A screening algorithm for the efficient exclusion of biliary atresia in infants with cholestatic jaundice.
BACKGROUND
Neonates with cholestasis may undergo many tests before biliary atresia (BA) or an alternative diagnosis is reached, and delayed intervention may worsen outcomes. An optimal diagnostic approach to reduce risk, cost, and delay has yet to be defined. The purpose of this study was to develop an algorithm that rapidly and accurately excludes BA for infants with cholestatic jaundice.


METHODS
A single-center retrospective comparison of diagnostic workup was made between cholestatic infants with BA, and those without BA who underwent hepatobiliary iminodiacetic acid (HIDA) scan during admission. Patients were born between 2000 and 2010 and those older than 100days at assessment were excluded. Sensitivity and specificity analysis of predictive variables was performed and an algorithm constructed.


RESULTS
There were 45 BA and 167 non-BA patients. Some variables were 100% sensitive for the exclusion of BA: conjugated bilirubin <2.5mg/dL, gamma-glutamyl transpeptidase <150U/L, excretion on HIDA, or a normal percutaneous cholangiogram. Clinical variables and ultrasound were less useful as screening tests owing to low specificity and sensitivity, respectively. Liver biopsy was 98% sensitive and 84% specific in the diagnosis of BA. An algorithm was constructed that rules out BA with a negative laparotomy rate of 3-22%.


CONCLUSIONS
We propose a screening algorithm for infants with conjugated hyperbilirubinemia that permits efficient exclusion of BA with minimal invasive testing and with a low risk of negative laparotomy. This algorithm now requires prospective evaluation to determine its diagnostic accuracy and its ability to reduce hospital costs, patient morbidity, and time to Kasai portoenterostomy in patients with BA.
Division of Pediatric Surgery, The Hospital for Sick Children, Toronto, Ontario, Canada; Department of Surgery, University of Toronto, Toronto, Ontario, Canada. Electronic address: tjancele@uthsc.edu.
Tim
T
Jancelewicz
Division of Pediatric Surgery, The Hospital for Sick Children, Toronto, Ontario, Canada; Department of Surgery, University of Toronto, Toronto, Ontario, Canada.
Rebecca
R
Barmherzig
Division of Pathology and Pediatric Laboratory Medicine, The Hospital for Sick Children, Toronto, Ontario, Canada; Department of Pediatrics, University of Toronto, Toronto, Ontario, Canada.
Catherine T-S
CT
Chung
Division of Gastroenterology, Hepatology and Nutrition, The Hospital for Sick Children, Toronto, Ontario, Canada; Department of Pediatrics, University of Toronto, Toronto, Ontario, Canada.
Simon C
SC
Ling
Division of Gastroenterology, Hepatology and Nutrition, The Hospital for Sick Children, Toronto, Ontario, Canada; Department of Pediatrics, University of Toronto, Toronto, Ontario, Canada.
Binita M
BM
Kamath
Division of Gastroenterology, Hepatology and Nutrition, The Hospital for Sick Children, Toronto, Ontario, Canada; Department of Pediatrics, University of Toronto, Toronto, Ontario, Canada.
Vicky L
VL
Ng
Department of Diagnostic Imaging, Division of Image Guided Therapy, The Hospital for Sick Children, Toronto, Ontario, Canada.
Joao
J
Amaral
Division of Gastroenterology, Hepatology and Nutrition, The Hospital for Sick Children, Toronto, Ontario, Canada; Department of Pediatrics, University of Toronto, Toronto, Ontario, Canada.
Constance
C
O'Connor
Division of Pediatric Surgery, The Hospital for Sick Children, Toronto, Ontario, Canada; Department of Surgery, University of Toronto, Toronto, Ontario, Canada.
Annie
A
Fecteau
Division of Pediatric Surgery, The Hospital for Sick Children, Toronto, Ontario, Canada; Department of Surgery, University of Toronto, Toronto, Ontario, Canada.
Jacob C
JC
Langer
D000465:Algorithms; D001656:Biliary Atresia / Q000150:complications / Q000175:diagnosis*; D001706:Biopsy; D002758:Cholangiography / Q000379:methods*; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D041781:Jaundice, Obstructive / Q000175:diagnosis* / Q000209:etiology; D007813:Laparotomy; D008297:Male; D012189:Retrospective Studies
Biliary atresia; Cholestasis; Cholestatic jaundice; Neonatal