Pediatric Rapunzel Syndrome Causing Pancreatitis and Cholangitis with Unique MRI Findings
Introduction: Gastrointestinal bezoars can cause unfortunate complications. This is a novel pediatric case of Rapunzel syndrome causing pancreatitis and progressing to cholangitis.
Case Description: An 11 year old malnourished female presented with persistent nausea, emesis, anorexia, and abdominal pain for weeks. She was dehydrated, ill-appearing, and had epigastric tenderness. Labs showed an elevated lipase, mild transaminitis, and a normal total bilirubin. A dilated common bile duct measuring 11.4mm (normal <4mm) was seen on abdominal ultrasound, but no signs of stones or pancreatic inflammation. Despite pain control, NPO status, and IV hydration her pancreatitis symptoms did not improve prompting a magnetic resonance cholangiopancreatography (MRCP) for possible obstruction.
The MRCP confirmed biliary duct dilation, without evidence of choledocholithiasis, and identified a non-specific “non-layering low signal void gastric substance” [Figure 1]. This surprisingly well circumscribed gastric substance coupled with the “fluid-filled loops of dilated small bowel without distinct transition” seen on MRI, increased suspicions of an obstructing mass.
Post-MRCP, the patient became septic with fever, abdominal distention, and peritonitis. With these symptoms, biliary duct dilation, increasing bilirubinemia and blood culture positive for E.coli, her illness had progressed to cholangitis. She underwent an endoscopic retrograde cholangiopancreatography, and was emergently taken for an exploratory laparotomy. Her common bile duct was found to be obstructed from a bezoar extending from the gastric antrum to the terminal ileum [Figure 2] with multiple areas of necrosis and perforation.
Discussion: A bezoar, a mass of indigestible exogenous materials, can accumulate within the gastrointestinal tract and cause abdominal pain, nausea, vomiting, anorexia, and weight loss. Though majority of bezoars are gastric, they can migrate to and accumulate in the small intestines. As bezoars grow, complications arise such as mucosal erosion/ulceration/perforation, pancreatitis, small bowel obstruction, and obstructive jaundice.
Bezoars are most commonly identified on computed tomography and have heterogenous material-like appearance. Since this patient’s workup focused on an intrabiliary source of pancreatitis, an ultrasound and MRCP were done instead. The appearance of a bezoar on MRCP is not well established, which made this patient’s bezoar difficult to conclusively diagnose on MRI.
Conclusion: While there is one documented case of a bezoar leading to cholangitis in an adult, there is no documented pediatric case. Based on this patient’s case, when clinical signs of pancreatitis or cholangitis arise in the absence of an obstructing stone, consider other masses, such as a bezoar. If an MRI is obtained for a patient with acute-on-chronic abdominal pain, signal voids circumscribed by fluid in the stomach and/or intestines raise suspicions for a mass. This novel case with these distinct MRI findings may help clinicians make quicker diagnoses of bezoars, and sheds light on the use of MRI in identifying such masses.
