Clinical Policy: Intestinal and Multivisceral Transplant Form

CENTENE® Corporation

Clinical Policy: Intestinal and Multivisceral Transplant Reference Number: CP.MP.58 Date of Last Revision: 05/24 Coding Implications Revision Log

See Important Reminder at the end of this policy for important regulatory and legal information.

Description This policy describes the medical necessity criteria for the review of intestinal and multivisceral transplant requests.

Policy/Criteria It is the policy of health plans affiliated with Centene Corporation® that any of the intestinal and/or multivisceral transplantation procedures listed in I are medically necessary for pediatric and adult members/enrollees to restore function in those with irreversible intestinal failure when meeting the criteria in section II:

I. Transplantation Procedures A. Isolated intestinal transplantation is indicated for members/enrollees who have only isolated intestinal failure and no liver disease. B. Combined intestinal and liver transplant is indicated in those with intestinal failure and end stage liver disease. C. Multivisceral transplant is indicated in those with intestinal failure and gastrointestinal motility disorders (e.g., chronic idiopathic intestinal pseudo-obstruction, visceral myopathy, visceral neuropathy, total intestinal aganglionosis, and some forms of mitochondrial respiratory chain disorders that affect gastrointestinal motor function), or extensive mesenteric thrombosis.

II. Procedure Criteria: Members/enrollees must have one of the indications in A and none of the contraindications in B: A. Indications, any one of the following:

1. Failure of total parenteral nutrition (TPN) as indicated by one of the following: a. Impending or overt liver failure due to TPN, indicated by elevated serum bilirubin and/or liver enzymes, splenomegaly, thrombocytopenia, gastro-esophageal varices, coagulopathy, peristomal bleeding, or hepatic fibrosis/cirrhosis; b. Thrombosis of two or more central veins, including jugular, subclavian, and femoral veins; c. Two or more episodes of systemic sepsis due to line infection, per year, or one episode of septic shock, acute respiratory distress syndrome, and/or line related fungemia; d. Frequent episodes of dehydration despite IV fluid supplementation; e. Other complications leading to loss of vascular access;
2. High risk of death if transplant is not performed;
3. Severe short bowel syndrome (gastrostomy, duodenostomy, and/or residual small bowel <10 cm in infants and <20 cm in adults);

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4. Frequent hospitalizations for complications directly related to intestinal failure (e.g. opioid dependency due to pain management for intestinal failure, or pseudo- obstruction);
5. Significant hepatic cirrhosis associated with diffuse post-mesenteric thrombosis;

B. Does not have ANY of the following contraindications:

1. Malignancy with high risk of recurrence or death related to cancer;
2. Other severe uncontrolled medical condition expected to limit survival after transplant;
3. Glomerular filtration rate < 30 mL/min/1.73m² unless being considered for multi-organ transplant;
4. HIV infection with detectable viral load unless all of the following are noted: a. CD4 cell count >200 cells/mm3; b. Absence of active AIDS-defining opportunistic infection (unless treated efficaciously or prevented, can be included on the heart transplant waiting list) or malignancy; c. Member/enrollee is currently on effective ART (antiretroviral therapy);
5. Presence of other GI diseases;
6. Acute liver failure, or cirrhosis with portal hypertension or synthetic dysfunction unless being considered for multi-organ transplant;
7. Septic shock;
8. Progressive cognitive impairment;
9. Stroke, acute coronary syndrome, or myocardial infarction (excluding demand ischemia) within 30 days;
10. Chronic infection with highly virulent and/or resistant microbes that are poorly controlled pre-transplant;
11. Inability to adhere to the regimen necessary to preserve the transplant, even with caregiver support;
12. Absence of an adequate or reliable social support system;
13. Active substance use or dependence including current tobacco use, vaping, marijuana use (unless prescribed by a licensed practitioner), or IV drug use without convincing evidence of risk reduction behaviors (unless urgent transplant timelines are present, in which case a commitment to reducing behaviors is acceptable). Serial blood and urine testing may be used to verify abstinence from substances that are of concern.

Background Intestinal transplantation is a therapeutic option for patients with intestinal failure. Intestinal failure is the loss of absorptive capacity of the small bowel secondary to severe primary gastrointestinal disease or surgically induced short bowel syndrome (SBS). The normal small intestine length varies widely, ranging from three to eight meters. SBS occurs when there is approximately < 200 cm of small bowel remaining.

Multi-visceral transplantation includes the stomach, duodenum, pancreas, liver, and small intestine. A modified version excludes the liver if the recipient’s liver is normal. A kidney transplant is occasionally included if the recipient has end-stage renal disease.⁴

Common indications for intestinal transplantation in children include:

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• Small bowel atresia • Gastroschisis • Aganglionosis (Hirschsprung’s disease) • Infections such as necrotizing enterocolitis and mesenteric ischemia • Intestinal pseudo-obstruction • Microvillus inclusion disease • Short gut syndrome • Trauma • Crohn’s disease • Midget volvulus • Massive resection secondary to tumor

Common indications for intestinal transplantation in adults include: • Short gut syndrome • Mesenteric ischemia following thrombosis, embolism, volvulus, or trauma • Crohn’s disease • Small bowel tumors • Small bowel secretory disorders • Tumors of mesenteric root and retroperitoneum • Trauma • Volvulus • Pseudo-obstruction • Radiation enteritis

Guideline Recommendations The British Society of Gastroenterology recommends patients with SBS, including irreversible intestinal failure, expected to die prematurely on TPN, should be referred for consideration of short bowel transplant where appropriate.¹³

The American Society of Transplantation issued a position paper on indications for pediatric intestinal transplantation. The AST recommends intestinal transplantation only for TPN- dependent children with intestinal failure who have or are likely to develop life-threatening TPN- related complications such as liver disease, recurrent sepsis, and threatened loss of central venous access. The AST stated that intestinal transplantation should not be performed solely because of continued dependence on TPN.⁸

Coding Implications This clinical policy references Current Procedural Terminology (CPT®). CPT® is a registered trademark of the American Medical Association. All CPT codes and descriptions are copyrighted 2023, American Medical Association. All rights reserved. CPT codes and CPT descriptions are from the current manuals and those included herein are not intended to be all-inclusive and are included for informational purposes only. Codes referenced in this clinical policy are for informational purposes only. Inclusion or exclusion of any codes does not guarantee coverage. Providers should reference the most up-to-date sources of professional coding guidance prior to the submission of claims for reimbursement of covered services.

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References

1. Buchman AL, Iyer K, Fryer J. Parenteral nutrition-associated liver disease and the role for<br> isolated intestine and intestine/liver transplantation. Hepatology. 2006;43(1):9-19.<br> doi:10.1002/hep.20997
2. National coverage determination: intestinal and multi-visceral transplantation (260.5).<br> Centers for Medicare and Medicaid Services Web site. https://www.cms.gov/medicare-<br> coverage-database/Search.aspx. Published May 11, 2006. Accessed March 26, 2024.
3. Khan FA, Selvaggi G. Overview of intestinal and multivisceral transplantation. UpToDate.<br> www.uptodate.com. Published August 17, 2022. Accessed March 26, 2024.
4. Gilroy RK. Intestinal and multivisceral transplantation. Medscape. https://reference.medscape.com/.<br> Published May 24, 2018 (updated November 1, 2023). Accessed March 26, 2024.
5. Andacoglu, OM. Intestinal transplantation. Medscape. https://reference.medscape.com/.<br> Published January 18, 2017 (updated March 16, 2021). Accessed March 26, 2024.
6. Kato T, Tzakis AG, Selvaggi F, et al. Intestinal and multivisceral transplantation in<br> children. Ann Surg. 2006;243(6):756-766. doi:10.1097/01.sla.0000219696.11261.13
7. Kaufman SS, Atkinson JB, Bianchi A, et al. Indications for pediatric intestinal<br> transplantation: a position paper of the American Society of Transplantation. Pediatr<br> Transplant. 2001;5(2):80-87. doi:10.1034/j.1399-3046.2001.005002080.x
8. Kubal CA, Mangus RS, Tector AJ. Intestine and multivisceral transplantation: current status<br> and future directions. Curr Gastroenterol Rep. 2015;17(1):427. doi:10.1007/s11894-014-<br> 0427-8
9. Levitsky J, Doucette K; AST Infectious Diseases Community of Practice. Viral hepatitis in<br> solid organ transplantation. Am J Transplant. 2013;13 Suppl 4:147-168.<br> doi:10.1111/ajt.12108
10. Lladó L, Fabregat J, Castellote J, et al. Management of portal vein thrombosis in liver<br> transplantation: influence on morbidity and mortality. Clin Transplant. 2007;21(6):716-721.<br> doi:10.1111/j.1399-0012.2007.00728.x
11. Mangus RS, Tector AJ, Kubal CA, Fridell JA, Vianna RM. Multivisceral transplantation:<br> expanding indications and improving outcomes. J Gastrointest Surg. 2013;17(1):179-p.187.<br> doi:10.1007/s11605-012-2047-7
12. Nightingale J, Woodward JM; Small Bowel and Nutrition Committee of the British Society<br> of Gastroenterology. Guidelines for management of patients with a short bowel. Gut.<br> 2006;55 Suppl 4(Suppl 4):iv1-iv12. doi:10.1136/gut.2006.091108
13. Holzheimer RG, Mannick JA, eds. Surgical Treatment: Evidence-Based and Problem-<br> Oriented. Munich: Zuckschwerdt; 2001.
14. Tzakis AG, Kato T, Levi DM, et al. 100 multivisceral transplants at a single center. Ann<br> Surg. 2005;242(4):480-493. doi:10.1097/01.sla.0000183347.61361.7a

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15. U.S. Department of Veteran Affairs. HIV. Laboratory Tests and HIV: Entire Lesson.<br> https://www.hiv.va.gov/patient/diagnosis/labtests-single-page.asp. Accessed March 26, 2024.
16. Vianna RM, Mangus RS, Kubal C, Fridell JA, Beduschi T, Tector AJ. Multivisceral<br> transplantation for diffuse portomesenteric thrombosis. Ann Surg. 2012;255(6):1144-1150.<br> doi:10.1097/SLA.0b013e31825429c0
17. Wu G, Cruz RJ. Liver inclusion improves outcomes of intestinal retransplantation in adults.<br> [Corrected] [published correction appears in Transplantation. 2015<br> Aug;99(8):e118]. Transplantation. 2015;99(6):1265-1272.<br> doi:10.1097/TP.0000000000000488
18. Bharadwaj S, Tandon P, Gohel TD, et al. Current status of intestinal and multivisceral<br> transplantation. Gastroenterol Rep (Oxf). 2017;5(1):20-28. doi:10.1093/gastro/gow045
19. Pironi L, Arends J, Bozzetti F, et al. ESPEN guidelines on chronic intestinal failure in adults<br> [published correction appears in Clin Nutr. 2017 Apr;36(2):619]. Clin Nutr. 2016;35(2):247-<br> 307. doi:10.1016/j.clnu.2016.01.020
20. Hawksworth JS, Desai CS, Khan KM, et al. Visceral transplantation in patients with<br> intestinal-failure associated liver disease: Evolving indications, graft selection, and<br> outcomes. Am J Transplant. 2018;18(6):1312-1320. doi:10.1111/ajt.14715
21. Camilleri M. Chronic intestinal pseudo-obstruction: Etiology, clinical manifestations, and<br> diagnosis. UpToDate. www.uptodate.com. Published February 17, 2022. Accessed March<br> 26, 2024.
22. Roest S, Hesselink DA, Klimczak-Tomanik D, et al. Incidence of end-stage renal disease<br> after heart transplantation and effect of its treatment on survival. ESC Heart Fail.<br> 2020;7(2):533-541. doi:10.1002/ehf2.12585
23. Harbell J, Terrault NA, Stock P. Solid organ transplants in HIV-infected patients. Curr<br> HIV/AIDS Rep. 2013;10(3):217-225. doi:10.1007/s11904-013-0170-z

Important Reminder This clinical policy has been developed by appropriately experienced and licensed health care professionals based on a review and consideration of currently available generally accepted standards of medical practice; peer-reviewed medical literature; government agency/program approval status; evidence-based guidelines and positions of leading national health professional organizations; views of physicians practicing in relevant clinical areas affected by this clinical policy; and other available clinical information. The Health Plan makes no representations and accepts no liability with respect to the content of any external information used or relied upon in developing this clinical policy. This clinical policy is consistent with standards of medical practice current at the time that this clinical policy was approved. “Health Plan” means a health plan that has adopted this clinical policy and that is operated or administered, in whole or in part, by Centene Management Company, LLC, or any of such health plan’s affiliates, as applicable.

The purpose of this clinical policy is to provide a guide to medical necessity, which is a component of the guidelines used to assist in making coverage decisions and administering benefits. It does not constitute a contract or guarantee regarding payment or results. Coverage decisions and the administration of benefits are subject to all terms, conditions, exclusions and limitations of the coverage documents (e.g., evidence of coverage, certificate of coverage, policy, contract of insurance, etc.), as well as to state and federal requirements and applicable Health Plan-level administrative policies and procedures.

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This clinical policy is effective as of the date determined by the Health Plan. The date of posting may not be the effective date of this clinical policy. This clinical policy may be subject to applicable legal and regulatory requirements relating to provider notification. If there is a discrepancy between the effective date of this clinical policy and any applicable legal or regulatory requirement, the requirements of law and state regulation shall govern. The Health Plan retains the right to change, amend or withdraw this clinical policy, and additional clinical policies may be developed and adopted as needed, at any time.

This clinical policy does not constitute medical advice, medical treatment or medical care. It is not intended to dictate to providers how to practice medicine. Providers are expected to exercise professional medical judgment in providing the most appropriate care, and are solely responsible for the medical advice and treatment of members/enrollees. This clinical policy is not intended to recommend treatment for members/enrollees. Members/enrollees should consult with their treating physician in connection with diagnosis and treatment decisions.

Providers referred to in this clinical policy are independent contractors who exercise independent judgment and over to whom the Health Plan has no control or right of control. Providers are not agents or employees of the Health Plan.

This clinical policy is the property of the Health Plan. Unauthorized copying, use, and distribution of this clinical policy or any information contained herein are strictly prohibited. Providers, members/enrollees and their representatives are bound to the terms and conditions expressed herein through the terms of their contracts. Where no such contract exists, members/enrollees and their representatives agree to be bound by such terms and conditions by providing services to members/enrollees and/or submitting claims for payment for such services.

Note: For Medicaid members/enrollees, when state Medicaid coverage provisions conflict with the coverage provisions in this clinical policy, state Medicaid coverage provisions take precedence. Please refer to the state Medicaid manual for any coverage provisions pertaining to this clinical policy.

Note: For Medicare members/enrollees, to ensure consistency with the Medicare National Coverage Determinations (NCD) and Local Coverage Determinations (LCD), all applicable NCDs, LCDs, and Medicare Coverage Articles should be reviewed prior to applying the criteria set forth in this clinical policy. Refer to the CMS website at http://www.cms.gov for additional information.

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