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Clinical Nutrition 40 (2021) 5196e5220
Contents lists available at ScienceDirect
Clinical Nutrition
journal homepage: http://www.elsevier.com/locate/clnu
ESPEN Guideline
ESPEN practical guideline: Clinical nutrition in chronic intestinal
failure
a, *, 2 b, c, 2 d e 3
Cristina Cuerda , Loris Pironi , Jann Arends , Federico Bozzetti , Lyn Gillanders ,
f g 3 h f
Palle Bekker Jeppesen , Francisca Joly , Darlene Kelly , Simon Lal , Michael Staun ,
i j k l
Kinga Szczepanek , Andre Van Gossum , Geert Wanten ,Stephane Michel Schneider ,
m
Stephan C. Bischoff , the Home Artificial Nutrition & Chronic Intestinal Failure Special
Interest Group of ESPEN1
a ~
Nutrition Unit, Hospital General Universitario Gregorio Maranon, Madrid, Spain
b Alma Mater Studiorum e University of Bologna, Department of Medical and Surgical Sciences, Italy
c IRCCS Azienda Ospedaliero-Universitaria di Bologna, Centre for Chronic Intestinal Failure e Clinical Nutrition and Metabolism Unit, Italy
d Department of Medicine I, Medical Center e University of Freiburg, Faculty of Medicine, University of Freiburg, Germany
e Faculty of Medicine, University of Milan, Milan, Italy
f Rigshospitalet, Department of Intestinal Failure and Liver Diseases Gastroenterology, Copenhagen, Denmark
g ^
Centre for Intestinal Failure, Department of Gastroenterology and Nutritional Support, Hopital Beaujon, Clichy, France
h Intestinal Failure Unit, Salford Royal Foundation Trust, Salford, UK
i General and Oncology Surgery Unit, Stanley Dudrick's Memorial Hospital, Skawina, Poland
j ^
Medico-Surgical Department of Gastroenterology, Hopital Erasme, Free University of Brussels, Belgium
k Intestinal Failure Unit, Department of Gastroenterology and Hepatology, Radboud University Nijmegen Medical Centre, Nijmegen, the Netherlands
l Gastroenterology and Clinical Nutrition, CHU of Nice, University of Nice Sophia Antipolis, Nice, France
mDepartment of Nutritional Medicine and Prevention, University of Hohenheim, Stuttgart, Germany
articleinfo summary
Article history: Background: This practical guideline is based on the ESPEN Guidelines on Chronic Intestinal Failure in
Received 23 June 2021 Adults.
Accepted 2 July 2021 Methodology: ESPEN guidelines have been shortened and transformed into flow charts for easier use in
clinical practice. The practical guideline is dedicated to all professionals including physicians, dieticians,
Keywords: nutritionists, and nurses working with patients with chronic intestinal failure.
Guideline Results: This practical guideline consists of 112 recommendations with short commentaries for the
Intestinal failure managementandtreatmentofbenignchronicintestinalfailure,including homeparenteral nutritionand
Homeparenteral nutrition its complications, intestinal rehabilitation, and intestinal transplantation.
Intestinal transplantation Conclusion: This practical guideline gives guidance to health care providers involved in the management
Short bowel syndrome
Intestinal pseudo-obstruction of patients with chronic intestinal failure.
©2021EuropeanSocietyforClinicalNutritionandMetabolism.PublishedbyElsevier.Allrightsreserved.
1. Introduction
* Corresponding author. Nutrition Unit, Hospital General Universitario Gregorio Intestinal failure (IF) is defined as the reduction of gut function
~ below the minimum necessary for the absorption of macronutri-
Maranon, Doctor Esquerdo 46, 28007 Madrid, Spain.
E-mail address: cuerda.cristina@gmail.com (C. Cuerda). ents and/or water and electrolytes, such that intravenous supple-
1 Based on ESPEN Guidelines on Chronic Intestinal Failure in Adults: Loris mentation is required to maintain health and/or growth.
Pironi, Jann Arends, Federico Bozzetti, Cristina Cuerda, Lyn Gillanders, Palle Bekker The reduction of the gut's absorptive function that does not
Jeppesen, Francisca Joly, Darlene Kelly, Simon Lal, Michael Staun, Kinga Szczepanek, require any intravenous supplementation to maintain health and/
Andre Van Gossum, Geert Wanten, Stephane Michel Schneider, the Home Artificial
Nutrition & Chronic Intestinal Failure Special Interest Group of ESPEN. Clin Nutri- or growth, can be considered as intestinal insufficiency.
tion 35: 247e307, 2016. IF can be classified according to different criteria:
2 C.C. and L.P. share co-first authorship.
3 Retired
https://doi.org/10.1016/j.clnu.2021.07.002
0261-5614/© 2021 European Society for Clinical Nutrition and Metabolism. Published by Elsevier. All rights reserved.
C. Cuerda, L. Pironi, J. Arends et al. Clinical Nutrition 40 (2021) 5196e5220
Abbreviations IFALD intestinal failure associated liver disease
ITx intestinal transplantation
CIPO chronic intestinal pseudo-obstruction LILT longitudinal intestinal lengthening and tailoring
CRBSI catheter-related bloodstream infection MCT medium-chain triglycerides
CRI catheter-related infection NST nutrition support team
CRVT catheter-related venous thrombosis ONS oral nutritional supplements
CVC central venous catheter PICC peripherally Inserted Central Venous Catheter
DXA dual-energy X-ray absorptiometry PN parenteral nutrition
EFA essential fatty acids PUFA poly-unsaturated fatty acids
EN enteral nutrition QoL quality of life
GLP-2 glucagon-like peptide-2 RCT randomized controlled trial
HEN homeenteral nutrition SBS short bowel syndrome
HPN homeparenteral nutrition SRSB segmental reversal of the small bowel
IF intestinal failure STEP serial transverse enteroplasty
Functional classification (type I or an acute, short-term condi- parenteral nutrition (HPN). The guideline process was funded
tion, type II or a prolonged acute condition, and type III a exclusively by the ESPEN society. The shortened guideline and
potentially chronic condition). dissemination were funded in part by the UEG society, and also by
Pathophysiological classification (short bowel, intestinal fistula, the ESPEN society.
intestinal dysmotility, mechanical obstruction, and extensive
small bowel mucosal disease). 3. Results
Clinical classification (on the basis of the energyand the volume
of the required intravenous supplementation) ManagementandTreatmentofbenignChronicIntestinalFailure
covers 112 recommendations structured in 4 main chapters and
The clinical condition associated with the remaining small diverse subchapters (Fig. 1).
bowel in continuity of less than 200 cm is defined as short bowel
syndrome(SBS).Dependingontheanatomyoftheremnantbowel, 3.1. Home parenteral nutrition (HPN)
threecategoriesofSBSareidentified:end-jejunostomy,jejunocolic
anastomosis, and jejunoileal anastomosis with both the ileo-cecal 3.1.1. Management of HPN (Fig. 2)
valve and the entire colon in continuity. The management of HPN is summarized in Fig. 2.
Chronicintestinalfailure(CIF)maybetheconsequenceofsevere
gastrointestinal or systemic benign diseases, or the end stage of 3.1.1.1. General recommendations (aims of HPN, audits, selection of
intra-abdominalorpelviccancer.Thepresentguidelineislimitedto patients, discharge from hospital). 1)Werecommendthattheaims
CIF due to benign disease in adults, where the term benign means of an HPN program include provision of evidence-based ther-
the absence of end-stage malignant disease. apy, preventionofHPNerelatedcomplicationssuchascatheter-
relatedinfections(CRI)andmetaboliccomplicationsandensure
2. Methodology quality of life (QoL) is maximized.
(R1, Grade of evidence: very low)
This practical guideline consists of 112 recommendations and is Commentary
based on the ESPEN Guidelines on Chronic Intestinal Failure in The aims of a safe and effective HPN program must focus on
Adults [1]. The original guideline was shortened by restricting the therapy outcomes. It is important that CRI are diagnosed early and
commentariestothegatheredevidenceandliteratureonwhichthe treatedeffectivelytominimizetheassociatedrisks.AllHPN-related
recommendations are based on. The recommendations were not complications including catheter obstruction, central venous
changed (except “artificial nutrition” was replaced by “medical thrombosis,liverdisease,andosteoporosis,shouldberecognizedas
nutrition” and language adaptions to American English), but the partofregularsurveillanceandtreatedearlywithinanexperienced
presentation of the content was transformed into a graphical pre- nutrition support team (NST) to prevent later irreversible
sentation consisting of decision-making flow charts wherever complications.
possible. The original guideline was developed according to the 2) We recommend regular audit of therapy and outcomes
ESPENmethodology[2]. The experts followed the GRADE method, against standards to ensure safety and efficacy of an HPN
whichisbasedondeterminationsofgradeofevidenceandstrength program.
of recommendation. Grading from High to Very Low was used to (R2, Grade of evidence: very low)
rate the quality of the underlying evidence and the level of cer- Commentary
tainty for effect. In brackets, the original recommendationnumbers To measure and provide evidence of the safety and efficacy of
(R1, R2, …) and the grading is indicated. The strength of recom- the HPN service, there should be regular audits of outcomes and
mendation (strong-weak resulting in “we recommend/do not scrutiny of results concerning HPN-related major complications,
recommend…”orin“wesuggest/donotsuggest…”)wasbasedon
including re-admission rates. Furthermore, a recognized instru-
a consensus discussion, which included expression and delibera- mentfor measuring QoL should be used regularly to monitor HPN
tionofexpertopinions,risk-benefitratioofrecommendation,costs, patients. Accreditation programs for HPN providers must also
and a review of supportive evidence, followed by Delphi rounds ensure regular audit against these quality measures.
and votes until agreement was reached. The working group 3)WerecommendthatpatientsselectedforanHPNprogram
included gastroenterologists, surgeons, endocrinologists, anesthe- have confirmed CIF that despite maximal medical therapy
siologists, and dietitians with long-term expertise in IF and home would lead to deterioration of nutrition and/or fluid status.
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C. Cuerda, L. Pironi, J. Arends et al. Clinical Nutrition 40 (2021) 5196e5220
Management and treatmentofbenign
Chronic IntesƟnal Failure (CIF)
HPN IntesƟnal IntesƟnal Tx ComplicaƟons
rehabilitaƟon of HPN
Figure 10
Manage-
mentof Diet
HPN
Figure 2 Figures 5 Catheter- Disease-
related related
Com- Figure 13
ponents Medical
of HPN
Figure 3 Figures 6
Venous
catheters Surgery
for HPN
Figure 4 Figure 7 Catheter Occlusions Liver Choleli- Renal Bone
Pseudo- infecƟons thrombosis disease thiasis failure / disease
obstruc- stones
Ɵon(CIPO) Special Figure 11 Figure 12
Figure 8
cases
RadiaƟon
enteriƟs
Figure 9
Fig. 1. Structure of the ESPEN practical guideline “Clinical nutrition in chronic intestinal failure” (CIF, chronic intestinal failure, CIPO, chronic intestinal pseudo-obstruction; HPN,
home parenteral nutrition; Tx, transplantation).
Management of HPN
General Devices Training and monitoring PaƟent support
1) We recommend that the aims of an HPN 5) We 6) We recommend that paƟent/caregiver training 10) We suggest that HPN paƟents
program include provision of evidence-based recommend that for HPN management is paƟent-centered with a are encouraged to join nonprofit
therapy, prevenƟon of HPN related complicaƟons HPN paƟents mulƟdisciplinary approach, together with wriƩen groups that provide HPN
such as CRI and metabolic complicaƟons and have access to guidelines. HPN training may take place in educaƟon, support and
ensure QoLismaximized. (R1) infusion pumps hospital or at home. (R6) networking among members. This
or devices with may be beneficial to paƟent
specified safety 7) We recommend regular contact by the HPN consumers of HPN with respect to
2) We recommend regular audit of therapy and features team with paƟents, scheduled according to QoL, depression scores, and
outcomes against standards to ensure safety and together with paƟents' clinical characterisƟcs and requirements. catheter infecƟons. (R10)
efficacy of an HPN program. (R2) ancillary (R7)
products, safe 11) We recommend that CIF
compounding 8) We recommend that laboratory tesƟng is done paƟents are cared for by a NST
3) We recommend that paƟents selected for an and delivery on a regular basis using appropriate tests and with skills and experience in
HPN program have confirmed CIF that despite systems. (R5) Ɵming relaƟve to PN infusion. (R8) intesƟnal failure and HPN
maximal medical therapy would lead to management. (R11)
deterioraƟon of nutriƟon and/or fluid status. (R3) 9) We recommend that QoL for HPN paƟents is
regularly measured using validated tools as part
4) We recommend that prior to discharge, of standard clinical care. Quality of care should be
paƟents are metabolically stable, able to assessed regularly according to recognized
physically and emoƟonally cope with the HPN criteria. (R9)
therapy, and have an adequate home
environment. (R4)
Fig. 2. Management of home parenteral nutrition. For details see text. Abreviations: CIF, chronic intestinal failure; CRI, catherter-related infection; HPN, home parenteral nutrition;
NST, nutrition support team.
(R3, Grade of evidence: very low) leadtodeterioratingnutritionaland/orfluidstatusandshouldhave
Commentary undergone an adequate trial of enteral nutrition (EN), if feasible
All patients who are considered for entry into an HPN program (except, for example, in the case of extreme short bowel). They
should have documentedprolongedCIFwhich,ifuntreated, would should be managed by a clinician and multidisciplinary nutrition
5198
C. Cuerda, L. Pironi, J. Arends et al. Clinical Nutrition 40 (2021) 5196e5220
support team (NST) that have an interest and experience in CIF. To demonstrate understanding of principles of asepsis and its
optimize safety and efficacy, evidence-based procedures and pro- importance together with sterile procedures for commencing
tocols should be used to educate patients and carers (including and discontinuing HPN.
hospital and home care provider staff) on catheter care and for demonstrate safe delivery of HPN according to institutional
monitoring the nutritional, metabolic, and clinical status of the protocol guidelines.
patient. recognize specific problems and symptoms and respond
4) We recommend that prior to discharge, patients are appropriately; these commonly include mechanical problems
metabolically stable, able to physically and emotionally cope with the lines or pumps and febrile episodes.
with the HPN therapy, and have an adequate home have a connected telephone for medical and nursing support,
environment. emergency services, and logistics planning and delivery.
(R4, Grade of evidence: very low) live independently or have adequate care and support.
Commentary haveahomeenvironmentthatprovidesacleanspaceforsterile
The patient and/or carers must be physically and emotionally additions, HPN setup, and connection.
able to undertake HPN training and demonstrate self-care com- have access to a dedicated refrigerator, if needed, for HPN so-
petency prior to discharge. The home situation must be stable and lution storage.
have adequate facilities for safe administration of HPN.
7) We recommend regular contact by the HPN team with
3.1.1.2. Devices. 5)WerecommendthatHPNpatientshaveaccess patients, scheduled according to patients’ clinical characteris-
to infusion pumps or devices with specified safety features tics and requirements.
together with ancillary products, safe compounding and de- (R7, Grade of evidence: very low)
livery systems. Commentary
(R5, Grade of evidence: very low) After hospital discharge it is critical that the HPN team contacts
Commentary thepatientsonaregularbasis,initiallyeveryfewdays,thenweekly
Electronic pumpswithappropriatedeliverysetsshouldbeused and eventually monthly as the patient gains confidence. The clini-
where possible to manage and monitor the delivery of HPN. An cianwhoisincontactshouldbepreparedtoclarifyconfusingissues
ambulatory pump further enables these individuals to achieve and also to follow weight, urine output, diarrhea or stoma output,
desired independence. The range of other sterile consumable temperatures before and within an hour of starting the parenteral
productsoraccessoriesrequiredforusebythepatientathomewill nutrition(PN)infusion,andgeneralhealth.Monitoringofhydration
vary, depending on the pump in use and individual patient status is particularly important to prevent hospitalization with
requirements. dehydration by early provision of extra intravenous fluid. If insulin
Parenteral nutrient admixtures can be compounded in single is required, capillary blood sugars should be performed frequently
bags, two chamberbagsorthreechamberbags.Vitaminsandtrace and also recorded by the HPN team clinicians.
elements can be added prior to infusion in the home setting. Two 8) We recommend that laboratory testing is done on a reg-
and three chamber bags have advantages for HPN patients as they ular basis using appropriate tests and timing relative to PN
have a longer shelf life. Some three chamber bags do not require infusion.
refrigeration which provides advantages for HPN patients while (R8, Grade of evidence: very low)
travelling.Stabilityisalsomarkedlyprolongedbyrefrigeration.This Commentary
þ þ 3-
requires a dedicated refrigerator for HPN solution storage. HPN Electrolytes, including Na ,K ,Cl , HCO , plus studies of renal
admixtures should be visually inspected for lipid emulsion coa- function (creatinine and blood urea nitrogen) should be measured
lescence as well as calcium phosphate precipitates prior to use. frequently until stable, then at regular intervals. Assays of liver
Delivery of HPN admixtures to patients should be in strong enzymes, bilirubin, albumin, and complete blood counts should
containers under known temperature/time conditions to ensure also be monitored on a regular basis. Vitamin levels and trace
safe storage requirements are not exceeded in transit. The ambient element levels are typically done less frequently, often once or
twice annually. Bone mineral densitometry should be done when
temperature of the HPN solution must be kept at 4e8C and air
excluded from a three-chamber bag. HPNisinitiated and at intervals thereafter.
9) We recommend that QoL for HPN patients is regularly
3.1.1.3. Training and monitoring. 6) We recommend that patient/ measuredusingvalidatedtoolsaspartofstandardclinicalcare.
caregiver training for HPN management is patient-centered Quality of care should be assessed regularly according to
with a multidisciplinary approach, together with written recognized criteria.
guidelines. HPN training may take place in hospital or at home. (R9, Grade of evidence: very low)
(R6, Grade of evidence: very low) Commentary
Commentary QoL should be patient-based rather than the clinician's
HPNpatients should be trained by a NST (medical, nursing, di- perspective. Studies acknowledgethe difficultyof trying toidentify
etetic, and pharmacy clinicians with experience in an HPN pro- the effects of the underlying illness, resulting in the need for HPN,
gram) as an inpatient in preparation for the home environment. andtheHPNitself.TheuseofdifferentQoLinstruments,scales,and
ThepatientwillneedtobestableontheHPNregimenbeforebeing lifestyle domains limit comparison among studies. The HPN-QoL®
discharged. is a treatment specific questionnaire for patients with benign un-
Initiation of HPN at home is of interest to patients, health care derlying disease [3]. It is a 48-item questionnaire that focuses on
providers, and third-party payers. The training process may take physical, emotional, and symptomatic issues.
from several days to weeks depending on the patients’ ability to Thequalityofcarecanbereflectedbymeasuringseveralfactors
learn the techniques to ensure safe practice in the home. In a few in practice such as the number of CRI, the incidence of hospital
instances, care in a residential care facility may be an option. readmission for the patient, the QoL, weight change, or the inci-
Before discharge the patient/carer(s) should be able to: dence of dehydration.
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