Early graft failure due to a veno‐occlusive disease after a pediatric living donor liver transplantation

Abstract

A 10-month-old boy with biliary atresia after Kasai procedure underwent a living donor liver transplantation (LDLT). Five days after the LDLT, high fever and increased ascites followed by poor bile drainage was accompanied by elevation of serum liver enzymes. Liver biopsy showed occlusion of the central veins by fibro-edematous endothelium and submassive necrosis of the parenchyma. Veno-occlusive disease (VOD) was suspected, and re-LDLT was urgently performed because of deterioration of hepatic failure. There are few cases of VOD after liver transplantation and this is the first one in an infant after LDLT.

Sinusoidal Obstruction Syndrome (Hepatic Veno-Occlusive Disease)

“…90 This is to be distinguished from post-transplant perivenulitis, as part of graft rejection. 91 If present, SOS is more likely to occur in living-related transplant grafts 92 or in patients receiving azothiaprine as part of their immunosuppressive regime. 93 Better optimization of immunosuppression is suggested as an approach to reduce the incidence of this rare event.…”

Untitled

“…Several cases with VOD after liver transplant have been reported, involving cases of deceased-donor and living-related, adult and pediatric recipients. [7][8][9][10][11][12] Two review articles of large series mentioned that about 1.9% to 2.3% of patients after deceased donor's liver transplant had VOD at various times after transplant. 8,9 The clinical symptoms related to VOD are nonspecific, which induces the fact that diagnosing of VOD in postliver transplant patients is more difficult and confused than that which occurs in other types of transplants.…”

“…It should be emphasized that recent researches tended to support the hypothesis that immunologic responses may participate in the onset of VOD after liver transplant (including acute cellular and humoral rejection). [7][8][9][10][11][12] Tacrolimus might be another possible agent to induce VOD because it precipitates dysregulation of endothelial cells. 10 But the cases of VOD presumably associated with tacrolimus, are even rarer than azathioprine and immunologic phenomenon.…”

Defibrotide in the treatment of hepatic veno-occlusive disease

“…A low incidence of SOS has been noted in patients affected by acute myeloid leukemia treated with the cytotoxic chemotherapeutic agent gemtuzumab ozogamicin 10,11. The rare occurrence of SOS due to liver graft dysfunction following liver transplantation,12 and SOS associated with immunodeficiency syndrome related to a mutation of the immunoregulatory gene SP110 have also been described 13. Finally, SOS typically develops following allogeneic hematopoietic stem-cell transplantation (HSCT) 14.…”