Clinical Gastroenterology and Hepatology
Volume 8, Issue 2 , Pages e18-e19, February 2010

Pancreatic Cystosis Complicating Cystic Fibrosis

  • Alvin J. Freeman

      Affiliations

    • Division of Pediatric Gastroenterology, University of Mississippi Medical Center, Jackson, Mississippi
    • ,
  • Henry W. Giles

      Affiliations

    • Department of Radiology, University of Mississippi Medical Center, Jackson, Mississippi
    • ,
  • Michael J. Nowicki

      Affiliations

    • Division of Pediatric Gastroenterology, University of Mississippi Medical Center, Jackson, Mississippi

published online 05 October 2009.

Article Outline

 

A 16-year-old African American boy with cystic fibrosis presented with a 3-day history of abdominal pain, constipation, and increased cough. The pain was poorly characterized, diffuse, and continuous, without exacerbating or relieving factors. He denied nausea and vomiting, but did report recent onset of anorexia with a 4-pound weight loss. He reported worsening constipation, with no bowel movement for 3 days despite increasing doses of milk of magnesia. Past medical history was significant for cystic fibrosis, pancreatic insufficiency, pulmonary disease, and severe constipation. Past surgical history revealed no abdominal surgeries. Medications included pancreatic enzyme supplements, proton-pump inhibitor, β-agonist, fat-soluble vitamin supplements, dornase alpha, and inhaled tobramycin. Physical examination was significant for diffuse abdominal tenderness to light palpation; no hepatosplenomegaly or masses were felt. Pulmonary examination revealed poor respiratory effort but clear breath sounds.

Evaluation included an abdominal ultrasound, which revealed a complex cystic mass with mild calcification arising at the root of the mesentery; the pancreas was not visualized (Figure A). There was normal sonographic appearance of the liver, kidneys, and splenic parenchyma. A magnetic resonance imaging study was performed to better delineate the mass.

Axial T1-weighted magnetic resonance imaging showed multiple low-signal, varying sized cysts in the expected region of the pancreas (Figure B); Coronal T2-weighted magnetic resonance imaging showed multiple bright-signal, varying sized cysts in the expected region of the pancreas (Figure C). The cystic mass displaced the portal venous structures and the superior mesenteric artery anteriorly. No pancreatic tissue was identified along the splenic vein or adjacent to the duodenum, which was displaced posteriorly by the mass.

Pancreatic cystosis is a rare condition typically seen in cystic fibrosis during the second decade of life. Presenting complaints include abdominal pain, postprandial fullness, and nausea; asymptomatic cases have been reported, discovered by a palpable mass on examination.1 Symptoms have been attributed to mass effect and/or hemorrhage into the cysts.2

Although microscopic pancreatic cysts are common in cystic fibrosis, pancreatic cystosis results in large cysts ranging from 1.5 to 12 cm.3 The cysts result from maintained secretory function despite ductal obstruction. Low levels of α-1-antitrypsin and high levels of immunoreactive trypsin suggest that an autodigestive process is not present.1 Histologically pancreatic cystosis is a true cystic lesion lined by epithelium, with little to no pancreatic tissue present between the cysts. Differential diagnosis includes lymphangioma, mesenteric teratoma, and multiple pancreatic serous cystadenoma. Multiple pancreatic serous cystadenomas have been described in von Hippel–Lindau syndrome and rarely are found in patients unaffected by this syndrome.4 Multiple serous cystadenoma may be differentiated from pancreatic cystosis by the findings of small cysts (<2 cm), calcification, and/or a central stellate scar.4

Treatment must be individualized to the patient because there is a paucity of data from which to make broad recommendations. Reports from the literature include providing symptomatic treatment to surgical resection, depending on severity and duration of symptoms.

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References 

  1. Hernanz-Schulman M, Teele RL, Pere-Atayde A, et al. Pancreatic cystosis in cystic fibrosis. Radiology. 1986;158:629–631
  2. van Rijn R, Schilte P, Wiarda B, et al. Pancreatic cystosis. Radiology. 2007;243:598–602
  3. Cahill ME, Parmentier JM, van Ruyssevelt C, et al. Pancreatic cystosis in cystic fibrosis. Abdom Imaging. 1997;22:313–314
  4. Leonard D, Baulieux J, Rode A, et al. Multiple synchronous serous cystadenomas of the pancreas: uncommon CT and MRI findings. J Hepatobiliary Pancreat Surg. 2007;14:600–603

 Conflicts of interest The authors disclose no conflicts.

PII: S1542-3565(09)00995-1

doi:10.1016/j.cgh.2009.09.027

Clinical Gastroenterology and Hepatology
Volume 8, Issue 2 , Pages e18-e19, February 2010