BlueCross and BlueShield of Montana Medical Policy/Codes
Small Bowel/Liver and Multivisceral Transplant
Chapter: Transplant
Current Effective Date: February 01, 2014
Original Effective Date: September 04, 2009
Publish Date: January 15, 2014
Revised Dates: August 13, 2012; September 27, 2013; January 15, 2014
Description

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.

Background

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):

General

  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

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.

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

Evidence of intolerance of total parenteral nutrition (TPN) includes, but is not limited to, multiple and prolonged hospitalizations to treat TPN-related complications, or the 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.

Policy

Each benefit plan or contract defines which services are covered, which are excluded, and which are subject to dollar caps or other limits.  Members and their providers have the responsibility for consulting the member's benefit plan or contract to determine if there are any exclusions or other benefit limitations applicable to this service or supply.  If there is a discrepancy between a Medical Policy and a member's benefit plan or contract, the benefit plan or contract will govern.

Coverage

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.

Rationale

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 and colleagues 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 and colleagues 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 and colleagues 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 and colleagues 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).

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. (9) 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. (10) 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.” (11)

No studies that reported on outcomes in HIV-positive patients who received small bowel/liver or multivisceral transplants have been identified in literature searches.

Retransplantation

In 2012, Trevizol and colleagues published a review of literature from the previous 5 years on intestinal and multivisceral retransplantation. (12) The authors found articles from two centers. Mazariegos and colleagues reported on 15 retransplantations in 14 pediatric patients. (13) 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 and colleagues, 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 and colleagues reported intestinal retransplantation data from the Organ Procurement and Transplant Network (OPTN) database. (14) 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.

Summary

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.

Coding

Disclaimer for coding information on Medical Policies           

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. 

Rationale for Benefit Administration

 

ICD-9 Codes

45.63, 46.97, 50.59, 570, 572.8, 579.3

ICD-10 Codes

K72.00-K72.01, K72.10-K72.11, K91.2, 0DY60Z0, 0DY80Z0, 0DYE0Z0, 0FY00Z0, 0FYG0Z0

Procedural Codes: 44132, 44133, 44135, 44136, 44137, 47133, 47135, 47136, 47140, 47141, 47142, S2053, S2054, S2055, S2152
References
  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.
  2. Blue Cross and Blue Shield Technology Evaluation Center (TEC). Small bowel transplants in adults and multivisceral transplants in adults and children. TEC Assessments 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.
  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.
  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.
  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.
  7. Wu G, Selvaggi G, Nishida S et al. Graft-versus-host disease after intestinal and multivisceral transplantation. Transplantation 2011; 91(2):219-24.
  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; In Press.
  9. 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.
  10. Bhagani S, Sweny P, Brook G. Guidelines for kidney transplantation in patients with HIV disease. HIV Med 2006; 7(3):133-9.
  11. United Network for Organ Sharing. Identification of transmissible diseases in organ recipients. Available online at: www.optn.transplant.hrsa.gov . Last accessed April, 2012.
  12. Trevizol AP, David AI, Yamashita ET et al. Intestinal and multivisceral retransplantation results: literature review. Transplant Proc 2013; 45(3):1133-6.
  13. Mazariegos GV, Soltys K, Bond G et al. Pediatric intestinal retransplantation: techniques, management, and outcomes. Transplantation 2008; 86(12):1777-82.
  14. Desai CS, Khan KM, Gruessner AC et al. Intestinal retransplantation: analysis of Organ Procurement and Transplantation Network database. Transplantation 2012; 93(1):120-5.
  15. Small Bowel/Liver and Multivisceral Transplant. Chicago, Illinois: Blue Cross Blue Shield Association Medical Policy Reference Manual (2013 June) Surgery: 7.03.05.
History
August 2012  Policy updated with literature review; reference numbers 4 and 5 added; other references renumbered/removed; rationale section extensively edited; policy statement unchanged
October 2013 Policy formatting and language revised.  Policy statement unchanged.  Title changed from "Transplant: Small Bowel/Liver and Multivisceral Transplant" to "Liver, Small Bowel, and Multivisceral Transplants".
February 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.
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Small Bowel/Liver and Multivisceral Transplant