Managing recurrent portal steal in auxiliary liver transplantation for non‐cirrhotic metabolic liver disease

Background APOLT has been proposed as a treatment modality for certain types of NCMLD. While the short‐term outcomes of this operation have been comparable with orthotopic LT, its long‐term outcomes have sparsely been reported. We present one such case of Citrullinemia type I who underwent APOLT and...

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Published in:Pediatric transplantation 2022-12, Vol.26 (8), p.n/a
Main Authors: Vasudevan, Anu K., Shanmugam, Naresh P., Rammohan, Ashwin, Rela, Mohamed
Format: Article
Language:English
Subjects:
Ammonia
CTLN1
Diagnosis
Diet
Embolization
Encephalopathy
Gene therapy
Liver
Liver diseases
Liver transplantation
Liver transplants
metabolic profile
Metabolism
NCMLD
Portal vein
PVE
Seizures
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Summary:Background APOLT has been proposed as a treatment modality for certain types of NCMLD. While the short‐term outcomes of this operation have been comparable with orthotopic LT, its long‐term outcomes have sparsely been reported. We present one such case of Citrullinemia type I who underwent APOLT and developed recurrent PS. Case Report A 2‐year‐old male child with a diagnosis of Citrullinemia type I underwent APOLT with a left lateral segment from a split deceased donor liver, and his postoperative period was unremarkable. Ammonia‐lowering agents were stopped 1 week following the operation and the child was discharged home on a normal diet. Four years following APOLT, the child presented with altered sensorium and seizures. A diagnosis of PS was made. Subsequent to an embolization of the native liver's right anterior portal vein his sensorium improved and he remained clinically stable on a normal diet. Six years following the APOLT, the child again presented with features of acute encephalopathy. Imaging was suggestive of PS. A portal vein embolization of the native portal vein was performed and the child's clinical condition improved. At 6 months' follow‐up, the child remains well on a normal diet. Conclusions While the early impediments in this technique may have been overcome, in the absence of any realistic clinical application gene therapy, the debate of long‐term phenotypic metabolic correction for NCMLD by APOLT needs to be revisited.
ISSN:1397-3142
1399-3046
DOI:10.1111/petr.14389