Medical Policy: 07.03.05
Original Effective Date: November 2009
Reviewed: November 2015
Revised: November 2015
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.
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).
Intestinal failure results from obstruction, dysmotility, surgical resection, congenital defect, or disease-associated loss of absorption and is characterized by the inability to maintain protein-energy, fluid, electrolyte, or micronutrient balance.
Short bowel syndrome results from surgical resection, congenital defect, or disease-associated loss of absorption and is characterized by the inability to maintain protein-energy, fluid, electrolyte, or micronutrient balances when on a conventionally accepted, normal diet.
Leading causes of intestinal failure differ between adult and pediatric populations. In children, the following are the leading causes of intestinal failure:
- Intestinal atresia
- Crohn disease
- Microvillus involution disease
- Necrotizing enterocolitis
- Midgut volvulus
- Chronic intestinal pseudo-obstruction
- Massive resection secondary to tumor
- Hirschsprung disease
The following are the leading causes of intestinal failure in adults:
- Crohn disease
- Superior mesenteric artery thrombosis
- Superior mesenteric vein thrombosis
- Desmoid tumor
- Massive resection secondary to tumor
- Radiation enteritis
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.
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.
A combined small bowel and liver retransplant or multivisceral retransplant may be considered medically necessary after failed primary combined small bowel and liver transplant or multivisceral transplant provided the individual meets the transplant criteria above.
Except as defined above, candidates for small bowel and liver or multivisceral transplants should meet the following general criteria:
- Adequate cardiopulmonary status
- Absence of active infection
- Documentation of patient compliance with medical management
The evaluation of a transplant candidate who has a history of cancer must consider the prognosis and risk of recurrence from available information including tumor type and stage, response to therapy, and time since therapy was completed. Although evidence is limited, patients in whom cancer is thought to be cured should not be excluded from consideration for transplant. UNOS has not addressed malignancy in current policies.
There is minimal data regarding long-term outcomes of liver transplantation in HIV-positive patients. The United Network for Organ Sharing (UNOS) believes that asymptomatic HIV-positive patients should not necessarily be excluded for candidacy for organ transplantation, stating, “A potential candidate for organ transplantation whose test for HIV is positive but who is in an asymptomatic state should not necessarily be excluded from candidacy for organ transplantation, but should be advised that he or she may be at increased risk of morbidity and mortality because of immunosuppressive therapy.” In 2001, the Clinical Practice Committee of the American Society of Transplantation proposed that the presence of AIDS could be considered a contraindication to kidney transplant unless the following criteria were present. These criteria may be extrapolated to other potential organ transplants:
- CD4 count ≥ 200 cells/mm-3 for > 6 months
- HIV-1 RNA undetectable
- On stable anti-retroviral therapy > 3 months
- No other complications from AIDS (e.g., opportunistic infection including aspergillus, tuberculosis, coccidioses mycosis, resistant fungal infections, Kaposi’s sarcoma, or other neoplasm)
- Meeting all other criteria for organ transplantation
It is likely that each individual transplant center will have explicit patient selection criteria for HIV-positive patients.
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
- 47136 Liver allotransplantation; heterotopic, partial or whole, from cadaver or living donor, any age
- S2053 Transplantation of small intestine and liver allografts
- S2054 Multivisceral transplant
- American Society of Transplantation (AST). Facts About Intestinal Transplantation. December 2006.
- 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 InstituteExternal Site; 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 ReferenceExternal Site. 2012.May:2 (14)
- American Gastroenterological AssociationExternal Site. Intestinal Transplantation for Gut Failure. June 2003. Volume 124, issue 6, pages 1516-1628.
- American Society of TransplantationExternal Site (AST). Facts About Intestinal Transplantation. December 2006.
- Siego Nishida, M.D., PhD., Pediatric Intestinal and Multivisceral Transplantation. Medscape ReferenceExternal Site. May 30, 2012.
- UpToDateExternal Site. Overview of Intestinal and Multivisceral Transplantation. Farrukh A. Khan, M.D., FACS, Gennaro Selvaggi, M.D.. Topic last updated October 18, 2012.
- Centers for Medicare & Medicaid ServicesExternal Site. 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
- NCCNExternal Site (National Comprehensive Cancer Network) Guidelines Version 2.2014 Desmoid Tumors (Aggressive Fibromatosis).
- UpToDateExternal Site. Desmoid Tumors: Systemic Therapy, Vinod Ravi, M.D., Shreyaskumar R. Patel, M.D., Topic last updated April 30, 2013.
- UpToDateExternal Site. 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 September 3, 2014.
- UpToDateExternal Site. 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 ReferenceExternal Site. Updated November 13, 2014.
- Richard K. Gilroy, MBBS, FRACP, Intestinal and Multivisceral Transplantation, Medscape ReferenceExternal Site. 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.
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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*Current Procedural Terminology © 2012 American Medical Association. All Rights Reserved.