Cholangitis secondary to obstructive choledocholithiases in an elderly woman with heterotaxy syndrome with polysplenia: report of a case and brief review of the literature

The present study aims to report a rare case of obstructive choledocholithiases found in an elderly female with heterotaxy syndrome (HS) with polysplenia, and to review the available literature on hepatobiliary pathologies associated with this syndrome. We present the case of an 82-year-old female patient with multiple comorbidities who presented to our hospital with abdominal pain and jaundice. An abdominal ultrasound and subsequent computed tomography showed several obstructive choledocholithiases. Incidentally, an abnormal visceral arrangement consistent with HS with polysplenia was observed, including midline liver, semi-annular pancreas, intestinal malrotation, preduodenal portal vein and other vascular anomalies. Accordingly, the patient was diagnosed with cholangitis secondary to obstructive choledocholithiases and HS with polysplenia. She was admitted to the hospital for endoscopic retrograde cholangiopancreatography after medical stabilization. Unfortunately, her condition rapidly worsened and died a few hours after hospitalization due to septic shock. Only one case of choledocholithiases in HS has been previously reported in the literature. It is unclear whether the abnormal arrangement of abdominal organs may have a significant role in the development of this complication. Although biliary drainage might be hindered in the presence of anatomical variants such as (semi-)annular pancreas, intestinal malrotation and preduodenal portal vein, further studies are needed to determine if there is an association between these anomalies and the development of choledocholithasis.