Background : Dubin-Johnson syndrome (DJS), a rare autosomal recessive liver disorder, is characterized by hyperbilirubinemia. We would like to report a case in which a left hemihepatectomy was performed on a patient with DJS presenting with a massive ruptured hepatocellular carcinoma (HCC)

Methods : A 54-year-old male who presented to the emergency room with sudden upper abdominal pain. Vital signs were stable and laboratory analysis revealed with a white blood cell count of 10,100/L, hemoglobin of 12.7 g/dL, platelets of 178,000/L, total bilirubin (TB) of 2.45 mg/dL, direct bilirubin (DB) of 1.26 mg/dL, AST of 42 U/L, ALT of 20 U/L, and PT of 1.24 INR. Imaging studies demonstrated a 13cm ruptured mass in the left hemiliver, prompting emergency angiography and subsequent embolization with gelfoam. Post-procedure, the patient exhibited fluctuating bilirubin levels (TB:2.5~10.7), eventually stabilizing at TB 2.5~3.5. Follow-up MRI revealed a necrotic, but partially viable tumor in the left hemiliver without cirrhosis, biliary problem, or metastatic findings.

Results : Considering the elevated TB levels observed in past health screenings, a suspicion of DJS, guiding the decision to proceed with left hemihepatectomy. Operative findings included a black liver color and omental adhesions around the ruptured tumor area. Postoperatively, the patient's peak TB levels was 5.5 but subsequently decreased. Pathological examination confirmed an 11x10cm HCC and DJS findings in the non-tumor liver area. The patient was discharged on the 12th hospital day without complications.

Conclusions : This case emphasizes the importance of considering DJS in cases of conjugated hyperbilirubinemia and the successful performance of hepatectomy for ruptured HCC.