Medical Policy: 07.03.04
Original Effective Date: November 2009
Reviewed: November 2015
Revised: November 2015
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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.
Small bowel transplant has evolved into an established therapeutic modality in the management of the patient with irreversible intestinal failure. It is performed mainly in patients with short bowel syndrome (SBS) and those who develop severe complications due to total parenteral nutrition (TPN). The goal of transplantation is to eliminate the need for TPN and to reverse or prevent TPN associated liver disease.
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
Total parenteral nutrition (TPN) is the current standard of care for patients with intestinal failure. The chronic use of TPN is often associated with life-threatening complications including:
- Catheter related sepsis
- Catheter related thrombosis
- Severe dehydration
- Parenteral nutrition associated liver disease (PNALD)
Small bowel transplant should be recommended in patients with the following conditions:
1). Failure of parenteral nutrition
• Impending or overt liver failure
• Thrombosis of 2 or more central veins
• Two ore more episodes per year of systemic sepsis secondary to line infections
• Frequent episodes of dehydration
2). High risk of death
3). Severe short bowel syndrome (gastrotomy, duodenostomy, residual small bowel <10 cm in infants and <20 cm in adults)
4). Frequent hospitalization, narcotic dependency or pseudoobstruction
5). Unwillingness to accept long-term parenteral nutrition (TPN)
Small bowel transplant may be considered a means to avoid end stage liver failure, thus avoiding a multivisceral organ transplant.
Cadaveric small bowel transplant is the most commonly performed transplant, there has been recent interest in using living donors. Living donor transplantation has been proposed as a means to increase the pool of donor organs and thereby reduce waiting time and wait list morbidity and mortality.
The most common causes of graft loss in small bowel transplant are infection, rejection (acute and chronic) and technical or clinical complications. Careful patient selection, post-transplant immunosuppression and patient management are essential for successful long term outcomes. Individuals undergoing repeat small bowel transplantation should meet all eligibility criteria and should not have contraindications to transplantation.
Small bowel transplant may be considered medically necessary in patients with intestinal failure who are developing severe total parenteral nutrition (TPN) – related complications, to obviate the subsequent need for multi-visceral transplant. Small bowel transplant using a living donor may be considered medically necessary only when a cadaveric intestinal transplant is not available. The available published survival data suggest that small bowel retransplant is reasonable option after a failed primary small bowel transplant; thus, this may be considered medically necessary.
Practice Guidelines or 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 transplant mirror those of Medicare in patients who fail TPN therapy for one of the following reasons:
- Impending or overt liver failure (increased serum bilirubin and/or liver enzyme levels, splenomegaly, thrombocytopenia, gastroesophageal varicies, coagulopathy, stomal bleeding, hepatic fibrosis or cirrhosis)
- Thrombosis of 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).
- 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.
See also medical policy 07.03.05 Small Bowel/Liver and Nultivisceral Transplant
Cadaveric Small Bowel Transplant
A small bowel transplant using a 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 TPN and are developing or have developed severe complications due to TPN (see criteria above under Description).
Small bowel transplant would be considered not medically necessary in adult or pediatric patients who are able to tolerate TPN.
Living Donor Small Bowel Transplant
Small bowel transplant using a living donor intestine may be considered medically necessary only when a cadaveric intestine is not available for transplantation in a patient who meets the above criteria for a cadaveric intestinal transplant.
Small bowel transplant using living donor is considered investigational in all other situations.
Based on peer reviewed literature living donors for small bowel transplantation is limited and the number of living donor transplants performed to date has been small. The literature is mostly limited to single center individual case reports and small case series. More large studies are needed to determine impact on net health outcomes to include that patient survival is comparable or better than those patients receiving cadaveric organs. However, small bowel transplant using a living donor does have a role in select cases where a cadaveric intestine is not available, see above.
Small Bowel Retransplant
Small bowel retransplant may be considered medically necessary in individuals with graft failure of an initial small bowel transplant who meet criteria for small bowel transplantation above.
Except as defined above, candidates for small bowel transplant 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.
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
- 44136 Intestinal allotransplantation from living donor
- 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.
- 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.
- American Gastroenterological Association. American Gastroenterological Association medical position statement: short bowel syndrome and intestinal transplantation. Gastroenterology. 2003 Apr;124(4):1105-10.
- 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.
- Giuliana Testa, M.D., Fabrizio Panaro, M.D., Stefano Schena, M.D., Mark Holterman, M.D., Herand Abcarian, M.D. and Enrico Benedetti, M.D. Annals of Surgery, 2004 November; 240(5); 779-784. Living Related Small Bowel Transplantation.
- CMS. National Coverage Determination (NCD) for Intestinal and Multi-Visceral Transplantation (260.5).
- UpToDateExternal Site. Overview of Intestinal an Multivisceral Transplantation. Farrukh A. Khan, M.D., FACS, Gennaro Selvaggi, M.D. Topic last updated October 18, 2012.
- MedscapeExternal Site. Intestinal Transplantation. Stuart M. Greenstein, M.D. Updated March 5, 2012.
- MedscapeExternal Site. Pediatric Intestinal and Multivisceral Transplantation. Seigo Nishida, M.D., PhD. Updated May 30, 2012.
- UpToDateExternal Site. Overview of Intestinal and Multivisceral Transplantation. Farrukh A Khan, M.D., FACS, Gennaro Selvaggi, M.D., Topic last updated June 4, 2015.
- UpToDateExternal Site. Management of the Short Bowel Syndrome in Children. John A. Vanderhoof, M.D., Rosemary J. Pauley-Hunter, NP-C, MS, RN. Topic last updated February 9, 2015.
- UpToDateExternal Site. Management of the Short Bowel Syndrome in Adults. Jon A. Vanderhoof, M.D., Rosemary J. Pauley-Hunter, NP-C, MS, RN. Topic last updated August 21, 2014.
- MedscapeExternal Site. Stuart M. Greestein, M.D. et. al. Intestinal Transplantation, updated August 17, 2014.
- Organ Procurement and Transplant NetworkExternal Site, Allocation of Livers and Liver-Intestines.
- Organ Procurement and Transplant NetworkExternal Site, Intestine,
- PubMed. Intestinal and Multivisceral Retransplantation Results: Literature Review. Transplant Proc. 2013 Apr:45(3):1133-6.
- PubMed. Kaufman SS, Atikinson JB, et. al. Indications for Pediatruc Intestinal Transplantation: A Position Paper of the American Society of Transplantation. Pediatr Transplant 2001 Apr 5(2):80-7\
- Benedetti Enrico, Holterman Mark, et. al. Living Related Segmental Bowel Transplantation from Experimental to Standardized Procedure. Ann Sug. 2006;244(5):694-699
- PubMed. Sudan D. Long Term Outcomes and Quality of Life after Intestine Transplantation, Curr Opin Organ Transplant 2010 Jun:15(3):357-60
- PubMed. Desai CS, Khan KM, et. al. Intestinal Retransplantation: Analysis of Organ Procurement and Transplantation Network Database. Transplantation 2012 Jan 15:93(1):120-5
- 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
- Trevizol AP, David AI, Yamashita ET. et.al. Intestinal and Multivisceral Retransplantation Results: Literature Review. Transplant Proc. Apr 2013;45(3):1133-1136
- 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
November 2015 - Annual Review, Policy Revised
December 2014 - Annual Review, Policy Revised
February 2014 - Annual Review, Policy Revised
March 2013 - Annual Review, Policy Renewed
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.