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 |
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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. |
doi_str_mv | 10.1186/s12893-020-00921-6 |
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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.</description><identifier>ISSN: 1471-2482</identifier><identifier>EISSN: 1471-2482</identifier><identifier>DOI: 10.1186/s12893-020-00921-6</identifier><identifier>PMID: 33121468</identifier><language>eng</language><publisher>England: BioMed Central Ltd</publisher><subject>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</subject><ispartof>BMC surgery, 2020-10, Vol.20 (1), p.257-257, Article 257</ispartof><rights>COPYRIGHT 2020 BioMed Central Ltd.</rights><rights>2020. This work is licensed under http://creativecommons.org/licenses/by/4.0/ (the “License”). Notwithstanding the ProQuest Terms and Conditions, you may use this content in accordance with the terms of the License.</rights><rights>The Author(s) 2020</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c563t-40ee709c2ba6e6b520765d577a07d4bc1802a5eec8cc2e75712db87973eb4fd43</citedby><cites>FETCH-LOGICAL-c563t-40ee709c2ba6e6b520765d577a07d4bc1802a5eec8cc2e75712db87973eb4fd43</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC7597044/pdf/$$EPDF$$P50$$Gpubmedcentral$$Hfree_for_read</linktopdf><linktohtml>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC7597044/$$EHTML$$P50$$Gpubmedcentral$$Hfree_for_read</linktohtml><link.rule.ids>230,314,727,780,784,864,885,2102,27924,27925,53791,53793</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/33121468$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Kawachi, Shigeyuki</creatorcontrib><creatorcontrib>Chiba, Naokazu</creatorcontrib><creatorcontrib>Nakagawa, Masashi</creatorcontrib><creatorcontrib>Kobayashi, Toshimichi</creatorcontrib><creatorcontrib>Hikita, Kosuke</creatorcontrib><creatorcontrib>Sano, Toru</creatorcontrib><creatorcontrib>Tomita, Koichi</creatorcontrib><creatorcontrib>Hirano, Hiroshi</creatorcontrib><creatorcontrib>Abe, Yuta</creatorcontrib><creatorcontrib>Obara, Hideaki</creatorcontrib><creatorcontrib>Shimazu, Motohide</creatorcontrib><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><title>BMC surgery</title><addtitle>BMC Surg</addtitle><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.</description><subject>Analysis</subject><subject>Aneurysm</subject><subject>Aneurysm - complications</subject><subject>Aneurysm - surgery</subject><subject>Aneurysms</subject><subject>Ascites</subject><subject>Calcification</subject><subject>Case Report</subject><subject>Cirrhosis</subject><subject>Complications</subject><subject>Constriction, Pathologic - complications</subject><subject>Constriction, Pathologic - surgery</subject><subject>Dialysis</subject><subject>Disease prevention</subject><subject>Encephalopathy</subject><subject>Esophagus</subject><subject>Extrahepatic portal vein stenosis</subject><subject>Female</subject><subject>Fistula</subject><subject>Grafting</subject><subject>Health aspects</subject><subject>Humans</subject><subject>Hyperplasia</subject><subject>Hypertension</subject><subject>Hypertension, Portal - complications</subject><subject>Hypertension, Portal - surgery</subject><subject>Idiopathic portal hypertension</subject><subject>Jaundice</subject><subject>Literature reviews</subject><subject>Liver</subject><subject>Liver cirrhosis</subject><subject>Liver Cirrhosis - complications</subject><subject>Liver Cirrhosis - surgery</subject><subject>Liver diseases</subject><subject>Liver Transplantation</subject><subject>Living donor liver transplantation</subject><subject>Living Donors</subject><subject>Middle Aged</subject><subject>Pancytopenia - complications</subject><subject>Pancytopenia - surgery</subject><subject>Patients</subject><subject>Portal hypertension</subject><subject>Portal vein</subject><subject>Portal Vein - pathology</subject><subject>Portal Vein - surgery</subject><subject>Prognosis</subject><subject>Reconstructive Surgical Procedures - methods</subject><subject>Renal function</subject><subject>Spleen</subject><subject>Splenectomy</subject><subject>Splenic artery</subject><subject>Splenic Artery - surgery</subject><subject>Splenic artery aneurysms</subject><subject>Splenomegaly - complications</subject><subject>Splenomegaly - surgery</subject><subject>Stenosis</subject><subject>Superficial femoral vein graft</subject><subject>Tissue donation</subject><subject>Transplantation</subject><subject>Transplants & implants</subject><subject>Vascular Surgical Procedures - 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complications</topic><topic>Aneurysm - surgery</topic><topic>Aneurysms</topic><topic>Ascites</topic><topic>Calcification</topic><topic>Case Report</topic><topic>Cirrhosis</topic><topic>Complications</topic><topic>Constriction, Pathologic - complications</topic><topic>Constriction, Pathologic - surgery</topic><topic>Dialysis</topic><topic>Disease prevention</topic><topic>Encephalopathy</topic><topic>Esophagus</topic><topic>Extrahepatic portal vein stenosis</topic><topic>Female</topic><topic>Fistula</topic><topic>Grafting</topic><topic>Health aspects</topic><topic>Humans</topic><topic>Hyperplasia</topic><topic>Hypertension</topic><topic>Hypertension, Portal - complications</topic><topic>Hypertension, Portal - surgery</topic><topic>Idiopathic portal hypertension</topic><topic>Jaundice</topic><topic>Literature reviews</topic><topic>Liver</topic><topic>Liver cirrhosis</topic><topic>Liver Cirrhosis - complications</topic><topic>Liver Cirrhosis - surgery</topic><topic>Liver diseases</topic><topic>Liver Transplantation</topic><topic>Living donor liver transplantation</topic><topic>Living Donors</topic><topic>Middle Aged</topic><topic>Pancytopenia - complications</topic><topic>Pancytopenia - surgery</topic><topic>Patients</topic><topic>Portal hypertension</topic><topic>Portal vein</topic><topic>Portal Vein - pathology</topic><topic>Portal Vein - surgery</topic><topic>Prognosis</topic><topic>Reconstructive Surgical Procedures - methods</topic><topic>Renal function</topic><topic>Spleen</topic><topic>Splenectomy</topic><topic>Splenic artery</topic><topic>Splenic Artery - surgery</topic><topic>Splenic artery aneurysms</topic><topic>Splenomegaly - complications</topic><topic>Splenomegaly - surgery</topic><topic>Stenosis</topic><topic>Superficial femoral vein graft</topic><topic>Tissue donation</topic><topic>Transplantation</topic><topic>Transplants & implants</topic><topic>Vascular Surgical Procedures - methods</topic><topic>Veins</topic><topic>Veins & arteries</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Kawachi, Shigeyuki</creatorcontrib><creatorcontrib>Chiba, Naokazu</creatorcontrib><creatorcontrib>Nakagawa, Masashi</creatorcontrib><creatorcontrib>Kobayashi, Toshimichi</creatorcontrib><creatorcontrib>Hikita, Kosuke</creatorcontrib><creatorcontrib>Sano, Toru</creatorcontrib><creatorcontrib>Tomita, Koichi</creatorcontrib><creatorcontrib>Hirano, Hiroshi</creatorcontrib><creatorcontrib>Abe, Yuta</creatorcontrib><creatorcontrib>Obara, Hideaki</creatorcontrib><creatorcontrib>Shimazu, Motohide</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>ProQuest Central (Corporate)</collection><collection>Biotechnology Research Abstracts</collection><collection>Calcium & Calcified Tissue Abstracts</collection><collection>Health & Medical Collection</collection><collection>ProQuest Central (purchase pre-March 2016)</collection><collection>Medical Database (Alumni Edition)</collection><collection>Technology Research Database</collection><collection>Hospital Premium Collection</collection><collection>Hospital Premium Collection (Alumni Edition)</collection><collection>ProQuest Central (Alumni) (purchase pre-March 2016)</collection><collection>ProQuest Central (Alumni Edition)</collection><collection>ProQuest Central UK/Ireland</collection><collection>ProQuest Central Essentials</collection><collection>ProQuest Central</collection><collection>ProQuest One Community College</collection><collection>ProQuest Central Korea</collection><collection>Engineering Research Database</collection><collection>Health Research Premium Collection</collection><collection>Health Research Premium Collection (Alumni)</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>Health & Medical Collection (Alumni Edition)</collection><collection>Medical Database</collection><collection>Biotechnology and BioEngineering Abstracts</collection><collection>Publicly Available Content Database</collection><collection>ProQuest One Academic Eastern Edition (DO NOT USE)</collection><collection>ProQuest One Academic</collection><collection>ProQuest One Academic UKI Edition</collection><collection>ProQuest Central China</collection><collection>MEDLINE - Academic</collection><collection>PubMed Central (Full Participant titles)</collection><collection>DOAJ Directory of Open Access Journals</collection><jtitle>BMC surgery</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Kawachi, Shigeyuki</au><au>Chiba, Naokazu</au><au>Nakagawa, Masashi</au><au>Kobayashi, Toshimichi</au><au>Hikita, Kosuke</au><au>Sano, Toru</au><au>Tomita, Koichi</au><au>Hirano, Hiroshi</au><au>Abe, Yuta</au><au>Obara, Hideaki</au><au>Shimazu, Motohide</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>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</atitle><jtitle>BMC surgery</jtitle><addtitle>BMC Surg</addtitle><date>2020-10-29</date><risdate>2020</risdate><volume>20</volume><issue>1</issue><spage>257</spage><epage>257</epage><pages>257-257</pages><artnum>257</artnum><issn>1471-2482</issn><eissn>1471-2482</eissn><abstract>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.</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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source | MEDLINE; DOAJ Directory of Open Access Journals; PubMed Central Open Access; Springer Nature OA Free Journals; EZB-FREE-00999 freely available EZB journals; PubMed Central; SpringerLink Journals - AutoHoldings |
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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