Latest Review Date: September 2024

Category: Surgery                                                                  

POLICY:

A small bowel/liver transplant or multivisceral transplant may be considered medically necessary for coverage for pediatric and adult individuals 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 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 for coverage after a failed primary small bowel/liver transplant or multivisceral transplant.

A small bowel/liver transplant or multivisceral transplant in all other situations is considered investigational.

POLICY GUIDELINES:

Potential contraindications for solid organ transplant that are subject to the judgment of the transplant center include the following:

• Known current malignancy, including metastatic cancer;
• Recent malignancy with a high incidence of recurrence;
• History of cancer with a moderate risk of recurrence;
• Systemic disease that could be exacerbated by immunosuppression;
• Untreated systemic infection making immunosuppression unsafe, including chronic infection;
• Other irreversible end-stage disease not attributed to intestinal failure; OR
• 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. Short bowel syndrome is an example of intestinal failure.

Candidates should meet the following criteria:

• Adequate cardiopulmonary status
• Documentation of individuals compliance with medical management

Small Bowel/Liver Specific

Evidence of intolerance to TPN includes, but 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 would thus avoid the necessity of a multivisceral transplant.

See policy #479 for Small Bowel Transplantation.

DESCRIPTION OF PROCEDURE OR SERVICE:

Solid organ transplantation offers a treatment option for individuals with different types of end-stage organ failure that can be lifesaving or provide significant improvements to an individual’s quality of life. Many advances have been made in the last several decades to reduce perioperative complications. Available data supports improvement in long-term survival as well as improved quality of life particularly for liver, kidney, pancreas, heart, and lung transplants. Allograft rejection remains a key early and late complication risk for any organ transplantation. Transplant recipients require life-long immunosuppression to prevent rejection. Individuals are prioritized for transplant by mortality risk and severity of illness criteria developed by Organ Procurement and Transplantation Network and United Network of Organ Sharing.

Small Bowel/Liver and Multivisceral Transplant

In 2023, 46,629 transplants were performed in the United States procured from 39,679 deceased donors and 6,950 living donors. Intestinal transplants occur less often than other organ transplants, with 10 or fewer individuals receiving liver-intestine transplant each year from 2008 to 2019. Small bowel and liver or multivisceral transplant is usually considered in adults and children who develop serious complications related to parenteral nutrition, including inaccessibility (e.g., due to thrombosis) of access sites, catheter-related sepsis, and cholestatic liver disease.

Short Bowel Syndrome

Small bowel transplants are typically performed in individuals 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.

Treatment

A small bowel/liver transplant or a multivisceral transplant, includes the small bowel and liver with one or more of the following organs: stomach, duodenum, jejunum, ileum, pancreas, and/or colon. The type of transplantation depends on the underlying etiology of intestinal failure, quality of native organs, presence or severity of liver disease, and history of prior abdominal surgeries. A multivisceral transplant is indicated when anatomic or other medical problems preclude a small bowel/liver transplant. Complications following small bowel/liver and multivisceral transplants include acute or chronic rejection, donor-specific antibodies, infection, post-transplant lymphoproliferative disorder (PTLD), graft-versus-host disease, and renal dysfunction.

KEY POINTS:

The most recent literature update was conducted through July 8, 2024.

Summary of Evidence

For individuals who have intestinal failure and evidence of impending end-stage liver failure who receive a small bowel and liver transplant alone or multivisceral transplant, the evidence includes a registry study and a limited number of case series. Relevant outcomes are overall survival (OS), morbid events, and treatment-related mortality and morbidity. However, results from the available literature has have revealed fairly high post-procedural survival rates. Given these results and the exceedingly poor survival rates of individuals who have exhausted all other treatments, transplantation may prove not only be the last option but also a beneficial one.  Transplantation is contraindicated for individuals in whom the procedure is expected to be futile due to comorbid disease or in whom post-transplantation care is expected to significantly worsen comorbid conditions. The evidence is sufficient to determine that the technology results in an improvement in the net health outcome.

For individuals who have a failed small bowel and liver or multivisceral transplant without contraindications for retransplant who receive a small bowel and liver retransplant alone or multivisceral retransplant, the evidence includes case series. Relevant outcomes are overall survival, morbid events, and treatment-related mortality and morbidity. Although limited in quantity, the available post-retransplantation data has suggested reasonably high survival rates. Given exceedingly poor survival rates without retransplantation of individuals who have exhausted other treatments, evidence of postoperative survival from uncontrolled studies is sufficient to demonstrate that retransplantation provides a survival benefit in appropriately selected individuals. Retransplantation is contraindicated for individuals in whom the procedure is expected to be futile due to comorbid disease or in whom post-transplantation care is expected to significantly worsen comorbid conditions. The evidence is sufficient to determine that the technology results in an improvement in the net health outcome.

Practice Guidelines and Position Statements

American Gastroenterological Association

In 2003, the American Gastroenterological Association (AGA) published a position statement on short bowel syndrome and intestinal transplantation. The statement noted that only individuals with life-threatening complications due to intestinal failure or long-term total parenteral nutrition have undergone intestinal transplantation. The statement recommended the following Medicare-approved indications, pending availability of additional data:

• Impending liver failure
• Thrombosis of major central venous channels
• Frequent central line associated sepsis
• Frequent severe dehydration.

The AGA published an expert review update in 2022. The update made the same statements as the 2003 position statement in their best practice advice for referral 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 Society listed the following disorders in children as being potentially treatable by intestinal transplantation: short bowel syndrome, defective intestinal motility, and impaired enterocyte absorptive capacity. Contraindications for intestinal transplant to treat pediatric patients with intestinal failure are similar to those of other solid organ transplants: profound neurologic disabilities, life-threatening comorbidities, severe immunologic deficiencies, non-resectable malignancies, autoimmune diseases, and insufficient vascular patency.

U.S Preventive Services Task Force Recommendations

Not applicable.

KEY WORDS:

Bowel Transplant, Intestine Transplant, Small Bowel Transplant, small bowel/liver and multivisceral transplant, multivisceral transplant, small bowel/liver transplant

APPROVED BY GOVERNING BODIES:

Small bowel/liver and multivisceral transplantation is a surgical procedure and as such is not subject to U.S Food and Drug Administration regulations.

The U.S. Food and Drug Administration regulates human cells and tissues intended for implantation, transplantation, or infusion through the Center for Biologics Evaluation and Research, under Code of Federal Regulation Title 21, parts 1270 and 1271. Solid organs used for transplantation are subject to these regulations.

BENEFIT APPLICATION:

Coverage is subject to member’s specific benefits.  Group specific policy will supersede this policy when applicable.

ITS: Home Policy provisions apply

FEP contracts: Special benefit consideration may apply.  Refer to member’s benefit plan. 

CURRENT CODING:

CPT Codes:  

44120	Enterectomy, resection of small intestine; single resection and anastomosis
44121	; each additional resection and anastomosis
44132	Donor enterectomy (including cold preservation), open; from cadaver donor
44133	Donor enterectomy (including cold preservation), open; partial, from living donor
44135	Intestinal allotransplantation; from cadaver donor
44136	Intestinal allotransplantation; from living donor
44715	Backbench standard preparation of cadaver or living donor intestine allograft prior to transplantation, including mobilization and fashioning of the superior mesenteric artery and vein
44720	Backbench reconstruction of cadaver or living donor intestine allograft prior to transplantation; venous anastomosis, each
44721	Backbench reconstruction of cadaver or living donor intestine allograft prior to transplantation; arterial anastomosis, each
44799	Unlisted procedure, small intestine
47133	Donor hepatectomy (including cold preservation), from cadaver donor
47135	Liver allotransplantation, orthotopic, partial or whole, cadaver or living donor, any age
47140	Donor hepatectomy (including cold preservation), from living donor; left lateral segment only (segments II and III)
47141	; total left lobectomy (segments II, III, or IV)
47142	; total right lobectomy (segments V, VI, VII, and VIII)
47143	Backbench standard preparation of cadaver donor whole liver graft prior to allotransplantation, including cholecystectomy, if necessary, and dissection and removal of surrounding tissues to prepare the vena cave, implantation; without tri-segment or lobe split
47144	Backbench standard preparation of cadaver donor whole liver graft prior to allotransplantation, including cholecystectomy, if necessary, and dissection and removal of surrounding tissues to prepare the vena cave, implantation; with tri-segment split of whole liver graft into tow partial liver grafts (i.e., left lateral segment (segments II and III) and right tri-segment (segments I and IV through VIII))
47145	Backbench standard preparation of cadaver donor whole liver graft prior to allotransplantation, including cholecystectomy, if necessary, and dissection and removal of surrounding tissues to prepare the vena cave, implantation; with lobe split of whole liver graft into two partial liver grafts (i.e., left lobe (segments II, III and IV) and right lobe (segments I and V through VIII))
47146	Backbench reconstruction of cadaver or living donor liver graft prior to allotransplantation; venous anastomosis
47147	Backbench reconstruction of cadaver or living donor liver graft prior to allotransplantation; arterial anastomosis, each

HCPCS Codes:                     

G6021	Unlisted procedure, small intestine
S2053	Transplantation of small intestine, and liver allografts
S2054	Transplantation of multivisceral organs
S2055	Harvesting of donor multivisceral organs, with preparation and maintenance of allografts; from cadaver donor

REFERENCES:

1. Abu-Elmgagd KM, Costa G, Bond GJ et al. Five hundred intestinal and multivisceral transplantations at a single center: major advances with new challenges. Ann Surg. Oct 2009; 250(4):567-581.
2. American Gastroenterological Association (AGA). American Gastroenterological Association medical position statement: short bowel syndrome and intestinal transplantation. Gastroenterology. Apr 2003; 124(4):1105-1110.
3. Bharadwaj S, Tandon P, Gohel TD, et al. Current status of intestinal and multivisceral transplantation. Gastroenterol Rep (Oxf). Feb 2017; 5(1):20-28.
4. Black CK, Termanini KM, Aguirre O, et al. Solid organ transplantation in the 21 st century. Ann Transl Med. Oct 2018; 6(20): 409. 
5. Blue Cross and Blue Shield Association Technology Evaluation Center (TEC). Small bowel transplants in adults and multivisceral transplants in adults and children. TEC Assessments. 1999; Volume 14: Tab 9.
6. 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.
7. Desai CS, Khan KM, Gruessner AC et al. Intestinal retransplantation: analysis of Organ Procurement and Transplantation Network database. Transplantation 2012; 93(1):120-125.
8. 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.
9. Ekser B, Kubal CA, Fridell JA, et al. Comparable outcomes in intestinal retransplantation: Single-center cohort study. Clin Transplant. Jun 2018 32(7): e13290. 
10. Florescu DF, Qiu F, Langnas AN, et al. Bloodstream infections during the first year after intestinal and multivisceral transplantation. Transplantation. Jan 27 2011; 91 (2): 219-24.
11. 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).
12. IOM (Institute of Medicine). 2011. Clinical Practice Guidelines We Can Trust. Washington, DC: The National Academies Press.
13. Iyer K, DiBaise JK, Rubio-Tapia A. AGA Clinical Practice Update on Management of Short Bowel Syndrome: Expert Review. Clin Gastroenterol Hepatol. Oct 2022; 20(10): 2185-2194.e2.
14. Kaufman SS, Atkinson JB, Bianchi A, et al. Indications for pediatric intestinal transplantation: a position paper of the American Society of Transplantation. Pediatr Transplant. Apr 2001; 5(2):80-87.
15. 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.
16. Lauro A, Zanfi C, Dazzi A et al. Disease-related intestinal transplant in adults: results from a single center. Transplant Proc 2014; 46(1):245-248. 
17. Loo L, Vrakas G, Reddy S, et al. Intestinal transplantation: a review. Curr Opin Gastroenterol. May 2017; 33(3): 203-211.
18. Mangus RS, Tector AJ, Kubal CA et al. Multivisceral transplantation: expanding indications and improving outcomes. J Gastrointest Surg 2013; 17(1):179-186; discussion p 86-7.
19. 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.
20. Organ Procurement and Transplantation Network (OPTN). National Data. optn.transplant.hrsa.gov/data/view-data-reports/national-data/.
21. Organ Procurement and Transplantation Network (OPTN). Organ Procurement and Transplantation Network Policies. 2023; optn.transplant.hrsa.gov/media/1200/optn_policies.pdf.
22. Raghu VK, Beaumont JL, Everly MJ et al. Pediatric intestinal transplantation: Analysis of the intestinal transplant registry. Pediatr Transplant.Dec 2019: 23(8): e13580.
23. 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.
24. Spence AB, Natarajan M, Fogleman S et al. Intra-abdominal infections among adult intestinal and multivisceral transplant recipients in the 2-year post-operative period. Transpl Infect Dis. 2020 Feb; 22(1).
25. 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.
26. Varkey J, Simren M, Bosaeus I et al. Survival of patients evaluated for intestinal and multivisceral transplantation - the Scandinavian experience. Scand J Gastroenterol. Jun 2013; 48(6):702-711.
27. Working Party of the British Transplantation Society. Kidney and Pancreas Transplantation in Patients with HIV. Second Edition (Revised). British Transplantation Society Guidelines. Macclesfield, UK: British Transplantation Society; 2017.
28. Wu GS, Cruz RJ, Jr., Cai JC. Acute antibody-mediated rejection after intestinal transplantation. World J Transplant. Dec 24 2016; 6(4):719-728.
29. Wu G, Selvaggi G, Nishida S et al. Graft-versus-host disease after intestinal and multivisceral transplantation. Transplantation. Jan 27 2011; 91(2):219-224.

POLICY HISTORY:

Medical Policy Group, August 2009 (2)

Medical Policy Administration Committee, September 2009

Available for comment September 4-October 19, 2009

Medical Policy Panel, June 2010

Medical Policy Group, June 2010 (2)

Medical Policy Administration Committee, July 2010

Available for comment July 2-August 16, 2010

Medical Policy Panel, June 2011

Medical Policy Group, September 2011 (2): Policy, Key Points, References updated

Medical Policy Administration Committee, September 2011

Available for comment September 22 through November 7, 2011

Medical Policy Group, July 2012 (2): 2012 Update – Key Points & References

Medical Policy Panel, June 2013

Medical Policy Group, June 2013 (3): 2013 Update to Key Points, References, and policy statement – added retransplant coverage criteria to policy

Available for comment July 18 through September 6, 2013

Medical Policy Panel, June 2014

Medical Policy Group, June 2014 (3):  2014 Updates to Key Points & References; policy statement updated to include “all other situations” as investigational.

Medical Policy Administration Committee, July 2014

Available for comment July 7 through August 20, 2014

Medical Policy Group, November 2014: 2015 Coding Updates – add word ‘small’ to code 44799

Medical Policy Panel, June 2015

Medical Policy Group, June 2015 (2): 2015 Updates to Key Points, Approved by Governing Bodies, Coding, and Policy section to include description of intolerance to TPN –small bowel and liver specific; no change to policy statement.

Medical Policy Group, November 2015: 2016 Annual Coding Update.  Added CPT code 43799 to current coding.  Created previous coding section and moved CPT code 47136 from current coding to previous coding.

Medical Policy Panel, December 2016

Medical Policy Group, May 2017 (7): 2017 Updates to Key Points and References. Policy Statement- clarified Absolute Contraindications and removed old policy criteria from 2013.

Medical Policy Administration Committee, June 2017

Available for comment May 20 through July 3, 2017

Medical Policy Panel, August 2017

Medical Policy Group, September 2017 (7): Updates to Description, Key Points, and References. Policy Statement updated; clarification to HIV criteria; no change in intent.

Medical Policy Panel, August 2018

Medical Policy Group, August 2018 (3): Updates to Description, Key Points, Approved by Governing Bodies and References. No change in policy statement or intent.

Medical Policy Panel, August 2019

Medical Policy Group, September 2019 (3): 2019 Updates to Key Points. No changes in policy statement or intent.

Medical Policy Panel, August 2020

Medical Policy Group, September 2020 (3): 2020 Updates to Description, Key Points, Practice Guidelines and Position Statements, Approved by Governing Bodies, and References. No changes to policy statement or intent.

Medical Policy Panel, August 2021

Medical Policy Group, August 2021 (3): 2021 Updates to Description, Key Points, and References. Policy statement updated to remove “not medically necessary”, no other changes to policy statement.

Medical Policy Panel, August 2022 (3):  2022 Updates to Description, Key Points, and References. Removed UNOS references that contained outdated policy guidelines and 1994-1995 TEC criteria. Removed HIV positive patient criteria from policy section to reflect standard of care guidelines from the American Society of Transplantation. Removed absolute contraindications, as defined by the American Society of Transplantation in policy guidelines and changed verbiage to “Potential contraindications to solid organ transplant (subject to the judgment of the transplant center).” No changes to policy statement or intent.

Medical Policy Panel, August 2023

Medical Policy Group, September 2023 (3): 2023 Updates Description, Key Points, Practice Guidelines and Position Statements, Benefit Applications, References, and removed Previous Coding section. No changes to policy statement or intent.

Medical Policy Panel, August 2024

Medical Policy Group, September 2024 (3): Updates Description, Key Points, and References. No change to the policy statement  or intent.

This medical policy is not an authorization, certification, explanation of benefits, or a contract. Eligibility and benefits are determined on a case-by-case basis according to the terms of the member’s plan in effect as of the date services are rendered. All medical policies are based on (i) research of current medical literature and (ii) review of common medical practices in the treatment and diagnosis of disease as of the date hereof. Physicians and other providers are solely responsible for all aspects of medical care and treatment, including the type, quality, and levels of care and treatment.

This policy is intended to be used for adjudication of claims (including pre-admission certification, pre-determinations, and pre-procedure review) in Blue Cross and Blue Shield’s administration of plan contracts.

The plan does not approve or deny procedures, services, testing, or equipment for our members. Our decisions concern coverage only. The decision of whether or not to have a certain test, treatment or procedure is one made between the physician and his/her patient. The plan administers benefits based on the member’s contract and corporate medical policies. Physicians should always exercise their best medical judgment in providing the care they feel is most appropriate for their patients. Needed care should not be delayed or refused because of a coverage determination.

As a general rule, benefits are payable under health plans only in cases of medical necessity and only if services or supplies are not investigational, provided the customer group contracts have such coverage.

The following Association Technology Evaluation Criteria must be met for a service/supply to be considered for coverage:

1. The technology must have final approval from the appropriate government regulatory bodies;

2. The scientific evidence must permit conclusions concerning the effect of the technology on health outcomes;

3. The technology must improve the net health outcome;

4. The technology must be as beneficial as any established alternatives;

5. The improvement must be attainable outside the investigational setting.

Medical Necessity means that health care services (e.g., procedures, treatments, supplies, devices, equipment, facilities or drugs) that a physician, exercising prudent clinical judgment, would provide to a patient for the purpose of preventing, evaluating, diagnosing or treating an illness, injury or disease or its symptoms, and that are:

1. In accordance with generally accepted standards of medical practice; and

2. Clinically appropriate in terms of type, frequency, extent, site and duration and considered effective for the patient’s illness, injury or disease; and

3. Not primarily for the convenience of the patient, physician or other health care provider; and

4. Not more costly than an alternative service or sequence of services at least as likely to produce equivalent therapeutic or diagnostic results as to the diagnosis or treatment of that patient’s illness, injury or disease.