1/11. Cystic anomalies of biliary tree in the fetus: is it possible to make a more specific prenatal diagnosis?BACKGROUND/PURPOSE: Biliary tree cystic malformations (BCM) occur rarely. Current progress in ultrasonography makes it possible to identify them prenatally. The aim of this study was to analyze retrospectively prenatal ultrasound patterns of 5 consecutive subhepatic cysts in an effort to differentiate the BCM before birth and onset of symptoms. methods: The prenatal ultrasound patterns of 5 consecutive BCM were correlated with the postnatal diagnosis. RESULTS: Case 1 showed a hyperechogenic cyst that underwent enlargement during fetal life; the diagnosis at birth was an obstructed choledochal cyst (CC). In cases 2 and 3, an anechoic cyst with small, stable dimensions during pregnancy was identified; a biliary atresia (BA) type III-C was confirmed at birth. A slightly hyperechogenic cyst with no increase in size at repeated prenatal scans, was observed in cases 4 and 5; a CC and a cystic lesion at the confluence of the hepatic ducts (without obstruction) were diagnosed postnatally, respectively. CONCLUSIONS: The ultrasound scan pattern and size allow to make a prenatal differential diagnosis of BCM. Anechoic, small cyst in the hepatic hilum is highly suspicious for BA. Large, echoic or enlarging cyst is strongly suggestive of "obstructed" CC. Echoic, small and stable cyst is more suggestive of "unobstructed" CC.- - - - - - - - - - ranking = 1keywords = tree (Clic here for more details about this article) |
2/11. Gastrointestinal malformations in two infants born to women with hyperthyroidism untreated in the first trimester.We report two infants with gastrointestinal anomalies: one with esophageal atresia and tracheo-esophageal fistula and the other with biliary tree atresia, born to hyperthyroid women diagnosed and treated with methimazole after 14 weeks' gestation. Euthyroidism was documented in both infants. These cases raise the issue of whether untreated hyperthyroidism and not methimazole intake is the teratogen.- - - - - - - - - - ranking = 0.2keywords = tree (Clic here for more details about this article) |
3/11. Clinical problems with developmental anomalies of the biliary tract.Cholestatic jaundice defined as conjugated hyperbilirubinemia is a typical feature of neonatal liver disease. biliary atresia is the most common disorder producing cholestasis during the first 2 months of life. Syndromic and non-syndromic paucity of the intralobular bile ducts and choledochal cysts can also present with cholestasis during early life. Liver dysfunction from obstruction of the biliary tree must be differentiated from numerous disorders affecting hepatocytes such as congenital infection and inborn errors of metabolism. Early recognition and a stepwise diagnostic evaluation of the cholestatic infant are essential in successfully treating many metabolic and infectious liver diseases of the infant as well as surgically relieving obstruction in patients with biliary atresia.- - - - - - - - - - ranking = 0.2keywords = tree (Clic here for more details about this article) |
4/11. Successful pregnancy outcome in a patient with congenital biliary atresia.Congenital biliary atresia is one of the most common congenital anomalies of the biliary tree. Without surgical correction, two-thirds of all cases of extrahepatic biliary atresia are fatal within 18 months. Historically, the preferred surgical procedure to correct this anomaly has been the Kasai procedure. Surgical treatment of congenital biliary atresia is associated with substantial perioperative morbidity and mortality, as well as long-term sequelae. Portal hypertension and hypersplenism can complicate the course of up to 50% of patients who survive for more than 2 years. Not surprisingly, few of these patients reach adulthood. We report the case of a woman with congenital biliary atresia surgically corrected with the Kasai operation who subsequently reached adulthood and became pregnant. Despite a prenatal course complicated by portal hypertension, bleeding esophageal varices, and hypersplenism, the woman delivered a healthy infant at 36.5 weeks' gestation.- - - - - - - - - - ranking = 0.2keywords = tree (Clic here for more details about this article) |
5/11. Disappearing cyst of the hepatic hilum in uncorrectable biliary atresia.A 2-month-old boy with biliary atresia (BA) with an extremely rare prenatal history is reported. A cystic lesion in the hepatic hilum was diagnosed during prenatal ultrasonography at 20 weeks' gestation. However, it disappeared during later prenatal observation. He was diagnosed with an uncorrectable type of BA after a massive subdural hemorrhage at the age of 66 days. A fetus suspected of having a cyst of the biliary tree should be followed even after the cyst has disappeared spontaneously.- - - - - - - - - - ranking = 0.2keywords = tree (Clic here for more details about this article) |
6/11. Biliary appendico-duodenostomy: a nonrefluxing conduit for biliary reconstruction.Biliary conduits constructed during operations for choledochal cysts or biliary atresia are frequently complicated by reflux of gastrointestinal contents, stasis, and obstruction with resulting cholangitis. We have used the appendix as a biliary conduit for cases of biliary atresia and choledochal cyst, adapting the urologic technique of a tunneled, nonrefluxing anastomosis for reconstruction of the biliary tree--biliary appendico-duodenostomy (BAD). From our preliminary experience with this technique, it appears promising.- - - - - - - - - - ranking = 0.2keywords = tree (Clic here for more details about this article) |
7/11. Idiopathic extrahepatic biliary atresia: recurrence in sibs in two families.Since 1855, 11 instances of familial extrahepatic biliary atresia (EHBA) have been described. Genetic, environmental, vascular, and infectious causes for this disorder have been proposed. We report on 2 families each with 2 sibs with EHBA. We suggest that a genetic susceptibility to an environmental insult to the biliary tree may be responsible for the genesis of this disease.- - - - - - - - - - ranking = 0.2keywords = tree (Clic here for more details about this article) |
8/11. Antenatal diagnosis of biliary atresia (type I cyst) at 19 weeks' gestation: differential diagnosis and etiologic implications.At 19 weeks' gestation, two cystic structures were first identified in the abdomen of a fetus. A repeat ultrasonography at 34 weeks confirmed a definite cyst communicating with the liver. The baby was born at 39 weeks, and serum direct bilirubin started to rise to 4.1 mg/dL. An operative cholangiogram at 23 days of life showed a cystically dilated choledochus with distal atresia and a relatively smooth yet hypoplastic intrahepatic biliary tree. Complete obliteration of the cystic duct was also noted. After excision of the cystic common bile duct, hepatico-jejunal anastomosis was performed, and the patient did well for 8 months postoperatively. Liver biopsy showed proliferation of the bile ductules, but no interlobular bile ducts were observed in any portal triad. A diagnosis of biliary atresia was established. Including the present case, five cases of antenatally diagnosed biliary atresia have been reported. All of them had type I cyst, and antenatal diagnosis was made at 19 to 32 weeks' gestation. Differential diagnosis between biliary atresia of type I cyst and choledochal cyst with complete distal obstruction has been a matter of discussion, and recognition of the entity of antenatally diagnosed biliary atresia is of significant importance from an etiological point of view.- - - - - - - - - - ranking = 0.2keywords = tree (Clic here for more details about this article) |
9/11. Percutaneous retrieval of a fractured biliary guidewire from a reduced liver graft.We describe a unique case of percutaneous retrieval of a broken guidewire in the biliary tree of a patient with a biliary stricture following liver transplantation with a reduced segment 2,3 graft using an Amplatz "goose-neck" snare.- - - - - - - - - - ranking = 0.2keywords = tree (Clic here for more details about this article) |
10/11. hyaline cartilage at porta hepatis in extrahepatic biliary atresia.hyaline cartilage was found on microscopy of sections of the extrahepatic biliary tree in two infants with extrahepatic biliary atresia (EHBA). Respiratory epithelium was not present, and the cartilage did not seem to block the bile duct lumen. hyperbilirubinemia was manifest in one infant on the second postnatal day, but clinical courses were otherwise unremarkable. In neither infant was the ductal plate malformation found on light microscopy of liver biopsy specimens, and in neither infant was visceral topography abnormal. hyaline cartilage at the porta hepatis appears to be a novel finding in EHBA. Its significance remains to be defined.- - - - - - - - - - ranking = 0.2keywords = tree (Clic here for more details about this article) |
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