Medical Policy: 07.03.05
Original Effective Date: November 2009
Reviewed: November 2017
Revised: November 2016
Benefit determinations are based on the applicable contract language in effect at the time the
services were rendered. Exclusions, limitations or exceptions may apply. Benefits may vary
based on contract, and individual member benefits must be verified. Wellmark determines medical
necessity only if the benefit exists and no contract exclusions are applicable. This medical
policy may not apply to FEP. Benefits are determined by the Federal Employee Program.
This Medical Policy document describes the status of medical technology at the time the document
was developed. Since that time, new technology may have emerged or new medical literature may
have been published. This Medical Policy will be reviewed regularly and be updated as scientific
and medical literature becomes available.
This policy addresses transplantation of the intestinal allograft in combination with liver allograft, either alone or in combination with one or more of the following organs: stomach, duodenum, jejunum, ileum, pancreas or colon.
Combined Small Bowel and Liver Transplant
Combined small bowel and liver transplant is generally indicated when intestinal failure is accompanied by irreversible liver failure, usually due to the long term complications of total parenteral nutrition (TPN).
Some conditions are more closely associated with pediatric intestinal failure while others are more common with intestinal failure in adults.
The following are pediatric conditions causing intestinal failure:
- Short bowel syndrome following extensive bowel surgeries (midgut volvulus)
- Congenital malformations (e.g. intestinal atresia, gastroschisis, aganglionosis)
- Absorptive impairment (e.g. microvillus involution disease, chronic intestinal pseudo-obstruction)
- Infections of gastrointestinal tract (e.g. necrotizing enterocolitis)
The following are the leading causes of intestinal failure in adults:
- Crohn’s disease
- Tumors of the mesenteric root and retroperitoneum (e.g. desmoid tumor)
- Short bowl syndrome following extensive surgeries secondary to mesenteric ischemia (following thrombosis, embolism, volvulus or trauma)
- Chronic intestinal pseudo-obstruction
- Small bowel tumors such as Gardner’s Syndrome (familial colorectal polyposis)
The chronic use of TPN is often associated with life-threatening complication including:
- Catheter related sepsis
- Catheter related thrombosis
- Severe dehydration
- Parenteral nutrition associated liver disease (PNALD)
Multivisceral transplant is generally indicated when anatomic or other medical problems preclude a small bowel/liver transplant when intestinal failure is accompanied by liver failure, usually due to complications of long term total parenteral nutrition (TPN). In addition, the patient usually has a history surgery, abdominal trauma, motility disorders, tumor or other etiologies that warrant the multivisceral transplant. The multivisceral transplant can include the liver, stomach, small intestine (duodenum, jejunum and ileum), pancreas and/or colon. Kidney transplant may also be included if the recipient has end stage renal failure.
The most common causes of graft loss may include acute and chronic rejection, post- transplant lymphoproliferative disorder, graft dysmotility or dysfunction, severe infection, arterial graft aneurysm or allograft liver failure. Careful patient selection, post-transplant immunosuppression and patient management are essential for successful long-term outcomes. Individuals undergoing retransplantgation for small bowel and liver or multivisceral transplantation should meet all of the eligibility criteria for primary transplantation and should not have contraindications to transplantation.
Evidence for combined small bowel/liver and multivisceral transplant and retransplant consists of case series. Though infrequently performed, the transplant procedures are demonstrated to provide a survival benefit, and the procedure is considered medically necessary for patients who have been managed with long-term parenteral nutrition and who have developed evidence of impending end stage liver failure.
Practice Guideline and Position Statement
In 2003, the American Gastroenterological Association produced a medical position statement on short bowel syndrome and intestinal transplantation. It recommends dietary, medical and surgical solutions. Indications for intestinal transplant mirror those of Medicare in patients who fail TPN therapy for one of the following reasons:
- Impending or overt liver failure as evidence by elevated serum bilirubin and/or liver enzymes, splenomegaly, thrombocytopenia, gastroesophageal varices, coagulopathy, stomal bleeding or hepatic fibrosis/cirrhosis
- Thromobosis of major central venous channels (2 thromboses in subclavian, jugular or femoral veins)
- Frequent central line related sepsis (2 episodes of systemic sepsis secondary to line infection per year, 1 episode of line related fungemia, septic shock or acute respiratory distress syndrome (ARDS))
- Frequent severe dehydration
Until better data become available, these parameters are likely to be widely recognized as the indications for intestinal transplantation.
American Society of Transplantation
In 2001, the American Society of Transplantation issued a position paper on indications for pediatric intestinal transplantation. The position paper included the following:
“Parenteral nutrition represents standard therapy for children with short bowel syndrome and other causes of intestinal failure. Most infants with short bowel syndrome eventually wean from parenteral nutrition, and most of those who do not wean tolerate parenteral nutrition for protracted periods. However, a subset of children with intestinal failure remaining dependent on parenteral nutrition will develop life-threatening complications arising from therapy. Intestinal transplantation can now be recommended for this select group. Life-threatening complications warranting consideration of intestinal transplantation include parenteral nutrition-associated liver disease, recurrent sepsis, and threatened loss of central venous access. Children with liver dysfunction should be considered for isolated intestinal transplantation before irreversible, advanced bridging fibrosis or cirrhosis supervenes, for which a combined liver and intestinal transplant is necessary. Irreversible liver disease is suggested by hyperbilirubinemia persisting beyond 3-4 months of age combined with features of portal hypertension such as splenomegaly, thrombocytopenia, or prominent superficial abdominal veins; esophageal varices, ascites, and impaired synthetic function are not always present.”
Organ Procurement and Transplantation Network (OPTN)/United Network for Organ Sharing (UNOS)
As of February 2013, the United Network for Organ Sharing (UNOS) policy on HIV-positive transplant candidates states “A potential candidate for organ transplantation whose test for HIV is positive should not be excluded from candidacy for organ transplantation unless there is a documented contraindication in transplantation based on local policy.”
As of October 2016, the Organ Procurement and Transplantation Network policy states “ To be eligible for an organ transplant, potential candidates must be tested for human immunodeficiency virus (HIV), hepatitis B, and hepatitis C, unless the testing would violate state or federal laws. Potential candidates who test positive for HIV, hepatitis B, or hepatitis C must be offered appropriate counseling.
OPTN permits HIV test positive individuals as organ candidates if permitted by the transplant hospital. Care of HIV positive organ candidate and recipients must not deviate from general medical practice.
Prior approval is required. Submit a prior approval now.
A combined small bowel and liver transplant may be considered medically necessary for pediatric and adult patients with intestinal failure (characterized by loss of absorption and the inability to maintain protein-energy, fluid, electrolyte, or micronutrient balance) who have been managed with long-term total parenteral nutrition (TPN) and who have developed evidence of impending end-stage liver failure.
A multivisceral transplant may be considered medically necessary for pediatric and adult patients who meet criteria above for the combined small bowel and liver transplant and require 1 or more abdominal visceral organs to be transplanted due to concomitant organ failure or anatomical abnormalities that preclude a small bowel and liver transplant.
A combined small bowel and liver transplant or multivisceral transplant for pediatric and adult patients performed for any other conditions not listed above will be considered not medically necessary.
Retransplantation in individuals with graft failure of a combined small bowel and liver transplant or multivisceral retransplant due to technical reasons, hyperacute or chronic rejection, or return of disease may be considered medically necessary if the transplant criteria above have been met.
Potential contraindications to solid organ transplant (subject to judgement of the transplant center):
- Known current malignancy, including metastatic cancer
- Recent malignancy with high risk of recurrence
- History of cancer with moderate risk of recurrence
- Untreated systemic infection making immunosuppression unsafe, including chronic infection
- Other irreversible end stage disease not attributed to intestinal failure
- Systemic disease that could be exacerbated by immunosuppression
- Psychosocial conditions or chemical dependency affecting ability to adhere to therapy
The Organ Procurement Transplant Network (OPTN) policy 15.2 permits HIV test positive individuals as organ candidates if permitted by the transplant hospital. Care of the HIV test positive organ candidate and recipients should not deviate from general medical practice.
Procedure Codes and Billing Guidelines:
- To report provider services, use appropriate CPT* codes, Modifiers, Alpha Numeric (HCPCS level 2) codes, Revenue codes, and/or diagnosis codes.
- 44135 Intestinal allotransplantation; from cadaver donor
- 47135 Liver allotransplantation; orthotopic, partial or whole, from cadaver or living donor, any age
- 47399 Unlisted procedure, liver (this code is used to represent liver allotransplantation; heterotopic, partial or whole, from cadaver or living donor, any age)
- S2053 Transplantation of small intestine and liver allografts
- S2054 Multivisceral transplant
- Abu-Elmagd KM. Intestinal transplantation for short bowel syndrome and gastrointestinal failure: current consensus, rewarding outcomes, and practical guidelines. Gastroenterology. 2006 Feb; 130(2 Suppl 1): S132-7.
- American Gastroenterological Association. American Gastroenterological Association medical position statement: short bowel syndrome and intestinal transplantation. Gastroenterology. 2003 Apr;124(4):1105-10.
- ECRI Institute. Hotline Response [database online]. Plymouth Meeting (PA): ECRI Institute 2008 Oct 21. Intestine and Intestine-Liver Transplantation.
- Kato T, Tzakis AG, Selvaggi G et al. Intestinal and multivisceral transplantation in children. Ann Surg. 2006 Jun;243(6):756-64.
- Bhagani S, Sweny P, Brook G; British HIV Association. Guidelines for kidney transplantation in patients with HIV disease. HIV Med. 2006; 7(3):133-9.
- Sudan DL. Treatment of intestinal failure: intestinal transplantation. Nat Clin Pract Gastroenterol Hepatol. 2007 Sep;4(9):503-10.
- Pironi L, Forbes A, Joly F et al. Survival of patients identified as candidates for intestinal transplantation: a 3-year prospective follow-up. Gastroenterology. 2008 Jul;138(1):61-71.
- Tzvetanov IG, Oberholzer J, Benedetti E. Current status of living donor small bowel transplantation. Curr Opin Organ Transplant. 2010 Jun;15(3):346-8.
- Gangemi A, Tzvetanov IG, Beatty E et al. Lessons learned in pediatric small bowel and liver transplantation from living-related donors. Transplantation. 2009 Apr 15;87(7):1027-30.
- Gilroy R., Shapiro R., Intestinal and multivisceral transplantation. Medscape Reference 2012.May:2 (14)
- American Gastroenterological Association Intestinal Transplantation for Gut Failure. June 2003. Volume 124, issue 6, pages 1516-1628.
- American Society of Transplantation (AST). Facts About Intestinal Transplantation. December 2006.
- Siego Nishida, M.D., PhD., Pediatric Intestinal and Multivisceral Transplantation. Medscape Reference May 30, 2012.
- UpToDate. Overview of Intestinal and Multivisceral Transplantation. Farrukh A. Khan, M.D., FACS, Gennaro Selvaggi, M.D.. Topic last updated January 8, 2016.
- Centers for Medicare & Medicaid Services National Coverage Determination (NCD) for Intestinal and Multi-Visceral Transplantation (260.5).
- Andreas G. Tzakis, M.D., PhD, et. al. 100 Multivisceral Transplants at a Single Center, Ann Surg 2005; 242:480-493
- Matthew Wheeler, David Mercer, et. al. Department of Surgery, University of Nebraska Medical Center, Surgical Treatment of Intra-Abdominal Desmoid Tumors Resulting in Short Bowel Syndrome. Cancers 2012, 4, 31-38; doi:10.3390/cancers4010031
- NCCN (National Comprehensive Cancer Network) Guidelines Version 2.2014 Desmoid Tumors (Aggressive Fibromatosis).
- UpToDate. Desmoid Tumors: Systemic Therapy, Vinod Ravi, M.D., Shreyaskumar R. Patel, M.D., Topic last updated April 30, 2013.
- UpToDate. Desmoid Tumors: Epidemiology, Risk Factors, Molecular Pathogenesis, Clinical Presentation and Local Therapy, Vinod Ravi, M.D., Shreyaskumar R. Patel, M.D., Chandrajit P. Raut, M.D., MSc, FACS, Thomas F. DeLaney, M.D., Topic last updated April 25, 2016.
- UpToDate. Overview of Intestinal and Multivisceral Transplantation. Farrukh A. Khan, M.D., FACS, Gennaro Selvaggi, M.D., Topic last updated June 4, 2015.
- Siego Nishida, M.D., PhD., Pediatric Intestinal and Multivisceral Transplantation, Medscape Reference Updated November 13, 2014.
- Richard K. Gilroy, MBBS, FRACP, Intestinal and Multivisceral Transplantation, Medscape Reference Updated April 7, 2015.
- Yildix Dogu Baris, Where Are We at With Short Bowel Syndrome and Small Bowel Transplant? World Journal of Transplantation, 2012 December 24;296):95-103
- O’Keefe SJ, Buchman A, Fishbein TM. et.al. Short Bowel Syndrome and Intestinal Failure: Consensus Definitions and Overview. Clin Gastroenterol Hepatol. Jan 2006;4(1):6-10
- Abu-Elmagd KM, Costa G, Bond GJ, et. al. Five Hundred Intestinal and Multivisceral Transplantations at a Single Center: Major Advances with New Challenges. Ann Surg. Oct2009;250(4):567-581
- Mangus RS, Tector AJ, Kubal CA, et. al. Multivisceral Transplantation: Expanding Indications and Improving Outcomes. J Gastrointest Surg. Jan 2013;17(1):179-186
- Trevizol AP, David AI, Yamashita ET. et.al. Intestinal and Multivisceral Retransplantation Results: Literature Review. Transplant Proc. Apr 2013;45(3):1133-1136Abu-Elmagd KM. Intestinal transplantation for short bowel syndrome and gastrointestinal failure: current consensus, rewarding outcomes, and practical guidelines. Gastroenterology. 2006 Feb; 130(2 Suppl 1): S132-7.
- Lauro A, Zanfi C. Dazzi A, et. al. Disease-related intestinal transplant in adults: results from a single center. Transplant Proc. Jan-Feb 2014;46(1):245-248. PMID 24507060
- Organ Procurement and Transplantation Network (OPTN) Policy 9 Allocation of Livers and Liver-Intestines. Effective October 2016.
- Organ Procurement and Transplantation Network (OPTN) Policy 15 Identification of Transmissible Diseases Effective October 2016.
- Blumberg E.A., Rogers C.C. The American Society of Transplantation Infectious Diseases Guidelines 3rd Edition, Human Immunodeficiency Virus in Solid Organ Transplantation. American Journal of Transplantation 2013 Volume 13, Issue s4, pages 169-178.
- UpToDate. Chronic intestinal pseudo-obstruction. Michael Camilleri M.D.. Topic last updated July 18, 2016.
- UpToDate. Management of short bowel syndrome in adults. Jon A. Vanderhoof M.D., Rosemary J Pauley-Hunter NP-C, MS, RN. Topic last updated August 21, 2014.
- UpToDate. Management of short bowel syndrome in children. Danielle A. Stamm R.N., MSN, FNP-BC, Christopher Duggan M.D., MPH. Topic last updated September 9, 2016.
- UpToDate. Gardner Syndrome. Randall W. Burt M.D.. Topic last updated January 6, 2015.
- Lacaille F, Irtan S, Dupic L, et. al. Twenty-eight years of intestinal transplantation in Paris: experience of the oldest European center. Transpl Int. Feb 2017;30(2):178-186. PMID 27889929
- Bharadwaj S, Tandon P, Gohel TD, et al. Current status of intestinal and multivisceral transplantation. Gastroenterol Rep (Oxf). Jan 26 2017. PMID 28130374
- Loo L, Vrakas G, Reddy S, et al. Intestinal transplantation: a review. Curr Opin Gastroenterol. May 2017;33(3):203-211. PMID 28282321
- Dore M, Junco PT, Andres AM, et al. Surgical rehabilitation techniques in children with poor prognosis short bowel syndrome. Eur J Pediatr Surg. Feb 2016;26(1):112-116. PMID 26535775
- Rutter CS, Amin I, Russell NK, et al. Adult intestinal and multivisceral transplantation: experience from a single center in the United Kingdom. Transplant Proc. Mar 2016;48(2):468-472. PMID 27109980
- Garcia Aroz S, Tzvetanov I, Hetterman EA, et al. Long-term outcomes of living-related small intestinal transplantation in children: A single-center experience. Pediatr Transplant. Jun 2017;21(4). PMID 28295952
- Nagai S, Mangus RS, Anderson E, et al. Cytomegalovirus infection after intestinal/multivisceral transplantation: a single-center experience with 210 cases. Transplantation. Feb 2016;100(2):451-460. PMID 26247555
- Timpone JG, Yimen M, Cox S, et al. Resistant cytomegalovirus in intestinal and multivisceral transplant recipients. Transpl Infect Dis. Apr 2016;18(2):202-209. PMID 26853894
- Wu GS, Cruz RJ, Jr., Cai JC. Acute antibody-mediated rejection after intestinal transplantation. World J Transplant. Dec 24 2016;6(4):719-728. PMID 28058223
- Cromvik J, Varkey J, Herlenius G, et al. Graft-versus-host disease after intestinal or multivisceral transplantation: a Scandinavian single-center experience. Transplant Proc. Jan-Feb 2016;48(1):185-190. PMID 26915866
- November 2017- Annual Review, Policy Renewed
- November 2016- Annual Review, Policy Revised
- November 2015 - Annual Review, Policy Revised
- December 2014 - Annual Review, Policy Revised
- February 2014 - Annual Review, Policy Revised
- March 2013 - Annual Review, Policy Revised
- March 2012 - Annual Review, Policy Renewed
- April 2011 - Annual Review, Policy Revised
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