PANCREATICOBILIARY MALJUNCTION ASSOCIATED WITH NONDILATATION OR MINIMAL DILATATION OF THE COMMON BILE-DUCT IN CHILDREN - DIAGNOSIS AND TREATMENT

It is known that the etiology of congenital biliary dilatation (CBD) i s closely associated with pancreaticobiliary maljunction (PBMJ). Treat ment of CBD today is primary excision of the cyst followed by hepatico enterostomy. However, PBMJ without dilatation of the biliary tract has recently been reported and its treatment is still controversial. In a dults, simple cholecystectomy without biliary reconstruction is often performed, because it usually presents as an anomaly of the gallbladde r. Over 30 years, we have encountered 8 patients with PBMJ without dil atation of the common bile duct in 180 pediatric cases of CBD. The pre senting symptoms of these patients were those of pancreatitis i.e. abd ominal pain associated with elevation of serum amylase levels. Five ca ses presented with jaundice or a history of pale colored stools. Endos copic retrograde cholangiopancreatography was performed to confirm the diagnosis. Five (62.5%) of these 8 patients were found to have dilata tion of the common channel, which was seen in 50 (29%) of the 172 othe r cases with biliary tract dilatation. Three cases (37.5%) had proven protein plugs or debris at the level of the common channel, and this w as observed in 28 (16%) of the 172 other cases. It is strongly suggest ed that the manifestation of clinical symptoms in these patients resul ts from stasis or obstruction at the level of the common channel. In c hildren who present with recurrent pancreatitis, PBMJ must be suspecte d even if the common bile duct appears to be normal. It is difficult t o resolve these anomalies without operative repair. The authors consid er that radical treatment of PBMJ is required in these children to pre vent serious long-term complications.