Atypical Presentation of Acute Pancreatitis in a Man with Pancreatic Insufficiency and Cystic Fibrosis: A Case Report

Malcolm Turner; Hugh Jackson; Robin Harle; Rob Bohmer; David W Reid

Disclosures

J Med Case Reports. 2010;4(298) 

In This Article

Case Presentation

A 52-year-old Australian Caucasian male with cystic fibrosis was admitted to our hospital with an exacerbation of pulmonary sepsis accompanied by a vague abdominal pain. Abdominal X-ray revealed faecal loading in his caecum and ascending colon with proximal small bowel dilatation consistent with meconium ileus equivalent. His relevant medical history consisted of pancreatic insufficiency and bronchiectasis with moderately severe lung function impairment (FEV1 2.18 L/s; 53% predicted). He had multiple hospital admissions over the preceding two years with exacerbations of chronic airway sepsis.

The diagnosis of cystic fibrosis had been made during his early childhood when he presented with failure to thrive, and this had been confirmed with an elevated sweat test and genotyping that revealed him to be a heterozygote for G542X, with the other allele unidentified. G542X is a Class I mutation that results in the complete failure to synthesize functional cystic fibrosis transmembrane conductance regulator (CFTR) and is usually associated with pancreatic insufficiency. On this particular admission, his chest and abdominal symptoms resolved after a course of intravenous antibiotics, fluid replacement, and oral administration of N-acetyl cysteine. His 24-hour fecal fat levels were elevated at 41 grams and his pancreatic enzymes were thus increased.

He presented again after six weeks due to a recurrence of severe abdominal pain, anorexia and weight loss, but without any alteration to his bowel habit. Examination revealed epigastric tenderness and active bowel sounds, but no guarding. Results of respiratory examination were unchanged. On this occasion, abdominal X-ray was normal, as were his full blood count, urea and electrolytes, serum amylase (46 IU/L; NR < 100 IU/L), and liver function tests. However, he continued to complain of severe abdominal pain radiating through his back and lower chest. An abdominal ultrasound demonstrated an increased echogenicity consistent with inspissated secretions in the pancreatic duct and an 8 mm common bile duct with no other abnormalities.

Two days after admission, our patient became jaundiced. Repeat blood tests demonstrated a cholestatic picture: total protein 66 g/L, albumin 36 g/L, alkaline phosphates (ALP) 809IU/L, alanine transaminase (ALT) 543IU/L, glutamyl transaminase (GGT) 334IU/L, and bilirubin 23 mmol/L. His serum amylase (42IU/L) and lipase (4IU/L; NR: < 10IU/L), as well as urinary lipase (96IU/L, NR: < 500IU/L) levels remained normal. An abdominal computed tomography (CT) scan demonstrated that he had a heterogeneous mass measuring 5×5×5 cm and located at the head of the pancreas with biliary and pancreatic duct dilation (Figure 1). Magnetic resonance cholangiopancreatography confirmed the enlargement of the head of his pancreas with dilated intrahepatic and extrahepatic biliary ducts. These findings were thought to be consistent with either acute pancreatitis or a pancreatic malignancy.

Figure 1.

Computed tomography scan demonstrating mass at the head of the pancreas.

Results of his liver function tests remained abnormal (ALP 1085IU/L, ALT 249IU/L, GGT 383IU/L, and bilirubin 37 μmol/L) and the patient proceeded to endoscopic retrograde cholangiopancreatography, where a narrowing of the common bile duct at the head of the pancreas was identified with more distal anatomical distortion of the entire pancreatic ductal system. A biliary stent was consequently inserted. CT guided biopsy of the pancreatic mass demonstrated reactive pancreatic ductal epithelium with an infiltrate of macrophages and lymphocytes, but no evidence of malignancy was found.

The patient continued to experience abdominal pain and ursodeoxycholic acid was introduced to treat any potential contribution of biliary sludging to cholestasis and also to minimize the risk of stent occlusion. Following stent insertion and commencement of ursodeoxycholic acid, his symptoms and liver function tests slowly improved, and then returned to normal over the next 6 weeks. A repeat CT scan showed a resolution of biliary dilation, but no change in the pancreatic mass was noted. After 18 months he remains well with normal liver function tests and no abdominal pain. A repeat CT scan at this time demonstrated a complete resolution of the pancreatic mass (Figure 2).

Figure 2.

Computed tomography scan at 18 months showing resolution of the pancreatic inflammatory mass.

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