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

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. We report the case of a 51-year-old female patient diagnose...

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Veröffentlicht in:BMC surgery 2020-10, Vol.20 (1), p.257-257, Article 257
Hauptverfasser: Kawachi, Shigeyuki, Chiba, Naokazu, Nakagawa, Masashi, Kobayashi, Toshimichi, Hikita, Kosuke, Sano, Toru, Tomita, Koichi, Hirano, Hiroshi, Abe, Yuta, Obara, Hideaki, Shimazu, Motohide
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container_issue 1
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container_title BMC surgery
container_volume 20
creator Kawachi, Shigeyuki
Chiba, Naokazu
Nakagawa, Masashi
Kobayashi, Toshimichi
Hikita, Kosuke
Sano, Toru
Tomita, Koichi
Hirano, Hiroshi
Abe, Yuta
Obara, Hideaki
Shimazu, Motohide
description 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. 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. 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.
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Furthermore, there are few reports of living donor liver transplantation (LDLT) for IPH with extrahepatic portal vein stenosis. 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. 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. 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Furthermore, there are few reports of living donor liver transplantation (LDLT) for IPH with extrahepatic portal vein stenosis. 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. 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.</abstract><cop>England</cop><pub>BioMed Central Ltd</pub><pmid>33121468</pmid><doi>10.1186/s12893-020-00921-6</doi><tpages>1</tpages><oa>free_for_read</oa></addata></record>
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subjects Analysis
Aneurysm
Aneurysm - complications
Aneurysm - surgery
Aneurysms
Ascites
Calcification
Case Report
Cirrhosis
Complications
Constriction, Pathologic - complications
Constriction, Pathologic - surgery
Dialysis
Disease prevention
Encephalopathy
Esophagus
Extrahepatic portal vein stenosis
Female
Fistula
Grafting
Health aspects
Humans
Hyperplasia
Hypertension
Hypertension, Portal - complications
Hypertension, Portal - surgery
Idiopathic portal hypertension
Jaundice
Literature reviews
Liver
Liver cirrhosis
Liver Cirrhosis - complications
Liver Cirrhosis - surgery
Liver diseases
Liver Transplantation
Living donor liver transplantation
Living Donors
Middle Aged
Pancytopenia - complications
Pancytopenia - surgery
Patients
Portal hypertension
Portal vein
Portal Vein - pathology
Portal Vein - surgery
Prognosis
Reconstructive Surgical Procedures - methods
Renal function
Spleen
Splenectomy
Splenic artery
Splenic Artery - surgery
Splenic artery aneurysms
Splenomegaly - complications
Splenomegaly - surgery
Stenosis
Superficial femoral vein graft
Tissue donation
Transplantation
Transplants & implants
Vascular Surgical Procedures - methods
Veins
Veins & arteries
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
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