Management of Established Small-for-size Syndrome in Post Living Donor Liver Transplantation: Medical, Radiological, and Surgical Interventions: Guidelines From the ILTS-iLDLT-LTSI Consensus Conference

Author:

Kirchner Varvara A.1,Shankar Sadhana2,Victor David W.3,Tanaka Tomohiro4,Goldaracena Nicolas5,Troisi Roberto I.6,Olthoff Kim M.7,Kim Jong Man8,Pomfret Elizabeth A.9,Heaton Nigel10,Polak Wojtek G.11,Shukla Akash12,Mohanka Ravi13,Balci Deniz14,Ghobrial Mark3,Gupta Subash15,Maluf Daniel16,Fung John J.17,Eguchi Susumu18,Roberts John19,Eghtesad Bijan20,Selzner Markus21,Prasad Raj22,Kasahara Mureo23,Egawa Hiroto24,Lerut Jan25,Broering Dieter26,Berenguer Marina27,Cattral Mark S.21,Clavien Pierre-Alain28,Chen Chao-Long29,Shah Samir R.30,Zhu Zhi-Jun31,Ascher Nancy19,Ikegami Toru32,Bhangui Prashant33,Rammohan Ashwin34,Emond Jean C.35,Rela Mohamed34

Affiliation:

1. Division of Abdominal Transplantation, Department of Surgery, Stanford University School of Medicine, Palo Alto, CA.

2. The Liver Unit, King’s College Hospital, London, United Kingdom.

3. Sherrie and Alan Conover Center for Liver Disease and Transplantation, Houston Methodist Hospital, Houston, TX.

4. Department of Internal Medicine, Gastroenterology and Hepatology, University of Iowa, Iowa City, IA.

5. Abdominal Organ Transplant and Hepatobiliary Surgery, University of Virginia Health System, Charlottesville, VA.

6. Division of Hepato-Bilio-Pancreatic, Minimally Invasive and Robotic Surgery, Department of Public Health, Federico II University Hospital, Naples, Italy.

7. Department of Surgery, Division of Transplant Surgery, Hospital of the University of Pennsylvania, Philadelphia, PA.

8. Department of Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.

9. Division of Transplant Surgery, Department of Surgery, Children’s Hospital Colorado, University of Colorado Anschutz Medical Campus, Aurora, CO.

10. The Institute of Liver Studies, King’s College Hospital, London, United Kingdom.

11. The Erasmus MC Transplant Institute, Erasmus MC, University Medical Center Rotterdam, Rotterdam, the Netherlands.

12. Department of Gastroenterology, Seth GS Medical College and KEM Hospital, Mumbai, India.

13. Institute of Liver Disease, HPB Surgery and Transplant, Global Hospital, Mumbai, Maharashtra, India.

14. Department of General Surgery and Organ Transplantation Bahcesehir University School of Medicine, Istanbul, Turkey.

15. Max Centre for Liver and Biliary Sciences, Max Saket Hospital, New Delhi, India.

16. Program in Transplantation, University of Maryland Medical Center, University of Maryland School of Medicine, Baltimore, MD.

17. Department of Surgery, University of Chicago Medicine Transplant Institute, Chicago, IL.

18. Department of Surgery, Graduate School of Biomedical Sciences, Nagasaki University, Nagasaki, Japan.

19. Department of Surgery, University of California San Francisco Medical Center, San Francisco, CA.

20. Digestive Disease and Surgery Institute, Cleveland Clinic; Clinical Assistant Professor, Cleveland Clinic Lerner College of Medicine of Case Western Reserve University, Cleveland, OH.

21. HPB and Multi-Organ Transplant Program, Department of Surgery, Toronto General Hospital, University Health Network, University of Toronto, Toronto, Canada.

22. Division of Transplantation, Department of Surgery, Medical College of Wisconsin, Milwaukee, WI.

23. National Center for Child Health and Development, Tokyo, Japan.

24. Department of Surgery, Tokyo Women’s Medical University, Tokyo, Japan.

25. Institute for Experimental and Clinical Research–Université catholique de Louvain, Brussels, Belgium.

26. King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia.

27. Liver Unit, CIBERehd, Instituto de Investigación Sanitaria La Fe, Hospital Universitario y Politécnico La Fe-Universidad de Valencia, Valencia, Spain.

28. Department of Surgery and Transplantation, University Hospital Zurich, Zurich, Switzerland.

29. Liver Transplant Center and Department of Surgery, Kaohsiung Chang Gung Memorial Hospital and Chang Gung University College of Medicine, Kaohsiung, Taiwan.

30. Institute of Liver Disease, HPB Surgery and Transplant, Global Hospitals, Mumbai, India.

31. Liver Transplantation Center, National Clinical Research Center for Digestive Diseases, Beijing Friendship Hospital, Capital Medical University; and Clinical Center for Pediatric Liver Transplantation, Capital Medical University, Beijing, China.

32. Divsion of Hepatobiliary Surgery and Pancreas Surgery, Department of Surgery, Jikei University School of Medicine, Tokyo, Japan.

33. Medanta Institute of Liver Transplantation and Regenerative Medicine, Medanta-The Medicity, New Delhi, India.

34. The Institute of Liver Disease and Transplantation, Dr Rela Institute and Medical Centre, Chennai, India.

35. Department of Surgery, Columbia University College of Physicians and Surgeons, New York, NY.

Abstract

Small-for-size syndrome (SFSS) following living donor liver transplantation is a complication that can lead to devastating outcomes such as prolonged poor graft function and possibly graft loss. Because of the concern about the syndrome, some transplants of mismatched grafts may not be performed. Portal hyperperfusion of a small graft and hyperdynamic splanchnic circulation are recognized as main pathogenic factors for the syndrome. Management of established SFSS is guided by the severity of the presentation with the initial focus on pharmacological therapy to modulate portal flow and provide supportive care to the patient with the goal of facilitating graft regeneration and recovery. When medical management fails or condition progresses with impending dysfunction or even liver failure, interventional radiology (IR) and/or surgical interventions to reduce portal overperfusion should be considered. Although most patients have good outcomes with medical, IR, and/or surgical management that allow graft regeneration, the risk of graft loss increases dramatically in the setting of bilirubin >10 mg/dL and INR>1.6 on postoperative day 7 or isolated bilirubin >20 mg/dL on postoperative day 14. Retransplantation should be considered based on the overall clinical situation and the above postoperative laboratory parameters. The following recommendations focus on medical and IR/surgical management of SFSS as well as considerations and timing of retransplantation when other therapies fail.

Publisher

Ovid Technologies (Wolters Kluwer Health)

Subject

Transplantation

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