Researchers reported the case of a patient with Alagille syndrome (ALGS) who underwent stenting for severe pulmonary stenosis and received a deceased donor liver transplantation, as published in the International Journal of Surgery.

Large-for-size syndrome is a potentially devastating complication of liver transplantation. It is usually reported among pediatric patients and surgeons who often reduce the graft size before surgery. The management of large-for-size syndrome in adult patients has rarely been discussed.

Jeong and Jung reported the case of an adult female with ALGS who had chronic hepatic failure. She previously had pulmonary stenting for pulmonary stenosis associated with ALGS. She had a Model for End-stage Liver Disease score of 40 and was on continuous hemodialysis.


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The patient underwent an emergency deceased donor liver transplantation. The donor was a brain-dead adult male. The graft was 2300 g, which translated to a graft-to-recipient weight ratio of 3.96%. Because the patient’s condition was critical, the surgery still went ahead. Her surgeons planned an ex-situ right posterior graft hepatectomy.

“During the donor operation, the posterior glissonean pedicle was clamped temporarily to mark the cutting line,” Jeong and Jung wrote. “The parenchymal transection which was performed on the back table took approximately 90 minutes using a cavitron ultrasonic surgical aspirator.”

The graft was successfully implanted without compromising the hepatic venous outflow. There was no bile leakage or definite bleeding. Primary abdominal closure was performed. Postoperatively, the patient did not experience any vascular or ductal complications. 

The surgery demonstrated the feasibility of performing ex-situ right posterior hepatectomy in patients with ALGS who have expected large-for-size syndrome and need liver transplantation critically.

Reference

Jeong I-J, Jung D-H. Deceased donor liver transplantation using an ex-situ reduced left trisection graft in an adult patient with Alagille syndrome who underwent stenting for severe pulmonary stenosis: a case report. Int J Surg. Published online April 30, 2022. doi:10.1016/j.issue.2022.106480