Orthotopic Liver Transplantation in a Patient with X-Linked Centronuclear Myopathy after Liver Hemorrhage due to Peliosis Hepatis

Jankofsky, Martin

doi:10.23880/ghij-16000105

Cited by 4 publications

(2 citation statements)

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“…To our knowledge, 14 cases of XLMTM with PH, including the present 2, have been reported, as shown in Table 1. [6][7][8]11 Massive hepatic hemorrhaging with shock, cardiopulmonary arrest (CPA), or death was observed in 12 of the 14 cases. TAE was effective in 3 of 6 cases.…”

Section: Discussionmentioning

confidence: 99%

“…In a recent case report, LT was successfully performed as curative therapy because massive liver damage after surgery to control bleeding led to liver failure. 8 We herein report 2 successful cases of emergent and scheduled living donor liver transplantation (LDLT) for patients with MTM due to liver hemorrhaging caused by PH at our institute. Furthermore, we review previous reports and describe the treatment strategy for hepatic bleeding caused by PH associated with MTM.…”

Section: Introductionmentioning

confidence: 99%

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Living-donor liver transplantation for liver hemorrhaging due to peliosis hepatis in X-linked myotubular myopathy: Two cases and a literature review

Shimizu

Sakamoto

Fukuda

et al. 2020

American Journal of Transplantation

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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.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%