Advertisement

Pediatric Radiology

, Volume 43, Issue 10, pp 1365–1375 | Cite as

Phenobarbital-enhanced hepatobiliary scintigraphy in the diagnosis of biliary atresia: two decades of experience at a tertiary center

  • Neha Kwatra
  • Eglal Shalaby-Rana
  • Srikala Narayanan
  • Parvathi Mohan
  • Sunil Ghelani
  • Massoud MajdEmail author
Original Article

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.

Keywords

Hepatobiliary scintigraphy Biliary atresia Cholestasis Phenobarbital Infants 

Notes

Conflicts of interest

None.

References

  1. 1.
    Sokol RJ, Mack C, Narkewicz MR et al (2003) Pathogenesis and outcome of biliary atresia: current concepts. J Pediatr Gastroenterol Nutr 37:4–21PubMedCrossRefGoogle Scholar
  2. 2.
    Landing BH (1974) Considerations of the pathogenesis of neonatal hepatitis, biliary atresia and choledochal cyst—the concept of infantile obstructive cholangiopathy. Prog Pediatr Surg 6:113–139PubMedGoogle Scholar
  3. 3.
    Kirks DR, Coleman RE, Filston HC et al (1984) An imaging approach to persistent neonatal jaundice. AJR Am J Roentgenol 142:461–465PubMedCrossRefGoogle Scholar
  4. 4.
    Makin E, Davenport M (2006) Biliary atresia. Curr Paediatr 16:59–63CrossRefGoogle Scholar
  5. 5.
    Pashankar D, Schreiber RA (2000) Neonatal cholestasis: a red alert for the jaundiced newborn. Can J Gastroenterol 14:67D–72DPubMedGoogle Scholar
  6. 6.
    Mieli-Vergani G, Howard ER, Portman B et al (1989) Late referral for biliary atresia – missed opportunities for effective surgery. Lancet 1:421–423PubMedCrossRefGoogle Scholar
  7. 7.
    Ohi R, Nio M, Chiba T et al (1990) Long-term follow-up after surgery for patients with biliary atresia. J Pediatr Surg 25:442–445PubMedCrossRefGoogle Scholar
  8. 8.
    Majd M, Reba RC, Altman RP (1981) Hepatobiliary scintigraphy with 99mTc-PIPIDA in the evaluation of neonatal jaundice. Pediatrics 67:140–145PubMedGoogle Scholar
  9. 9.
    Majd M, Reba RC, Altman RP (1981) Effect of phenobarbital on 99mTc-IDA scintigraphy in the evaluation of neonatal jaundice. Semin Nucl Med 11:194–204PubMedGoogle Scholar
  10. 10.
    Gerhold JP, Klingensmith WC 3rd, Kuni CC et al (1983) Diagnosis of biliary atresia with radionuclide hepatobiliary imaging. Radiology 146:499–504PubMedGoogle Scholar
  11. 11.
    Spivak W, Sarkar S, Winter D et al (1987) Diagnostic utility of hepatobiliary scintigraphy with 99mTc-DISIDA in neonatal cholestasis. J Pediatr 110:855–861PubMedCrossRefGoogle Scholar
  12. 12.
    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 6:885–891PubMedCrossRefGoogle Scholar
  13. 13.
    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 20:153–163PubMedCrossRefGoogle Scholar
  14. 14.
    Gilmour SM, Hershkop M, Reifen R et al (1997) Outcome of hepatobiliary scanning in neonatal hepatitis syndrome. J Nucl Med 38:1279–1282PubMedGoogle Scholar
  15. 15.
    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 32:1555–1559PubMedCrossRefGoogle Scholar
  16. 16.
    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 12:30–33PubMedCrossRefGoogle Scholar
  17. 17.
    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 4:8–12PubMedGoogle Scholar
  18. 18.
    Johnson K, Alton HM, Chapman S (1998) Evaluation of mebrofenin hepatoscintigraphy in neonatal-onset jaundice. Pediatr Radiol 28:937–941PubMedCrossRefGoogle Scholar
  19. 19.
    Esmaili J, Izadyar S, Karegar I et al (2007) Biliary atresia in infants with prolonged cholestatic jaundice: diagnostic accuracy of hepatobiliary scintigraphy. Abdom Imaging 32:243–247PubMedCrossRefGoogle Scholar
  20. 20.
    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 33:439–446PubMedCrossRefGoogle Scholar
  21. 21.
    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:S189–194PubMedGoogle Scholar
  22. 22.
    Poddar U, Bhattacharya A, Thapa BR et al (2004) Ursodeoxycholic acid-augmented hepatobiliary scintigraphy in the evaluation of neonatal jaundice. J Nucl Med 45:1488–1492PubMedGoogle Scholar
  23. 23.
    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 39:115–128PubMedCrossRefGoogle Scholar
  24. 24.
    Howman-Giles R, Uren R, Bernard E et al (1998) Hepatobiliary scintigraphy in infancy. J Nucl Med 39:311–319PubMedGoogle Scholar
  25. 25.
    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 41:1209–1213PubMedGoogle Scholar
  26. 26.
    Mowat AP, Psacharopoulos HT, Williams R (1976) Extrahepatic biliary atresia versus neonatal hepatitis. Review of 137 prospectively investigated infants. Arch Dis Child 51:763–770PubMedCrossRefGoogle Scholar
  27. 27.
    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 141:217–222PubMedCrossRefGoogle Scholar
  28. 28.
    Caton AR, Druschel CM, McNutt LA (2004) The epidemiology of extrahepatic biliary atresia in New York State, 1983–98. Paediatr Perinat Epidemiol 18:97–105PubMedCrossRefGoogle Scholar
  29. 29.
    Krantz ID, Piccoli DA, Spinner NB (1997) Alagille syndrome. J Med Genet 34:152–157PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2013

Authors and Affiliations

  • Neha Kwatra
    • 1
  • Eglal Shalaby-Rana
    • 1
  • Srikala Narayanan
    • 1
  • Parvathi Mohan
    • 2
  • Sunil Ghelani
    • 3
  • Massoud Majd
    • 1
    Email author
  1. 1.Division of Diagnostic Imaging and Radiology, Children’s National Medical CenterThe George Washington University Medical CenterWashingtonUSA
  2. 2.Department of Gastroenterology, Hepatology and Nutrition Children’s National Medical CenterWashingtonUSA
  3. 3.Department of PediatricsChildren’s National Medical CenterWashingtonUSA

Personalised recommendations