Case Report

A rare cause of haemoptysis S. C. Loof1, M. D. van Borsel2, G. F. Joos1, J. P. van Meerbeeck1,3 1

Department of Respiratory Medicine, Ghent University Hospital, Ghent, Belgium, 2Department of Radiology, Ghent University Hospital, Ghent, Belgium, 3Thoracic Oncology, MOCA, Antwerp University Hospital, Antwerp, Belgium A 59-year-old man was admitted with haemoptysis, several months after a car accident. A diagnostic workup including laboratory testing, chest radiograph and bronchoscopy could not explain the haemoptysis. A pancreatic-pulmonary fistula was suspected by additional CT scan. Magnetic resonance cholangiopancreaticography confirmed the diagnosis, followed by surgical exploration and repair.

Keywords: Haemoptysis, Pancreatic, Fistula

Introduction Haemoptysis is caused by airway disease, pulmonary parenchymal disease or pulmonary vascular disease. Even small amounts of haemoptysis are alarming and can represent serious pathology. We report a case of a 59-year-old-man with progressive haemoptysis of uncommon aetiology: a post-traumatic pancreaticpulmonary fistula. Pancreatic-pleural fistula is a complication of pancreatitis and it usually presents as a large pleural effusion and therefore respiratory symptoms. The traumatic origin in this case makes it unique. We review the literature on the causes and management of pancreatic-pleural fistulae.

Case Report A 59-year-old man was admitted to the Emergency Unit with complaints of progressive haemoptysis and respiratory-related stabbing pain at the left hemithorax, radiating to the left side of his neck. The patient had been involved in a car accident five months ago, which resulted in an abdominal polytrauma, requiring a splenectomy. Because of an inflammatory lesion, the pancreatic tail needed an external drainage for 3 weeks, followed by granulation of the tissue with wicks. The patient had a history of stenting of the arteria iliaca communis 8 years ago necessitating a lifelong treatment with clopidogrel because of aspirin intolerance. The patient is an active smoker, of 25 pack years. There is no history of alcohol abuse. About three months after the accident, the patient developed symptoms of severe coughing and haemoptysis, both more pronounced in the morning or when Correspondence to: S. C. Loof, Department of Respiratory Medicine, Ghent University Hospital, De Pintelaan 185, 9000 Ghent, Belgium. Email: [email protected]

ß Acta Clinica Belgica 2014 DOI 10.1179/0001551213Z.0000000007

lying down. The haemoptysis was initially limited, yet increased progressively. He also had multiple episodes of fever. These complaints were first treated as a pleuropneumonia; however, there was little improvement of the symptoms despite prolonged antibiotic treatment with clindamycin and clarithromycin. Patient suffered a weight loss of 13 kg since the accident, corresponding to a reduction in body mass index from 26.0 kg/m2 to 21.8 kg/m2. The physical examination revealed a patient in good general shape. Lung auscultation gave basal crepitations on both sides, however more pronounced left than right. Abdominal examination showed a large incisional hernia and a non-tense, painless abdomen. Laboratory tests at admission showed a high inflammatory component: 70 mm/h (normal value ,28 mm/h), a C-reactive protein level of 23.8 mg/dl (,5 mg/dl) and significant leukocytosis at 33 500/ml (3650–9300/ml) with neutrophilic shift. Hematocrit level was 31.0%. Patient furthermore had elevated pancreatic lipases 415U/l (0–60), but not amylases. D-dimers and cardiac enzymes were negative. Bacteriological analysis of expectorated sputum showed the presence of Candida non-glabrata, considered colonization. The chest radiograph showed a pleural effusion and parenchymal consolidation in the left lower lobe, suspicious for a bronchopneumonic infiltrate (Fig. 1).

Differential Diagnosis Haemoptysis is an alarming symptom and often indicates serious underlying disease. In the differential diagnosis of haemoptysis causes are bronchitis, bronchiectasis, pneumonia, lung abscess, tuberculosis or lung cancer. Any patient with novel haemoptysis should be evaluated by chest X-ray, complemented with CT scan or bronchoscopy.1

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Figure 1 Chest X-ray shows a pleural effusion and parenchymal consolidation in the left lower lobe.

Since in this case no clear explanation for the haemoptysis was found and as the patient was an active smoker, a bronchoscopy was indeed performed. This showed traces of blood on the vocal cords, extending to the left lower lobe. An active bleeding site or endobronchial tumour was not visualized. A cardiologic exam by electrocardiogram and echocardiography could neither explain the chest pain. In addition, an angio CT scan of the thorax was performed as the character of the pleuritic chest pain raised suspicion of pulmonary embolism but was negative. What was noticed on the CT scan were bibasal consolidations with air bronchogram (Fig. 2). In addition, upper abdominal slices of the CT scan showed a collection in the splenectomy region, with enhanced edges under the left diaphragm dome. There was a suspicion of a fistula through the diaphragm into the left lung base and of secondary necrosis of the lung parenchyma. The subdiaphragmatic collection extended up to the pancreatic tail, suggesting a pancreatic-pulmonary fistula as cause of the haemoptysis and pleural pain. At thoracotomy, an inflammatory mass was found in the left lower lobe, adherent to the left hemidiaphragm and in continuity with a subdiaphragmatic abscess which required external drainage. The lower part of the left lobe was necrotic and therefore removed (wedge resection), and the diaphragm restored by primary closure. The procedure was uncomplicated, however postoperative a new sub-

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diaphragmatic collection developed, despite external drainage. The fluid from this collection tested positive for lipase (99560U/L). Because of the suspicion on CT scan of a persisting pancreatic fistula originating from the pancreatic tail into the subdiaphragmatic collection and even further to the left costodiaphragmatic recess, a magnetic resonance cholangiopancreaticography was carried out, which confirmed the presence of a persisting pancreatic-pulmonary fistula (Fig. 3). A distal pancreatic resection was done in combination with a closing of the fistula and external drainage of the collection, three weeks after thoracotomy. Pathological findings of the resected piece indicated very limited chronic pancreatitis with focal granulation tissue. The patient made a full recovery, was discharged and remained well since.

Discussion A literature search using the PubMed database with the keywords ‘haemoptysis’, ‘pancreatic fistula’, ‘pancreatic-pulmonary fistula’ and ‘pancreatico-pulmonary fistula’ reveals three previous case reports of a pancreatic-pulmonary fistula, with necrosis of the lung parenchyma. This concerns one patient in Slovakia and two patients in Italy, one of which had a chronic pancreatitis.2 No further English information is available. Anyhow, there are multiple case reports of pancreatic-bronchial and pancreatic-pleural fistulae, as rare complications of chronic and severe pancrea-

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Figure 2 Computed tomography on admission. The upper slices (axonal section) show necrosis of the lung parenchyma in the left lower lobe. The lower slices (coronal section) show a collection subdiaphragmatic with suspicion of break through the diaphragm, based on the presence of air in both the collection as the infiltrate.

titis and presenting with haemoptysis as a recurrent symptom, besides dyspnea, cough and chest pain. Abdominal symptoms are often absent. Because these clinical manifestations are misleading, many patients go through extensive pulmonary evaluation before the pancreas can be identified as the focus of primary pathology. Delay in the diagnosis of a pancreaticpulmonary fistula is a critical issue. The symptoms of this patient were also first treated as a pleuropneumonia; there was a delay of 3 months to the correct diagnosis. Our patient did not have pancreatitis, the fistula arose-post-traumatic which makes this case unique. The pathofyiosology is the same, pancreatic enzymes drain directly into the main bronchi, the pleural cavity or in this case the lung parenchyma. The diagnosis is usually made by CT scan or endoscopic retrograde cholangiopancreaticography

(ERCP).3 ERCP leads to the diagnosis in 80% and demonstrates the fistulous tract in 59% of the cases. Ali et al. conclude that MRCP is superior to CT scan or ERCP for the diagnosis of a pancreatic fistula.4 The use of secretin in MRCP improves the visualization of the pancreatic ducts, by increasing the pancreatic exocrine secretions and temporary dilatation of the ducts. Secretin-enhanced MRCP can probably parallel ERCP in delineating pancreatic ductal injuries5. However, secretin is an expensive product and it is very rarely used in our medical center. Therapeutic recommendations concerning pancreatic-pleural fistulae are based on case reports. There are no clinical trials given the rare occurrence of this entity. Currently there is a preference for thoracocentesis or ERCP guided stenting with or without association of a somatostatin analogue6,7.

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Figure 3 Magnetic resonance images showing the fistula originating from the pancreatic tail (A), continuing to the subdiaphragmatic collection (B, C) and even further through the diaphragm into the left lung base (D).

In a case report of Safadi et al. a successful intervention with ERCP guided stenting is described. The authors conclude that, since usually there is a disruption of the main pancreatic duct, stenting is the treatment of first choice, whereby the stent has to be placed so as to bridge the site of disruption8. Dhebri et al. agree that this is first-line therapy, although they notice that ERCP guided stenting may not be possible in patients in whom the fistula arises from the tail of the pancreas6. Immediate surgical intervention was preferred for our patient, because of the amount of haemoptysis and the clear destruction of lung parenchyma, leading to a worsening of his general condition. A wedge resection was performed in combination with clearing out the subdiaphragmatic abscess and restoring the diaphragm. Ideally, good imaging of the fistula had to be performed in advance of the initial surgery and the pancreas tail had to be removed in the same time. Initially there was some uncertainty about the fistula as aetiology of the parenchyma destruction, therefore the pancreas tail resection was only performed three weeks later, as the patient developed a new subdiaphragmatic abscess. Adequate, non-invasive imaging of the pancreas fistula was carried out before this second operation. In this case the fistula was definitely originating from the atrophic pancreas tail, although it was not possible to identify a clear disruption of the pancreatic duct by MRCP. An ERCP could have

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given additional information, but it was not performed as ERCP guided stenting in this particular case would have been very difficult. Alternative therapeutic management by means of thoracocentesis and somatostatin analogue in this context was not a good option because of the total destruction of the lung parenchyma in the left lower lobe, which had to be removed. Most case reports in which this therapeutic approach has achieved a good outcome, were patients reported with pleural effusion but without lung parenchyma destruction. Association of octeotride with any kind of therapy (thoracocentesis, ERCP guided stenting or surgery) may accelerate the recovery of the patient. Again, these are no hard data, but repeated findings in case reports. Octeotride is a long-acting synthetic somatostatin analogue that can be administered subcutaneous because of its long half-life. The aim of this medical treatment is to reduce stimulation of the pancreatic exocrine function, leading to a decrease in production of the fistula and shortening of the closing time9. Conservative therapy (medical treatment or ERCP guided stenting) fails in almost 50% of the described cases, necessitating surgical intervention later on. Since there is almost no literature concerning traumatic pancreatic-pulmonary fistulae, there are no diagnostic or therapeutic recommendations. We presume that the recommendations for pancreatic-

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pleural fistulae due to pancreatitis, named above, can be followed. The clinical manifestations are often misleading. Early diagnosis and intervention is necessary to prevent further destruction of the lung parenchyma caused by pancreatic enzymes and superinfection.

Acknowledgements The authors declare that they have no competing financial interests.

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A rare cause of haemoptysis

2 Matejicek E, Kraus L, Szabo Nacko J. A pancreatic-pleuralpulmonary fistula as a complication of chronic pancreatitis. Rozhl Chir. 1990;69:65–70. 3 Yasuda T, Ueda T, Fujino Y, Matsumoto I, Nakajima T, Sawa H, et al. Pancreaticobronchial fistula associated with chronic pancreatitis: Report of a case. Surg Today. 2007;37:338–41. 4 Ali LT, Srinivasan N, Le V, Chimpiri AR, Tierney WM. Pancreaticopleural fistula. Pancreas 2009;38:26–31. 5 Bashin DK, Rana S, Rawal P. Endoscopic retrograde pancreatography in pancreatic trauma: need to break the mental barrier. J Gastroenterol Hepatol. 2009;24: 720–728. 6 Dhebri AR, Ferran N. Nonsurgical management of pancreaticopleural fistula. J Pancreas. 2005;6(2):152–161. 7 King JC, Reber HA, Shiraga S, Hines OJ. Pancreatic-pleural fistula is best managed by early operative intervention. Surgery. 2010;147(1):154–9. 8 Safadi BY, Marks JM. Pancreatic-pleural fistula: the role of ERCP in diagnosis and treatment. Gastrointest Endosc. 2000;51(2):213–215. 9 Rockey DC, Cello JP. Pancreaticopleural fistula. Report of 7 patients and review of the literature. Medicine (Baltimore). 1990;69(6):332–344.

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A rare cause of haemoptysis.

A 59-year-old man was admitted with haemoptysis, several months after a car accident. A diagnostic work-up including laboratory testing, chest radiogr...
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