METHODS: A patient with maple syrup urine disease (MSUD) underwent a living donor liver transplant, and the explanted liver was divided in situ into right and left lobes and transplanted to 2 separate patients. Demographic data, surgical techniques, postoperative period, and patient follow-ups were evaluated.
RESULTS: The father\'s left lobe liver graft was transplanted into a 12-year-old boy with MSUD. The removed liver was divided in situ into right and left lobes. The left lobe was transplanted to a 14-year-old male patient, whereas the right lobe was transplanted to a 67-year-old male patient. The donor and the first recipient were discharged on postoperative days 5 and 22. The second pediatric patient who underwent domino split-left lobe transplantation was discharged on postoperative day 23. The adult patient who underwent domino split-right lobe transplantation died on postoperative day 12 owing to massive esophageal variceal bleeding.
CONCLUSIONS: Patients who underwent liver transplantation due to MSUD are among the best donor choices for domino liver transplantation. If the extracted liver has a sufficient volume and anatomic features for a split, it can be used in \"selected cases.\"
方法:一名患有枫糖浆尿病(MSUD)的患者接受了活体肝移植,移植的肝脏被原位分为右叶和左叶,并移植给2例患者。人口统计数据,外科技术,术后期间,并对患者随访情况进行评估。
结果:将父亲的左叶肝移植物移植到一个12岁的MSUD男孩体内。将取出的肝脏原位分成右叶和左叶。左叶移植到一名14岁的男性患者身上,而右叶移植到一名67岁的男性患者身上。供体和第一受体在术后第5天和第22天出院。第二例接受多米诺左叶分裂移植的儿科患者在术后第23天出院。接受多米诺骨牌右叶移植的成年患者在术后第12天因食管静脉曲张大出血而死亡。
结论:因MSUD而接受肝移植的患者是多米诺肝移植的最佳供体选择之一。如果提取的肝脏有足够的体积和解剖特征进行分裂,它可以在“选定的情况下使用。\"