Gut, 1990,31,561-563

CASE REPORT

Pancreatic pseudocyst causing portal veinthrombosis and pancreatico-pleural fistula

P A McCormick, N Chronos, A K Burroughs, N McIntyre, J E McLaughlin

AbstractPortal vein thrombosis and pancreatico-pleural fistula are unusual complications ofchronic pancreatitis. We describe a patientwith chronic alcoholic pancreatitis in whomerosion of the splenic vein led to portal veinthrombosis and to the development of apancreatico-pleural fistula. We suggest thatfistula formation may occur over a consider-able time period as the portal vein thrombosiswas diagnosed three years before the amylase-rich pleural effusions.

Royal Free HospitalSchool of MedicineLondon, AcademicDepartment of MedicineP A McCormickN ChronosA K BurroughsN McIntyre

Department ofHistopathologyJ E McLaughlinCorrespondence to:Dr P A McCormick, RoyalFree Hospital, LondonNW3 2QG.Accepted for publication21 August 1989

Pancreatico-pleural fistula` and portal veinthrombosis4 are rare, but well described, compli-cations of chronic pancreatitis. Pancreaticfistulae are thought to be caused by disruptionof the pancreatic duct in patients with chronicpancreatitis. Little is known of the naturalhistory of pancreatic fistulae before clinicalpresentation and one of the puzzling features ofthe condition is that it may appear in the absenceof symptoms or signs of pancreatic inflamma-tion.' We present a patient in whom the fistuloustrack involved the portal venous system andprobably caused portal vein thrombosis. As theportal vein thrombosis was noted three yearsbefore the patient presented with pancreaticpleural effusions we suggest that this lesion mayhave a very long presymptomatic phase.

Figure 1: Abdominal computed tomography (CT) scan with oral and intravenous contrastshowing a kidney shaped pseudocyst in the pancreatic head (arrowed).

Case reportA 36 year old man, with a history of excessivealcohol intake, initially presented to anotherhospital in October 1984 with acute abdominalpain. Serum amylase was 1800 IU/l and a diag-nosis of pancreatitis was made. Ultrasoundshowed an enlarged head of pancreas but nopseudocyst. Repeat ultrasound three monthslater showed similar findings. After dischargefrom hospital he reduced his alcohol intake butexperienced similar, milder symptoms on twofurther occasions. These were treated at homnewith oral analgesics and a liquid diet. In August1985 he was referred to our unit because ofcontinuing abdominal discomfort. Serumamylase, in out-patients, was raised at 876 IU/l.An abdominal computed, tomography scanrevealed a cyst (3x2x 5 cm) in the head of thepancreas (Fig 1) and a possible portal veinthrombosis. He was admitted to hospital where asplenic venogram confirmed portal vein throm-bosis. An ERCP showed distortion of the pan-creatic duct consistent with chronic pancreatitis,a small cyst in the head of pancreas communicat-ing with the ductal system, and a normal biliarytree. In view of the small size of the cyst and thepresence of portal hypertension, surgery to drainthe cyst was not performed. In October 1987 hewas admitted after a minor haematemesis. Adiagnosis of bleeding from gastritis was made onendoscopy. Small white oesophageal variceswere also noted. He was treated with H2 blockersand discharged.

In January 1988 he was admitted with a oneweek history of gradually increasing shortness ofbreath and chest discomfort. He admitted todrinking approximately 284 ml of lager daily forthe previous three years. On examination he wastachypnoeic, had a tachycardia and largebilateral pleural effusions. He was not jaundicedand there was not evidence of ascites. Serumamylase was 2663 IU/l and pleural fluid amylase73 000 IU/l. An abdominal computed tomo-graphy scan showed an irregular pancreas withcalcification and a few tiny cystic areas, andconfirmed the absence of ascites. He was treatedinitially with H2 blockers and oral pancreaticsupplements to suppress pancreatic secretionand with total parenteral nutrition and regularthoracentesis for symptom relief. Intravenousantibiotics were added subsequently when hebecame febrile. He required repeated largevolume thoracentesis for relief of dyspnoea andbilateral large bore chest drains were inserted. As

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McCormick, Chronos, Burroughs, McIntyre, McLaughlin

Figure 2: Endoscopicretrogradecholangiopancreatography(ERCP) showing a smallcavity in the head ofpancreas (arrowed)communicating with thepancreatic ductal system.

Figure 3: Latefilm (20 mins)after the ERCP illustratedin Figure 2 showing contrastfilling the inferior portion ofthe fistulous tract (arrowed)between the pancreas and thepleural cavities.

he was not responding after three weeks ofconservative therapy a subtotal pancreatectomywas planned. Before surgery an ERCP was

performed which showed a grossly distortedpancreatic ductal system with a small cyst in thehead of the pancreas (Fig 2). Late films showedthat contrast from this cystic lesion flowedupwards towards the mediastrinum in a fistuloustrack (Fig 3). Unfortunately, before surgery

could be performed he developed respiratoryfailure and required ventilation. He subse-quently developed severe intrathoracic sepsiswith abscess formation, adult respiratorydistress syndrome and renal failure. Despiteventilation, haemofiltration, intravenous anti-biotics and numerous attempts to drain theintrathoracic abscesses he died nine weeks afteradmission.

Post mortem examination confirmed the diag-nosis of chronic pancreatitis with duct ectasiaand ulceration of the wall of the distal part of thesplenic vein. The ulcer extended to involve theproximal part of the portal vein and was con-tinuous with the wall of a haemorrhagic cavityleading up into the posterior mediastinumthrough the oesophageal hiatus (Fig 4). Distallythe portal vein showed evidence of previousthrombosis and recanalisation. Numerousloculated abscesses were present in the leftpleural cavity.

DiscussionThis patient had chronic alcoholic pancreatitiscomplicated by pseudocyst formation, portalvein thrombosis and a pancreatico-pleuralfistula. We are not aware that a previous casewith this combination of rare complications ofchronic pancreatitis has been reported. Atnecropsy it appeared the pseudocyst initiallyeroded into the splenic vein causing portal veinthrombosis and subsequently progressedupwards into the mediastinum to rupture intothe pleural cavity. The fistula may haveeroded along the line of the left gastric (coronary)vein to reach the perioesophageal venouscollaterals. These events occurred over a threeyear observation period which suggests thatpancreatico-pleural fistulae may develop veryslowly. It is possible that initially the portal veinthrombosis was unrelated directly to the pseudo-cyst and the fistula and that by chance the fistulaeroded through the already thrombosed vein.This latter possibility appears less likely as inmost cases of extrahepatic portal hypertensioncomplicating pancreatitis the splenic vein aloneis involved because of its close proximity to thepancreas.'

Pancreatic fistulae causing pancreatic ascitesor pancreatic pleural effusions have been increas-ingly recognised in the last 15 years particularlyin patients with alcoholic pancreatitis.'3 Theyoccur because of disruption of the duct inpatients with chronic pancreatitis. The diagnosismay easily be missed if amylase is not measuredin the pleural fluid as many patients have noabdominal symptoms or signs. Indeed one of thepuzzling features of this condition has been thenumber of patients reported with no previoushistory of pancreatitis. Even in those with ahistory of acute pancreatitis the reportedepisode, in many cases, occurred months oryears before the presentation of the fistula. ' Thisdiscrepancy between the abdominal symptomsand the manifestation of the fistula may beexplained if fistulae have a very long pre-symptomatic phase, as we suggest. In thosepatients with no history of an acute attack ofpancreatitis a previous attack of abdominalpain may have been misdiagnosed, ignored, orforgotten.

Because of its rarity there are no controlledtrials concerning management of pancreatico-pleural fistula. Most authors suggest a trial ofconservative therapy for two to three weeks,'3 68aimed at reducing pancreatic secretion andimproving patient nutrition. If this fails excisionor drainage of the damaged pancreas is recom-

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Pancreatic pseudocyst causingportal vein thrombosis andpqncreatico-pleuralfistula 563

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Figure 4: Posterior dissection ofthe portal venous system showing ulceration ofthe distal parts ofthe splenic vein (SV) and part ofthe cavity ofthefistula (CF). Strands ofconnective tissue are seen in the lumen of the recanalised portal vein (PV). SMV=superior mesentenic vein.

mended. It is also recommended that the fistulabe visualised before surgery so that the appro-priate procedure may be planned preoperatively.In many cases the fistula may be visualised byERCP,79 computed tomography scanning,2 orthe injection of lipiodol into the pleural cavity.'"There is one report of successful treatment usinglow dose radiotherapy.9 Conservative treatmentfailed in our patient and surgery was planned.

Drainage of the pseudocyst after its identifica-tion in 1985 might have averted the tragicsequelae in our patient. Most authors nowrecommend early drainage of pseudocysts."1Conservative treatment may be justified inpatients with small pseudocysts, however,'2 par-ticularly ifthey occur in association with an acuteattack of pancreatitis.'3 The presence of portalhypertension4 and the location of the pseudocystin the head of the pancreas would have added tothe difficulties of surgery in our patient.

We would like to thank Ms Doris Elliott for secretarial assistance.

1 Cameron JL. Chronic pancreatic ascites and pancreatic pleuraleffusions. Gastroenterology 1978; 74: 134-40.

2 Pottmeyer EW, Frey CF, Matsuno S. Pancreaticopleuralfistulas. Arch Surg 1987; 122: 648-54.

3 Bedingfield JA, Anderson MC. Pancreaticopleural fistula.Pancreas 1986; 1: 283-90.

4 Warshaw AL, Jin GL, Ottinger LW. Recognition andclinical implications of mesenteric and portal vein obstruc-tion in chronic pancreatitis. Arch Surg 1987; 122: 410-5.

5 Madsen MS, Petersen TH, Sommer H. Segmental portalhypertension. Ann Surg 1986; 204: 72-7.

6 Liedberg G, Lindmark G, Struwe E. Pleural effusion withdyspnoea as the presenting symptom in chronic pancreatitis.Acta Chir Scand 1983; 149: 209-1 1.

7 Rotman N, Fagniez PL. Chronic pancreaticopleural fistulas.Arch Surg 1984; 119: 1204-6.

8 Dewan NA, Kinney WW, O'Donoghue WJ. Chronic massivepancreatic pleural effusion. Chest 1984; 85: 497-501.

9 Greenwald RA, Deluca RF, Raskin JB. Pancreatic-pleuralfistula. Demonstration by endoscopic retrograde cholangio-pancreatography (ERCP) and successful treatment withradiation therapy. Dig Dis Sci 1979; 24: 240-4.

10 Tombroff M, Loicq A, De Koster JP, et al. Pleural effusionwith pancreaticopleural fistula. BrMedJ 1973; 1: 330-1.

11 Mullins RJ, Malangoni MA, Bergamini TM, Casey JM,Richardson JD. Controversies in the management of pan-creatic pseudocysts. AmJ Surg 1988; 155: 165-72.

12 Sankaran S, Walt AJ. The natural and unnatural history ofpancreatic pseudocysts. BrJ7 Surg 1975; 62: 37-44.

13 London NJM, Neoptolemos JP, Lavelle J, Bailey I, James D.Serial computed tomography scanning in acute pancreatitis;a prospective study. Gut 1989; 30: 397-403.

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