A case of pleural effusion leading to a pancreaticojejunostomy

Abstract

Pancreaticopleural fistula (PPF) is a rare complication of chronic pancreatitis, with a described incidence rate of about 0.4% in alcoholic chronic pancreatitis. Hereditary pancreatitis (HP) is an established cause of chronic pancreatitis. It usually presents as recurrent bouts of acute abdominal pain in childhood, typically at the age of 10–12 years. We report an uncommon case of HP presenting with recurrent pleural effusions due to an underlying PPF in a young patient. A 17-year-old Maori female patient had two consecutive hospital admissions for shortness of breath and worsening exercise tolerance. She was found to have a large left-sided pleural effusion on chest imaging requiring a pleural drain on both instances. Computed tomography (CT) of the chest/upper abdomen showed an atrophic calcified pancreas with a dilated pancreatic duct prompting further abdominal imaging. Magnetic resonance cholangiopancreatography (MRCP) revealed a calcified pancreas with pronounced dilatation of pancreatic duct and a pancreaticolith measuring up to 13 mm (Fig. 1). The pleural fluid was exudative with a lipase level of 68 900 U/L raising the suspicion of an underlying PPF. No personal or family history of recurrent pancreatitis and no risk factors was found. The PPF was not identified on performed imaging. Genetic testing was conducted, confirming a diagnosis of autosomal recessive HP with SPINK1 homozygous mutation. Her PRSS1 gene was normal. She was referred to hepatobiliary surgical team and successfully underwent a Roux-en-Y pancreaticojejunostomy. Intraoperative ultrasound confirmed dilated pancreatic duct and multiple palpable parenchymal stones throughout pancreas. She had a smooth post-operative recovery. PPF most commonly presents with a recurrent leftsided pleural effusion. Diagnosis can be challenging as it requires high index of suspicion. Pleural fluid amylase level is the most important test with usual level of above 10 000 U/L (normal level <150 μ/L) and although high levels are characteristic for PPF, they are not diagnostic. Different imaging modality that can be used to identify PPF tract with varying sensitivities includes CT scan (47% sensitivity), MRCP (80% sensitivity) and endoscopic retrograde cholangiopancreatography (78% sensitivity). It can be managed conservatively, endoscopically or surgically but in the presence of large pancreaticolith, surgical intervention is the best option. HP is the most common cause of chronic pancreatitis in children. It is an autosomal dominant disorder commonly manifesting as recurrent acute pancreatitis in childhood leading to chronic pancreatitis by second or third decade of life. It commonly affects Caucasians, most of the families identified being from Europe and Northern America. Genes that have been identified to be pathogenic include serine protease inhibitor Kazal type 1 (SPINK1), cystic fibrosis transmembrane conductance regulator gene (CFTR), cationic trypsinogen protease serine 1 (PRSS1) and the CTRC. In the present case, large recurrent pleural effusions due to PPF were the only manifestation of hereditary chronic pancreatitis in an otherwise healthy young woman. Early diagnosis is important to prevent recurrent episodes of pleural effusion through appropriate endoscopic and surgical interventions. Despite high risk