Premium
Living‐donor liver transplantation for liver hemorrhaging due to peliosis hepatis in X‐linked myotubular myopathy: Two cases and a literature review
Author(s) -
Shimizu Seiichi,
Sakamoto Seisuke,
Fukuda Akinari,
Yanagi Yusuke,
Uchida Hajime,
Takeda Masahiro,
Yamada Yohei,
Nakano Noriyuki,
Yoshioka Takako,
Kasahara Mureo
Publication year - 2020
Publication title -
american journal of transplantation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.89
H-Index - 188
eISSN - 1600-6143
pISSN - 1600-6135
DOI - 10.1111/ajt.15978
Subject(s) - medicine , porta hepatis , liver transplantation , surgery , complication , peliosis hepatis , transplantation , pathological
X‐linked myotubular myopathy (MTM) (OMIM 310400) is a severe neuromuscular disorder caused by mutations in the myotubularin ( MTM1 ) gene. Liver hemorrhaging due to peliosis hepatis (PH) is a fatal complication. We herein report 2 successful cases of living‐donor liver transplantation (LDLT) for MTM patients due to liver hemorrhaging caused by PH and review previous reports. A boy who was 9 years and 4 months old initially underwent left lateral segmentectomy due to massive hepatic and intraperitoneal hemorrhaging. As bleeding from the remnant liver continued after hepatectomy, this patient emergently underwent LDLT using a left lateral segment graft from his father. Another boy who was 1 year and 7 months old underwent transcatheter arterial embolization due to hepatic hemorrhaging and was referred to our hospital for LDLT using a left lateral segment graft from his father. The pathological findings in both cases showed sinusoidal dilatation with degenerative changes in reticular fiber and hematoma in the explanted liver, which were consistent with PH associated with MTM. LT should be considered as a treatment option for patients with episodes of hepatic hemorrhaging due to MTM in order to protect against fatal bleeding.