Terlipressin Therapy for Portal Hyperperfusion Secondary to Portal Vein Size Discrepancy After Pediatric Liver Transplant.

backgroundCongenital portosystemic shunts (CPSS) are rare vascular malformations in which portal venous blood bypasses the liver and drains directly into systemic circulation. Type I Abernethy malformations, characterized by absent intrahepatic portal veins, rarely close spontaneously and often require liver transplantation. Small-for-size syndrome (SFSS), typically described in adult living donor liver transplantation, is increasingly recognized in pediatric recipients, particularly with graft-to-recipient weight ratio (GRWR) < 1.5%.

methodsWe report the case of a 5-year-old female with Type I Abernethy malformation who underwent deceased donor whole graft liver transplantation. Intraoperative findings demonstrated marked size discrepancy between the donor portal vein and the patient's large portosystemic shunt. Postoperatively, she developed acute liver dysfunction concerning for relative hyperperfusion and SFSS physiology.

resultsDespite a GRWR of 1.78%, the patient exhibited rapid elevations in transaminases, INR, and bilirubin postoperatively, raising concern for SFSS physiology. Octreotide therapy resulted in partial biochemical improvement. The subsequent initiation of terlipressin resulted in a marked and sustained decline in liver enzymes and normalization of coagulation parameters. At 18 months post-transplant, the patient remains clinically well without hepatic dysfunction.

conclusionsThis is the first reported pediatric case of successful use of terlipressin, bridged by octreotide, for management of suspected SFSS physiology following liver transplantation. Terlipressin may represent a safe and effective therapeutic option to modulate portal pressures in pediatric patients with graft hyperperfusion.