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Agenesis of the dorsal pancreas in a patient with recurrent acute pancreatitis: case report and review

2004; Elsevier BV; Volume: 60; Issue: 3 Linguagem: Inglês

10.1016/s0016-5107(04)01733-x

1097-6779

Mustafa Güçlü, Ender Serin, Şerife Ulucan, Kemal Kul, Birol Özer, Yüksel Gümürdülü, Cengiz Pata, Arif Coşar, Gürden Gür, Sedat Boyacıoğlu,

Pancreatitis Pathology and Treatment

Complete agenesis of the dorsal pancreas is extremely rare. The embryologic development of the pancreas is complex, and several morphologic anomalies related to this process have been documented. 1. Schulte S.J. Embryology, normal variation, and congenital anomalies of the pancreas. in: Freeny P.C. Stevenson G.W. Margulis and Burhenne's alimentary tract radiology. 5th ed. Mosby-Year Book, St. Louis1994: 1039-1049 Google Scholar , 2. Wilding R. Schnedl W.J. Reisinger E.C. Schreiber F. Lipp R.W. Lederer A. et al. Agenesis of the dorsal pancreas in a woman with diabetes mellitus and in both of her sons. Gastroenterology. 1993; 104: 1182-1186 PubMed Google Scholar , 3. Bret P.M. Reinhold C. Taourel P. Pancreas divisum: evaluation with MR cholangiopancreatography. Radiology. 1996; 199: 99-103 Crossref PubMed Scopus (268) Google Scholar Complete agenesis of the pancreas is incompatible with life. 4. Lehman G.A. Kopecky K.K. Rogge J.D. Partial pancreatic agenesis combined with pancreas divisum and duodenum reflexum. Gastrointest Endosc. 1987; 33: 445-448 Abstract Full Text PDF PubMed Scopus (14) Google Scholar The most common congenital anomaly of the pancreas is pancreas divisum. 5. Cotton P.B. Congenital anomaly of pancreas divisum as cause of obstructive pain and pancreatitis. Gut. 1980; 21: 105-114 Crossref PubMed Scopus (392) Google Scholar , 6. Cotton P.B. Pancreas divisum: curiosity or culprit [editorial]?. Gastroenterology. 1985; 89: 1431-1435 PubMed Google Scholar The frequency of the latter anomaly in patients with primary biliary tract disease is 3.6%, whereas the frequency in patients with unexplained recurrent pancreatitis is 25.6%. 5. Cotton P.B. Congenital anomaly of pancreas divisum as cause of obstructive pain and pancreatitis. Gut. 1980; 21: 105-114 Crossref PubMed Scopus (392) Google Scholar Agenesis of the dorsal pancreas results in characteristic CT findings. 7. Shah K.K. Deriddor P.H. Schwab R.E. Alexander T.J. Case report. CT diagnosis of dorsal pancreas agenesis. J Comput Assist Tomogr. 1987; 11: 170-171 Crossref PubMed Scopus (30) Google Scholar Early reports of complete agenesis of the dorsal pancreas were based on findings at autopsy. 8. Ghon A. Roman B. Ein fall von bilding des pancreas mit diabetes mellitus. Prager Medizinische Wochenschrift. 1913; 38 ([German]): 524-526 Google Scholar , 9. Priesel A. Sitzing der Vereinigung der pathologischen Anatomen Wiens. Wien Klin Wochenschr. 1923; 22 ([German]): 407 Google Scholar , 10. Duschl L. Ein beitrag zu den pankreas bildungen: fehlendes pankreaskopfes und-schwanzes [German]. München Med Wochenschrift. 1923; 70: 1388-1389 Google Scholar , 11. Kriss B. Zur kenntnis der hypoplasie des pankreas. Virchow Arch Path. 1926; 263: 591-598 Google Scholar The present report describes the ERCP, MRCP, and CT findings in a patient with agenesis of the dorsal pancreas who presented with acute pancreatitis.