Case Study Asian Cardiovascular & Thoracic Annals 22(1) 98–101 ß The Author(s) 2013 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492312474453 aan.sagepub.com
Pancreaticopleural fistula: an overlooked entity Turki Altasan1, Yasser Aljehani2, Ahmad Almalki3, Saleh Algamdi4, Ali Talag1 and Khalid Alkattan2
Abstract Pleuropulmonary complications of pancreatitis are well known. Less commonly encountered is pancreaticopleural fistula. We describe the case of a 15-year-old boy with a presumed episode of pancreatitis, complicated by pseudocyst and development of a pancreaticopleural fistula. Successful medical management was achieved, and he made a full recovery. This case demonstrates that the rarity of such a condition leads to delay as well as challenges in diagnosis and management.
Keywords Pancreaticopleural, Pancreatic ducts, Pancreatic fistula, Pleural effusion
Introduction Pancreaticopleural fistula (PPF) is a rare complication of pancreatitis. It is seen in cases of acute and chronic pancreatitis. The pathogenesis includes the herniation of a pancreatic pseudocyst and subsequent rupture or direct communication between the pancreatic duct and the pleural cavity. The management includes conservative medical, endoscopic, or surgical approaches.1
Case report A 15-year-old boy who was known to be autistic with mental retardation but no previous medical illnesses, presented with shortness of breath, generalized weakness, and weight loss over a period of 1 month. There was no history productive cough, hemoptysis, or fever. There was no abdominal pain or gastrointestinal symptoms. His surgical, medical, drug, family and social histories as well as a review of systems were unremarkable. Physical examination revealed a thin and mute boy with no respiratory distress signs. His vital signs were stable. A cardiac examination was within normal limits. Chest examination found decreased chest movement, decreased air entry, and stony dullness on the right side. Abdominal examination did not disclose any pain, tenderness, or organomegaly. A neurological examination did not elicit any deficit, and psychological
findings were consistent with autism and mental retardation. A full blood count, renal, and hepatic profiles were normal. An immune profile and sputum acidfast bacillus culture were negative. Chest radiography revealed massive right-sided pleural effusion (Figure 1). Ultrasound-guided thoracocentesis was performed and the pleural fluid analysis was consistent with exudative lymphocytic pleural effusion. Partial pleural drainage was achieved. Computed tomography of the chest and upper abdomen showed minimal residual rightsided pleural effusion and an element of atelectasis, no mediastinal lymphadenopathy, and no parenchymal lung lesions. The abdomen had an encapsulated retropancreatic fluid collection, a possible pancreatic
1 Department of Medicine, Pulmonary Medicine, Prince Sultan Medical City, Riyadh, Saudi Arabia 2 Department of Surgery, Thoracic Section, King Faisal Specialist Hospital & Research Center, Riyadh, Saudi Arabia 3 Department of Medicine, Gastroenterology, Prince Sultan Medical City, Riyadh, Saudi Arabia 4 Department of Surgery, Thoracic Section, Prince Sultan Medical City, Riyadh, Saudi Arabia
Corresponding author: Yasser Aljehani, MD, King Faisal Specialist Hospital & Research Center, Riyadh, Kingdom of Saudi Arabia. Email: [email protected]
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Figure 1. Chest radiograph demonstrating right-sided massive pleural effusion.
pseudocyst, fluid collection between the small bowel loop, and some bowel loops were matted, raising the suspicion of tuberculosis (TB) but a polymerase chain reaction test was negative. A chest tube was inserted, yielding a persistent and large amount of drainage. The patient underwent video-assisted thoracoscopic drainage and pleural biopsy. No specific inflammatory pattern was seen. At this point, there was a diagnostic dilemma with the possibility of TB, lymphoma, or other malignancy. The possibility of starting anti-TB medication empirically was entertained because TB is endemic in this area. The patient developed abdominal pain in the early postoperative period, and laboratory investigations showed a raised blood amylase level (>1000 U mL 1; 9-fold). He was treated conservatively for pancreatitis. Pleural fluid amylase was found to be very high (35,536 U mL 1). Magnetic resonance pancreatography confirmed a fistulous tract between the pancreas and the right pleural space, which had formed during 2 weeks from the time of the initial abdominal computed tomography (Figure 2). A thoracic intervention was undertaken to decorticate and drain the loculated fluid because it was persistent. Medical treatment was started in the form of total parenteral nutrition and intravenous octreotide. Endoscopic retrograde cholangiopancreatography (ERCP) and sphincterotomy, which helps in reducing ductal pressure as well as easing stent introduction, and endoprosthesis stenting were carried out (Figure 3). The stent was inserted over the assumed site of the fistula. The patient’s condition improved dramatically with resolution of his radiologic and laboratory parameters. The final diagnosis was PPF as a complication of pancreatitis. The cause of the initial pancreatitis with pseudocyst complication remains unknown. The stent was removed in the 5th week.
Figure 2. Magnetic resonance imaging demonstrating a fistula between the pancreas and the right pleural cavity. The arrow indicates the possible fistula tract.
Discussion Pleuropulmonary complications associated with pancreatitis are common. They include atelectasis, pneumonia, pleural effusion, and acute respiratory distress syndrome. In acute pancreatitis, 5%–15% of cases are complicated by pseudocyst, necrosis, abscess, or PPF. Pleural effusion is seen in 3%–17% of cases, and it indicates a worse prognosis.2 In the setting of chronic pancreatitis where history of alcoholism can be elicited, 20%–40% of patients develop pseudocysts.3 PPF is seen in less than 1% of acute pancreatitis cases but in 0.4%–4.5% of chronic pancreatitis cases. Moreover, PPF is associated with pseudocyst in 77% of cases.4 It affects the age group 20–60 years in 95% of cases. Patients usually present with respiratory rather than abdominal symptoms. Respiratory symptoms include chest pain, shortness of breath, cough, fever, and even septicemia. Abdominal symptoms are seen in 24% of cases.5,6 Pancreatic symptoms in PPF are almost never recorded.1 This creates a delay in diagnosis which requires a high index of suspicion. The average time to diagnose PPF is around 5 weeks. Pleural effusion is usually present, associated with ascites in 20% of cases; it has been reported with pericarditis in 4% of cases.6 Pleural effusion in acute pancreatitis is not uncommon, usually small, transient, and left-sided, resulting from a lymphatic or sympathetic inflammatory reaction.
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Figure 3. Endoscopic retrograde cholangiopancreatography demonstrating the fistula tract (arrow).
In cases of PPF, associated effusions tend to be large and recurrent. They are seen at the left side in 76% of cases. As pancreatitis erodes intraperitoneally as well as retroperitoneally, plane dissections are created, reaching the pleura. A leaking pancreatic duct or pseudocyst can access the pleural cavity through a diaphragmatic or aortic hiatus or directly transdiaphragmatically.1,6,7 The effusion is typically exudate with a high amylase level as a hallmark, compared to serum.3 In general, the most common cause of amylase-rich effusion is malignancy; it was first reported in 1951 in association with lung cancer. Several subsequent reports associated it with ovarian or metastatic tumors, lymphoma, or leukemia.4 Others included post-coronary artery bypass, pneumonia, liver cirrhosis, TB, and hydronephrosis. Only pancreatitis-related PPF has the pancreatic-type amylase; the others have salivary-type amylase that can be produced by salivary glands, lung, or fallopian tubes, or ectopically by tumors.7,8 The pathogenesis of PPF involves a pancreatic duct stenosis or leak, which can result from trauma, surgery, ERCP, or pancreatitis.1,5 It is very difficult to demonstrate the fistula radiologically. ERCP is mandatory in such cases to document duct pathology and aid decision making.1,6 It has been reported that 80% of duct pathology and 59% of fistulae can be demonstrated. Magnetic resonance imaging has been reported to be sensitive with variable ranges.5 Some cases of mediastinal pancreatic pseudocyst have been reported wrongfully as a mediastinal mass on chest radiography.2 There are no clear guidelines for PPF management because it is a rare entity. It has been reported that 50% of PPF will close with conservative measures, with subsequent resolution of effusion. Form our literature review, we concur with the notion that management
has to be individualized according to fistula characteristics, but the work of Wronski and colleagues9 simplified the management of PPF based on categorizing the patients into 3 groups according to ERCP findings: complete stenosis, partial stenosis, or no stenosis. Location, obstruction, and extent determine the line of management. Since the first endoscopic pancreatic stenting in 1993, the role of surgery has fallen back. Such stenting reduces the intraductal pressure and seals the leak.2,5,6 Endoscopic intervention in conjunction with total parenteral nutrition, octreotide, and chest drainage constitute the medical management. It has been reported that such an approach closes the fistula in 2–3 weeks in 48% of cases, with concurrent management of the underlying cause. Pancreatic duct stents can be left for 4 weeks and up to 6 months. Limitations of stenting are a distally located fistula and completely ruptured or obstructed duct.1,5 Complications of medical management include malnutrition, central catheter infection, deep vein thrombosis, and sepsis. Surgical management is indicated in failure of medical management (3–4 weeks are considered) or obstructed pancreatic duct.1,6 Surgery includes either distal pancreatectomy or pancreaticojejunostomy. Operative mortality is 3%–5%. Recurrence has been reported in up to 11% of cases. The long-term outcome is good in 80%–95% of cases, with overall PPF mortality of 5%.1,6,7 PPF is a rare complication mainly in the setting of chronic pancreatitis. Extensive investigations should be carried out, but a delay in diagnosis may lead to sepsis. Routine pleural effusion amylase levels are not indicated from a cost-effective point of view, and management has to be individualized according to ERCP ductal pathology. Funding This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.
References 1. Wakefield S, Tutty B and Britton J. Pancreaticopleural fistula: a rare complication of chronic pancreatitis. Postgrad Med J 1996; 72: 115–116. 2. Subrt Z, Ferko A, Papı´ k Z, Vacek Z and Sedla´cek Z. A rare cause of mediastinal expansion with a massive pleural effusion. Postgrad Med J 2005; 81: e18. 3. Materne R, Vranckx P, Pauls C, Coche EE, Deprez P and Van Beers BE. Pancreaticopleural fistula: diagnosis with magnatic resonance pancreatography. Chest 2000; 117: 912–914. 4. Joseph J, Viney S, Beck P, Strange C, Sahn SA and Basran GS. A prospective study of amylase-rich pleural effusions with special reference to amylase isoenzyme analysis. Chest 1992; 102: 1455–1459.
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5. Kiewiet JJ, Moret M, Blok WL, Gerhards MF and de Wit LT. Two patients with chronic pancreatitis complicated by a pancreaticopleural fistula. Case Rep Gastroenterol 2009; 3: 36–42. 6. Dhebri AR and Ferran N. Nonsurgical management of pancreaticopleural fistula. JOP 2005; 6: 152–162. 7. Williams SG, Bhupalan A, Zureikat N, et al. Pleural effusions associated with pancreaticopleural fistula. Thorax 1993; 48: 867–868.
8. Branca P, Rodriguez RM, Rogers JT, Ayo DS, Moyers JP and Light RW. Routine measurement of pleural fluid amylase in not indicted. Arch Intern Med 2001; 161: 228–232. 9. Wronski M, Slodkowski M, Cebulski W, Moronczyk D and Krasnodebski IW. Optimizing the management of pancreaticopleural fistulas. World J Gastroenterol 2011; 17: 4696–4703.
Downloaded from aan.sagepub.com by guest on June 8, 2015
Pancreaticopleural fistula: an overlooked entity Turki Altasan1, Yasser Aljehani2, Ahmad Almalki3, Saleh Algamdi4, Ali Talag1 and Khalid Alkattan2
Abstract Pleuropulmonary complications of pancreatitis are well known. Less commonly encountered is pancreaticopleural fistula. We describe the case of a 15-year-old boy with a presumed episode of pancreatitis, complicated by pseudocyst and development of a pancreaticopleural fistula. Successful medical management was achieved, and he made a full recovery. This case demonstrates that the rarity of such a condition leads to delay as well as challenges in diagnosis and management.
Keywords Pancreaticopleural, Pancreatic ducts, Pancreatic fistula, Pleural effusion
Introduction Pancreaticopleural fistula (PPF) is a rare complication of pancreatitis. It is seen in cases of acute and chronic pancreatitis. The pathogenesis includes the herniation of a pancreatic pseudocyst and subsequent rupture or direct communication between the pancreatic duct and the pleural cavity. The management includes conservative medical, endoscopic, or surgical approaches.1
Case report A 15-year-old boy who was known to be autistic with mental retardation but no previous medical illnesses, presented with shortness of breath, generalized weakness, and weight loss over a period of 1 month. There was no history productive cough, hemoptysis, or fever. There was no abdominal pain or gastrointestinal symptoms. His surgical, medical, drug, family and social histories as well as a review of systems were unremarkable. Physical examination revealed a thin and mute boy with no respiratory distress signs. His vital signs were stable. A cardiac examination was within normal limits. Chest examination found decreased chest movement, decreased air entry, and stony dullness on the right side. Abdominal examination did not disclose any pain, tenderness, or organomegaly. A neurological examination did not elicit any deficit, and psychological
findings were consistent with autism and mental retardation. A full blood count, renal, and hepatic profiles were normal. An immune profile and sputum acidfast bacillus culture were negative. Chest radiography revealed massive right-sided pleural effusion (Figure 1). Ultrasound-guided thoracocentesis was performed and the pleural fluid analysis was consistent with exudative lymphocytic pleural effusion. Partial pleural drainage was achieved. Computed tomography of the chest and upper abdomen showed minimal residual rightsided pleural effusion and an element of atelectasis, no mediastinal lymphadenopathy, and no parenchymal lung lesions. The abdomen had an encapsulated retropancreatic fluid collection, a possible pancreatic
1 Department of Medicine, Pulmonary Medicine, Prince Sultan Medical City, Riyadh, Saudi Arabia 2 Department of Surgery, Thoracic Section, King Faisal Specialist Hospital & Research Center, Riyadh, Saudi Arabia 3 Department of Medicine, Gastroenterology, Prince Sultan Medical City, Riyadh, Saudi Arabia 4 Department of Surgery, Thoracic Section, Prince Sultan Medical City, Riyadh, Saudi Arabia
Corresponding author: Yasser Aljehani, MD, King Faisal Specialist Hospital & Research Center, Riyadh, Kingdom of Saudi Arabia. Email: [email protected]
Downloaded from aan.sagepub.com by guest on June 8, 2015
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Figure 1. Chest radiograph demonstrating right-sided massive pleural effusion.
pseudocyst, fluid collection between the small bowel loop, and some bowel loops were matted, raising the suspicion of tuberculosis (TB) but a polymerase chain reaction test was negative. A chest tube was inserted, yielding a persistent and large amount of drainage. The patient underwent video-assisted thoracoscopic drainage and pleural biopsy. No specific inflammatory pattern was seen. At this point, there was a diagnostic dilemma with the possibility of TB, lymphoma, or other malignancy. The possibility of starting anti-TB medication empirically was entertained because TB is endemic in this area. The patient developed abdominal pain in the early postoperative period, and laboratory investigations showed a raised blood amylase level (>1000 U mL 1; 9-fold). He was treated conservatively for pancreatitis. Pleural fluid amylase was found to be very high (35,536 U mL 1). Magnetic resonance pancreatography confirmed a fistulous tract between the pancreas and the right pleural space, which had formed during 2 weeks from the time of the initial abdominal computed tomography (Figure 2). A thoracic intervention was undertaken to decorticate and drain the loculated fluid because it was persistent. Medical treatment was started in the form of total parenteral nutrition and intravenous octreotide. Endoscopic retrograde cholangiopancreatography (ERCP) and sphincterotomy, which helps in reducing ductal pressure as well as easing stent introduction, and endoprosthesis stenting were carried out (Figure 3). The stent was inserted over the assumed site of the fistula. The patient’s condition improved dramatically with resolution of his radiologic and laboratory parameters. The final diagnosis was PPF as a complication of pancreatitis. The cause of the initial pancreatitis with pseudocyst complication remains unknown. The stent was removed in the 5th week.
Figure 2. Magnetic resonance imaging demonstrating a fistula between the pancreas and the right pleural cavity. The arrow indicates the possible fistula tract.
Discussion Pleuropulmonary complications associated with pancreatitis are common. They include atelectasis, pneumonia, pleural effusion, and acute respiratory distress syndrome. In acute pancreatitis, 5%–15% of cases are complicated by pseudocyst, necrosis, abscess, or PPF. Pleural effusion is seen in 3%–17% of cases, and it indicates a worse prognosis.2 In the setting of chronic pancreatitis where history of alcoholism can be elicited, 20%–40% of patients develop pseudocysts.3 PPF is seen in less than 1% of acute pancreatitis cases but in 0.4%–4.5% of chronic pancreatitis cases. Moreover, PPF is associated with pseudocyst in 77% of cases.4 It affects the age group 20–60 years in 95% of cases. Patients usually present with respiratory rather than abdominal symptoms. Respiratory symptoms include chest pain, shortness of breath, cough, fever, and even septicemia. Abdominal symptoms are seen in 24% of cases.5,6 Pancreatic symptoms in PPF are almost never recorded.1 This creates a delay in diagnosis which requires a high index of suspicion. The average time to diagnose PPF is around 5 weeks. Pleural effusion is usually present, associated with ascites in 20% of cases; it has been reported with pericarditis in 4% of cases.6 Pleural effusion in acute pancreatitis is not uncommon, usually small, transient, and left-sided, resulting from a lymphatic or sympathetic inflammatory reaction.
Downloaded from aan.sagepub.com by guest on June 8, 2015
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Figure 3. Endoscopic retrograde cholangiopancreatography demonstrating the fistula tract (arrow).
In cases of PPF, associated effusions tend to be large and recurrent. They are seen at the left side in 76% of cases. As pancreatitis erodes intraperitoneally as well as retroperitoneally, plane dissections are created, reaching the pleura. A leaking pancreatic duct or pseudocyst can access the pleural cavity through a diaphragmatic or aortic hiatus or directly transdiaphragmatically.1,6,7 The effusion is typically exudate with a high amylase level as a hallmark, compared to serum.3 In general, the most common cause of amylase-rich effusion is malignancy; it was first reported in 1951 in association with lung cancer. Several subsequent reports associated it with ovarian or metastatic tumors, lymphoma, or leukemia.4 Others included post-coronary artery bypass, pneumonia, liver cirrhosis, TB, and hydronephrosis. Only pancreatitis-related PPF has the pancreatic-type amylase; the others have salivary-type amylase that can be produced by salivary glands, lung, or fallopian tubes, or ectopically by tumors.7,8 The pathogenesis of PPF involves a pancreatic duct stenosis or leak, which can result from trauma, surgery, ERCP, or pancreatitis.1,5 It is very difficult to demonstrate the fistula radiologically. ERCP is mandatory in such cases to document duct pathology and aid decision making.1,6 It has been reported that 80% of duct pathology and 59% of fistulae can be demonstrated. Magnetic resonance imaging has been reported to be sensitive with variable ranges.5 Some cases of mediastinal pancreatic pseudocyst have been reported wrongfully as a mediastinal mass on chest radiography.2 There are no clear guidelines for PPF management because it is a rare entity. It has been reported that 50% of PPF will close with conservative measures, with subsequent resolution of effusion. Form our literature review, we concur with the notion that management
has to be individualized according to fistula characteristics, but the work of Wronski and colleagues9 simplified the management of PPF based on categorizing the patients into 3 groups according to ERCP findings: complete stenosis, partial stenosis, or no stenosis. Location, obstruction, and extent determine the line of management. Since the first endoscopic pancreatic stenting in 1993, the role of surgery has fallen back. Such stenting reduces the intraductal pressure and seals the leak.2,5,6 Endoscopic intervention in conjunction with total parenteral nutrition, octreotide, and chest drainage constitute the medical management. It has been reported that such an approach closes the fistula in 2–3 weeks in 48% of cases, with concurrent management of the underlying cause. Pancreatic duct stents can be left for 4 weeks and up to 6 months. Limitations of stenting are a distally located fistula and completely ruptured or obstructed duct.1,5 Complications of medical management include malnutrition, central catheter infection, deep vein thrombosis, and sepsis. Surgical management is indicated in failure of medical management (3–4 weeks are considered) or obstructed pancreatic duct.1,6 Surgery includes either distal pancreatectomy or pancreaticojejunostomy. Operative mortality is 3%–5%. Recurrence has been reported in up to 11% of cases. The long-term outcome is good in 80%–95% of cases, with overall PPF mortality of 5%.1,6,7 PPF is a rare complication mainly in the setting of chronic pancreatitis. Extensive investigations should be carried out, but a delay in diagnosis may lead to sepsis. Routine pleural effusion amylase levels are not indicated from a cost-effective point of view, and management has to be individualized according to ERCP ductal pathology. Funding This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.
References 1. Wakefield S, Tutty B and Britton J. Pancreaticopleural fistula: a rare complication of chronic pancreatitis. Postgrad Med J 1996; 72: 115–116. 2. Subrt Z, Ferko A, Papı´ k Z, Vacek Z and Sedla´cek Z. A rare cause of mediastinal expansion with a massive pleural effusion. Postgrad Med J 2005; 81: e18. 3. Materne R, Vranckx P, Pauls C, Coche EE, Deprez P and Van Beers BE. Pancreaticopleural fistula: diagnosis with magnatic resonance pancreatography. Chest 2000; 117: 912–914. 4. Joseph J, Viney S, Beck P, Strange C, Sahn SA and Basran GS. A prospective study of amylase-rich pleural effusions with special reference to amylase isoenzyme analysis. Chest 1992; 102: 1455–1459.
Downloaded from aan.sagepub.com by guest on June 8, 2015
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5. Kiewiet JJ, Moret M, Blok WL, Gerhards MF and de Wit LT. Two patients with chronic pancreatitis complicated by a pancreaticopleural fistula. Case Rep Gastroenterol 2009; 3: 36–42. 6. Dhebri AR and Ferran N. Nonsurgical management of pancreaticopleural fistula. JOP 2005; 6: 152–162. 7. Williams SG, Bhupalan A, Zureikat N, et al. Pleural effusions associated with pancreaticopleural fistula. Thorax 1993; 48: 867–868.
8. Branca P, Rodriguez RM, Rogers JT, Ayo DS, Moyers JP and Light RW. Routine measurement of pleural fluid amylase in not indicted. Arch Intern Med 2001; 161: 228–232. 9. Wronski M, Slodkowski M, Cebulski W, Moronczyk D and Krasnodebski IW. Optimizing the management of pancreaticopleural fistulas. World J Gastroenterol 2011; 17: 4696–4703.
Downloaded from aan.sagepub.com by guest on June 8, 2015
