Diagnostic efficacy of ERCP in cholestatic infants and neonates – a retrospective study on a large series

Background and study aims: Cholestatic jaundice in infants is a serious condition, requiring timely and accurate diagnostic evaluation. Our aim was to determine the safety and diagnostic efficacy of endoscopic retrograde cholangiopancreatography (ERCP) in the diagnosis of cholestatic liver disease in neonates and infants.

Patients and methods: ERCP procedures in cholestatic infants performed in our endoscopy unit between December 1998 and March 2008 were reviewed retrospectively (n = 104 children, 48 boys, 56 girls; mean age 7 weeks, range 3 – 25 weeks; mean weight 4.05 kg, range 1.5 – 4.8 kg). Endoscopic findings were compared with final diagnoses. Statistical analysis was performed and sensitivity, specificity, positive (PPV) and negative (NPV) predictive values of ERCP were calculated both separately for each diagnosis and on aggregate.

Results: Cannulation of the papilla was successful in 95 of 104 patients (success rate 91.3 %). Biliary atresia of any type was found in 51 children (53.7 %), with a sensitivity of 86 %, a specificity of 94 %, a PPV of 96 %, and a NPV of 100 %. Choledochal cysts were found in seven children (7.4 %), with a sensitivity of 100 %, a specificity of 90 %, PPV of 86 %, and NPV of 100 %. Biliary stones were found in seven patients (7.4 %). Other structural pathology was found in six patients, and no abnormality was seen in 24 patients. No severe complications occurred during or after ERCP.

Conclusions: ERCP in cholestatic infants, when performed in an expert center, is a safe and reliable procedure that can detect biliary tract abnormalities (e. g. biliary atresia, bile duct stones or choledochal cysts) with high sensitivity and specificity. Laparotomies can be prevented in infants by demonstrating normal patency of the biliary tract by ERCP or by magnetic resonance cholangiography if improvements in this technique are made.

References

• 1 Shan H A, Spivak W. Neonatal cholestasis of infancy.  Pediatric Clinic of North America. 1994;  43 943-965
• 2 Bassett M D, Murray K F. Biliary atresia: recent progress.  J Clin Gastroenterol. 2008;  42 720-729
• 3 Kelly D A, Davenport M. Current management of biliary atresia.  Arch Dis Child. 2007;  92 1132-1135
• 4 Davenport M, Caponcelli E, Livesey E. et al . Surgical outcome in biliary atresia: etiology affects the influence of age at surgery.  Ann Surg. 2008;  247 694-698
• 5 Wadhwani S I, Turmelle Y P, Nagy R. et al . Prolonged neonatal jaundice and the diagnosis of biliary atresia: a single-center analysis of trends in age at diagnosis and outcomes.  Pediatrics. 2008;  121 e1438-1440
• 6 Mieli-Vergani G, Howard E R, Portman B. et al . Late referral for biliary atresia: missed opportunities for effective surgery.  Lancet. 1989;  1 421-423
• 7 Kieling C O, dos Santos J L, Vieira S M. et al . Biliary atresia: we still operate too late.  J Pediatr (Rio J). 2008;  84 436-441
• 8 Guelrud M, Jaen D, Mendoza S. et al . ERCP in the diagnosis of extrahepatic biliary atresia.  Gastrointest Endosc. 1991;  37 522-526
• 9 Ohnuma N, Takahashi H, Tanabe M. et al . Endoscopic retrograde cholangiopancreatography (ERCP) in biliary tract disease of infants less than one year old.  Tohoku J Exp Med. 1997;  181 67-74
• 10 Teng R, Yokohata K, Utsunomiya N. et al . Endoscopic retrograde cholangiopancreatography in infants and children.  J Gastroenterol. 2000;  35 39-42
• 11 Durakbasa Ç U, Balik E, Yamaner S. et al . Diagnostic and therapeutic endoscopic retrograde cholangiopancreatography (ERCP) in children and adolescents: experience in a single institution.  Eur J Pediatr Surg. 2008;  18 241-244
• 12 Keil R, Snajdauf J, Stuj J. et al . Endoscopic retrograde cholangiopancreatography in infants and children.  Indian J Gastroenterol. 2000;  19 175-177
• 13 Nourallah H, Issa H, Al-Salem A H. The role of ERCP in the evaluation, diagnosis, and therapy of biliary and pancreatic diseases in children.  Ann Saudi Med. 1999;  19 163-166
• 14 Cheng C L, Fogel E L, Sherman S. et al . Diagnostic and therapeutic endoscopic retrograde cholangiopancreatography in children: a large series report.  J Pediatr Gastroenterol Nutr. 2005;  41 445-453
• 15 Iinuma Y, Narisawa R, Makoto I. et al . The role of endoscopic retrograde cholangiopancreatography in infants with cholestasis.  J Pediatr Surg. 2000;  35 545-549
• 16 Derkx H HF, Huibregtse K, Taminiau J AJM. The role of endoscopic retrograde cholangiopancreatography in cholestatic infants.  Endoscopy. 1994;  26 724-728
• 17 Woo M, Patel S, Walter M H, Godil A. Role of diagnostic and therapeutic ERCP in infants, children and adolescents.  Am J Gastroenterol. 2000;  95 2488
• 18 Takamizawa S, Zaima A, Muraji T. et al . Efficacy of ERCP in infancy and childhood.  Klin Padiatr. 2007;  219 271-276
• 19 Reinshagen K, Müldner A, Manegold B, Kähler G. Efficacy of ERCP in infancy and childhood.  Klin Padiatr. 2007;  219 271-276
• 20 Choi S O, Park W H, Lee H J. Ultrasonographic “triangular cord”: the most definitive finding for noninvasive diagnosis of extrahepatic biliary atresia.  Eur J Pediatr Surg. 1998;  8 12-16
• 21 Kotb M A, Kotb A, Sheba M F. et al . Evaluation of the triangular cord sign in the diagnosis of biliary atresia.  Pediatrics. 2001;  108 416-420
• 22 Roquete M L, Ferreira A R, Fagundes E D. et al . Accuracy of echogenic periportal enlargement image in ultrasonographic exams and histopathology in differential diagnosis of biliary atresia.  J Pediatr (Rio J). 2008;  84 331-336
• 23 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. 2004;  39 115-128. Erratum in: J Pediatr Gastroenterol Nutr 2004; 39: 306
• 24 Norton K I, Glass R B, Kogan D. et al . MR cholangiography in the evaluation of neonatal cholestasis: initial results.  Radiology. 2002;  222 687-691
• 25 Han S J, Kim M J, Han A. et al . Magnetic resonance cholangiography for the diagnosis of biliary atresia.  J Pediatr Surg. 2002;  37 599-604
• 26 Takaya J, Nakano S, Imai Y. et al . Usefulness of magnetic resonance cholangiopancreatography in biliary structures in infants: a four-case report.  Eur J Pediatr. 2007;  166 211-214
• 27 Chavhan G B, Babyn P S, Manson D, Vidarsson L. Pediatric MR cholangiopancreatography: principles, technique, and clinical applications.  Radiographics. 2008;  28 1951-1962
• 28 Ryeom H K, Choe B H, Kim J Y. et al . Biliary atresia: feasibility of mangafodipir trisodium-enhanced MR cholangiography for evaluation.  Radiology. 2005;  235 250-258
• 29 Schaefer J F, Kirschner H J, Lichy M. et al . Highly resolved free-breathing magnetic resonance cholangiopancreatography in the diagnostic workup of pancreaticobiliary diseases in infants and young children – initial experiences.  J Pediatr Surg. 2006;  41 1645-1651
• 30 Brough A J, Bernstein J. Conjugated hyperbilirubinemia in early infancy: a reassessment of liver biopsy.  Hum Pathol. 1974;  5 507-516
• 31 Landing B H, Wells T R, Ramicone E. Time course of the intrahepatic lesion of extrahepatic biliary atresia: a morphometric study.  Pediatr Pathol. 1985;  4 309-319
• 32 Fox V F, Cohen M B, Whitington P F. et al . Outpatient liver biopsy in children.  J Pediatr Gastroenterol Nutr. 1996;  23 213-216