Selective portal blood flow diversion in auxiliary partial orthotopic liver transplantation to induce regeneration of the graft.
Experience with auxiliary partial orthotopic liver transplantation (APOLT) is still very limited and many questions remain to be solved. In this article, we present the case of a 5-year-old girl with ornithine transcarbamylase deficiency who initially did well after APOLT. During a severe rejection episode 16 months after transplantation, she developed encephalopathy and hyperammonemia. Despite a good clinical and histopathological response to antirejection therapy, the graft had become smaller and the native liver had undergone compensatory hypertrophy. After we surgically ligated the right portal branch, the graft recovered and the patient was able to stop her medication 1 month after surgery. We have estimated that the minimum volume of normal liver required to correct the metabolic defect in ornithine transcarbamylase deficiency is 8 cm3/kg. The ligation of the right portal branch was a safe and effective method of inducing a gradual and progressive involution of the hypertrophic native liver and regeneration of the atrophic graft.
Kaibori
M
M
Department of Transplantation and Immunology, Kyoto University Graduate School of Medicine, Kyoto University Hospital, Japan.
Egawa
H
H
Inomata
Y
Y
Uemoto
S
S
Asonuma
K
K
Kiuchi
T
T
Varela-Fascinetto
G
G
Matsukura
T
T
Kasahara
M
M
Uryuhara
K
K
Inui
A
A
Komatsu
H
H
Fujisawa
T
T
Tanaka
K
K
D001284:Atrophy; D002675:Child, Preschool; D005260:Female; D006084:Graft Rejection / Q000000981:diagnostic imaging / Q000628:therapy; D006801:Humans; D008026:Ligation; D008099:Liver / Q000000981:diagnostic imaging / Q000503:physiopathology; D008115:Liver Regeneration / Q000502:physiology*; D016031:Liver Transplantation; D020163:Ornithine Carbamoyltransferase Deficiency Disease; D011168:Portal System / Q000503:physiopathology*; D011183:Postoperative Complications / Q000628:therapy*; D012086:Reoperation; D014057:Tomography, X-Ray Computed