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Liver transplantation (LT), especially auxiliary partial LT (APOLT) is an excellent treatment option for patients with propionic academia (PA). It avoids the demerits and morbidity associated with long-term dietary modifications. Portal steal however, remains a major issue with APOLT, and is one of the reasons for it not being more widely accepted. We report the case of a child with PA who underwent APOLT. Nine months, following the transplant, he had an episode of acute cellular rejection resulting in metabolic decompensation. This was diagnosed to be due to portal steal as a consequence of a rejection induced stiff graft, causing preferential portal blood flow to the native liver. A simple radiological segmental native portal vein embolization caused compensatory hypertrophy of the graft, leading to a complete recovery of the child. While it does remain a major concern, as shown by us portal steal phenomenon can be managed effectively in APOLT for metabolic liver diseases. There is, however the need for long term surveillance, allowing for an early diagnosis of portal steal, should it occur. This article is protected by copyright. All rights reserved.
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