Archived Policies - Surgery
Small Bowel/Liver and Multivisceral Transplant
A small bowel/liver transplant or multivisceral 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 small bowel/liver retransplant or multivisceral retransplant may be considered medically necessary after a failed primary small bowel/liver transplant or multivisceral transplant.
A small bowel/liver transplant or multivisceral transplant is considered experimental, investigational and/or unproven in all other situations.
Refer to SUR703.001, Organ and Tissue Transplantation for general donor and recipient information.
Small bowel/liver transplantation is transplantation of an intestinal allograft in combination with a liver allograft, either alone or in combination with one or more of the following organs: stomach, duodenum, jejunum, ileum, pancreas, or colon.
Small bowel transplants are typically performed in patients with short bowel syndrome, defined as an inadequate absorbing surface of the small intestine due to extensive disease or surgical removal of a large portion of small intestine. In some instances, short bowel syndrome is associated with liver failure, often due to the long-term complications of total parenteral nutrition (TPN). These patients may be candidates for a small bowel/liver transplant or a multivisceral transplant, which includes the small bowel and liver with 1 or more of the following organs: stomach, duodenum, jejunum, ileum, pancreas, and/or colon. A multivisceral transplant is indicated when anatomic or other medical problems preclude a small bowel/liver transplant.
Potential contraindications to solid organ transplant (subject to the judgment of the transplant center):
1. Known current malignancy, including metastatic cancer;
2. Recent malignancy with high risk of recurrence;
3. History of cancer with a moderate risk of recurrence;
4. Systemic disease that could be exacerbated by immunosuppression;
5. Untreated systemic infection making immunosuppression unsafe, including chronic infection;
6. Other irreversible end-stage disease not attributed to intestinal failure;
7. Psychosocial conditions or chemical dependency affecting ability to adhere to therapy.
Candidates should meet the following criteria:
• Adequate cardiopulmonary status;
• Documentation of patient compliance with medical management.
HIV [human immunodeficiency virus]-positive patients who meet the following criteria, as stated in the 2001 guidelines of the American Society of Transplantation, could be considered candidates for small bowel/liver or multivisceral transplantation:
• CD4 count greater than 200 cells per cubic millimeter for greater than 6 months;
• HIV-1 RNA undetectable;
• On stable anti-retroviral therapy >3 months;
• No other complications from AIDS [acquired immune deficiency syndrome] (e.g., opportunistic infection, including aspergillus, tuberculosis, coccidiosis mycosis, resistant fungal infections, Kaposi’s sarcoma, or other neoplasm), and meeting all other criteria for transplantation.
Small Bowel/Liver Specific
Intestinal failure 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 balance (adapted from reference 1). (1) Short bowel syndrome is one case of intestinal failure.
Evidence of intolerance of total parenteral nutrition (TPN) includes, but is not limited to, the following:
• Multiple and prolonged hospitalizations to treat TPN-related complications; or
• Development of progressive but reversible liver failure.
In the setting of progressive liver failure, small bowel transplant may be considered a technique to avoid end-stage liver failure related to chronic TPN, thus avoiding the necessity of a multivisceral transplant.
The policy was originally created in 1996 and updated with searches of the MedLine database. The most recent literature search was conducted through August 2015.
A 1999 Blue Cross Blue Shield Association (BCBSA) Technology Evaluation Center (TEC) Assessment focused on multivisceral transplantation and offered the following conclusions: “Multivisceral transplantation in patients with small bowel syndrome, liver failure, and/or other gastrointestinal problems such as pancreatic failure, thromboses of the celiac axis and the superior mesenteric artery, or pseudo-obstruction affecting the entire gastrointestinal tract associated with poor patient and graft survival. Pediatric and adult patients have a similar 2- and 5-year survival of 33–50%. However, without this procedure, it is expected that these patients would face 100% mortality.” (2)
The published literature consists of case series, mainly reported by single centers. Authors of these reports, as well as reviews, observe that while outcomes continue to improve, recurrent and chronic rejection and complications of immunosuppression continue to be obstacles to long-term survival.
In 2010, Nayyar et al. reported that there had been improvements in 5-year actuarial patient and graft survival after liver/small bowel transplant since the use of rabbit antithymocyte globulin (rATG) induction began to be used in their pediatric center in 2002 (81% vs. 58% and 76% vs. 52%, respectively). (3) In addition to innovations in immunosuppressive therapy, the authors cited new approaches to management of short gut syndrome including hypoallergenic formulas and modification of enteral nutrition to prevent total parenteral nutrition (TPN)-induced cholestasis. The authors noted that better understanding of the protective role of the liver in preventing chronic rejection of the small bowel allograft could improve long-term survival after isolated small bowel transplantation.
Other survival data include a 2009 report by Abu-Elmagd et al. reporting on their experience with 500 intestinal and multivisceral transplantations. (4) The study found 1- and 5-year patient survival of 92% and 70%, respectively. A 2013 study from a single center in Sweden included 30 patients accepted for intestinal and multivisceral transplantation. (5) One- and 3-year survival rates were 68% and 61%, respectively. Among patients awaiting transplantation after being accepted as candidates, there was a 34% survival rate. In 2013, Mangus et al. reported on 95 patients who underwent multivisceral transplantation with or without liver transplantation at one site in the U.S. (6) One-year patient survival was 72% and 3-year survival was 57%. The authors noted a learning curve, with a 48% survival rate for transplants performed between 2004 and 2007 and a 70% survival rate for operations between 2008 and 2010.
Several case series have focused on complications after small bowel and multivisceral transplantation. For example, in 2011 Wu et al. reported on 241 patients who underwent intestinal transplantation. (7) Of these, 147 (61%) had multivisceral transplants, 65 (27%) had small bowel transplants, and 12% had small bowel/liver transplants. There were 151 children (63%) and 90 adults. A total of 22 patients (9%) developed graft-versus-host disease (GVHD). Children younger than 5-years-old were more likely to develop GVHD; the incidence in this age group was 16 of 121 (13.2%) compared to 2 of 30 (6.7%) in children between 5 and 18 years and 9 of 90 (4.4%) in adults older than 18 years. In addition, a 2012 article retrospectively reported on bloodstream infections among 98 children younger than age 18 years with small bowel/combined organ transplants. (8) Seventy-seven (79%) patients underwent small bowel transplant in combination with a liver, kidney or kidney-pancreas, and 21 had an isolated small bowel transplant. After a median follow-up of 52 months, 58 (59%) patients remained alive. The 1-year survival rate was similar in patients with combined small bowel transplant (75%) and those with isolated small bowel transplant (81%). In the first year after transplantation, 68 patients (69.4%) experienced at least one episode of bloodstream infection. The 1-year survival rate for patients with bloodstream infections was 72% compared to 87% in patients without bloodstream infections (p value= .056 for difference in survival in patients with and without bloodstream infections).
A 2014 single-center Italian case series reported on transplants in 45 patients who received either intestinal transplants alone or combined transplant procedures. (9) Twelve of the patients had small bowel/multivisceral transplants. Five of these had the procedure due to short-bowel syndrome, 2 had chronic intestinal pseudo-obstruction, and 5 had Gardner syndrome. Survival rates for the entire patient population were 77% at 1 year, 58% at 3 years, 53% at 5 years, and 37% at 10 years.
HIV-Positive Transplant Recipients
This subgroup of recipients has long been controversial, due to the long-term prognosis for HIV positivity and the impact of immunosuppression on HIV disease. Although HIV-positive transplant recipients may be a research interest of some transplant centers, the minimal data regarding long-term outcome in these patients primarily consist of case reports and abstract presentations of liver and kidney recipients. Nevertheless, some transplant surgeons would argue that HIV positivity is no longer an absolute contraindication to transplant due to the advent of highly active antiretroviral therapy (HAART), which has markedly changed the natural history of the disease. 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. (10) These criteria may be extrapolated to other organs:
• CD4 count greater than 200 cells per cubic millimeter for more than 6 months;
• HIV-1 RNA undetectable;
• On stable antiretroviral therapy for more than 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 transplantation.
In 2006, the British HIV Association and the British Transplantation Society Standards Committee published guidelines for kidney transplantation in patients with HIV disease. (11) As described above, these criteria may be extrapolated to other organs.
The guidelines, which are similar to those cited here, recommend that any patient with end-stage organ disease with a life expectancy of at least 5 years is considered appropriate for transplantation under the following conditions:
• CD4 greater than 200 cells/mL for at least 6 months;
• Undetectable HIV viremia (<50 HIV-1 RNA copies/mL) for at least 6 months;
• Demonstrable adherence and a stable HAART regimen for at least 6 months;
• Absence of AIDS-defining illness following successful immune reconstitution after HAART.
Furthermore, as of November 2010, the United Network for Organ Sharing (UNOS) policy on identification of transmissible diseases in organ recipients 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 to transplantation based on local policy.” (12)
No studies that reported on outcomes in HIV-positive patients who received small bowel/liver or multivisceral transplants have been identified in literature searches.
In 2012, Trevizol et al. published a review of literature from the previous 5 years on intestinal and multivisceral retransplantation. (13) The authors found articles from two centers. Mazariegos et al. reported on 15 retransplantations in 14 pediatric patients. (14) By the end of follow-up, 4 patients had died and 10 patients had a normal graft function. TPN was weaned at a mean of 32 days after retransplantation. A 2009 study by Abu-Elmagd et al., discussed earlier, (4) reported 47 retransplants after 500 intestinal and multivisceral transplantations in adults and children Included were 31 intestinal retransplants, 9 multivisceral retransplants and 7 intestinal/liver retransplants. For all types of retransplants combined, there is a 5-year survival rate of 47% for all retransplants.
Desai et al. reported intestinal retransplantation data from the Organ Procurement and Transplant Network (OPTN) database. (15) Between October 1987 and August 2009, there were 31 cases of small bowel/liver retransplants in adults and 49 in children. Among adults, 1-, 3- and 5-year survival rates after retransplantation were 63.1%, 56.1% and 46.8%, respectively. This compares to survival rates after primary small bowel/liver transplants of 67%, 53.3% and 46% at 1-, 3- and 5-years. Among children, there was a 42.1% survival rate at 1-, 3- and 5 years after retransplantation. Survival rates after primary small bowel/liver transplantation was 67.6%, 56.1% and 51.4%, respectively.
Ongoing and Unpublished Clinical Trials
A search of ClinicalTrials.gov in August 2015 did not identify any ongoing or unpublished trials that would likely influence this coverage.
Practice Guidelines and Position Statements
No relevant guidelines or position statements were identified.
Evidence for 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 total parenteral nutrition and who have developed evidence of impending end-stage liver failure.
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Procedure and diagnosis codes on Medical Policy documents are included only as a general reference tool for each policy. They may not be all-inclusive.
The presence or absence of procedure, service, supply, device or diagnosis codes in a Medical Policy document has no relevance for determination of benefit coverage for members or reimbursement for providers. Only the written coverage position in a medical policy should be used for such determinations.
Benefit coverage determinations based on written Medical Policy coverage positions must include review of the member’s benefit contract or Summary Plan Description (SPD) for defined coverage vs. non-coverage, benefit exclusions, and benefit limitations such as dollar or duration caps.
The following codes may be applicable to this Medical policy and may not be all inclusive.
44120, 44121, 44132, 44133, 44135, 44136, 44137, 44715, 44720, 44721, 44799, 47133, 47135, 47140, 47141, 47142, 47143, 47144, 47145, 47146, 47147, [Deleted 1/2016: 47136]
S2053, S2054, S2055, S2152
ICD-9 Diagnosis Codes
Refer to the ICD-9-CM manual
ICD-9 Procedure Codes
Refer to the ICD-9-CM manual
ICD-10 Diagnosis Codes
Refer to the ICD-10-CM manual
ICD-10 Procedure Codes
Refer to the ICD-10-CM manual
The information contained in this section is for informational purposes only. HCSC makes no representation as to the accuracy of this information. It is not to be used for claims adjudication for HCSC Plans.
The Centers for Medicare and Medicaid Services (CMS) does have a national Medicare coverage position.
A national coverage position for Medicare may have been changed since this medical policy document was written. See Medicare's National Coverage at <http://www.cms.hhs.gov>.
1. O'Keefe SJ, Buchman AL, Fishbein TM, et al. Short bowel syndrome and intestinal failure: consensus definitions and overview. Clin Gastroenterol Hepatol. 2006; 4(1):6-10. PMID 1641298
2. Small bowel transplants in adults and multivisceral transplants in adults and children. Blue Cross and Blue Shield Technology Evaluation Center (TEC) Program Assessments: Chicago, Illinois. 1999; Volume 14, Tab 9.
3. Nayyar N, Mazariegos G, Ranganathan S, et al. Pediatric small bowel transplantation. Semin Pediatr Surg. 2010; 19(1):68-77. PMID 20123276
4. 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. 2009; 250(4):567-81. PMID 19730240
5. Varkey J, Simren M, Bosaeus I, et al. Survival of patients evaluated for intestinal and multivisceral transplantation - the Scandinavian experience. Scand J Gastroenterol. 2013; 48(6):702-11. PMID 23544434
6. Mangus RS, Tector AJ, Kubal CA, et al. Multivisceral transplantation: expanding indications and improving outcomes. J Gastrointest Surg. 2013; 17(1):179-86; discussion p 86-7. PMID 23070622
7. Wu G, Selvaggi G, Nishida S, et al. Graft-versus-host disease after intestinal and multivisceral transplantation. Transplantation. 2011; 91(2):219-24. PMID 21076376
8. Florescu DF, Qiu F, Langnas AN, et al. Bloodstream Infections during the First Year after Pediatric Small Bowel Transplantation. Pediatr Infect Dis J. 2012; 31(7): 700-4. PMID 22466325
9. Lauro A, Sanfi C, Dassi A, et al. Disease-related intestinal transplant in adults: results from a single center. Transplat Proc. Jan-Feb 2014; 46(1):245-8. PMID 24507060
10. Steinman TI, Becker BN, Frost AE et al. Guidelines for the referral and management of patients eligible for solid organ transplantation. Transplantation. 2001; 71(9):1189-204. PMID 11397947
11. Bhagani S, Sweny P, Brook G. Guidelines for kidney transplantation in patients with HIV disease. HIV Med. 2006; 7(3):133-9. PMID 16494626
12. United Network for Organ Sharing. Identification of transmissible diseases in organ recipients. Available online at: <www.optn.transplant.hrsa.gov> (accessed August 2015).
13. Trevizol AP, David AI, Yamashita ET, et al. Intestinal and multivisceral retransplantation results: literature review. Transplant Proc. 2013; 45(3):1133-6. PMID 23622645
14. Mazariegos GV, Soltys K, Bond G, et al. Pediatric intestinal retransplantation: techniques, management, and outcomes. Transplantation. 2008; 86(12):1777-82. PMID 19104421
15. Desai CS, Khan KM, Gruessner AC, et al. Intestinal retransplantation: analysis of Organ Procurement and Transplantation Network database. Transplantation. 2012; 93(1):120-5. PMID 22113492
16. Small Bowel/Liver and Multivisceral Transplant. Chicago, Illinois: Blue Cross Blue Shield Association Medical Policy Reference Manual (2015 June) Surgery: 7.03.05.
|11/1/2016||Reviewed. No changes.|
|2/1/2016||Document updated with literature review. The following was added: A small bowel/liver transplant or multivisceral transplant is considered experimental, investigational and/or unproven in all other situations.|
|2/1/2014||Document updated with literature review. The following changes were made to coverage: 1) “short bowel syndrome” changed to “intestinal failure”. 2) Intestinal failure defined. 3) A small bowel/liver retransplant or multivisceral retransplant may be considered medically necessary after a failed primary small bowel/liver transplant or multivisceral transplant. Title changed from: Liver, Small Bowel, and Multivisceral Transplants. CPT/HCPCS codes updated.|
|4/1/1996||New medical document|
|Title:||Effective Date:||End Date:|
|Small Bowel/Liver and Multivisceral Transplant||06-01-2017||10-14-2018|
|Small Bowel/Liver and Multivisceral Transplant||11-01-2016||05-31-2017|
|Small Bowel/Liver and Multivisceral Transplant||02-01-2016||10-31-2016|
|Small Bowel/Liver and Multivisceral Transplant||02-01-2014||01-31-2016|
|Liver, Small Bowel, and Multivisceral Transplants||07-01-2004||01-31-2014|
|Liver-Small Bowel and Multivisceral Transplant||03-01-2000||06-30-2004|