What’s the Connection? A 12-year-old Female with Shortness of Breath and Abdominal Pain

Introduction: Severe pancreatitis may lead to pancreatic duct defects, pseudocysts, and fistulas to the pleural space. Pancreaticopleural fistulas are a rare cause of exudative effusions that, despite a possibly impressive presentation, can be diagnostically challenging in the absence of an overt history of pancreatitis.

Case Description: A 12-year-old female presented for two days of dyspnea, right chest and lower back pain, which she developed after having two months of mid-epigastric and periumbilical pain. Physical exam was significant for substernal retractions, absent lung sounds on the right, and mild epigastric tenderness without organomegaly or Murphy’s sign. Chest x-ray showed complete opacification of right hemithorax (Figure 1). CTA chest revealed large right pleural effusion with fluid extension into the abdomen. Thoracentesis drained 1230 mL of brown, hazy, fluid with studies consistent with exudative effusion per Light’s Criteria. Cytology and cultures for bacteria, fungi and AFB were negative. Pleural effusion rapidly reaccumulated on serial imaging. CT abdomen revealed dilated pancreatic duct (1.2cm) and peripancreatic fluid collections. MRCP demonstrated a tubular structure near the pancreatic fluid collection, extending toward right hemithorax (Figure 2). Serum and pleural fluid amylase and lipase were elevated. ERCP/EUS revealed chronic pancreatitis, ansa pancreatica type C anatomy, and pancreatic duct (PD) stricture. A PD stent was placed and the patient had a video-assisted thoracoscopic surgery, decortication and multiple chest tubes with eventual resolution of her pleural effusion.

Discussion: Our patient had a large right exudative pleural effusion without infection or malignancy. Her imaging findings and elevated pleural pancreatic enzymes were consistent with a pancreaticopleural fistula (PPF), which is an extremely rare complication of pancreatitis. Of 22 pediatric case reports, many required endoscopic intervention, as did our patient. Common risk factors for pediatric chronic pancreatitis include genetic predisposition, obstruction (i.e., PD anomaly), autoimmunity, medications and metabolic abnormalities. Our patient’s variant pancreatic anatomy may have predisposed her to chronic pancreatitis, which led to the PPF. Genetic testing for hereditary chronic pancreatitis also revealed she was homozygous for an increased risk allele in SPINK1.

Conclusion: This rare case highlights the importance of a broad differential for pediatric pleural effusion. While respiratory symptoms predominated the initial presentation and the clinical history did not suggest acute pancreatitis, the co-occurrence of abdominal pain, an exudative pleural effusion, and re-accumulation of the effusion prompted consideration of chronic pancreatitis and evaluation for anatomic abnormalities. A work-up to assess for predisposition to chronic pancreatitis should include genetic evaluation, which also allows for assessment of increased risk for pancreatic cancer. The extent of this patient’s pancreatic necrosis will require serial PD stents, with possible resection and transplant long-term. This case will hopefully remind clinicians to keep a broad differential for pediatric pleural effusions.