Definitive exclusion of biliary atresia in infants with cholestatic jaundice: the role of percutaneous cholecysto-cholangiography

Abstract

Definitive exclusion of biliary atresia in the infant with cholestatic jaundice usually requires operative cholangiography. This approach suffers from the disadvantage that sick infants are subjected to a time-consuming and potentially negative surgical exploration. The purpose of this study was to determine if percutaneous cholecystocholangiography (PCC) prevents unnecessary laparotomy in infants whose cholestasis is caused by diseases other than biliary atresia. This study is a 10 year retrospective review of all infants with persistent direct hyperbilirubinemia and inconclusive biliary nuclear scans who underwent further evaluation for suspected biliary atresia. A gallbladder ultrasound (US) was obtained in all patients. When the gallbladder was visualized, further imaging by PCC was done under intravenous sedation; otherwise, the standard operative cholangiogram (OCG) was performed, with liver biopsy as indicated. The primary outcome was the diagnostic accuracy of PCC, especially with respect to preventing a laparotomy. There were 35 infants with suspected biliary atresia, with a mean age of 8 weeks (range 1-14 weeks). Nine infants whose gallbladder was visualized by ultrasound underwent PCC that definitively excluded biliary atresia. Of this group, the most frequent diagnosis (five patients) was total parenteral nutrition-associated cholestasis. The other 26 infants with absent or decompressed gallbladder had laparotomy and OCG, which identified biliary atresia in 16 patients (61%). Laparotomy was avoided in all 9 patients who underwent PCC, thus reducing the negative laparotomy rate by 47%. There were no complications associated with PCC. Several alternative techniques to operative cholangiogram have been described for the definitive exclusion of biliary atresia, but many of these have distinct drawbacks. Advances in interventional radiology techniques have permitted safe percutaneous contrast evaluation of the biliary tree. Identification of a normal gall bladder on sonogram is highly predictive of the absence of biliary atresia. Further confirmation can be accurately obtained by a combination of PCC and percutaneous liver biopsy.

Common misdiagnoses of biliary atresia

“…The traditional approach is to perform laparotomy and IOC, and to proceed with immediate portoenterostomy if BA is confirmed. Unfortunately, some infants with cholestatic jaundice may be misdiagnosed and will be subjected to a potentially negative surgical exploration [7].…”

A screening algorithm for the efficient exclusion of biliary atresia in infants with cholestatic jaundice

“…Since it was reported in BA patients in 1988, PC has become a valuable preoperative test in this setting, and its safety and ease-of-use have improved [6,22]. Though invasive, improved technique by interventional radiologists has made this modality possible even in patients with a small GB, and PC may exclude BA in up to 47% of infants studied [23]. In some selected centers, endoscopic retrograde cholangiopancreatography (ERCP) is done as an alternative to PC and there are data supporting its feasibility and accuracy in the pediatric population [24,25].…”

Laparoscopic cholecystostomy and bile duct lavage for treatment of inspissated bile syndrome: a single-center experience

“…DBIL: direct bilirubin; AST: aspartate aminotransferase. cholangiography (PCC) [8] and percutaneous transhepatic cholangiography (PTTC) [9] with contemporary therapeutic saline lavage of the biliary tree could avoid unnecessary laparotomy, but they are difficult procedures for infants. Moreover, it is sometimes diffi cult to obtain a suffi cient fl ushing pressure.…”