The incidence of pancreas divisum in children

Abstract

Pancreas divisum is a congenital anomaly caused by failure of fusion of the dorsal and ventral endodermal buds during the sixth and seventh week of gestation. Each duct drains via its own separate orifice, the major papilla of Vater for the ventral duct of Wirsung, and the minor accessory papilla for the dorsal duct of Santorini. Pancreas divisum is the most common congenital anomaly of the pancreas. It has been found in 5% to 14% of autopsy series and 0.3% to 8% of ERCP studies.1Cotton PB. Congenital anomaly of pancreas divisum as a cause of obstructive pain and pancreatitis.Gut. 1980; 21: 105-114Crossref PubMed Scopus (388) Google Scholar, 2Delhaye M Engelholm L Cremer M. Pancreas divisum: congenital anatomic variant or anomaly? Contribution of endoscopic retrograde dorsal pancreatography.Gastroenterology. 1985; 89: 951-958Abstract Full Text PDF PubMed Scopus (241) Google Scholar The clinical significance of pancreas divisum is controversial, and not all patients with this anomaly have clinical symptoms. Several authors1Cotton PB. Congenital anomaly of pancreas divisum as a cause of obstructive pain and pancreatitis.Gut. 1980; 21: 105-114Crossref PubMed Scopus (388) Google Scholar, 3Richter JM Shapiro RH Mulley AG Warshaw AL. Association of pancreas divisum and pancreatitis, and its treatment by sphincterotomy of the accessory ampulla.Gastroenterology. 1981; 81: 1104-1110Abstract Full Text PDF PubMed Scopus (143) Google Scholar have suggested an association between pancreas divisum and pancreatitis, on the basis that the minor papilla could be too small to allow adequate drainage. However, others2Delhaye M Engelholm L Cremer M. Pancreas divisum: congenital anatomic variant or anomaly? Contribution of endoscopic retrograde dorsal pancreatography.Gastroenterology. 1985; 89: 951-958Abstract Full Text PDF PubMed Scopus (241) Google Scholar have considered it to be a coincidental finding by reviewing large series of ERCP. These series dealt specifically with adult patients. Many authors have argued that if pancreas divisum causes pancreatitis, one might expect the disease to occur much earlier in life. Some reports3Richter JM Shapiro RH Mulley AG Warshaw AL. Association of pancreas divisum and pancreatitis, and its treatment by sphincterotomy of the accessory ampulla.Gastroenterology. 1981; 81: 1104-1110Abstract Full Text PDF PubMed Scopus (143) Google Scholar, 4Chevillote G Sahel J Pietri H Sarles H. Recurrent acute pancreatitis associated with pancreas divisum. A clinical study of 12 cases.Gastroenterol Clin Biol. 1984; 8: 352-358PubMed Google Scholar suggest that pancreatitis associated with pancreas divisum occurs earlier in life than other forms. This has been denied by others.5Mitchell CJ Lintott DJ Ruddell WSJ et al.Clinical relevance of an unfused pancreatic duct system.Gut. 1979; 20: 1066-1071Crossref PubMed Scopus (76) Google Scholar In children with recurrent pancreatitis, pancreas divisum has been found in 12% of patients (Table 1). Table 1Frequency of pancreas divisum in children with recurrent pancreatitisPatients with pancreas divisumAuthor (year)ReferenceNo. of patientsNo.%Forbes (1984)625416Lemmel (1994)72927Brown (1994)89222Guelrud (1994)950612Total1131412 Open table in a new tab In our experience with 272 consecutive cases of successful ERCP performed in children at the Gastroenterology Department in the Hospital General del Oeste in Caracas, Venezuela, nine (3.3%) children with pancreas divisum were encountered. Two patient groups were identified on the basis of the age at which ERCP was performed. Group 1 included 147 neonates or young infants in which ERCP was performed to evaluate neonatal cholestasis. Two (1.4%) patients had pancreas divisum, one a 30-day-old neonate with neonatal hepatitis and the other a 26-day-old neonate with biliary atresia (Fig. 1). Fig. 1A, Biliary atresia in a 26-day-old neonate. A distal, narrowed, and irregular common bile duct is seen. The gallbladder is normal. No opacification of the common hepatic duct or of the intrahepatic ducts is seen. B, A normal dorsal pancreas is visualized.View Large Image Figure ViewerDownload Hi-res image Download (PPT)To our knowledge, this is the youngest infant in the current literature to be reported with pancreas divisum. Group 2 included 125 children and adolescents with an age range of 1 to 19 years in which ERCP was done to evaluate pancreatic and biliary disorders. Seven (5.6%) patients had pancreas divisum. In this group, there was a high incidence of the anomaly 12% (6 of 50) in patients with recurrent pancreatitis, when compared with the incidence of 1.3% (1 of 75) in patients with biliary disease. It appears that in children and adolescents, as well as in adults, pancreas divisum is associated with a high incidence of recurrent pancreatitis.