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Isolated Small Bowel Transplant

Policy Number: MP-479

Latest Review Date: August 2021

Category:  Surgery                                                                 

Policy Grade: A

POLICY:

Effective for dates of service on or after July 4, 2017:

A small bowel transplant using cadaveric intestine may be considered medically necessary in adult and pediatric patients with intestinal failure (characterized by loss of absorption and the inability to maintain protein-energy, fluid, electrolyte, or micronutrient balance), who have established long-term dependency on total parenteral nutrition* (TPN) and are developing or have developed severe complications due to TPN and meet all the following criteria:

  • Adequate cardiopulmonary status; and
  • Documentation of patient compliance with medical management.

A small bowel transplant using a living donor may be considered medically necessary

only when a cadaveric intestine is not available for transplantation in a patient who meets the criteria noted above for a cadaveric intestinal transplant.

A small bowel retransplant may be considered medically necessary after a failed primary small bowel transplant.

A small bowel transplant is considered  investigational for adults and pediatric patients with intestinal failure who are able to tolerate TPN.

Small bowel transplant may be considered medically necessary for HIV-positive patients when:

  • Adherent with treatment, particularly antiretroviral therapy; and
  • CD4 count greater than 100 cells/mL (ideally >200 cells/mL) for at least 3 months; and
  • Undetectable HIV viremia (<50 HIV-1 RNA copies/mL) for at least 6 months; and
  • No opportunistic infections for at least 6 months; and
  • No history of progressive multifocal leukoencephalopathy, chronic intestinal cryptosporidiosis, or lymphoma.

A small bowel transplant in patients with the following absolute contraindications, as defined by the American Society of Transplantation in Guidelines for the Referral and Management of Patients Eligible for Solid Organ Transplantation is considered not medically necessary:

  • Serious cardiac or other ongoing insufficiencies that create an inability to tolerate transplant surgery;
  • 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.

A small bowel transplant using living donors is considered not medically necessary in all other situations.

POLICY GUIDELINES:

*Small Bowel 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. Short bowel syndrome is one cause of intestinal failure.

Patients who are developing or have developed severe complications due to total parenteral nutrition (TPN) include, but are not limited to, the following: multiple and prolonged hospitalizations to treat TPN-related complications (especially repeated episodes of catheter-related sepsis) or the development of progressive 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. In those receiving TPN, liver disease with jaundice (total bilirubin >3 mg/dL) is often associated with development of irreversible, progressive liver disease. The inability to maintain venous access is another reason to consider small bowel transplant in those who are dependent on TPN.

Effective for dates of service on or after December 12, 2013 through July 3, 2017:

A small bowel transplant using cadaveric intestine may be considered medically necessary in adult and pediatric patients with intestinal failure (characterized by loss of absorption and the inability to maintain protein-energy, fluid, electrolyte, or micronutrient balance), who have established long-term dependency on total parenteral nutrition* (TPN) and are developing or have developed severe complications due to TPN and meet all the following criteria:

  • Adequate cardiopulmonary status; and
  • Absence of significant infection that could be exacerbated by immunosuppressive therapy (e.g., chronic active viral hepatitis B, hepatitis C, and human immunodeficiency virus); and
  • No history of malignancy within 5 years of transplantation, excluding non-melanomatous skin cancers; and
  •  Documentation of patient compliance with medical management.

*Severe complications due to total parenteral nutrition (TPN) include, but are not limited, to the following:

  • Multiple and prolonged hospitalizations to treat TPN-related complications (especially repeated episodes of catheter-related sepsis) or
  • The development of progressive 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.

In those receiving TPN, liver disease with jaundice (total bilirubin above 3 mg/dL) is often associated with development of irreversible progressive liver disease.

The inability to maintain venous access is another reason to consider small bowel transplant in those who are dependent on TPN.

A small bowel transplant using a living donor may be considered medically necessary

only when a cadaveric intestine is not available for transplantation in a patient who meets the criteria noted above for a cadaveric intestinal transplant.

A small bowel retransplant may be considered medically necessary after a failed primary small bowel transplant.

A small bowel transplant using living donors is considered not medically necessary in all other situations.

A small bowel transplant is considered not medically necessary and investigational

for adults and pediatric patients with intestinal failure who are able to tolerate TPN.

*Small Bowel Specific

Patients who are developing or have developed severe complications due to total parenteral nutrition (TPN) include, but are not limited to, the following: multiple and prolonged hospitalizations to treat TPN-related complications (especially repeated episodes of catheter-related sepsis) or the development of progressive 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. In those receiving TPN, liver disease with jaundice (total bilirubin >3 mg/dL) is often associated with development of irreversible progressive liver disease. The inability to maintain venous access is another reason to consider small bowel transplant in those who are dependent on TPN.

DESCRIPTION OF PROCEDURE OR SERVICE:

Solid organ transplantation offers a treatment option for patients with different types of end stage organ failure that can be lifesaving or provide significant improvements to a patient’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. Patients are prioritized for transplant by mortality risk and severity of illness criteria developed by the Organ Procurement and Transplantation Network (OPTN) and United Network of Organ Sharing (UNOS).

Short Bowel Syndrome

Short bowel syndrome is a condition in which the absorbing surface of the small intestine is inadequate due to extensive disease or surgical removal of a large portion of the small intestine. The spectrum of clinical disease is widely variable from only single micronutrient malabsorption to complete intestinal failure, defined as the reduction of gut function below the minimum necessary for the absorption of macronutrients and/or water and electrolytes. In adults, etiologies of short bowel syndrome include ischemia, trauma, volvulus, and tumors. In children, gastroschisis, volvulus, necrotizing enterocolitis, and congenital atresia are predominant causes. Although the actual prevalence of short bowel syndrome is not clear primarily due to under-reporting and a lack of reliable patient databases, its prevalence is estimated to be 30 cases per million in the U.S.

Treatment

The small intestine, particularly the ileum, can adapt to some functions of the diseased or removed portion over a period of 1 to 2 years. Prognosis for recovery depends on the degree and location of small intestine damage. Therapy focuses on achieving adequate macro- and micronutrient uptake in the remaining small bowel. Pharmacologic agents have been studied to increase villous proliferation and slow transit times, and surgical techniques have been advocated to optimize remaining small bowel.

However, some patients with short bowel syndrome are unable to obtain adequate nutrition from enteral feeding and become chronically dependent on total parenteral nutrition (TPN). For patients with short bowel syndrome, the rate of parenteral nutrition dependency at 1, 2, and 5 years has been reported to be 74%, 64%, and 48%, respectively. Patients with complications from TPN may be considered candidates for a small bowel transplant. Complications include catheter-related mechanical problems, infections, hepatobiliary disease, and metabolic bone disease. While cadaveric intestinal transplant is the most commonly performed transplant, there has been a recent interest in using living donors.

Intestinal transplants (including multivisceral and bowel/liver) represent a small minority of all solid organ transplants. In 2019, 81 intestinal transplants were performed in the U.S. (50% of which were intestine-liver transplants). Overall, both the number of new patients added to the intestinal transplant waiting list (n=103) and the number of intestinal transplants performed declined to their lowest levels in 2019.

KEY POINTS:

The policy has been updated on a regular basis with literature reviews; the most recent update was with a search of the MEDLINE database through June 14, 2021.

Summary of Evidence

For individuals who have intestinal failure who receive a small bowel transplant, the evidence includes case series. Relevant outcomes are overall survival, morbid events, and treatment-related mortality and morbidity. Small bowel transplant is infrequently performed, and only relatively small case series, generally single-center, are available. Risks after small bowel transplant are high, particularly related to infection, but may be balanced against the need to avoid the long-term complications of total parenteral nutrition dependence. In addition, early small bowel transplant may obviate the need for a later combined liver/small bowel transplant. Transplantation is contraindicated in patients 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. Guidelines and U.S. federal policy no longer view HIV infection as an absolute contraindication for solid organ transplantation. The evidence is sufficient to determine that the technology results in an improvement in the net health outcome.

For individuals who have failed small bowel transplant without contraindication(s) for re-transplant who receive a small bowel re-transplant, the evidence includes case series. Relevant outcomes are overall survival, morbid events, and treatment-related mortality and morbidity. Data from only a small number of patients undergoing retransplantation are available. Although limited in quantity, the available data after retransplantation have suggested a reasonably high survival rate after small bowel in patients who continue to meet criteria for transplantation. 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 produced a medical position statement on short bowel syndrome and intestinal transplantation. It recommends dietary, medical, and surgical solutions. Indications for intestinal transplantation mirror those of CMS. The guidelines acknowledge the limitations of transplant for these patients. The statement recommended the following Medicare-approved indications, pending availability of additional data:

  • “Impending or overt liver failure.…
  • Thrombosis of major central venous channels….
  • Frequent central line-related sepsis….
  • Frequent severe dehydration.”

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 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, nonresectable malignancies, autoimmune diseases, and insufficient vascular patency.

U.S. Preventive Services Task Force Recommendations

Not Applicable.

KEY WORDS:

Isolated small bowel transplant, small bowel transplant, small bowel, Intestinal Transplantation (IT), Isolated Intestinal Transplant

APPROVED BY GOVERNING BODIES:

Solid organ transplants are a surgical procedure and, as such, are not subject to regulation by the U.S. Food and Drug Administration.

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: Special benefit consideration may apply. Refer to member’s benefit plan. FEP does not consider investigational if FDA approved and will be reviewed for medical necessity.

CURRENT CODING:

CPT Codes:

44132

Donor enterectomy (including clod preservation), open: from cadaver donor

44133 

; 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

REFERENCES:

  1. 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. Oct 2009; 250(4):567-581.
  2. American Gastroenterological Association medical position statement: short bowel syndrome and intestinal transplantation. Gastroenterology 2003; 124(4):1105-1110.
  3. Benedetti E, Holterman M, Asolati M et al. Living related segmental bowel transplantation: from experimental to standardized procedure. Ann Surg 2006; 244(5):694-699.
  4. Bhagani S, Sweny P, Brook G. Guidelines for kidney transplantation in patients with HIV disease. HIV Med 2006; 7(3):133-139.
  5. Black CK, Termanini KM, Aguirre O, et al. Solid organ transplantation in the 21 st century. Ann Transl Med. Oct 2018; 6(20): 409.
  6. Blue Cross and Blue Shield Association Technology Evaluation Center (TEC). Small bowel transplant. TEC Assessments 1995; Volume 10, Tab 27.
  7. Blue Cross and Blue Shield Association Technology Evaluation Center (TEC). Small bowel transplants in adults and multivisceral transplants. TEC Assessments 1999; Volume 14, Tab 9.
  8. Boyer O, Noto C, De Serre NP et al. Renal function and histology in children after small bowel transplantation. Pediatr Transplant 2013; 17(1):65-72.
  9. Calvo Pulido J, Jimenez Romero C, Morales Ruiz E, et al. Renal failure associated with intestinal transplantation: our experience in Spain. Transplant Proc. Jul-Aug 2014; 46(6):2140-2142.
  10. Colfax G. HIV Organ Policy Equity (HOPE) Act Is Now Law. 2013; www.obamawhitehouse.archives.gov/blog/2013/11/21/hiv-organ-policy-equity-hope-act-now-law. Accessed July 26, 2018.
  11. Desai CS, Khan KM, Gruessner AC et al. Intestinal retransplantation: analysis of Organ Procurement and Transplantation Network database. Transplantation 2012; 93(1):120-125.
  12. 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.
  13. Florescu DF, Islam KM, Grant W et al. Incidence and outcome of fungal infections in pediatric small bowel transplant recipients. Transpl Infect Dis 2010; 12(6):497-504.
  14. Florescu DF, Langnas AN, Grant W et al. Incidence, risk factors, and outcomes associated with cytomegalovirus disease in small bowel transplant recipients. Pediatr Transplant 2012; 16(3):294-301.
  15. Florescu DF, Qui F, Langnas AN et al. Bloodstream infections during the first year after pediatric small bowel transplantation. Pediatr Infect Dis J 2012; 31(7):700-704.
  16. Fujimoto Y, Uemoto S, Inomata Y et al. Living-related small bowel transplant: management of rejection and infection. Transplant Proc 1998; 30(1):149.
  17. Gangemi A, Benedetti E. Living donor small bowel transplantation: Literature review 2003-2006 Pediatric Transplantation 2006; 10(8):875-878.
  18. 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).
  19. Gruessner RW, Sharp HL. Living-related intestinal transplantation: first report of a standardized surgical technique. Transplantation 1997; 64(11):1605-1607.
  20. Jaffe BM, Beck R, Flint L et al. Living-related small bowel transplantation in adults: a report of two patients. Transplant Proc 1997; 29(3):1851-1852.
  21. 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.
  22. 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.
  23. 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.
  24. Massironi S, Cavalcoli F, Rausa E, et al. Understanding short bowel syndrome: Current status and future perspectives. Dig Liver Dis. Mar 2020; 52(3): 253-261.
  25. Matarese LE, Costa G, Bond G et al. Therapeutic efficacy of intestinal and multivisceral transplantation: survival and nutrition outcome. Nutr Clin Pract 2007; 22(5):474-481.
  26. 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.
  27. Organ Procurement and Transplantation Network. Policies. 2017 September 12; optn.transplant.hrsa.gov/governance/policies/.
  28. Organ Procurement and Transplantation Network. www.optn.transplant.hrsa.gov/latestData/viewDataReports.asp. Accessed July 26, 2018.
  29. Organ Procurement and Transplantation Network. www.optn.transplant.hrsa.gov/policiesAndBylaws/policies.asp. Accessed July 26, 2018.
  30. 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.
  31. Sudan D. Long-term outcomes and quality of life after intestine transplantation. Curr Opin Organ Transplant 2010; 15(3):357-360.
  32. Sudan D. The current state of intestine transplantation: indications, techniques, outcomes and challenges. Am J Transplant. Sep 2014; 14(9): 1976-84.
  33. Tesi R, Beck R, Lambiase L et al. Living-related small-bowel transplantation: donor evaluation and outcome. Transplant Proc 1997; 29(1-2):686-687.
  34. Ueno T, Wada M, Hoshino K, et al. Impact of intestinal transplantation for intestinal failure in Japan. Transplant Proc. Jul-Aug 2014; 46(6):2122-2124.
  35. United Network for Organ Sharing (UNOS). OPTN policies, procedures implemented to support HOPE Act. 2015; www.unos.org/optn-policies-procedures-implemented-to-support-hope-act/. Accessed July 26, 2018.
  36. U. S. Department of Health and Human Services (DHHS). Organ Procurement and Transplantation Network National Data. 2018; www.optn.transplant.hrsa.gov/data/. Accessed July 26, 2018.
  37. Vianna RM, Mangus RS, Tector AJ. Current status of small bowel and multivisceral transplantation. Adv Surg 2008; 42:129-150.
  38. 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.
  39. Wu GS, Cruz RJ, Jr., Cai JC. Acute antibody-mediated rejection after intestinal transplantation. World J Transplant. Dec 24 2016; 6(4):719-728.

POLICY HISTORY:

Medical Policy Group, September 2011 (2): New policy

Medical Policy Administration Committee, September 2011

Available for comment September 22 through November 7, 2011

Medical Policy Group, June 2012 (2): Updated Key Points and References.

Medical Policy Panel, October 2013

Medical Policy Group, October 2013 (3): 2013 Updates to Description, Key Points and References; no change in policy statement

Medical Policy Panel, December 2013

Medical Policy Group, January 2014 (3):  2013 Update to Policy statement to include coverage for small bowel retransplant after a failed primary small bowel transplant. Key Points and References also updated.

Medical Policy Administration Committee, January 2014

Available for comment January 9 through February 24, 2014

Medical Policy Panel, May 2014

Medical Policy Group, June 2014 (3):  2014 Updates – editing changes only; no new literature available.

Medical Policy Panel, May 2015

Medical Policy Group, June 2015 (2): 2015 Updates to Key Points, Approved by Governing Bodies, and References; no change to policy statement.

Medical Policy Panel, December 2016

Medical Policy Group, May 2017 (7): 2017 Updates to Key Points, Policy statement- Added HIV criteria, clarified absolute contraindications; removed old 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): Clarification to HIV Criteria. Updates to Key Points, and References. No change in intent.

Medical Policy Panel, August 2018

Medical Policy Group, August 2018 (3): Updates to Description, Key Points, Approved by Governing Bodies, References and Key Words: added Intestinal Transplantation (IT) and Isolated Intestinal Transplant. No change to policy statement or intent.

Medical Policy Panel, August 2019

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

Medical Policy Panel, August 2020

Medical Policy Group, September 2020 (3): 2020 Updates to Description, Key Points, 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 and Key Points. Policy statement updated to remove "not medically necessary", no other changes to policy statement.

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.