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
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...
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doaj-ca58872cb6e34123a3715500236e707a2020-11-25T04:02:07ZengBMCBMC Surgery1471-24822020-10-012011610.1186/s12893-020-00921-6Living donor liver transplantation for idiopathic portal hypertension with extrahepatic portal vein stenosis and splenic artery aneurysms: a case report and review of the literatureShigeyuki Kawachi0Naokazu Chiba1Masashi Nakagawa2Toshimichi Kobayashi3Kosuke Hikita4Toru Sano5Koichi Tomita6Hiroshi Hirano7Yuta Abe8Hideaki Obara9Motohide Shimazu10Department of Digestive and Transplantation Surgery, Tokyo Medical University Hachioji Medical CenterDepartment of Digestive and Transplantation Surgery, Tokyo Medical University Hachioji Medical CenterDepartment of Digestive and Transplantation Surgery, Tokyo Medical University Hachioji Medical CenterDepartment of Digestive and Transplantation Surgery, Tokyo Medical University Hachioji Medical CenterDepartment of Digestive and Transplantation Surgery, Tokyo Medical University Hachioji Medical CenterDepartment of Digestive and Transplantation Surgery, Tokyo Medical University Hachioji Medical CenterDepartment of Digestive and Transplantation Surgery, Tokyo Medical University Hachioji Medical CenterDepartmet of Diagnostic Pathology, Tokyo Medical University Hachioji Medical CenterDepartment of Surgery, Keio University School of MedicineDepartment of Surgery, Keio University School of MedicineDepartment of Digestive and Transplantation Surgery, Tokyo Medical University Hachioji Medical CenterAbstract 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.http://link.springer.com/article/10.1186/s12893-020-00921-6Idiopathic portal hypertensionExtrahepatic portal vein stenosisLiving donor liver transplantationSplenic artery aneurysmsSuperficial femoral vein graft |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Shigeyuki Kawachi Naokazu Chiba Masashi Nakagawa Toshimichi Kobayashi Kosuke Hikita Toru Sano Koichi Tomita Hiroshi Hirano Yuta Abe Hideaki Obara Motohide Shimazu |
spellingShingle |
Shigeyuki Kawachi Naokazu Chiba Masashi Nakagawa Toshimichi Kobayashi Kosuke Hikita Toru Sano Koichi Tomita Hiroshi Hirano Yuta Abe Hideaki Obara Motohide Shimazu 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 BMC Surgery Idiopathic portal hypertension Extrahepatic portal vein stenosis Living donor liver transplantation Splenic artery aneurysms Superficial femoral vein graft |
author_facet |
Shigeyuki Kawachi Naokazu Chiba Masashi Nakagawa Toshimichi Kobayashi Kosuke Hikita Toru Sano Koichi Tomita Hiroshi Hirano Yuta Abe Hideaki Obara Motohide Shimazu |
author_sort |
Shigeyuki Kawachi |
title |
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 |
title_short |
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 |
title_full |
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 |
title_fullStr |
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 |
title_full_unstemmed |
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 |
title_sort |
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 |
publisher |
BMC |
series |
BMC Surgery |
issn |
1471-2482 |
publishDate |
2020-10-01 |
description |
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. |
topic |
Idiopathic portal hypertension Extrahepatic portal vein stenosis Living donor liver transplantation Splenic artery aneurysms Superficial femoral vein graft |
url |
http://link.springer.com/article/10.1186/s12893-020-00921-6 |
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