnant of the common bile duct, a separate fibrous bile duct remnant running in parallel on the left, was identified. Histologically, there was bile duct proliferation at the porta hepatis of the left bile duct remnant. A diagnosis of BA with duplicated common bile duct was made. Postoperative course was uneventful, and at a follow-up assessment at 7 months, she was jaundice free, and her progress has been unremarkable.- - - - - - - - - - ranking = 0.040624189607235keywords = bile duct, bile, duct (Clic here for more details about this article) |
9/106. portal vein phlebolithiasis found post-liver transplantation in the native liver of a child with biliary atresia.biliary atresia is defined as partial or total obliteration of the extra-hepatic bile ducts. In advanced cases, liver transplantation (LTx) is considered the most appropriate treatment. This report describes a female patient whose biliary atresia and subsequent cirrhosis required LTx at 1 yr of age. Macroscopic inspection of the hilar region of the native liver post-Tx revealed the formation of a pouch in the hepatic duct and a stone in the lumen of the portal vein. x-ray diffraction analysis showed that the stone was composed of cholesteryl cinnamate, gluconic acid phenylhydrazide, Na beta broma-allyl mercaptomethyl penicillinate, and Al2O3 crystals. While the cholesterol component is a known element of gallstones, we attributed the Na beta broma-allyl mercaptomethyl penicillinate to the patient's drug therapy. Our literature search revealed no previous record or crystallographic analysis of portal vein phlebolithiasis. In this report we describe this rare finding.- - - - - - - - - - ranking = 0.0042713125051838keywords = bile duct, bile, duct (Clic here for more details about this article) |
10/106. association of biliary atresia and urogenital sinus.A case of an association of extrahepatic biliary atresia (EHBA) and urogenital sinus (UGS) anomaly that was diagnosed as an urachal remnant antenatally is reported. Diagnostic laparoscopy of the abdominal cavity in the postnatal period was the most helpful step for making the diagnosis. biliary atresia and urogenital sinus, which was the cause of bladder outlet obstruction, were treated successfully. To the authors' knowledge, this is the first case of this type of association introduced to the literature. J Pediatr Surg 36:635-637.- - - - - - - - - - ranking = 1.0001052805936keywords = extrahepatic, obstruction (Clic here for more details about this article) |
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