Gut: first published as 10.1136/gut.28.3.358 on 1 March 1987. Downloaded from

Gut, 1987, 28, 358-362 Case report Periampullary cyst: a surgically remediable cause of

I KALVARIA, P C BORNMAN, A H GIRDWOOD, AND I N MARKS From the Gastrointestinal Clinic, Groote Schuur Hospital and the Departments ofMedicine and Surgery University ofCape Town, Cape Town, South Africa

SUMMARY We report two patients with periampullary cysts associated with recurrent attacks of . 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 cysts to the biliary and pancreatic ductal systems. Simple marsupialisation of the cysts resulted in long term relief of symptoms. Congenital cystic anomalies in the second part of the of the duodenum should be diligently sought in patients with pancreatitis of unexplained cause, as surgical therapy is safe and effective.

With the advent of endoscopic retrograde cholangio- sions and raised on both occasions (6100 and 4780 U/l pancreatography (ERCP), surgically remediable respectively) (normal <300 U/l). Two ultrasono- conditions such as biliary calculi, ampullary stenosis, graphic examinations of the upper abdomen and one http://gut.bmj.com/ and local obstruction can be identi- barium meal had been interpreted as normal. There fied in up to two-thirds of patients with recurrent non- was no other relevant past medical history, family alcoholic pancreatitis.' ' Endoscopic retrograde history or drug history and she denied alcohol intake. cholangiopancreatography has also contributed to On examination, two weeks after her last episode of the recognition of an association between relapsing , the only abnormal finding was a pancreatitis and duodenal duplication,` choledo- small, non-tender, firm epigastric mass. chocoele,87 and other rare structural anomalies.8 We The serum amylase was 305 U/I and the total serum report two parients who presented with recurrent bilirubin 20 ,imol/l. The transaminase and alkaline on September 29, 2021 by guest. Protected copyright. attacks of acute pancreatitis caused by duodenal cysts phosphatase concentrations, the serum calcium and in relation to the accessory and main papilla respect- the plasma lipids were normal. Review of the barium ively, highlighting the delay in diagnosis of these meal done two years previously revealed an intra- cysts, the importance of ERCP in their assessment luminal filling defect in the second part of the and the long term response to appropriate surgical duodenum. The lesion was cystic on computed tomo- management. graphic scanning (Fig. 1). At ERCP a 4x 5 cm smooth cystic mass projected into the duodenal lumen Case reports immediately proximal to the major papilla (Fig. 2). Cholangiography was normal and divisum PATIENT 1 was demonstrated on pancreatography. A 21 year old woman was investigated for recurrent Laparotomy was carried out in view of the pancreatitis. She had been admitted to hospital on suspected association between the cyst adjacent to four occasions in the preceding two years for episodes the ampulla of Vater and the history of relapsing of right upper quadrant and epigastic pain radiating pancreatitis. The pancreas and gall bladder were to the back, relieved by adopting the 'jack-knife' macroscopically normal but a longitudinal duo- posture. Serum amylase was measured on two admis- denotomy revealed a 3-4 cm tense cyst attached to the medial border of the second part of the duo- Address for correspondence: Dr I Kalvaria. Gastrointestinal Clinic, Giroote denum just proximal to the major papilla (Fig. 3). Schuur Hospital, Observatory 7925, Capc Town. South Africa. The cyst overlay the expected site of the accessory Received for publication 2(0 June 1986. papil1a which could not be clearly identified. Aspira- 358 Gut: first published as 10.1136/gut.28.3.358 on 1 March 1987. Downloaded from

Periampullary cyst: a surgically remediable cause ofpaticreatiti.s 3593

Fig. 1 Computed tomographyv scan I..... v %i(patient0; I) showing a cyst in the duodenum (marker).

tion of the cyst produced clear fluid with low amylase concentration, and contrast injection revealed no communication with the common or pancreatic duct. The cyst wall was excised except for the portion attached to the duodenal wall which was marsupialised. Postoperative recovery was unevent- ful. Histology of the excised wall of the cyst revealed normal duodenal mucosa on both surfaces, separated

by a thin muscularis mucosa but no muscularis http://gut.bmj.com/ propria. Six months later the accessory papilla was identified at ERCP and a dorsal pancreatogram was normal. Two years after surgery the patient has experienced no further episodes of pancreatitis. on September 29, 2021 by guest. Protected copyright.

Fig. 2 Endoscopic view ofsecondpart ofthe duodenum at Fig. 3 Operative appearance (patient 1) of cystprojecting ERCP (patient 1) showing a large mass protruding into the from the medial wall ofthe duodenum justproximal'to the lumen. cannulated majorpapilla (arrow). Gut: first published as 10.1136/gut.28.3.358 on 1 March 1987. Downloaded from

^360( Kalvaria, Bornman, Girdwood, and Marks

PATIFINT 2 ampulla of Vater was identified (Fig. 4) and laid A 60 year old man.was admitted for investigation of open. Both the common bile duct and pancreatic recurrent abdominal pain. For 13 years he had ducts opened into the floor of the cyst and were experienced two to three episodes per year of severe cannulated separately. The cyst was marsupialised epigastric pain radiating to the back, associated with and the patient made a good recovery. He has jaundice on one occasion. Abdominal ultrasono- remained asymptomatic three years after surgery. graphy, oral cholecystography, and upper gastro- Histology of the cyst wall was inadequate to deter- intestinal barium radiology were previously normal, mine its mucosal origin. but endoscopy done two weeks before his admission showed a cystic swelling in the region of the ampulla Discussion of Vater. There was no relevant family history and no drug or alcohol intake. On admission he was Cystic lesions in the periampullary region may be due asymptomatic and physical examination was unre- to a number of different causes, some of which carry markable. The serum amylase was 821 U/l, the total a bewildering array of synonyms. These include serum bilirubin 21 [smol/l and the alkaline phos- enterogenous cyst of the duodenum,9 "' duodenal phatase, serum calcium and blood lipids were duplication,'5 juxtampullary bile filled duodenal normal. At ERCP a swelling was noted bulging into duplication cyst,'9 intraluminal duodenal the lumen of the second part of the duodenum on its diverticulum,'4 'i type 111 choledochal cyst medial aspect. A tiny opening on the mass was (choledochocoele),"'6 diverticulum of the common cannulated and contrast injection filled a large cystic bile duct'7 and cyst of the ampulla of Vater.'` The lesion which did not appear to communicate with terminology is further complicated by the suggestion either the biliary or pancreatic ductal systems. It was that the intraluminal duodenal diverticulum and the suspected, however, that the cyst occupied the enterogenous or duplication cyst are varieties of the ampulla of Vater, as no other structure resembling a same entity.'9 Similarly it has been considered that major papilla, could be found. the choledochocoele represents another form of At laparotomy and duodenotomy a cyst of the duodenal duplication, occurring at the ampulla.'" http://gut.bmj.com/ on September 29, 2021 by guest. Protected copyright.

Fig. 4 Operative view (patient2) ofduodenotomyshowing an ampullary cyst. Gut: first published as 10.1136/gut.28.3.358 on 1 March 1987. Downloaded from Periamplallary cyst: a surgically remediable caluse ofpancreatitis 361

This contention is supported by the observation that References duodenal rather than biliary tract mucosa lines the majority of choledochocoeles.2' 1 Katon RM, Bilbao MK, Eidemiller LR, Benson JA Jr. Our patients amply illustrate the difficulties Endoscopic retrograde cholangiopancreatography in encountered in placing duodenal wall cysts into the diagnosis and management of non-alcoholic precise diagnostic categories. Patient 1 does not fulfil pancreatitis. Surg Gynecol Obstet 1978; 147: 333-8. the criteria for a duplication cyst22 in view of the 2 Geenen JE, Hogan WJ. Endoscopic access to the papilla absence of a circular muscle layer within the cyst wall. of Vater. Endoscopy 1980: suppl. 47-56. Furthermore the cyst did not fill with barium and 3 Holstege A, Barner S, Brambs HJ, Wenz W, Gerok W, cannot therefore be classified as an intraluminal Farthmann EH. Relapsing pancreatitis associated with duodenal diverticulum.2 A precise diagnosis is even duodenal wall cysts. Diagnostic approach and treatment. more difficult in patient 2 whose lesion could repre- Gastroenterology 1985; 88: 814-9. 4 Abrams J, Connon JJ. Duodenal duplication presenting sent a duplication cyst or diverticulum occurring at as relapsing pancreatitis in an adult. Am J Gastroenterol the ampulla of Vater, or even a choledochocoele. 1984; 79: 360-2. Therefore, in the absence of specific diagnostic 5 Luckman KF, Welch RW, Schwesinger W, Oswalt C, features, we have simply termed the lesions Bannayan G. Symptomatic duodenal duplication cyst in 'periampullary cysts'. an adult demonstrated by endoscopic retrograde The clinical presentation, investigation, differen- cholangiopancreatography. Am J Gastroenterol 1979; tial diagnosis and management of duodenal wall cysts 72: 153-9. have recently been reviewed.' Relapsing pancreatitis 6 Greene FL, Brown JJ, Rubinstein P, Anderson MC. as exemplified by the patients in the present report, is Choledochocele and recurrent pancreatitis. Diagnosis uncommon but well described in association with and surgical management. Am J Surg 1985; 149: 306-9. duodenal duplication cyst34 intraluminal duodenal 7 Venu RP, Greene JE, Hogan WJ, et al. Role of diverticulum'4 15 and choledochocoele."'" A recent endoscopic retrograde cholangiopancreatography in the review4 of eight previously reported cases of acute diagnosis and treatment of choledochocele. Gastro- pancreatitis associated with duodenal duplication enterology 1984; 87: 1144-9. that pancreatitis results from obstruc- 8 Hatfield ARW, Dent DM, Marks IN. An intrapapillary cysts suggests pancreatic duct diverticulum: a rare but surgically tion of the ampulla of Vater caused by intermittent correctable cause of juvenile pancreatitis. Br J Surg distension of the duplication, or to duodenal obstruc- 1982; 69: 430-1. http://gut.bmj.com/ tion. Pancreatitis and jaundice in patient 2 of the 9 Kirtley JA Jr, Matuska RA. Enterogenous cyst of the present report may have been the consequence of an duodenum. Ann Surg 1957; 145: 265-8. intermittent increase in pressure within the ampul- 10 Ortiz VM, Nealon TF, Mitty WF. Enterogenous cyst of lary cyst resulting in ductal stasis because of obstruc- the duodenum. Am J Gastroenterol 1974; 61: 276-81. tion or distortion. 11 Basu R, Forshall 1, Rickham PP. Duplications of the The association of periampullary cyst with alimentary tract. Br J Surg 1960; 47: 477-84. pancreas divisum (patient 1) has not previously been 12 Browning RW. Duodenal duplications. Rev Surg 1963; on September 29, 2021 by guest. Protected copyright. documented. The absence of further attacks of 20: 226-9. pancreatitis after surgical treatment of the cyst 13 Jones PA, Rayter Z, Knight MJ, Rosswick RPR. related to the accessory duct opening suggests that Juxtampullary, bile-filled duodenal duplication cyst: the latter, rather than the pancreas divisum'4 was another surgically correctable cause of acute responsible for recurrent pancreatitis. Periampullary pancreatitis. J R Soc Med 1982; 75: 662-4. cysts are treated by surgery. In all cases a clear 14 Nosher JL, Seaman WB. Association of intraluminal preoperative definition of the relationship between duodenal diverticulum with acute pancreatitis. the cyst and the biliary and pancreatic ducts must be Radiology 1975; 115: 21-2. obtained, and is best achieved by ERCP. Although 15 Griffin M, Carey WD, Hermann R, Buonocore E. various surgical approaches have been used' the Recurrent acute pancreatitis and intussusception procedure currently recommended is internal drain- complicating an intraluminal duodenal diverticulum. age of the cyst into the duodenum3 5 a simple, safe, Gastroenterology 1981; 81: 345-8. and effective operation. 16 Wheeler WI DeC. An unusual case of obstruction to the common bile-duct (choledochocele ?). Br J Surg 1940; 27: 446-8. Dr Kalvaria is a Boehringer Ingelheim Research 17 Alden JF, Sterner ER. Diverticulum of the common bile Fellow in Gastroenterology. We thank the Chief duct: a case report. Ann Surg 1957; 145: 269-70. Medical Superintendent, Dr H Reeve-Sanders, for 18 Brooks B, Weinstein A. Cyst of the ampulla of Vater. permission to publish. We also gratefully acknow- Ann Surg 1943; 117: 728-34. ledge the secretarial assistance provided by Mrs 19 Wiot JF, Spiro E. Intraluminal diverticulum: a form of Helena Essel. duplication. Radiology 1963; 80: 46-9. Gut: first published as 10.1136/gut.28.3.358 on 1 March 1987. Downloaded from

362 Kalvaria, Bornman, Girdwood, and Marks

20 Reinus FZ, Weingarten G. Choledochocele of the sis, management. Philadelphia: Saunders, 1983: common bile duct. Am J Surg 1976; 132: 646-8. 780-811. 21 Alonso-Lej F, Rever WB Jr, Pessagno DJ. Congenital 23 Perrin RW. Communicating enterogenous cyst of the choledochal cyst, with a report of 2, and an analysis of duodenum receiving the termination of the common bile 94, cases. Int Abstr Surg 1959; 108: 1-30. duct demonstrated by intravenous cholangiography. 22 Trier JS, Krone CL, Sleisenger MH. , embry- Radiology 1969; 93: 675-6. ology and developmental abnormalities of the small 24 Cotton PB. Congenital anomaly of pancreas divisum as intestine and colon. In: Sleisenger MH, Fordtran JS, cause of obstructive pain and pancreatitis. Gut 1980: 21: eds. Gastrointestinal disease: pathophysiology, diagno- 105-14. http://gut.bmj.com/ on September 29, 2021 by guest. Protected copyright.