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

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Agenesis of the dorsal pancreas in a patient with recurrent acute pancreatitis: case report and review
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  Agenesis of the dorsal pancreas ina patient with recurrent acutepancreatitis: case report and review Mustafa Gu¨ c¸lu¨ , MD, Ender Serin, MD, S    ˛ erife Ulucan, MD,Kemal Kul, MD, Birol O¨  zer, MD, Yu¨ kselGu¨mu¨rdu¨lu¨, MD, Cengiz Pata, MD, Arif Cos    ˛ ar, MD,Gu¨ rden Gu¨ r, MD, Sedat Boyaciog ˘ lu, MD Complete agenesis of the dorsal pancreas isextremely rare. The embryologic development of thepancreas is complex, and several morphologic anom-aliesrelatedtothisprocesshavebeendocumented. 1-3 Complete agenesis of the pancreas is incompatiblewith life. 4 The most common congenital anomaly of  thepancreasispancreasdivisum. 5,6 Thefrequencyof the latter anomaly in patients with primary biliarytract disease is 3.6%, whereas the frequency inpatients with unexplained recurrent pancreatitis is25.6%. 5  Agenesis of the dorsal pancreas results incharacteristicCTfindings. 7 Earlyreportsofcompleteagenesis of the dorsal pancreas were based onfindings at autopsy. 8-11 The present report describesthe ERCP, MRCP, and CT findings in a patient withagenesis of the dorsal pancreas who presented withacute pancreatitis. CASE REPORT  A 33-year-old Turkish woman presented with severeepigastric abdominal pain, nausea, and vomiting. Anepisode of acute pancreatitis had occurred 1 year earlier.There also was a history of frequent urinary-tract in-fections over the previous 15 years; a diagnosis of vesicoureteral reflux was made 3 years earlier. Peritonealdialysis on an ambulatory basis was begun 2 years beforepresentation because of chronic renal failure secondary tochronic pyelonephritis. Noninsulin-dependent diabetesmellitus was diagnosed 1 year before presentation. Upperabdominal tenderness was elicited on examination atadmission. Laboratory test results at admission were thefollowing: serum amylase, 1512 IU/L (normal: 30-110 IU/ L); lipase, 1563 IU/L (23-300 IU/L); calcium, 8.9 mg/dL(8.40-10.0 mg/dL); triglycerides, 430 mg/dL (55-175mg/dL); aspartate aminotransferase, 26 IU/L (12-50 IU/L);alanine aminotransferase, 12 IU/L (10-70); gammaglutamyl transferase, 154 IU/L (7-40 IU/L); alkalinephosphatase, 188 IU/L (25-100 IU/L); total bilirubin,0.4 mg/dL (0.4-1.35 mg/dL); direct bilirubin, 0.2 mg/dL(0.10-0.50mg/dL);C-reactiveprotein,96mg/dL(0-6mg/dL);and blood glucose, 333 mg/dL (70-110 mg/dL). Analysisof peritoneal dialysis fluid revealed 10 white blood cellsper high-power field.On transabdominal US, the head of the pancreasappeared as a hypoechoic glandular structure in thenormal location, but the body and the tail were not visible. Afewmillimeter-sizedpolypswerenotedinthegallbladderwall;freeintraperitonealfluidwasattributedtoperitonealdialysis. CT with and without contrast revealed onlya prominent head of the pancreas (Fig. 1). At ERCP, the orifice of the major duodenal papilla was in the normalposition, but careful examination revealed no minorpapilla.Injectionofcontrastmaterialintothemajorpapillademonstrated a short, branching duct that was notconsistent with a normal main pancreatic duct (Fig. 2). MRCP also was obtained in an attempt to visualize thepancreas and bile ducts in greater detail. The head of thepancreas appeared normal on cross-sections, but no otherpancreatic tissue was evident. The scan also revealeda dilated common bile duct and a short main pancreaticductofnormaldiameterintheheadoftheglandbutnoductin the body and the tail (Fig. 3). Triphasic dynamic CT revealed only the head portion of the pancreas. Based onthecollectiveimagingstudies,adiagnosisofagenesisofthedorsal pancreas was made.With conservative treatment, the clinical status of thepatient and the laboratory parameters of pancreaticinflammation improved rapidly and her overall statushad returned to baseline after 3 days of hospitalization.Blood pressure, fundic examination, and neurologic func-tion remained normal throughout follow-up. DISCUSSION Patientswithagenesisofthedorsalpancreastendto present with non-specific abdominal pain, makingit difficult to distinguish this anomaly from pancre-atitis and other pancreatic abnormalities. Only 14cases (8men, 6 women; mean age 31.6years,range1month to 56 years) of complete agenesis of the dorsalpancreas were reported between 1913 and 1999(Table1). 12 Ninepatientshaddiabetesmellitus;onlyone had chronic pancreatitis.Complete agenesis of the dorsal pancreas isthought to be because of abnormalities of embryo-genesis. 7,13 The pancreas develops from dorsal andventralbuds.Duringembryogenesis,theventralbudrotates posteriorly and to the left and fuses with thedorsal bud during the 7th gestational week. 1 Theventral bud forms the major portion of  the head andthe uncinate process of the pancreas, 13,14 whereasthe neck, the body, and the tail srcinate from thedorsal bud. 1 The dorsal bud also may contribute tothe formation of the uncinate process. 1 The most common anomaly of pancreatic develop-ment is pancreas divisum, which has a frequency as Current affiliations: Department of Gastroenterology, Departmentof Radiology, Bas    ˛  kent University, Adana Teaching and Medical Research Center, Adana, Turkey. Reprint requests: Mustafa Gu¨c¸lu¨, MD, Bas    ˛  kent U ¨ niversitesi Adana Uygulama, ve Aras    ˛ tirma Hastanesi Dadalog˘ lu, Mah. 39.Sk. No: 6 Yu¨reg˘ ir, Adana, Turkey 01250.Copyright    2004 by the American Society for Gastrointestinal Endoscopy 0016-5107/$30.00 PII: S0016-5107(04)01733-X  472  GASTROINTESTINAL ENDOSCOPY VOLUME 60, NO. 3, 2004  high as 14% in autopsy series. 3 Other pancreaticanomalies include annular pancreas and agenesis of either the ventral or the dorsal bud. 1 There are fewpublished reports of absence of the ventral pancreas. Agenesis of  the dorsal pancreas may be partial orcomplete. 15 Partialagenesisofthedorsalpancreasisrelatively more common than agenesis of the ventralportion, 16 but complete agenesis of the dorsalpancreas is extremely rare. 12 The exact cause of agenesis of the ventral or the dorsal pancreas isunknown,buttwopossible explanationsareprimarydysgenesis of the dorsal bud and ischemic insult tothedevelopingpancreas. 17 Inpatientswithcompleteagenesis of the dorsal pancreas, the neck, the body,andthetailofthepancreas,theaccessorypancreaticduct (duct of Santorini), and the minor duodenalpapilla are absent. 15 In cases of partial agenesis orhypoplasiaofthedorsalpancreas,thesizeofthebodyof the pancreas varies, there is a remnant of the ductof Santorini, and the minor duodenal papilla ispresent. 15 Patientswithagenesisofthedorsalpancreasoftenpresentwithnon-specificabdominalpain,whichmayor may not be caused by pancreatitis. 16 Manypatients also have diabetes mellitus. 2  Abdominalpain may be more common when agenesis of thedorsal pancreas is partial, and diabetes may be morecommon when it is complete. 15  A bsence of the dorsalpancreas also is reported as part of the polyspleniasyndrome, 17,18 and this may be related to the prox-imity of the developing pancreas and spleen in thedorsal mesogastrium. 18 Figure 1. A-C,  Sequence of CT images showing head of pancreas  (arrows)   with absence of body and tail in all images. VOLUME 60, NO. 3, 2004 GASTROINTESTINAL ENDOSCOPY   473  Agenesis of the dorsal pancreas and recurrent acute pancreatitis M Gu¨c¸lu¨, E Serin, S    ˛ Ulucan, et al.   Agenesis of the dorsal pancreas usually is discov-ered when imaging studies are obtained to evaluateabdominal pain. 19 When the diagnosis of agenesis of the dorsal pancreas is suspected, it is critical to ruleout pancreatic carcinoma with proximal atrophy of the gland.Transabdominal US is of limited value for thediagnosis of agenesis of the dorsal pancreas, becausethe body and the tail may not be visible because of interference from overlying bowel gas and/or othertechnicalproblems. 19  AlthoughCTisvaluableforthediagnosisofpancreaticdisease,itfrequentlydoesnotdelineate the detailed anatomy of the pancreaticduct. In contrast, ERCP provides detailed informa-tionaboutthepancreaticductbutisinvasive,andtheidentification and cannulation of the minor papillacanbedifficult.MRCPclearlydemonstratesthemainandtheaccessorypancreaticductsandthusisusefulfor the diagnosis of abnormalities of the pancreas. 16 The sonographic appearance of agenesis of thedorsal pancreas has been described in a number of studies. 15-19  At the site where it normally joins the Figure 2.  Retrograde pancreatogram made by injection ofcontrast medium at major duodenal papilla, showing a shortmain duct with normal-appearing branches but no accessoryduct or main duct in body or tail of pancreas. Table 1. Characteristics of the 14 reported casesof agenesis of the dorsal pancreas (20) Patientno. Gender Age (y)DiagnosticmethodDiabetesmellitus Pancreatitis1 M 14 At autopsy + Unknown2 F 56 At autopsy    Unknown3 M 21 At autopsy + Unknown4 M 1 mo At autopsy    Unknown5 M 26 At autopsy + Unknown6 F 1 mo At autopsy    Unknown7 M 40 CT, ERCP +   8 M 54 ERCP,angiography+   9 F 32 CT, ERCP +   10 M 54 US,CT, ERCP,at surgery+ +11 F 39 US,CT, ERCP +   12 F 46 CT, ERCP +   13 M 15 CT, ERCP    14 F 47 US, CT, ERCP,MRCP atsurgery   Figure 3. A  and  B,  Magnetic resonance images showing normal appearing head of pancreas but no other pancreatic tissue. 474  GASTROINTESTINAL ENDOSCOPY VOLUME 60, NO. 3, 2004 M Gu¨c¸lu¨, E Serin, S    ˛ Ulucan, et al. Agenesis of the dorsal pancreas and recurrent acute pancreatitis  neck of the pancreas, the hypoechoic head is sharplydemarcated fromthemore echogenic retroperitonealfat. 19 On CT, the finding of a normal head but noother pancreatic tissue strongly suggested agenesisof the dorsal pancreas. 2,16,19 However, as noted bySchnedletal., 15 thepossibilityofneoplasmcannotbeexcluded unless pancreatic duct morphology isclearly delineated; therefore, ERCP is necessary toconfirm the diagnosis. In the present case, noevidence of pancreatic neoplasm was found by CT,ERCP, and MRCP.ERCP is considered the reference standard forevaluation of the pancreatic and the biliary ducts;however, this procedure is operator-dependent, andfailure to opacify the pancreatic and biliary treeoccurs with 3% to 10% of procedures, in which case,MRCP can be extremely helpful. 20 Improved tech-niques, including body phased-array coils andbreath-hold imaging, allow normal pancreatic ductsto be visualized in 90% of cases. 3 However, MRCP does not permit therapeutic intervention. 3,20 Ourpatienthadnoevidenceofexocrinepancreaticinsufficiency, but acute pancreatitis had occurredtwice within 1 year. In the present case, it is difficultto determine whether the absence of the dorsalpancreas contributed to the development of non-insulin-dependent diabetes mellitus. CT revealedonly the head of the pancreas, and ERCP demon-strated a short main pancreatic duct with absence of theaccessory duct andthemainduct proximaltotheheadofthegland.MRCPalsowasconsistentwiththediagnosisofcompleteagenesisofthedorsalpancreas. REFERENCES 1. Schulte SJ. Embryology, normal variation, and congenitalanomalies of the pancreas. In: Freeny PC, Stevenson GW,editors. Margulis and Burhenne’s alimentary tract radiology.5th ed. St. Louis: Mosby-Year Book; 1994. p. 1039-49.2. Wilding R, Schnedl WJ, Reisinger EC, Schreiber F, Lipp RW,Lederer A, et al. Agenesis of the dorsal pancreas in a womanwith diabetes mellitus and in both of her sons. Gastroenter-ology 1993;104:1182-6.3. Bret PM, Reinhold C, Taourel P. Pancreas divisum: evalua-tion with MR cholangiopancreatography. Radiology 1996;199:99-103.4. Lehman GA, Kopecky KK, Rogge JD. Partial pancreaticagenesis combined with pancreas divisum and duodenumreflexum. Gastrointest Endosc 1987;33:445-8.5. Cotton PB. Congenital anomaly of pancreas divisum as causeof obstructive pain and pancreatitis. Gut 1980;21:105-14.6. Cotton PB. Pancreas divisum: curiosity or culprit [editorial]?Gastroenterology 1985;89:1431-5.7. Shah KK, Deriddor PH, Schwab RE, Alexander TJ. Casereport. CT diagnosis of dorsal pancreas agenesis. J Comput Assist Tomogr 1987;11:170-1.8. Ghon A, Roman B. Ein fall von bilding des pancreas mitdiabetes mellitus [German]. Prager Medizinische Wochens-chrift 1913;38:524-6.9. Priesel A. Sitzing der Vereinigung der pathologischen Anatomen Wiens [German]. Wien Klin Wochenschr 1923;22:407.10. Duschl L. Ein beitrag zu den pankreas bildungen: fehlendespankreaskopfes und-schwanzes [German]. Mu¨nchen MedWochenschrift 1923;70:1388-9.11. Kriss B. Zur kenntnis der hypoplasie des pankreas. Virchow Arch Path 1926;263:591-8.12. Fukuoka K, Ajiki T, Yamamoto M, Fujwara H, Onoyama H,Fujita T, et al. Complete agenesis of the dorsal pancreas.J Hepatobiliary Pancreat Surg 1999;6:94-7.13. Adda G, Hannoun L, Loygue J. Development of the humanpancreas: variations and pathology. A tentative classification. Anat Clin 1984;5:275-83.14. Howat HT, Sarles H. The exocrine pancreas. London: WBSaunders; 1979. p. 402-82.15. Schnedl WJ, Reisinger EC, Schreiber F. Complete and partialagenesis of the dorsal pancreas in one family. GastrointestEndosc 1995;42:485-7.16. Gold RP. Agenesis and pseudo agenesis of the dorsalpancreas. Abdom Imaging 1993;18:141-4.17. Sener RN, Alper H. Polysplenia syndrome: a case associatedwith transhepatic portal vein, short pancreas and left inferiorvena cava with hemiazygos continuation. Abdom Imaging1994;19:64-6.18. Herman TE, Siegel MJ. Polysplenia syndrome with congen-ital short pancreas. AJR Am J Roentgenol 1991;156:799-800.19. Deignon RW, Nizzero A, Malone DE. Case report: agenesis of the dorsal pancreas—a cause of diagnostic error on abdom-inal sonography. Clin Radiol 1996;51:145-7.20. Soto JA, Yucel EK, Barish MA. MR cholangiopancreatogra-phy after unsuccessful or incomplete ERCP. Radiology 1996;199:91-8. VOLUME 60, NO. 3, 2004 GASTROINTESTINAL ENDOSCOPY   475  Agenesis of the dorsal pancreas and recurrent acute pancreatitis M Gu¨c¸lu¨, E Serin, S    ˛ Ulucan, et al.
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