A Japanese woman with a history of Kasai operation for biliary atresia had living-donor liver transplantation at the age of 22. The first episode of refractory HE and late cellular rejection was treated by a high dose of methylprednisolone. The second episode of refractory HE was treated by balloon-occluded retrograde transvenous obliteration for a spleno-renal shunt. However, the third episode of refractory HE occurred 11 years after liver transplantation. The liver cirrhosis and hypersplenism were present with a Child–Pugh score of C-10. Although portal vein flow was hepatopetal, superior mesenteric vein flow regurgitated. We performed proximal total splenic artery embolization (TSAE). Superior mesenteric vein flow changed to a hepatopetal direction and she became clear. At a year after proximal TSAE, her spleen volume had decreased to 589 mL (20% decrease) on computed tomography. She is well and has a Child–Pugh score of 8 without overt HE. We report the first case of refractory HE treated by proximal TSAE that is a possible less invasive treatment option for a selected patient.
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