Abstract
Background
Hepatobiliary scintigraphy is highly sensitive for diagnosing biliary atresia; however, its specificity has varied in the literature from 35% to 97%.
Objective
The purpose of this study was to re-evaluate the accuracy of phenobarbital-enhanced hepatobiliary scintigraphy in differentiating biliary atresia from other causes of neonatal cholestasis.
Materials and methods
We retrospectively reviewed all hepatobiliary scans of infants with cholestasis at our institution from December 1990 to May 2011. Per our routine protocol the scans were obtained after pretreatment with phenobarbital (5 mg/kg/day for 5 days) to achieve a serum level of ≥15 mcg/ml. Normal hepatic uptake with no biliary excretion by 24 h was considered consistent with biliary atresia.
Results
One hundred eighty-six infants with 210 hepatobiliary scans composed the study group. Forty-three (23%) infants had the final diagnosis of biliary atresia. Hepatobiliary scintigraphy was 100% sensitive, 93% specific and 94.6% accurate in diagnosing biliary atresia. Of the 186, 39/111 (35.1%) term and 2/68 (2.9%) preterm infants had biliary atresia; two of seven children with unknown gestational age also had biliary atresia. Other diagnoses included neonatal hepatitis, total parenteral nutrition cholestasis, Alagille syndrome, cystic fibrosis, choledochal cyst, hypothyroidism, alpha-1 antitrypsin deficiency and persistent cholestasis of unknown etiology.
Conclusion
Phenobarbital-enhanced hepatobiliary scintigraphy is highly accurate in differentiating biliary atresia from other causes of neonatal cholestasis. Biliary atresia is rare in premature infants.
Similar content being viewed by others
References
Sokol RJ, Mack C, Narkewicz MR et al (2003) Pathogenesis and outcome of biliary atresia: current concepts. J Pediatr Gastroenterol Nutr 37:4–21
Landing BH (1974) Considerations of the pathogenesis of neonatal hepatitis, biliary atresia and choledochal cyst—the concept of infantile obstructive cholangiopathy. Prog Pediatr Surg 6:113–139
Kirks DR, Coleman RE, Filston HC et al (1984) An imaging approach to persistent neonatal jaundice. AJR Am J Roentgenol 142:461–465
Makin E, Davenport M (2006) Biliary atresia. Curr Paediatr 16:59–63
Pashankar D, Schreiber RA (2000) Neonatal cholestasis: a red alert for the jaundiced newborn. Can J Gastroenterol 14:67D–72D
Mieli-Vergani G, Howard ER, Portman B et al (1989) Late referral for biliary atresia – missed opportunities for effective surgery. Lancet 1:421–423
Ohi R, Nio M, Chiba T et al (1990) Long-term follow-up after surgery for patients with biliary atresia. J Pediatr Surg 25:442–445
Majd M, Reba RC, Altman RP (1981) Hepatobiliary scintigraphy with 99mTc-PIPIDA in the evaluation of neonatal jaundice. Pediatrics 67:140–145
Majd M, Reba RC, Altman RP (1981) Effect of phenobarbital on 99mTc-IDA scintigraphy in the evaluation of neonatal jaundice. Semin Nucl Med 11:194–204
Gerhold JP, Klingensmith WC 3rd, Kuni CC et al (1983) Diagnosis of biliary atresia with radionuclide hepatobiliary imaging. Radiology 146:499–504
Spivak W, Sarkar S, Winter D et al (1987) Diagnostic utility of hepatobiliary scintigraphy with 99mTc-DISIDA in neonatal cholestasis. J Pediatr 110:855–861
Cox KL, Stadalnik RC, McGahan JP et al (1987) Hepatobiliary scintigraphy with technetium-99m disofenin in the evaluation of neonatal cholestasis. J Pediatr Gastroenterol Nutr 6:885–891
Ben-Haim S, Seabold JE, Kao SC et al (1995) Utility of Tc-99m mebrofenin scintigraphy in the assessment of infantile jaundice. Clin Nucl Med 20:153–163
Gilmour SM, Hershkop M, Reifen R et al (1997) Outcome of hepatobiliary scanning in neonatal hepatitis syndrome. J Nucl Med 38:1279–1282
Park WH, Choi SO, Lee HJ et al (1997) A new diagnostic approach to biliary atresia with emphasis on the ultrasonographic triangular cord sign: comparison of ultrasonography, hepatobiliary scintigraphy, and liver needle biopsy in the evaluation of infantile cholestasis. J Pediatr Surg 32:1555–1559
Lin WY, Lin CC, Changlai SP et al (1997) Comparison technetium of Tc-99m disofenin cholescintigraphy with ultrasonography in the differentiation of biliary atresia from other forms of neonatal jaundice. Pediatr Surg Int 12:30–33
El-Desouki M, Mohamadiyah M, Al Rabeeah A et al (1998) Hepatobiliary scintigraphy in the distinction between biliary hypoplasia and biliary atresia. Saudi J Gastroenterol 4:8–12
Johnson K, Alton HM, Chapman S (1998) Evaluation of mebrofenin hepatoscintigraphy in neonatal-onset jaundice. Pediatr Radiol 28:937–941
Esmaili J, Izadyar S, Karegar I et al (2007) Biliary atresia in infants with prolonged cholestatic jaundice: diagnostic accuracy of hepatobiliary scintigraphy. Abdom Imaging 32:243–247
Yang J-G, Ma D-Q, Peng Y et al (2009) Comparison of different diagnostic methods for differentiating biliary atresia from idiopathic neonatal hepatitis. Clin Imaging 33:439–446
Charearnrad P, Chongsrisawat V, Tepmongkol S et al (2003) The effect of phenobarbital on the accuracy of technetium-99m diisopropyl iminodiacetic acid hepatobiliary scintigraphy in differentiating biliary atresia from neonatal hepatitis syndrome. J Med Assoc Thai 86:S189–194
Poddar U, Bhattacharya A, Thapa BR et al (2004) Ursodeoxycholic acid-augmented hepatobiliary scintigraphy in the evaluation of neonatal jaundice. J Nucl Med 45:1488–1492
Moyer V, Freese DK, Whitington PF et al (2004) Guideline for the evaluation of cholestatic jaundice in infants: recommendations of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition. J Pediatr Gastroenterol Nutr 39:115–128
Howman-Giles R, Uren R, Bernard E et al (1998) Hepatobiliary scintigraphy in infancy. J Nucl Med 39:311–319
Lee CH, Wang PW, Lee TT et al (2000) The significance of functioning gallbladder visualization on hepatobiliary scintigraphy in infants with persistent jaundice. J Nucl Med 41:1209–1213
Mowat AP, Psacharopoulos HT, Williams R (1976) Extrahepatic biliary atresia versus neonatal hepatitis. Review of 137 prospectively investigated infants. Arch Dis Child 51:763–770
Fischler B, Haglund B, Hjern A (2002) A population-based study on the incidence and possible pre- and perinatal etiologic risk factors of biliary atresia. J Pediatr 141:217–222
Caton AR, Druschel CM, McNutt LA (2004) The epidemiology of extrahepatic biliary atresia in New York State, 1983–98. Paediatr Perinat Epidemiol 18:97–105
Krantz ID, Piccoli DA, Spinner NB (1997) Alagille syndrome. J Med Genet 34:152–157
Conflicts of interest
None.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Kwatra, N., Shalaby-Rana, E., Narayanan, S. et al. Phenobarbital-enhanced hepatobiliary scintigraphy in the diagnosis of biliary atresia: two decades of experience at a tertiary center. Pediatr Radiol 43, 1365–1375 (2013). https://doi.org/10.1007/s00247-013-2704-3
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00247-013-2704-3