A successful case of deceased-donor liver transplantation from a donor with Marfan syndrome: a case report
Abstract Background Liver transplantation is the definitive therapy for patients with decompensated cirrhosis. Marfan syndrome is a systemic inheritable connective tissue disease associated with fibrillin-1 gene mutations, which cause abnormalities in connective tissue. Vascular changes due to Marfa...
| Main Authors: | , , , , , , , , , , |
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| Format: | Article |
| Language: | English |
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SpringerOpen
2024-01-01
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| Series: | Surgical Case Reports |
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| Online Access: | https://doi.org/10.1186/s40792-024-01807-y |
| _version_ | 1797355573547630592 |
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| author | Takuma Ishikawa Shinji Itoh Takeo Toshima Yuki Nakayama Katsuya Toshida Yuriko Tsutsui Norifumi Iseda Takuma Izumi Shohei Yoshiya Mizuki Ninomiya Tomoharu Yoshizumi |
| author_facet | Takuma Ishikawa Shinji Itoh Takeo Toshima Yuki Nakayama Katsuya Toshida Yuriko Tsutsui Norifumi Iseda Takuma Izumi Shohei Yoshiya Mizuki Ninomiya Tomoharu Yoshizumi |
| author_sort | Takuma Ishikawa |
| collection | DOAJ |
| description | Abstract Background Liver transplantation is the definitive therapy for patients with decompensated cirrhosis. Marfan syndrome is a systemic inheritable connective tissue disease associated with fibrillin-1 gene mutations, which cause abnormalities in connective tissue. Vascular changes due to Marfan syndrome occur mostly in the main vessels due to the high amount of connective tissue within the vessel wall and the high pressure and blood flow to which they are exposed. The incidence of changes in visceral arteries is about 0.42% and usually presents with cystic medial necrosis. This report is the first deceased-donor liver transplantation with a donor with Marfan syndrome with a history of abdominal surgery. Case presentation A patient in his 50s underwent liver transplantation for decompensated alcoholic cirrhosis. The donor, a 50s male with Marfan syndrome, was diagnosed with brain-death due to a cerebral hemorrhage caused by a cerebral aneurysm. The donor’s clinical presentation as Marfan syndrome was aortic dissection, with multiple surgical procedures performed from the aortic root to the abdominal aorta. An intraoperative biopsy of the hepatic artery showed no abnormality, so this organ was considered appropriate. The surgery was completed without any problems of the arterial anastomosis. The patient’s postoperative course was uneventful, and he was transferred to a hospital for recuperation on the 18th postoperative day. One year after the surgery, the patient is still alive without any complications from the transplantation or arterial problems. Conclusions Even if the patient had a history of surgery for vascular anomalies extending to the abdominal aorta due to Marfan syndrome, the patient can be a donor for liver transplantation under appropriate judgment, including intraoperative biopsy. |
| first_indexed | 2024-03-08T14:13:06Z |
| format | Article |
| id | doaj.art-59fcaf23fecb4806bde3f68d50994107 |
| institution | Directory Open Access Journal |
| issn | 2198-7793 |
| language | English |
| last_indexed | 2024-03-08T14:13:06Z |
| publishDate | 2024-01-01 |
| publisher | SpringerOpen |
| record_format | Article |
| series | Surgical Case Reports |
| spelling | doaj.art-59fcaf23fecb4806bde3f68d509941072024-01-14T12:34:22ZengSpringerOpenSurgical Case Reports2198-77932024-01-011011410.1186/s40792-024-01807-yA successful case of deceased-donor liver transplantation from a donor with Marfan syndrome: a case reportTakuma Ishikawa0Shinji Itoh1Takeo Toshima2Yuki Nakayama3Katsuya Toshida4Yuriko Tsutsui5Norifumi Iseda6Takuma Izumi7Shohei Yoshiya8Mizuki Ninomiya9Tomoharu Yoshizumi10Department of Surgery and Science, Graduate School of Medical Sciences, Kyushu UniversityDepartment of Surgery and Science, Graduate School of Medical Sciences, Kyushu UniversityDepartment of Surgery and Science, Graduate School of Medical Sciences, Kyushu UniversityDepartment of Surgery and Science, Graduate School of Medical Sciences, Kyushu UniversityDepartment of Surgery and Science, Graduate School of Medical Sciences, Kyushu UniversityDepartment of Surgery and Science, Graduate School of Medical Sciences, Kyushu UniversityDepartment of Surgery and Science, Graduate School of Medical Sciences, Kyushu UniversityDepartment of Surgery and Science, Graduate School of Medical Sciences, Kyushu UniversityDepartment of Surgery and Science, Graduate School of Medical Sciences, Kyushu UniversityDepartment of Surgery and Science, Graduate School of Medical Sciences, Kyushu UniversityDepartment of Surgery and Science, Graduate School of Medical Sciences, Kyushu UniversityAbstract Background Liver transplantation is the definitive therapy for patients with decompensated cirrhosis. Marfan syndrome is a systemic inheritable connective tissue disease associated with fibrillin-1 gene mutations, which cause abnormalities in connective tissue. Vascular changes due to Marfan syndrome occur mostly in the main vessels due to the high amount of connective tissue within the vessel wall and the high pressure and blood flow to which they are exposed. The incidence of changes in visceral arteries is about 0.42% and usually presents with cystic medial necrosis. This report is the first deceased-donor liver transplantation with a donor with Marfan syndrome with a history of abdominal surgery. Case presentation A patient in his 50s underwent liver transplantation for decompensated alcoholic cirrhosis. The donor, a 50s male with Marfan syndrome, was diagnosed with brain-death due to a cerebral hemorrhage caused by a cerebral aneurysm. The donor’s clinical presentation as Marfan syndrome was aortic dissection, with multiple surgical procedures performed from the aortic root to the abdominal aorta. An intraoperative biopsy of the hepatic artery showed no abnormality, so this organ was considered appropriate. The surgery was completed without any problems of the arterial anastomosis. The patient’s postoperative course was uneventful, and he was transferred to a hospital for recuperation on the 18th postoperative day. One year after the surgery, the patient is still alive without any complications from the transplantation or arterial problems. Conclusions Even if the patient had a history of surgery for vascular anomalies extending to the abdominal aorta due to Marfan syndrome, the patient can be a donor for liver transplantation under appropriate judgment, including intraoperative biopsy.https://doi.org/10.1186/s40792-024-01807-yLiver transplantationDeceased-donor liver transplantationMarfan syndrome |
| spellingShingle | Takuma Ishikawa Shinji Itoh Takeo Toshima Yuki Nakayama Katsuya Toshida Yuriko Tsutsui Norifumi Iseda Takuma Izumi Shohei Yoshiya Mizuki Ninomiya Tomoharu Yoshizumi A successful case of deceased-donor liver transplantation from a donor with Marfan syndrome: a case report Surgical Case Reports Liver transplantation Deceased-donor liver transplantation Marfan syndrome |
| title | A successful case of deceased-donor liver transplantation from a donor with Marfan syndrome: a case report |
| title_full | A successful case of deceased-donor liver transplantation from a donor with Marfan syndrome: a case report |
| title_fullStr | A successful case of deceased-donor liver transplantation from a donor with Marfan syndrome: a case report |
| title_full_unstemmed | A successful case of deceased-donor liver transplantation from a donor with Marfan syndrome: a case report |
| title_short | A successful case of deceased-donor liver transplantation from a donor with Marfan syndrome: a case report |
| title_sort | successful case of deceased donor liver transplantation from a donor with marfan syndrome a case report |
| topic | Liver transplantation Deceased-donor liver transplantation Marfan syndrome |
| url | https://doi.org/10.1186/s40792-024-01807-y |
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