1/14. 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 = 1keywords = gestation (Clic here for more details about this article) |
2/14. 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 = gestation (Clic here for more details about this article) |
3/14. Extrahepatic biliary atresia with laterality sequence anomalies.The patient was the first child of first cousin parents. He was born at term after an uneventful pregnancy with normal height, weight and head circumference. jaundice appeared at 15 days of age. Ventricular septal defects and valvular pulmonary stenosis were diagnosed. An hepatic workup revealed extrahepatic biliary atresia and abdominal situs inversus. Hepatic biopsy showed cirrhosis with intrahepatic cholestasis. Genetic factors are suggested in extrahepatic biliary atresia. Analysis of segregation patterns suggested the existence of two major groups, one with various combinations of anomalies within the laterality sequence and the other with one or two anomalies mostly involving the cardiac, gastrointestinal, and urinary systems. This patient belongs to the first group.- - - - - - - - - - ranking = 0.044187825032826keywords = pregnancy (Clic here for more details about this article) |
4/14. prenatal diagnosis of extrahepatic biliary duct atresia.A rare case of extrahepatic biliary atresia was diagnosed by a combination of prenatal ultrasound and measurements of fetal digestive enzymes in amniotic fluid. Ultrasound at 15 and 18 weeks' gestation failed to detect the gall bladder, and amniotic fluid digestive enzyme values were below the fifth percentile. The patient decided to terminate the pregnancy. Post-abortal pathological examination confirmed the diagnosis.- - - - - - - - - - ranking = 1.0441878250328keywords = gestation, pregnancy (Clic here for more details about this article) |
5/14. 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 = 0.044187825032826keywords = pregnancy (Clic here for more details about this article) |
6/14. 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 = 1keywords = gestation (Clic here for more details about this article) |
7/14. Rapid progression of intrapulmonary arteriovenous shunting in polysplenia syndrome associated with biliary atresia.This report describes a patient with biliary atresia (BA) associated with polysplenia syndrome who showed a rapid progression of intrapulmonary arteriovenous shunting (IPS), resulting in a fatal outcome. Intrauterine ultrasonography at 36 weeks of gestation revealed fetal abnormalities, including situs inversus, absent retrohepatic inferior vena cava, and azygous connection. She was diagnosed postnatally as BA because of persistent acholic stool and neonatal jaundice. She underwent hepatic portoenterostomy at age 158 days. The gallbladder and the hepatic ducts were hypoplastic, and the common bile duct was absent. Magnetic resonance image and operative findings also identified polysplenia and an absent portal trunk. Liver histology showed cirrhotic changes and bile duct proliferation. Postoperatively, she achieved good bile secretion, with gradual decrease of total bilirubin. However, she had repeated febrile episodes, and computerized tomography at age 7 months showed multiple liver cysts. Thereafter, she presented with exertional dyspnea. Contrast-enhanced echocardiography showed IPS with a degree of 2/III at age 8 months and 3/III at 10 months. (99m)technetium-labeled macroaggregated albumin ((99m)Tc-MAA) scintigraphy revealed a shunt ratio of 25.5% at 9 months and 39.7% at 10 months. Percutaneous transhepatic drainage of the bile cysts was performed without success. Sludged bile was obtained. However, respiratory distress rapidly progressed, and she died at age 11 months. In the present patient, the association of polysplenia syndrome and absent portal vein with BA, as well as liver cirrhosis, seemed to be contributing factors to rapid progression of IPS in early life.- - - - - - - - - - ranking = 1keywords = gestation (Clic here for more details about this article) |
8/14. Complete absence of bile and pancreatic ducts in a newborn: a new entity of congenital anomaly in hepato-pancreatic development.We examined a newborn who had no bile and pancreatic ducts. Hydrops was evident after 29 weeks of gestation and she died shortly after birth, weighing 1,368 g. One of her siblings had died of hydrops at about six months of gestation, and there were two more miscarriages of unknown cause. At autopsy on the newborn, the liver had an abnormally round shape and the pancreas was not in the normal position. There was an ectopic small pancreas with normally developed islets. Histological analysis revealed the complete absence of extra- and intra-hepatic bile and pancreatic ducts. Immunostaining of these tissues showed no positive bile duct marker staining using epithelial membrane antigen and cytokeratin 19 in the liver. Albumin and alpha-fetoprotein staining was positive in the liver, and insulin and glucagon staining was positive in the remaining islets. Thus, this case is characterized by complete absence of bile and pancreatic ducts. These findings suggest the existence of a gene linked to the development of bile and pancreatic ducts.- - - - - - - - - - ranking = 2keywords = gestation (Clic here for more details about this article) |
9/14. Simultaneous correction of biliary atresia and small bowel atresia in the neonate.The coexistence of biliary atresia and small bowel atresia has been described. However, attempts to correct the biliary atresia at the same time as small bowel atresia repair have not been detailed. In 1986, a 2,170-g female was delivered at 36 weeks' gestation. Postpartum, duodenal obstruction was suspected after oral feedings were not tolerated. At 37 hours after birth, laparotomy showed unsuspected biliary atresia in addition to intestinal malrotation, duodenal web, and type IIIb jejunal atresia with an apple-peel configuration of the upper jejunum and mesentery supplied by the superior mesenteric artery with normal small bowel length and normal ileal mesentery. A modified Roux-en-Y hepatic portoenterostomy was performed using the blind end of the apple-peel configuration of jejunum, with a side-to-side duodenojejunostomy 25 cm from the portoenterostomy. Microscopic examination of the fibrous extrahepatic biliary tissue showed a disarray of small bile ductules. The maximal ductal size at the portoenterostomy site was 600 microns. A liver biopsy specimen showed normal architecture with eosinophils infiltrating the portal triads and periductal areas. Postoperatively, she has maintained normal growth and development with no episodes of cholangitis. Liver biopsies at 1 year and 3 years were normal. At 4 years, total and conjugated bilirubin, serum proteins, liver enzymes, coagulation factors, and trace elements were normal. We conclude that simultaneous correction of biliary atresia and small bowel atresia can be accomplished in the neonate with excellent early results.- - - - - - - - - - ranking = 1keywords = gestation (Clic here for more details about this article) |
10/14. 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 = 1.1767513001313keywords = gestation, pregnancy (Clic here for more details about this article) |
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