eMedicine Specialties > Pediatrics: Surgery > Transplantation

Intestinal and Multivisceral Transplantation

Author: Seigo Nishida, MD, PhD, Associate Director of Adult Intestinal Transplant Program, Associate Professor of Clinical Surgery, Division of Liver/GI Transplantation, Department of Surgery, University of Miami School of Medicine
Coauthor(s): Andreas G Tzakis, MD, PhD, Professor, Department of Surgery, Miller School of Medicine, University of Miami; David M Levi, MD, Associate Professor of Clinical Surgery, Department of Surgery, Division of Liver and Gastrointestinal Transplantation, Division of General Surgery, Jackson Memorial Hospital, Miller School of Medicine, University of Miami; Tomoaki Kato, MD, Associate Professor of Clinical Surgery, Department of Surgery, Division of Liver and Gastrointestinal Transplantation, Miller School of Medicine, University of Miami; Jose R Nery, MD, Associate Professor, Department of Surgery, Division of Liver and Gastrointestinal Transplantation, Miller School of Medicine, University of Miami; Juan Madariaga, MD, Professor, Department of Surgery, Miller School of Medicine, University of Miami; John F Thompson, MD, Associate Professor, Department of Pediatrics, Miller School of Medicine, University of Miami; Phillip Ruiz, Jr, MD, PhD, Professor of Pathology, Department of Pathology and Surgery, Miller School of Medicine, University of Miami; G Patricia Cantwell, MD, Associate Clinical Professor, Department of Pediatrics, University of Miami; Director of Pediatric Critical Care Medicine, Miller School of Medicine, Jackson Children's Hospital
Contributor Information and Disclosures

Updated: Jan 24, 2008

Introduction

Total parenteral nutrition (TPN) is the treatment of choice for patients with intestinal failure. Intestinal and multivisceral transplantation provides an alternative for patients who have life-threatening complications of TPN. Recent improvements in surgical technique, the monitoring and diagnosis of rejection, and cytomegalovirus (CMV) prophylaxis and the development of improved immunosuppression have paved the way for significant improvements in patient and graft survival rates. This article reviews the current status of intestinal and multivisceral transplantation, with emphasis on the authors' experience at the University of Miami.

History of the Procedure

Carrel performed the first experimental intestinal transplantation in 1902.1 Half a century later, in 1959, Lillehei demonstrated successful intestinal autotransplantation after cold preservation.2 Experimental multivisceral transplantation (termed polysplanchnic transplantation) was pioneered by Starzl in 1960.3 The earliest attempts at clinical intestinal transplantation were unsuccessful because of what appeared to be insurmountable barriers: graft rejection and infectious complications.4,5

The first successful intestinal transplantations occurred in the late 1980s. Grant reported successful intestinal transplantation in pigs with cyclosporine use in 1988.6 Starzl reported the first clinical successful multivisceral transplantation in 1987.7 In 1988, Goulet and Deltz independently reported the first successful isolated intestine transplantations.6 Grant reported the first combined liver and intestine transplantation under cyclosporine-based immunosuppression in 1990.8 Starzl, Todo, and Tzakis pioneered intestinal transplantation with tacrolimus in the early 1990s.7,9,10,11,12 As a result, the number of centers performing the procedures increased.13,14

The intestinal and multivisceral transplantation program at the University of Miami was started in 1994. Through September 2007, 278 intestinal transplantations have been performed, including 247 primary intestinal transplantations (65 isolated intestine, 32 combined liver-intestine, and 150 multivisceral transplantations) and 31 retransplantations (11 isolated intestine, 2 combined liver-intestine, and 18 multivisceral transplantations).

Problem

Short bowel syndrome is often the result of extensive intestinal resection for multiple pathophysiologies, such as volvulus, trauma, tumor, and thrombosis. An inadequate absorptive surface results in an inadequate energy intake and malabsorption of vitamin B-12 and other vitamins. Calcium and magnesium deficiencies can lead to neurologic complications such as encephalopathy, tetany, and convulsions. Intestinal failure with hyperalimentation causes liver failure. Patient with long-term hyperalimentation usually have complications, including line sepsis, thrombosed veins, and liver dysfunction.

Frequency

No database reports the exact incidence of short bowel syndrome. However, the incidence is estimated to be 1.8-2 patients per one million general population.

Etiology

The causes of intestinal failure include the following:

At the University of Miami, all patients with intestinal failure that necessitated transplantation had TPN-related complications, and most had concurrent TPN-induced liver failure. The causes of intestinal failure and the number of patients affected are as follows:

  • Mesenteric thrombosis - 29
  • Necrotizing enterocolitis - 32
  • Gastroschisis - 36
  • Volvulus - 21
  • Desmoid tumor - 14
  • Intestinal atresia - 17
  • Trauma - 16
  • Hirschsprung disease - 16
  • Crohn disease - 7
  • Pseudoobstruction - 13
  • Megacystic microcolon -10
  • Microvillus inclusion - 8
  • Others - 30
  • Retransplant - 31

Pathophysiology

Extensive resection of the small bowel can cause short bowel syndrome. Extensive loss of the intestinal mucosa surface results in an inadequate absorptive surface. An inadequate caloric intake and malabsorption of vitamins, including vitamin B-12, can result in severe malnutrition and neurological symptoms. Inadequate electrolyte absorption (eg, calcium, magnesium) also results in severe neurological complications, including encephalopathy, tetany, and convulsions.

Presentation

All patients who received intestinal or multivisceral transplants at the University of Miami had life-threatening TPN-related complications, such as sepsis, central vein thrombosis, and cholestatic TPN-induced liver failure.

The recipients of isolated intestinal transplants had short bowel syndrome with severe central vein thrombosis and line sepsis. Liver functions were preserved.

The recipients of liver and small bowel transplants had short bowel syndrome and cholestatic TPN-induced liver failure. Total bilirubin levels were usually higher than 20 mg/dL.

The recipients of multivisceral transplants had short bowel syndrome and cholestatic TPN-induced liver failure. In addition, they usually had a history of surgery, abdominal trauma, motility disorders, tumors, and other etiologies.

Indications

The role of TPN in short bowel syndrome is established, and long-term TPN is the treatment of choice for these patients because of its well-documented long-term safety.15 A subset of patients with short bowel syndrome cannot tolerate TPN because of frequent episodes of catheter-associated sepsis, catheter-associated vascular thrombosis, or TPN-induced cholestasis. This is the subset of patients who need intestinal or multivisceral transplantation.
 
Line infection is the most common cause of fever and chills for patients with TPN. Gram-positive bacteria are the common pathogens. TPN-induced liver failure is the most common cause of jaundice in TPN therapy. If liver failure progresses, intestinal transplantation or intestinal and liver transplantation is indicated. See Etiology for the causes of intestinal failure and the experience at the University of Miami.

Liver function test findings must be carefully evaluated for derangements that suggest the need for biopsy evaluation. If significant fibrosis, macrosteatosis, or cirrhosis is present on liver biopsy findings, then liver transplantation is necessary. Evaluation of renal function is critical because of the need for long-term use of tacrolimus postoperatively.

Relevant Anatomy

For isolated intestinal transplantation in patients with normal anatomy, the superior mesenteric artery (SMA) of the donor is procured with the aortic cuff. The portal vein is cut above the confluence of the superior mesenteric and splenic vein. If a right replaced hepatic artery from the SMA is present, the SMA is cut distal to the takeoff of the replaced hepatic artery. The SMA of the donor is anastomosed to the recipient SMA or the abdominal aorta. The portal vein of the donor is anastomosed to the portal vein or the inferior vena cava of the recipient.

For liver and intestinal or multivisceral transplantation, the celiac trunk and SMA are procured with the abdominal aorta and thoracic aorta. The inferior vena cava is procured from the iliac vein bifurcation to above the diaphragm. The anastomosis is performed between the donor abdominal aorta and the recipient abdominal aorta in an end-to-side fashion.

Contraindications

Transplantation is contraindicated if the recipient has active infection such as pneumonia, sepsis, or fungal infection. Because of the immunosuppressive medication used in the postoperative period, the infection must be controlled by antibiotics or antifungal therapy before transplantation.

More on Intestinal and Multivisceral Transplantation

Overview: Intestinal and Multivisceral Transplantation
Workup: Intestinal and Multivisceral Transplantation
Treatment: Intestinal and Multivisceral Transplantation
Follow-up: Intestinal and Multivisceral Transplantation
Multimedia: Intestinal and Multivisceral Transplantation
References
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References

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  2. Lillehei RC, Goott B, Miller FA. The physiological response of the small bowel of the dog to ischemia including prolonged in vitro preservation of the small bowel with successful replacement and survival. Ann Surg. 1959;150:543-60.

  3. Starzl TE, Kaupp HA Jr. Mass homotransplantation of abdominal organs in dogs. Surg Forum. 1960;11:28-30.

  4. Lillehei RC, Idezuki Y, Feemster JA, et al. Transplantation of stomach, intestine, and pancreas: experimental and clinical observations. Surgery. Oct 1967;62(4):721-41. [Medline].

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  7. Starzl TE, Rowe MI, Todo S. Transplantation of multiple abdominal viscera. JAMA. Mar 10 1989;261(10):1449-57. [Medline].

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Further Reading

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Keywords

intestinal transplantation, multivisceral transplantation, polysplanchnic transplantation, intestinal failure, short bowel syndrome, total parenteral nutrition, TPN, cytomegalovirus, CMV, polysplanchnic transplantation, volvulus, thrombosis, encephalopathy, tetany, calcium deficiency, magnesium deficiency, hyperalimentation, necrotizing enterocolitis, mesenteric thrombosis, gastroschisis, desmoid tumor, intestinal atresia, Hirschsprung disease, Crohn disease, pseudoobstruction, microvillus inclusion disease, central vein thrombosis, cholestatic TPN-induced liver failure, catheter-associated sepsis, catheter-associated vascular thrombosis

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Contributor Information and Disclosures

Author

Seigo Nishida, MD, PhD, Associate Director of Adult Intestinal Transplant Program, Associate Professor of Clinical Surgery, Division of Liver/GI Transplantation, Department of Surgery, University of Miami School of Medicine
Seigo Nishida, MD, PhD is a member of the following medical societies: American Society of Transplant Surgeons, Japan Surgical Society, Society for Surgery of the Alimentary Tract, and Transplantation Society
Disclosure: Nothing to disclose.

Coauthor(s)

Andreas G Tzakis, MD, PhD, Professor, Department of Surgery, Miller School of Medicine, University of Miami
Andreas G Tzakis, MD, PhD is a member of the following medical societies: American Association for the Study of Liver Diseases, American College of Angiology, American College of Surgeons, American Medical Association, American Society of Transplant Surgeons, American Spinal Injury Association, International College of Surgeons, Pennsylvania Medical Society, and Society of University Surgeons
Disclosure: Nothing to disclose.

David M Levi, MD, Associate Professor of Clinical Surgery, Department of Surgery, Division of Liver and Gastrointestinal Transplantation, Division of General Surgery, Jackson Memorial Hospital, Miller School of Medicine, University of Miami
Disclosure: Nothing to disclose.

Tomoaki Kato, MD, Associate Professor of Clinical Surgery, Department of Surgery, Division of Liver and Gastrointestinal Transplantation, Miller School of Medicine, University of Miami
Tomoaki Kato, MD is a member of the following medical societies: American Gastroenterological Association, American Medical Association, and American Society of Transplant Surgeons
Disclosure: Nothing to disclose.

Jose R Nery, MD, Associate Professor, Department of Surgery, Division of Liver and Gastrointestinal Transplantation, Miller School of Medicine, University of Miami
Disclosure: Nothing to disclose.

Juan Madariaga, MD, Professor, Department of Surgery, Miller School of Medicine, University of Miami
Juan Madariaga, MD is a member of the following medical societies: American Medical Association, American Society of Transplant Surgeons, Medical Society of the State of New York, and Society of Surgical Oncology
Disclosure: Nothing to disclose.

John F Thompson, MD, Associate Professor, Department of Pediatrics, Miller School of Medicine, University of Miami
John F Thompson, MD is a member of the following medical societies: Alpha Omega Alpha, American Gastroenterological Association, and North American Society for Pediatric Gastroenterology and Nutrition
Disclosure: Nothing to disclose.

Phillip Ruiz, Jr, MD, PhD, Professor of Pathology, Department of Pathology and Surgery, Miller School of Medicine, University of Miami
Phillip Ruiz, Jr, MD, PhD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Immunologists, American Society of Clinical Pathologists, American Society of Nephrology, American Society of Transplant Surgeons, American Society of Transplantation, Clinical Immunology Society, Florida Medical Association, New York Academy of Sciences, Pan American Medical Association, Southern Medical Association, and United States and Canadian Academy of Pathology
Disclosure: Nothing to disclose.

G Patricia Cantwell, MD, Associate Clinical Professor, Department of Pediatrics, University of Miami; Director of Pediatric Critical Care Medicine, Miller School of Medicine, Jackson Children's Hospital
G Patricia Cantwell, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Emergency Physicians, American Heart Association, American Trauma Society, National Association of EMS Physicians, Society of Critical Care Medicine, and Wilderness Medical Society
Disclosure: Nothing to disclose.

Medical Editor

Casimir F Firlit, MD, PhD, Consulting Staff, Department of Urology, Cardinal Glennon Children's Hospital
Casimir F Firlit, MD, PhD is a member of the following medical societies: American Academy of Pediatrics, American College of Surgeons, American Medical Association, American Society of Transplant Surgeons, American Urological Association, and Illinois State Medical Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine.com, Inc
Disclosure: Pfizer Inc Stock Investment from broker recommendation; Avanir Pharma Stock Investment from broker recommendation

Managing Editor

Brian F Gilchrist, MD, Chief, Division of Pediatric Surgery, Tufts-New England Medical Center; Associate Professor, Department of Surgery, Tufts University School of Medicine
Disclosure: Nothing to disclose.

CME Editor

Ron Shapiro, MD, Professor of Surgery, University of Pittsburgh; Director, Kidney, Pancreas, and Islet Transplantation, Thomas E Starzl Transplantation Institute, University of Pittsburgh Medical Center
Ron Shapiro, MD is a member of the following medical societies: American College of Surgeons, American Society of Transplant Surgeons, Association for Academic Surgery, Central Surgical Association, and Society of University Surgeons
Disclosure: Nothing to disclose.

Chief Editor

Stuart M Greenstein, MD, Professor of Surgery, Albert Einstein College of Medicine; Consulting Surgeon, Department of Surgery, Division of Transplantation, Montefiore Medical Center
Stuart M Greenstein, MD is a member of the following medical societies: American Association for the Advancement of Science, American College of Surgeons, American Society of Transplant Surgeons, American Society of Transplantation, Association for Academic Surgery, International College of Surgeons, Medical Society of New Jersey, National Kidney Foundation, New York Academy of Sciences, and Southeastern Surgical Congress
Disclosure: Nothing to disclose.

 
 
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