Liver-Intestine Transplant from a Pediatric Donor with Unrecognized Mitochondrial Succinate Cytochrome C Reductase Deficiency

The demand for pediatric solid organs for transplantation exceeds the available supply. Transplant surgeons may elect to use organs from a donor whose cause of death is uncertain, especially when the recipient is deteriorating. In such circumstances, it is possible that organs from a patient with a systemic metabolic disorder may be transplanted into the recipient, leading to an adverse outcome. We report the first case in which liver and small bowel were procured from a donor with an unsuspected mitochondrial respiratory transport chain defect (succinate cytochrome C reductase deficiency). We describe the subsequent course of the recipient, who died 10 weeks later of multiorgan failure, and unusual findings at autopsy. In the absence of a clear cause of death in a potential pediatric organ donor, factors such as parental consanguinity should prompt physicians to acknowledge the increased possibility of an inherited metabolic disorder and to take this into consideration before proceeding with the transplant procedure.