NDLI: Living donor liver transplantation for idiopathic portal hypertension with extrahepatic portal vein stenosis and splenic artery aneurysms: a case report and review of the literature

Content Provider	Springer Nature : BioMed Central
Author	Kawachi, Shigeyuki Chiba, Naokazu Nakagawa, Masashi Kobayashi, Toshimichi Hikita, Kosuke Sano, Toru Tomita, Koichi Hirano, Hiroshi Abe, Yuta Obara, Hideaki Shimazu, Motohide
Abstract	Background Idiopathic portal hypertension (IPH) generally has a good prognosis and rarely results in liver transplantation. Furthermore, there are few reports of living donor liver transplantation (LDLT) for IPH with extrahepatic portal vein stenosis. Case presentation We report the case of a 51-year-old female patient diagnosed with IPH more than 20 years ago. She suffered severe jaundice, massive ascites, and encephalopathy at the time of her visit to our hospital. The patient’s extrahepatic portal vein showed a scar-like stenosis, and the portal flow was completely hepatofugal. Collateral circulation such as the splenorenal shunt was well developed, and multiple splenic artery aneurysms up to 2 cm were observed in the splenic hilum. Her Model for End-Stage Liver Disease score increased to over 40 because of renal dysfunction, requiring temporary dialysis. We performed LDLT using her husband’s right lobe graft and splenectomy. The extrahepatic stenotic portal vein was completely resected, and the superficial femoral vein (SFV) graft collected from the recipient’s right leg was used for portal reconstruction as an interposition graft. Although the clinical course after LDLT had many complications, the patient was discharged on postoperative day 113 and has been fine for 2 years after LDLT. Histopathologically, the explanted liver had obliterative portal venopathy, nodular regenerative hyperplasia, and incomplete septal cirrhosis. Conclusion This case showed that severe IPH is occasionally associated with extrahepatic portal vein stenosis and can be treated with LDLT with portal vein reconstruction using an interposition graft. It was also suggested that the SFV is a useful choice for the interposition graft.
Related Links	https://bmcsurg.biomedcentral.com/counter/pdf/10.1186/s12893-020-00921-6.pdf
Ending Page	6
Page Count	6
Starting Page	1
File Format	HTM / HTML
ISSN	14712482
DOI	10.1186/s12893-020-00921-6
Journal	BMC Surgery
Issue Number	1
Volume Number	20
Language	English
Publisher	BioMed Central
Publisher Date	2020-10-29
Access Restriction	Open
Subject Keyword	Surgery Internal Medicine Idiopathic portal hypertension Extrahepatic portal vein stenosis Living donor liver transplantation Splenic artery aneurysms Superficial femoral vein graft
Content Type	Text
Resource Type	Case study
Subject	Surgery
Journal Impact Factor	1.6/2023
5-Year Journal Impact Factor	1.9/2023

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