Living donor liver transplantation for an infant with osteogenesis imperfecta and intrahepatic cholestasis: report of a case.
Young Rok ChoiNam-Joon YiJeong Kee SeoJung Min KoUng Sik JinHee Soo KimKook Hyun LeeTae-Joon ChoSuk-Won SuhTae YooKwang-Woong LeeKyung-Suk SuhPublished in: Journal of Korean medical science (2014)
Osteogenesis imperfecta (OI) is a group of genetic disorders characterized by bone fragility and connective tissue manifestations. We report a successful liver transplantation (LT) in an 8-month-old boy with OI and cholestatic biliary cirrhosis. After 4 cycles of intravenous pamidronate, LT was performed under intravenous anesthesia using a left lateral section from his mother without mechanical retractors. The operation time was 420 min and estimated blood loss was 520 mL requiring one unit of RBC transfusion. He was discharged without surgical complications. Therefore, LT should be considered for patients with end stage liver disease and OI under organic multidisciplinary cooperation.