Short bowel syndrome is defined as an inadequate absorbing surface of the small intestine due to extensive disease or surgical removal of a large portion of the 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.^1, 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, lymphoproliferative disorder, graft-versus-host disease, and renal dysfunction.^2,

Regulatory Status

Small bowel/liver and multivisceral transplantation are surgical procedures 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. Pancreas transplants are included in these regulations.

Related Policies

• Isolated Small Bowel Transplant (Policy #095 in the Surgery Section)

A. member has intestinal failure characterized by loss of absorption and the inability to maintain protein-energy, fluid, electrolyte, or micronutrient balance;
B. member has been managed with long-term total parenteral nutrition (TPN); and
C. member has developed evidence of impending end-stage liver failure.

For members enrolled in Medicaid and NJ FamilyCare plans, Horizon BCBSNJ applies the above medical policy.

FIDE SNP:

For members enrolled in a Fully Integrated Dual Eligible Special Needs Plan (FIDE-SNP): (1) to the extent the service is covered under the Medicare portion of the member’s benefit package, the above Medicare Coverage statement applies; and (2) to the extent the service is not covered under the Medicare portion of the member’s benefit package, the above Medicaid Coverage statement applies.

Policy Guidelines: (Information to guide medical necessity determination based on the criteria contained within the policy statements above.)

General Criteria
Potential contraindications to solid organ transplant are subject to the judgment of the transplant center include the following:

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.

To assess whether the evidence is sufficient to draw conclusions about the net health outcome of technology, two domains are examined: the relevance, and quality and credibility. To be relevant, studies must represent one or more intended clinical use of the technology in the intended population and compare an effective and appropriate alternative at a comparable intensity. For some conditions, the alternative will be supportive care or surveillance. The quality and credibility of the evidence depend on study design and conduct, minimizing bias and confounding that can generate incorrect findings. The randomized controlled trial is preferred to assess efficacy; however, in some circumstances, nonrandomized studies may be adequate. Randomized controlled trials are rarely large enough or long enough to capture less common adverse events and long-term effects. Other types of studies can be used for these purposes and to assess generalizability to broader clinical populations and settings of clinical practice.

Transplantation of Small Bowel and Liver or Multivisceral Organs

Clinical Context and Test Purpose

The purpose of small bowel and liver transplant alone or multivisceral transplant in patients who have intestinal failure and evidence of impending end-stage liver failure is to provide a treatment option that is an alternative to or an improvement on existing therapies.

The question addressed in this policy is: Does small bowel and liver transplant alone or multivisceral transplant improve the net health outcome in individuals with intestinal failure and evidence of impending end-stage liver failure?

The following PICOs were used to select literature to inform this policy.

Patients

The relevant population of interest are individuals with intestinal failure and evidence of impending end-stage liver failure.

Interventions

The therapy being considered is small bowel and liver transplant alone or multivisceral transplant.

Comparators

The following practices are currently being used to make decisions about intestinal failure and evidence of impending end-stage liver failure: medical management and parenteral nutrition.

Outcomes

The general outcomes of interest are overall survival (OS), morbid events, and treatment-related mortality and morbidity, including short- and long-term graft survival and 1- and 5-year OS.

Systematic Reviews

A TEC Assessment (1999) 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 is associated with poor patient and graft survival. Pediatric and adult patients have a similar 2- and 5-year survival of 33% to 50%. However, without this procedure, it is expected that these patients would face 100% mortality."^3,

Case Series

The published literature consists of case series, mainly reported by single-centers in the U. S. and Europe. Tables 1 and 2 summarize the characteristics and results of the case series, respectively. Many case series have included isolated small bowel transplantations (see separate policy on 'Isolated Small Bowel Transplant' - Policy #095 in the Surgery Section).

Reasons for transplantations were mainly short bowel syndrome. Other reasons included congenital enteropathies and motility disorders. Most common outcomes reported were survival rates and weaning off total parenteral nutrition. Several studies have presented survival rates by type of transplantation, while others have combined all types of transplants when reporting survival rates. When rates were reported by type of transplant, isolated transplantations had higher survival rates than multivisceral transplants (see Table 2).

Several investigators have reported higher survival rates in transplants conducted more recently than those conducted earlier.^4,5,6, Reasons for improved survival rates in more recent years have been attributed to the development of more effective immunosuppressive drugs and the learning curve for the complex procedure.

Authors of these series, as well as related reviews, have observed that while outcomes have improved over time, recurrent and chronic rejection and complications of immunosuppression continue to be obstacles to long-term survival. A separate discussion of complications follows the evidence tables.

Table 1. Summary of Key Case Series Characteristics for Transplantations

Study	Country	N	Median Age (Range), y	Interventions		Follow-Up (Range)
				Treatment	n
Lacaille et al (2017)7,	France	110	5.3 (0.4-19)	Isolated IT Combined liver IT Multivisceral graft	45 60 5	Of 55 alive: 17 at <5 y 17 at 5-10 y 21 at ≥10 y
Garcia Aroz et al (2017)8,,a	U.S.	10	1.5 (0.7-13)	Isolated IT Combined liver IT	7 3	6/7 alive at ≥10 y
Dore et al (2016)9,	U.S.	30	0.2 (0.1-18)	Isolated IT Combined liver IT Multivisceral graft	6 6 18	28 (4-175) mo
Rutter et al (2016)10,	U.K.	60	1.8 (0-8)	Isolated IT Combined liver IT Modified multivivceral	16 35 9	21.3 (0-95) mo
Lauro et al (2014)11,	Italy	46	34 (NR)	Isolated IT Combined liver IT Multivisceral graft	34 6 6	51.3 mo
Varkey et al (2013)12,	Sweden	20	Adults: 44 (20-67) Children: 6 (0.5-13)	Isolated IT Combined liver IT Multivisceral graft	4 1 15	NR
Mangus et al (2013)4,	U.S.	100	Adults: 48 (NR to 66) Children: 1 (0.6 to NR)	Multivisceral graft Modified multivisceral	84 16	25 mo

IT: intestinal transplantation; NR: not reported.

^a Living donors.

Table 2. Summary of Key Case Series Results for Transplantations

Study	Interventions		Survival	Off TPN
	Treatment	n
Lacaille et al (2017)7,	Isolated IT Combined liver IT Multivisceral graft	60 45 5	59% at 10 y; 54% at 18 y 48% at 10 y NR	All treatments combined: 73% at last follow-up
Garcia Aroz et al (2017)8,,a	Isolated IT Combined liver IT	7 3	All transplantations combined: 70%	All treatments combined: 100% at last follow-up
Dore et al (2016)9,	Isolated IT Combined liver IT Multivisceral graft	6 6 18	83% at 9 y 33% at 10 y 67% at 2.5 y	All treatments combined: 71% in 31 d 62% at last follow-up
Rutter et al (2016)10,	Isolated IT Multivisceral graft Modified multivisceral	16 35 9	92% at 1 y; 37% at 5 y 71% at 1 y; 33% at 5 y 85% at 1 y; 65% at 5 y	NR
Lauro et al (2014)11,	Isolated IT Combined liver IT Multivisceral graft	34 6 6	All transplantations combined: 77% at 1 y 58% at 3 y 53% at 5 y 37% at 10 y	NR
Varkey et al (2013)12,	Isolated IT Combined liver IT Multivisceral graft	4 1 15	All transplantations combined: 78% at 1 y 50% at 5 y	NR
Mangus et al (2013)4,	Multivisceral graft Modified multivisceral	84 16	All transplantations combined: 72% at 1 y 57% at 5 y	NR

IT: intestinal transplantation; NR: not reported; TPN: total parenteral nutrition.

^a Living donors.

Complications

Several case series have focused on complications after small bowel and multivisceral transplantation. For example, Nagai et al (2016) reported on cytomegalovirus (CMV) infection after intestinal or multivisceral transplantation at a single-center in the U. S.^13, A total of 210 patients had either an intestinal transplant, multivisceral transplant, or modified multivisceral transplant between 2003 and 2014. The median length of follow-up was 2.1 years. Thirty-four (16%) patients developed CMV infection at a median of 347 days after transplantation. Nineteen patients had tissue-invasive CMV disease. CMV infection was significantly associated with rejection (odds ratio, 2.6; p<0.01) and adversely affected patient survival (hazard ratio, 2.7; p<0.001). In a 2016 report from another U.S. center, Timpone et al (2016) reported that 16 (19%) of 85 patients undergoing intestinal or multivisceral transplantation developed CMV infection a mean of 139 days (range, 14-243 days) postoperatively.^14,

Wu et al (2016) investigated the incidence and risk factors of acute antibody-mediated rejection (ABMR) among patients undergoing intestinal transplantation (n=175).^15, All patients were 25 years of age. Acute ABMR was diagnosed by clinical evidence; histologic evidence of tissue damage; focal or diffuse linear C4d deposition; and circulating anti-human leukocyte antigen antibodies. Of the 175 intestinal transplants, 58% were liver-free grafts, 36% included a liver graft, and 6.3% were retransplantations. Eighteen cases of acute ABMR were identified-14 (14%) among the patients undergoing first liver-free transplantation, 2 (3%) among patients undergoing liver and small bowel transplantations, and 2 (18%) among the patients undergoing retransplantation. Graft failure occurred in 67% of patients with acute ABMR. The presence of a donor-specific antibody and a liver-free graft were associated with the development of acute ABMR.

In a series by Cromvik et al (2016), 5 (19%) of 26 patients were diagnosed with graft-versus-host disease after intestinal or multivisceral transplantation.^16, Risk factors for graft-versus-host disease were: malignancy as a cause of transplantation; neoadjuvant chemotherapy; or brachytherapy before transplantation.

In a retrospective study, Florescu et al (2012) reported on bloodstream infections among 98 children (>18 years) with small bowel and combined organ transplants.^17, Seventy-seven (79%) underwent small bowel transplant in combination with a liver, kidney, or kidney and pancreas, and 21 had an isolated small bowel transplant. After a median follow-up of 52 months, 58 (59%) patients had survived. 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 (69.4%) patients experienced at least 1 episode of bloodstream infection. The 1-year survival rate for patients with bloodstream infections was 72% compared with 87% in patients without bloodstream infections (p=0.056 for the difference in survival in patients with and without bloodstream infections).

Wu et al (2011) reported on 241 patients who underwent intestinal transplantation.^18, Of these, 147 (61%) had multivisceral transplants, 65 (27%) had small bowel transplants, and 29 (12%) had small bowel/liver transplants. Recipients included 151 (63%) children and 90 (37%) adults. Twenty-two (9%) patients developed graft-versus-host disease. Children younger than 5 years old were most likely to develop this condition (13.2% [16/121]) than children between 5 and 18 years (6.7% [2/30]) and adults older than 18 years (4.4% [9/90]).

Human Immunodeficiency Virus-Positive Transplant Recipients

Solid-organ transplant for patients who are HIV-positive was historically controversial, due to the long-term prognosis for HIV positivity and the impact of immunosuppression on HIV disease. No studies reporting on outcomes in HIV-positive patients who received small bowel and liver or multivisceral transplants were identified in literature reviews.

Current Organ Procurement Transplantation Network policy permits HIV-positive transplant candidates.^19,

The British HIV Association and the British Transplantation Society (2017) updated their guidelines on kidney transplantation in patients with HIV disease.^20, These criteria may be extrapolated to other organs:

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

Intestinal transplantation procedures are infrequently performed and only relatively small case series, generally, single-center, are available. For patients experiencing significant complications from total parenteral nutrition , which can lead to liver failure and repeated infections, these case series have shown reasonably high posttransplant survival rates in patients who have a high probability of death without treatment. Guidelines and U.S. federal policy no longer view HIV infection as an absolute contraindication for solid organ transplantation.

Retransplantation of Small Bowel and Liver or Multivisceral Organs

Clinical Context and Test Purpose

The purpose of small bowel and liver retransplant alone or multivisceral retransplant in patients who have a failed small bowel and liver or multivisceral transplant without contraindications for retransplant is to provide a treatment option that is an alternative to or an improvement on existing therapies.

The question addressed in this policy is: Does small bowel and liver retransplant alone or multivisceral retransplant improve the net health outcome in individuals with a failed small bowel and liver or multivisceral transplant and no contraindications to retransplant?

The following PICOs were used to select literature to inform this policy.

Patients

The relevant population of interest are individuals with a failed small bowel and liver or multivisceral transplant without contraindications for retransplant.

Interventions

The therapy being considered is small bowel and liver retransplant alone or multivisceral retransplant.

Comparators

The following practices are currently being used to make decisions about failed small bowel and liver or multivisceral transplant when there are no contraindications for retransplant: medical management and parenteral nutrition.

Outcomes

The general outcomes of interest are OS, morbid events, treatment-related mortality, and treatment-related morbidity, including short- and long-term graft survival and 1- and 5-year OS.

Case Series

Evidence for the use of retransplantation to treat individuals who have failed intestinal transplantations includes several case series, mostly from single institutions. The case series by Desai et al (2012) analyzed records from the United Network for Organ Sharing database.^6, Among the case series described in Table 3, reasons for retransplantations included: acute rejection, chronic rejection, CMV, liver failure, lymphoproliferative disorder, and graft dysfunction. Survival rates for retransplantations are listed in Table 4.

Table 3. Summary of Key Case Series Characteristics for Retransplantations

Study	Country	N	Median Age (Range), y	Interventions		Follow-Up, (Range), mo
				Treatment	n
Ekser et al (2018)21,	U.S.	18b	27.0 (17.4)a (0.9 to 57)	Isolated IT Modified MVT Multivisceral graft	1 1 16	NR
Lacaille et al (2017)7,	France	10	13 (5-16)	Isolated IT Combined liver IT	3 7	4
Desai et al (2012)6,	U.S.	72 (adults) 77 (children)	NR	Adults: Isolated IT Combined liver IT Children: Isolated IT Combined liver IT	41 31 28 49	NR
Abu-Elmagd et al (2009)5,	U.S.	47	NR	Isolated IT Combined liver IT Multivisceral graft	31 7 9	NR
Mazariegos et al (2008)22,	U.S.	14	9.4 (3.2-22.7)	Isolated IT Combined liver IT Multivisceral graft	1 3 10	55.9

IT: intestinal transplantation; MVT: multivisceral transplantation; NR: not reported.

^a Mean (standard deviation).

^b Of a cohort of 218 transplants or retransplant procedures.

Study	Interventions		Survival	Off TPN
	Treatment	n
Ekser et al (2018)21,	Isolated IT Modified MVT Multivisceral graft	1 1 16	Graft survival: 71% at 1 y; 56% at 3 y; 44% at 5 y Patient survival: 71% at 1 y; 47% at 3 y; 37% at 5 y	NR
Lacaille et al (2017)7,	Isolated IT Combined liver IT	3 7	All transplantations combined: 30% at last follow-up	NR
Desai et al (2012)6,	Adults: Isolated IT Combined liver IT Children: Isolated IT Combined liver IT	41 31 28 49	Adults: 80% at 1 y; 47% at 3 y; 29% at 5 y 63% at 1 y; 56% at 3 y; 47% at 5 y Children: 81% at 1 y; 74% at 3 y; 57% at 5 y 42% at 1 y; 42% at 3 y; 42% at 5 y	NR
Abu-Elmagd et al (2009)5,	Isolated IT Combined liver IT Multivisceral graft	31 7 9	All transplantations combined: 69% at 1 y 47% at 5 y	NR
Mazariegos et al (2008)22,	Isolated IT Combined liver IT Multivisceral graft	1 3 10	All transplantations combined: 71% at last follow-up	100%

Section Summary: Retransplantation of Small Bowel and Liver or Multivisceral Organs

Evidence for retransplantations derives mostly from single-center case series, though one series used records from the United Network for Organ Sharing database. Although limited in quantity, the available follow-up data after retransplantation have suggested reasonably high survival rates after small bowel and liver transplants and multivisceral retransplantation in patients who continue to meet criteria for transplantation.

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 limited number of case series. The relevant outcomes are OS, morbid events, and treatment-related mortality and morbidity. These transplant procedures are infrequently performed and few reported case series exist. However, results from the available case series have revealed fairly high postprocedural survival rates. Given these results and the exceedingly poor survival rates of patients who exhaust all other treatments, transplantation may prove not only to be the last option but also a beneficial one. Transplantation is contraindicated for patients in whom the procedure is expected to be futile due to comorbid disease, or in whom posttransplantation care is expected to significantly worsen comorbid conditions. The evidence is sufficient to determine that the technology results in a meaningful 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. The relevant outcomes are OS, morbid events, and treatment-related mortality and morbidity. Although limited in quantity, the available post retransplantation data have suggested reasonably high survival rates. Given exceedingly poor survival rates without retransplantation of patients 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 patients. Retransplantation is contraindicated for patients in whom the procedure is expected to be futile due to comorbid disease or in whom posttransplantation care is expected to significantly worsen comorbid conditions. The evidence is sufficient to determine that the technology results in a meaningful improvement in the net health outcome.

SUPPLEMENTAL INFORMATION

Practice Guidelines and Position Statements

American Gastroenterological Association

The American Gastroenterological Association (2003) published a position statement on short bowel syndrome and intestinal transplantation.^23,The statement noted that only patients 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 American Society of Transplantation (2001) issued a position paper on indications for pediatric intestinal transplantation.^24,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, nonresectable malignancies, autoimmune diseases, and insufficient vascular patency.

U.S. Preventive Services Task Force Recommendations

Not applicable.

Ongoing and Unpublished Clinical Trials

A search of ClinicalTrials.gov in June 2019 did not identify any ongoing or unpublished trials that would likely influence this review.]
________________________________________________________________________________________

Horizon BCBSNJ Medical Policy Development Process:

This Horizon BCBSNJ Medical Policy (the “Medical Policy”) has been developed by Horizon BCBSNJ’s Medical Policy Committee (the “Committee”) consistent with generally accepted standards of medical practice, and reflects Horizon BCBSNJ’s view of the subject health care services, supplies or procedures, and in what circumstances they are deemed to be medically necessary or experimental/ investigational in nature. This Medical Policy also considers whether and to what degree the subject health care services, supplies or procedures are clinically appropriate, in terms of type, frequency, extent, site and duration and if they are considered effective for the illnesses, injuries or diseases discussed. Where relevant, this Medical Policy considers whether the subject health care services, supplies or procedures are being requested primarily for the convenience of the covered person or the health care provider. It may also consider whether the services, supplies or procedures are more costly than an alternative service or sequence of services, supplies or procedures that are at least as likely to produce equivalent therapeutic or diagnostic results as to the diagnosis or treatment of the relevant illness, injury or disease. In reaching its conclusion regarding what it considers to be the generally accepted standards of medical practice, the Committee reviews and considers the following: all credible scientific evidence published in peer-reviewed medical literature generally recognized by the relevant medical community, physician and health care provider specialty society recommendations, the views of physicians and health care providers practicing in relevant clinical areas (including, but not limited to, the prevailing opinion within the appropriate specialty) and any other relevant factor as determined by applicable State and Federal laws and regulations.

___________________________________________________________________________________________________________________________

Index:
Small Bowel/Liver and Multivisceral Transplant
Small Bowel/Liver Transplant
Multivisceral Transplant, Small Bowel/Liver
Liver/Small Bowel and Multivisceral Transplant
Transplant, Multivisceral
Transplant, Small Bowel/Liver

References:
1. Bharadwaj S, Tandon P, Gohel TD, et al. Current status of intestinal and multivisceral transplantation. Gastroenterol Rep (Oxf). Feb 2017;5(1):20-28. PMID 28130374.

2. Loo L, Vrakas G, Reddy S, et al. Intestinal transplantation: a review. Curr Opin Gastroenterol. May 2017;33(3):203-211. PMID 28282321.

3. 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.

4. Mangus RS, Tector AJ, Kubal CA, et al. Multivisceral transplantation: expanding indications and improving outcomes. J Gastrointest Surg. Jan 2013;17(1):179-186; discussion p 186-177. PMID 23070622.

5. 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. PMID 19730240.

6. Desai CS, Khan KM, Gruessner AC, et al. Intestinal retransplantation: analysis of Organ Procurement and Transplantation Network database. Transplantation. Jan 15 2012;93(1):120-125. PMID 22113492.

7. 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. PMID 27889929.

8. 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). PMID 28295952.

9. 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. PMID 26535775.

10. 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. PMID 27109980.

11. 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. PMID 24507060.

12. 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. PMID 23544434.

13. 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. PMID 26247555.

14. 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. PMID 26853894.

15. Wu GS, Cruz RJ, Jr., Cai JC. Acute antibody-mediated rejection after intestinal transplantation. World J Transplant. Dec 24 2016;6(4):719-728. PMID 28058223.

16. 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. PMID 26915866.

17. Florescu DF, Qiu F, Langnas AN, et al. Bloodstream infections during the first year after pediatric small bowel transplantation. Pediatr Infect Dis J. Mar 29 2012;31(7):700-704. PMID 22466325.

18. 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. PMID 21076376.

19. Organ Procurement and Transplantation Network (OPTN). Organ Procurement and Transplantation Network Policies. 2018; https://optn.transplant.hrsa.gov/media/1200/optn_policies.pdf. Accessed August 29, 2019.

20. 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.

21. Ekser B, Kubal CA, Fridell JA, et al. Comparable outcomes in intestinal retransplantation: Single-center cohort study. Clin Transplant. May 21 2018:e13290. PMID 29782661.

22. Mazariegos GV, Soltys K, Bond G, et al. Pediatric intestinal retransplantation: techniques, management, and outcomes. Transplantation. Dec 27 2008;86(12):1777-1782. PMID 19104421.

23. American Gastroenterological Association (AGA). American Gastroenterological Association medical position statement: short bowel syndrome and intestinal transplantation. Gastroenterology. Apr 2003;124(4):1105-1110. PMID 12671903.

24. 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. PMID 11328544.

25. Center for Medicare & Medicaid Services. National Coverage Determination (NCD) for Intestinal and Multi- Visceral Transplantation (260.5). 2006; https://www.cms.gov/medicare-coverage-database/details/ncd- details.aspx?NCDId=280&ncdver=2&CoverageSelection=National&KeyWord=intestinal&KeyWordLookUp=Title& KeyWordSearchType=And&generalError=Thank+you+for+your+interest+in+the+Medicare+Coverage+Database. +You+may+only+view+the+page+you+attempted+to+access+via+normal+usage+of+the+Medicare+Coverage+ Database.&bc=gAAAACAAAAAAAA%3d%3d&. Accessed August 29, 2019.

Codes:
(The list of codes is not intended to be all-inclusive and is included below for informational purposes only. Inclusion or exclusion of a procedure, diagnosis, drug or device code(s) does not constitute or imply authorization, certification, approval, offer of coverage or guarantee of payment.)

CPT*

44120
44121
44132
44133
44715
44720
44721
44799
47133
47135
47140
47141
47142
47143
47144
47145
47146
47147

S2053
S2054
S2055