Todani type IV-A choledochal cyst associated with heterotaxy syndrome and polysplenia: a case report".

: Introduction Choledochal cysts are rare congenital dilatations of the biliary tract that usually require surgical management in childhood. Their association with heterotaxy syndrome (situs ambiguus) and polysplenia is exceptionally uncommon and poses major diagnostic and operative challenges due to complex thoracoabdominal anomalies. Case Report A 2-year-old girl with a prenatal diagnosis of left isomerism heterotaxy syndrome underwent abdominal ultrasonography, which incidentally revealed a fusiform dilatation of the common bile duct. The patient was completely asymptomatic, with a normal physical examination. Magnetic Resonance cholangiopancreatography (MRCP) confirmed a Todani type IV-A choledochal cyst (common bile duct 31 × 17 mm with intrahepatic dilatation up to 12 mm). Extended right subcostal laparotomy revealed midline liver, right-sided polysplenia, preduodenal portal vein, and mirror image nonrotation. Complete cyst excision with preservation of the pancreatic duct was performed, followed by Roux-en-Y hepaticojejunostomy. The postoperative course was uneventful. The patient was discharged on day 7 with no complications at 6-month follow-up. Conclusion Patients with heterotaxy and polysplenia may benefit from long-term surveillance of the biliary system, since although rare, dilatation of the biliary system may develop over time.