Introduction : Situs ambiguous known as heterotaxy syndrome or isomerism can be classified into situs ambiguous with polysplenia or asplenia. Situs ambiguous with asplenia is a second subcategory of situs ambiguous that is generally characterized by an abnormal arrangement of the abdominal organs and absence of the spleen. Laparoscopic cholecystectomy for acute cholecystitis in patient with this disorder is challenging.

Methods : A 19-year-old female patient was a situs ambiguous with asplenia patient with transverse liver with gallbladder, left-side stomach, congenital heart disease, and right isomerism. This patient underwent Fontan operation at 4 years of age due to double outlet right ventricle with ventricular septal defect and dextrocardia. This patient had undergone heart transplantation and re-heart transplantation one year ago. Six months ago, Acute pancreatitis with peripancreatic infiltration and gall bladder stone with common bile duct stone were identified on MRCP. We determined Laparoscopic cholecystectomy after ERCP through multidiscipline care.

Results : We performed laparoscopic cholecystectomy for these patients with 4-port insertion. The operation time was 59 minutes and the operation was completed without any special event. The patient went on a sips of water and soft diet on POD 1 and was discharged without complications on POD 2. The pathologic result was chronic cholecystitis.

Conclusions : Laparoscopic treatment is a feasible option for selective patients with these disease entity. Surgeons need to be careful because of reversed anatomy, and unaccustomed working hand.