Introduction: In intestinal transplantation (ITx), bleeding from the intestinal tract is a rare complication and the differential diagnosis include infection, rejection, and PTLD. We report on a case series with bleeding from ectopic varices

Methods:

Case 1: 15 year old girl (intestinal pseudo-obstruction) presented with bleeding from the stoma, 8 years after Liver ITx . Graft biopsies excluded rejection and PTLD. An upper GI endoscopy (UGIE) showed no evidence of active bleeding. Repeat UGIE 3 wks later showed a cluster of varices at the upper graft anastomosis . ATIPPS was performed which stopped the bleeding. Two mths after TIPPS, she had recurrent bleeding from the jejunal varices, despite an patent TIPPS. She developed multi-organ failure and she eventually died.

Case 2: A 8 year boy (MVID), presented with malaena & splenomegaly 4 years after isolated ITx. CT Angio revealed a patent portal vein & intra-abdominal varices in small bowel. A further UGIE revealed varices at upper graft anastomosis and were injected with thrombin. A TIPPS was performed, which stopped bleeding. He developed multiorgan failure and subsequently died.

Case 3: 3 year old girl (gastroschisis) presented with recurrent intermittent bleeding from the stoma 1 year after Liver ITx. A initial UGIE showed no bleeding point and graft biopsies were normal . Video-capsule endoscopy done showed no varices. CT angio could not pinpoint the location of bleeding, but demonstrated occlusion of porta-caval shunt. Due to ongoing blood transfusion requirements, a further UGIE showed varices at the junction of the upper graft anastomosis. The child continues to have intermittent episodes of bleeding but the family has declined a definitive surgical option.

Discussion: The bleeding episodes in our children originated from the ectopic varices that developed at level of the upper graft anastomosis due to altered hemodynamics following portacaval shunt blockage in two liver ITxgrafts . In the child with isolated ITx, it was due to progression of liver disease and possibly because of adhesions from repeated abdominal surgeries, which then predisposes to the development of collaterals at the upper graft anastomosis.

Conclusion: Ectopic varices should be in the differential diagnosis of children presenting with bleeding episodes following ITx especially when the graft biopsies are normal.