H.H.F. Derkx
ERCP in children with EHBA
THE VALUE OF ENDOSCOPIC RETROGRADE CHOLANGIO- PANCREATOGRAPHY (ERCP) IN THE DIAGNOSIS OF EXTRAHEPATIC BILIARY ATRESIA
Derkx HHF, Bosman DK, Ter Keurs E, Huibregtse K2
Academic Medical Centre, Emma Children's Hospital, Dept. Of Pediatric Gastroenterology and Nutrition, 2Dept. Of Gastroenterology, Academic Medical Centre, Amsterdam, The Netherlands
Introduction and Aim: Since the results of hepatoportoenterostomy (KASAI) in infants with extra hepatic biliary atresia (BA) are more successful if the procedure is performed before the age of 8 weeks, a rapid diagnostic work up for infants suspected for BA is needed. Unfortunately with exception of the preoperative cholangiography all diagnostic tools (ultrasound, hida/iodida-scan, liver biopsy) are mostly not conclusive and a diagnostic laparotomy is indicated. In this context the value of ERCP was evaluated.
Methods: In this retrospective study 41 children were included. Since in all these infants both ultrasound and hepatobiliary-scan could not exclude BA additional liver biopsy and ERCP were performed. Only 16 of these children appeared to have BA, leaving the unique opportunity to evaluate the value of both ERCP and liver biopsy in terms of sensitivity and specificity.
Results: Table I shows the results of liver biopsy and ERCP in relation to the final diagnosis. Unfortunately not all liver biopsies could be traced.
Table 1:
LIVERBIOPSY
Suggesting BA
No certain diagnosis
No BA
BA-patients
Patients without BA
ERCP
ERCP suggesting BA
ERCP suggesting no BA
BA-patients
Patients without BA
Liver biopsy suggested BA correctly only in 7 out 17 patients. The ERCP correctly diagnosed BA in 18 of the 19 patients. In 18 out of 22 patients the ERCP excluded BA correctly. In the group of patients in which ERCP suggested BA not correctly (n=4), 3 patients turned out to have Alagille syndrome. ERCP reached a sensitivity and specificity of 95 and 82% whereas the results for liver biopsy were as follows: 41% (7/17), and 41 % (7/17).
Conclusion: In skilled hands ERCP has proven to be associated with a high specificity and sensitivity and can be recommended as a useful procedure in the work-up of children suspected of BA. In this study ERCP resulted in a prevention of laparotomy in 47% of the patients.
