Prediction of Fetal Biliary Atresia Based on Second and Third-Trimester Ultrasound Characteristics

Abstract

Objective To explore the diagnostic performance of prenatal ultrasound in the prediction of biliary atresia (BA).

Methods We prospectively collected cases of suspected biliary abnormalities in the 2^nd trimester of pregnancy and performed a series (at least 3) of prenatal ultrasound examinations in the 2^nd and 3^rd trimester. The presence of the gallbladder was examined each time, and its size and shape were assessed if the gallbladder was visible. The existence of other abnormalities was carefully evaluated. Neonatal ultrasound examination was conducted within 1 month after birth, and clinical data were followed-up for 6 months after birth.

Results Among the 41 895 patients, 298 were suspected to have biliary abnormalities, while 82 patients were excluded due to loss to follow-up or induced labor caused by other abnormalities. A total of 216 patients were included in this study, and 15 were diagnosed with BA. We summarized the ultrasound findings of the gallbladders and defined a high-risk gallbladder for the prenatal diagnosis of BA. This was demonstrated to have the best diagnostic performance as a single parameter, with an area under the curve of 0.914 (95 %CI: 0.869–0.948). In addition, higher incidences of biliary cysts, right hepatic artery dilation, echogenic bowel, and ascites were observed in BA fetuses. Logistic regression analysis showed that the combination of 5 parameters had better diagnostic performance, with an area under the curve of 0.995 (95 %CI: 0.973–0.999).

Conclusion The fetal gallbladder was found to be a critical feature for the identification of BA. Concomitant abnormalities could be helpful to improve the accuracy of the diagnosis.

Zusammenfassung

Ziel Untersuchung der diagnostischen Leistung des pränatalen Ultraschalls bei der Vorhersage einer Gallengangatresie (BA).

Methoden Wir sammelten prospektiv Fälle mit Verdacht auf Gallenanomalien im 2. Trimenon der Schwangerschaft und führten eine Serie von (mindestens 3) pränatalen Ultraschalluntersuchungen im 2. und 3. Trimenon durch. Das Vorhandensein der Gallenblase wurde jedes Mal überprüft, und wenn die Gallenblase sichtbar war, wurden ihre Größe und Form beurteilt. Auch das Vorhandensein anderer Anomalien wurde sorgfältig geprüft. Eine Ultraschalluntersuchung des Neugeborenen wurde innerhalb des ersten Lebensmonats durchgeführt, und die klinischen Daten wurden 6 Monate postpartum nachverfolgt.

Ergebnisse Bei 298 von 41 895 Patienten bestand der Verdacht auf Gallenanomalien, wobei 82 Patienten wegen fehlender Nachbeobachtung oder wegen eingeleiteter Wehen aufgrund anderer Anomalien ausgeschlossen wurden. Insgesamt wurden 216 Patienten in diese Studie eingeschlossen, bei 15 wurde eine BA diagnostiziert. Wir fassten die Ultraschallbefunde der Gallenblasen zusammen und definierten eine Hochrisikogallenblase für die pränatale Diagnose einer BA. Es zeigte sich, dass dieser Parameter mit einer Fläche unter der Kurve (AUC) von 0,914 (95 %-KI 0,869–0,948) die beste diagnostische Leistung als Einzelparameter aufwies. Darüber hinaus wurde bei BA-Föten eine höhere Inzidenz von Gallenzysten, Dilatation der rechten Leberarterie, echogenem Darm und Seroperitoneum beobachtet. Die logistische Regressionsanalyse zeigte, dass die Kombination von 5 Parametern eine bessere diagnostische Leistung aufwies, mit einer AUC von 0,995 (95 %-KI 0,973–0,999).

Schlussfolgerung Die fetale Gallenblase erwies sich als entscheidender Marker für die Identifizierung einer BA. Begleitende Anomalien könnten hilfreich sein, um die diagnostische Genauigkeit zu verbessern.

Key words

biliary atresia - gallbladder - fetus - prenatal diagnosis - ultrasound

Publication History

Received: 05 December 2020

Accepted: 15 July 2021

Article published online:
08 March 2022

• References

• 1 Hartley JL, Davenport M, Kelly DA. Biliary atresia. Lancet 2009; 374: 1704-1713 DOI: 10.1016/S0140-6736(09)60946-6.
• 2 Serinet MO, Wildhaber BE, Broue P. et al. Impact of age at Kasai operation on its results in late childhood and adolescence: a rational basis for biliary atresia screening. Pediatrics 2009; 123: 1280-1286 DOI: 10.1542/peds.2008-1949.
• 3 Berger Y, Superina RA, Zbar A. et al. A case series of congenital hepatic hilar cyst: recommendations for diagnosis and management. Isr Med Assoc J 2015; 17: 32-36
• 4 Casaccia G, Bilancioni E, Nahom A. et al. Cystic anomalies of biliary tree in the fetus: is it possible to make a more specific prenatal diagnosis?. J Pediatr Surg 2002; 37: 1191-1194 DOI: 10.1053/jpsu.2002.34470.
• 5 Chalouhi GE, Muller F, Dreux S. et al Prenatal non-visualization of fetal gallbladder: beware of biliary atresia!. Ultrasound Obstet Gynecol 2011; 38: 237-238 ; author reply 238–240. DOI: 10.1002/uog.9058.
• 6 Wang Y, Li SL, Chen CY. et al. Diagnosis and clinical evaluation of gallbladder anomalies by prenatal ultrasound screening. Chinese Journal of Medical Ultrasound 2012; 9: 433-438
• 7 Tanaka H, Sasaki H, Wada M. et al. Postnatal management of prenatally diagnosed biliary cystic malformation. J Pediatr Surg 2015; 50: 507-510 DOI: 10.1016/j.jpedsurg.2014.08.002.
• 8 Salomon LJ, Alfirevic Z, Berghella V. et al. Practice guidelines for performance of the routine mid-trimester fetal ultrasound scan. Ultrasound Obstet Gynecol 2011; 37: 116-126 DOI: 10.1002/uog.8831.
• 9 Goldstein I, Tamir A, Weisman A. et al. Growth of the fetal gall bladder in normal pregnancies. Ultrasound Obstet Gynecol 1994; 4: 289-293 DOI: 10.1046/j.1469-0705.1994.04040289.x.
• 10 Hsiao CH, Chang MH, Chen HL. et al. Universal screening for biliary atresia using an infant stool color card in Taiwan. Hepatology 2008; 47: 1233-1240 DOI: 10.1002/hep.22182.
• 11 Balistreri WF, Grand R, Hoofnagle JH. et al. Biliary atresia: current concepts and research directions. Summary of a symposium. Hepatology 1996; 23: 1682-1692 DOI: 10.1002/hep.510230652.
• 12 Harpavat S, Finegold MJ, Karpen SJ. Patients with biliary atresia have elevated direct/conjugated bilirubin levels shortly after birth. Pediatrics 2011; 128: e1428-e1433 DOI: 10.1542/peds.2011-1869.
• 13 Di Pasquo E, Kuleva M, Rousseau A. et al. Outcome of non-visualization of fetal gallbladder on second-trimester ultrasound: cohort study and systematic review of literature. Ultrasound Obstet Gynecol 2019; 54: 582-588 DOI: 10.1002/uog.20252.
• 14 Zhou LY, Wang W, Shan QY. et al. Optimizing the US Diagnosis of Biliary Atresia with a Modified Triangular Cord Thickness and Gallbladder Classification. Radiology 2015; 277: 181-191 DOI: 10.1148/radiol.2015142309.
• 15 Rozel C, Garel L, Rypens F. et al. Imaging of biliary disorders in children. Pediatr Radiol 2011; 41: 208-220 DOI: 10.1007/s00247-010-1829-x.
• 16 Zhou LY, Guan BY, Li L. et al. Objective differential characteristics of cystic biliary atresia and choledochal cysts in neonates and young infants: sonographic findings. J Ultrasound Med 2012; 31: 833-841 DOI: 10.7863/jum.2012.31.6.833.
• 17 Aziz S, Wild Y, Rosenthal P. et al. Pseudo gallbladder sign in biliary atresia – an imaging pitfall. Pediatr Radiol 2011; 41: 620-626 DOI: 10.1007/s00247-011-2019-1.
• 18 Kim WS, Cheon JE, Youn BJ. et al. Hepatic arterial diameter measured with US: adjunct for US diagnosis of biliary atresia. Radiology 2007; 245: 549-555 DOI: 10.1148/radiol.2452061093.
• 19 Caponcelli E, Knisely AS, Davenport M. Cystic biliary atresia: an etiologic and prognostic subgroup. J Pediatr Surg 2008; 43: 1619-1624 DOI: 10.1016/j.jpedsurg.2007.12.058.
• 20 Muise AM, Turner D, Wine E. et al. Biliary atresia with choledochal cyst: implications for classification. Clin Gastroenterol Hepatol 2006; 4: 1411-1414 DOI: 10.1016/j.cgh.2006.07.005.
• 21 Kasai M, Sawaguchi M, Akiyama T. et al. A proposal of new classification of biliary atresia. J Jpn Soc Pediatr Surg 1976; 12: 327-331
• 22 Davenport M, Tizzard SA, Underhill J. et al. The biliary atresia splenic malformation syndrome: a 28-year single-center retrospective study. J Pediatr 2006; 149: 393-400 DOI: 10.1016/j.jpeds.2006.05.030.
• 23 Davenport M, Savage M, Mowat AP. et al. Biliary Atresia Splenic Malformation syndrome: an etiologic and prognostic subgroup. Surgery 1993; 113: 662-668
• 24 Aldeiri B, Giamouris V, Pushparajah K. et al. Cardiac-associated biliary atresia (CABA): a prognostic subgroup. Arch Dis Child 2020; DOI: 10.1136/archdischild-2020-319122.