Gut, 1987, 28, 358-362

Case report

Periampullary cyst: a surgically remediable cause ofpancreatitisI KALVARIA, P C BORNMAN, A H GIRDWOOD, AND I N MARKS

From the Gastrointestinal Clinic, Groote Schuur Hospital and the Departments ofMedicine and SurgeryUniversity ofCape Town, Cape Town, South Africa

SUMMARY We report two patients with periampullary cysts associated with recurrent attacks ofacute pancreatitis. In both patients the diagnosis was made preoperatively by upper gastro-intestinal endoscopy and ERCP, which was also useful in determining the relationship of the cyststo the biliary and pancreatic ductal systems. Simple marsupialisation of the cysts resulted in longterm relief of symptoms. Congenital cystic anomalies in the second part of the of the duodenumshould be diligently sought in patients with pancreatitis of unexplained cause, as surgical therapy issafe and effective.

With the advent of endoscopic retrograde cholangio-pancreatography (ERCP), surgically remediableconditions such as biliary calculi, ampullary stenosis,and local pancreatic duct obstruction can be identi-fied in up to two-thirds of patients with recurrent non-alcoholic pancreatitis.' ' Endoscopic retrogradecholangiopancreatography has also contributed tothe recognition of an association between relapsingpancreatitis and duodenal duplication,` choledo-chocoele,87 and other rare structural anomalies.8 Wereport two parients who presented with recurrentattacks of acute pancreatitis caused by duodenal cystsin relation to the accessory and main papilla respect-ively, highlighting the delay in diagnosis of thesecysts, the importance of ERCP in their assessmentand the long term response to appropriate surgicalmanagement.

Case reports

PATIENT 1A 21 year old woman was investigated for recurrentpancreatitis. She had been admitted to hospital onfour occasions in the preceding two years for episodesof right upper quadrant and epigastic pain radiatingto the back, relieved by adopting the 'jack-knife'posture. Serum amylase was measured on two admis-

Address for correspondence: Dr I Kalvaria. Gastrointestinal Clinic, GirooteSchuur Hospital, Observatory 7925, Capc Town. South Africa.

Received for publication 2(0 June 1986.

sions and raised on both occasions (6100 and 4780 U/lrespectively) (normal <300 U/l). Two ultrasono-graphic examinations of the upper abdomen and onebarium meal had been interpreted as normal. Therewas no other relevant past medical history, familyhistory or drug history and she denied alcohol intake.On examination, two weeks after her last episode ofabdominal pain, the only abnormal finding was asmall, non-tender, firm epigastric mass.The serum amylase was 305 U/I and the total serum

bilirubin 20 ,imol/l. The transaminase and alkalinephosphatase concentrations, the serum calcium andthe plasma lipids were normal. Review of the bariummeal done two years previously revealed an intra-luminal filling defect in the second part of theduodenum. The lesion was cystic on computed tomo-graphic scanning (Fig. 1). At ERCP a 4x 5 cm smoothcystic mass projected into the duodenal lumenimmediately proximal to the major papilla (Fig. 2).Cholangiography was normal and pancreas divisumwas demonstrated on pancreatography.Laparotomy was carried out in view of the

suspected association between the cyst adjacent tothe ampulla of Vater and the history of relapsingpancreatitis. The pancreas and gall bladder weremacroscopically normal but a longitudinal duo-denotomy revealed a 3-4 cm tense cyst attached tothe medial border of the second part of the duo-denum just proximal to the major papilla (Fig. 3).The cyst overlay the expected site of the accessorypapil1a which could not be clearly identified. Aspira-

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Periampullary cyst: a surgically remediable cause of paticreatiti.s

Fig. 1 Computed tomographyv scanI..... v 0;%i(patient I) showing a cyst in the

duodenum (marker).

tion of the cyst produced clear fluid with low amylaseconcentration, and contrast injection revealed nocommunication with the common bile duct orpancreatic duct. The cyst wall was excised except forthe portion attached to the duodenal wall which wasmarsupialised. Postoperative recovery was unevent-ful. Histology of the excised wall of the cyst revealednormal duodenal mucosa on both surfaces, separatedby a thin muscularis mucosa but no muscularispropria. Six months later the accessory papilla wasidentified at ERCP and a dorsal pancreatogram wasnormal. Two years after surgery the patient hasexperienced no further episodes of pancreatitis.

Fig. 2 Endoscopic view ofsecondpart ofthe duodenum atERCP (patient 1) showing a large mass protruding into thelumen.

Fig. 3 Operative appearance (patient 1) of cyst projectingfrom the medial wall ofthe duodenum just proximal'to thecannulated majorpapilla (arrow).

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Kalvaria, Bornman, Girdwood, and Marks

PATIFINT 2

A 60 year old man.was admitted for investigation ofrecurrent abdominal pain. For 13 years he hadexperienced two to three episodes per year of severeepigastric pain radiating to the back, associated withjaundice on one occasion. Abdominal ultrasono-graphy, oral cholecystography, and upper gastro-intestinal barium radiology were previously normal,but endoscopy done two weeks before his admissionshowed a cystic swelling in the region of the ampullaof Vater. There was no relevant family history andno drug or alcohol intake. On admission he wasasymptomatic and physical examination was unre-markable. The serum amylase was 821 U/l, the totalserum bilirubin 21 [smol/l and the alkaline phos-phatase, serum calcium and blood lipids werenormal. At ERCP a swelling was noted bulging intothe lumen of the second part of the duodenum on itsmedial aspect. A tiny opening on the mass wascannulated and contrast injection filled a large cysticlesion which did not appear to communicate witheither the biliary or pancreatic ductal systems. It wassuspected, however, that the cyst occupied theampulla of Vater, as no other structure resembling amajor papilla, could be found.At laparotomy and duodenotomy a cyst of the

ampulla of Vater was identified (Fig. 4) and laidopen. Both the common bile duct and pancreaticducts opened into the floor of the cyst and werecannulated separately. The cyst was marsupialisedand the patient made a good recovery. He hasremained asymptomatic three years after surgery.Histology of the cyst wall was inadequate to deter-mine its mucosal origin.

Discussion

Cystic lesions in the periampullary region may be dueto a number of different causes, some of which carrya bewildering array of synonyms. These includeenterogenous cyst of the duodenum,9 "' duodenalduplication,'5 juxtampullary bile filled duodenalduplication cyst,'9 intraluminal duodenaldiverticulum,'4 'i type 111 choledochal cyst(choledochocoele),"'6 diverticulum of the commonbile duct'7 and cyst of the ampulla of Vater.'` Theterminology is further complicated by the suggestionthat the intraluminal duodenal diverticulum and theenterogenous or duplication cyst are varieties of thesame entity.'9 Similarly it has been considered thatthe choledochocoele represents another form ofduodenal duplication, occurring at the ampulla.'"

Fig. 4 Operative view (patient2) ofduodenotomyshowing an ampullary cyst.

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Periamplallary cyst: a surgically remediable caluse ofpancreatitis 361

This contention is supported by the observation thatduodenal rather than biliary tract mucosa lines themajority of choledochocoeles.2'Our patients amply illustrate the difficulties

encountered in placing duodenal wall cysts intoprecise diagnostic categories. Patient 1 does not fulfilthe criteria for a duplication cyst22 in view of theabsence of a circular muscle layer within the cyst wall.Furthermore the cyst did not fill with barium andcannot therefore be classified as an intraluminalduodenal diverticulum.2 A precise diagnosis is evenmore difficult in patient 2 whose lesion could repre-sent a duplication cyst or diverticulum occurring atthe ampulla of Vater, or even a choledochocoele.Therefore, in the absence of specific diagnosticfeatures, we have simply termed the lesions'periampullary cysts'.The clinical presentation, investigation, differen-

tial diagnosis and management of duodenal wall cystshave recently been reviewed.' Relapsing pancreatitisas exemplified by the patients in the present report, isuncommon but well described in association withduodenal duplication cyst34 intraluminal duodenaldiverticulum'4 15 and choledochocoele."'" A recentreview4 of eight previously reported cases of acutepancreatitis associated with duodenal duplicationcysts suggests that pancreatitis results from obstruc-tion of the ampulla of Vater caused by intermittentdistension of the duplication, or to duodenal obstruc-tion. Pancreatitis and jaundice in patient 2 of thepresent report may have been the consequence of anintermittent increase in pressure within the ampul-lary cyst resulting in ductal stasis because of obstruc-tion or distortion.The association of periampullary cyst with

pancreas divisum (patient 1) has not previously beendocumented. The absence of further attacks ofpancreatitis after surgical treatment of the cystrelated to the accessory duct opening suggests thatthe latter, rather than the pancreas divisum'4 wasresponsible for recurrent pancreatitis. Periampullarycysts are treated by surgery. In all cases a clearpreoperative definition of the relationship betweenthe cyst and the biliary and pancreatic ducts must beobtained, and is best achieved by ERCP. Althoughvarious surgical approaches have been used' theprocedure currently recommended is internal drain-age of the cyst into the duodenum3 5 a simple, safe,and effective operation.

Dr Kalvaria is a Boehringer Ingelheim ResearchFellow in Gastroenterology. We thank the ChiefMedical Superintendent, Dr H Reeve-Sanders, forpermission to publish. We also gratefully acknow-ledge the secretarial assistance provided by MrsHelena Essel.

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