Spontaneous rupture of pancreatic pseudocyst into the pericardial cavity with tamponade, gastro-pericardial fistula and febrile neutrophilic dermatosis (Sweet syndrome): a systematic review

Abstract

Introduction: Pancreatic pseudocysts are enzyme-rich fluid collections that form as a complication of acute or chronic pancreatitis, with an incidence of 5-16% and 20-40% respectively. Spontaneous rupture of a pancreatic pseudocyst is an exceptional complication, occurring in only 3-5% of cases. When rupture occurs toward adjacent structures, it can give rise to internal fistulas, including pancreaticopericardial fistulas (PPF), an extraordinarily rare entity with only 15 cases documented over a 40-year period. PPF can manifest with cardiac tamponade, heart failure, and shock. Sweet syndrome or febrile neutrophilic dermatosis is a rare inflammatory dermatosis that has been associated with neoplastic processes and, exceptionally, with pancreatic pathology. Objective: To synthesize the available evidence on spontaneous rupture of pancreatic pseudocyst into the pericardial cavity, gastro-pericardial fistula, and its association with febrile neutrophilic dermatosis (Sweet syndrome). Methodology: Systematic review following PRISMA 2020 guidelines (Page et al., 2021). A systematic search was conducted in PubMed, LILACS, SciELO and Cochrane for studies published between 2000 and 2026 on pancreaticopericardial fistulas, cardiac tamponade secondary to pancreatic pathology, and Sweet syndrome associated with pancreatic disease. Results: Eight relevant studies documenting the association between pancreatic pseudocysts and pericardial complications were identified. Pancreaticopericardial fistula is an extremely rare complication of chronic pancreatitis, with an unknown incidence but with only 15 cases reported in 40 years. Cardiac tamponade was present in most cases, with dyspnea as the main symptom (78%), followed by hypotension (53%), chest pain (50%), and fever (43%). Management includes pericardiocentesis, percutaneous or endoscopic drainage, and octreotide therapy, with surgery reserved for refractory cases. Sweet syndrome associated with pancreatic neoplasm is exceptional, with only one documented case of association with intraductal papillary mucinous neoplasm (IPMN). Conclusion: Rupture of pancreatic pseudocyst into the pericardial cavity is an exceptional but potentially lethal complication that requires a high index of suspicion. The association with Sweet syndrome is extremely rare and suggests a possible common pathophysiological mechanism related to systemic inflammation and neutrophilic response. Multidisciplinary management and early diagnosis are essential to reduce associated morbidity and mortality.