Purpose: Heterotaxy is a rare congenital anomaly that describes abnormal symmetry of the thoracic and abdominal organs and vasculature. Heterotaxy includes anomalies such as situs inversus and situs ambiguous. We report a rare case of recurrent acute pancreatitis in which the clinical picture is complicated by underlying situs ambiguous. Case Report: A 67-year-old woman was admitted with acute abdominal pain and vomiting. Clinical presentation, laboratory values and imaging with CAT scan revealed acute pancreatitis as well as incidental situs ambiguous, polysplenia, and intestinal malrotation. Abdominal ultrasound did not demonstrate any evidence of cholecystitis or cholelithiasis. Further diagnostic workup revealed normal serum triglyceride levels, ANA, and IgG4 levels. All medications were reviewed and medication-induced pancreatitis was ruled out. Patient was treated with intravenous fluids and electrolyte supplementation and discharged home. She presented back one month later with the recurrent abdominal pain. At this time, a CAT scan of the abdomen and pelvis revealed worsening pancreatitis with extensive pararenal phlegmon and pancreatic necrosis. MRCP did not reveal any intrahepatic or extrahepatic biliary ductal dilatation, and no pancreatic ductal abnormalities were noted. Patient was managed conservatively with intravenous fluids and electrolyte supplementation and recovered uneventfully. Conclusion: Recurrent acute pancreatitis has not been reported in the literature in a patient with situs ambiguous. We present a rare case of an adult patient with situs ambiguous, polysplenia, and pancreatitis without any duodenal or biliary malformations directly contributory to her pancreatitis. Our case describes a common clinical problem presenting in the setting of a rare anatomical anomaly. Any patient with heterotaxy/polysplenia syndrome with pancreatitis should be aggressively investigated for structural and biochemical abnormalities that could potentially contribute to pancreatitis.Figure: No Caption available.
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