Clinical Nutrition 34 (2015) 171e180
`
`Contents lists available at ScienceDirect
`
`Clinical Nutrition
`
`j o u r n a l h o m e p a g e : h t t p : / / w w w . e l s e v i e r . c o m / l o c a t e / c l n u
`
`ESPEN endorsed recommendation
`ESPEN endorsed recommendations. Definition and classification
`of intestinal failure in adults
`Loris Pironi a, *, Jann Arends b, Janet Baxter c, Federico Bozzetti d, Rosa Burgos Pelaez e,
`Cristina Cuerda f, Alastair Forbes g, Simon Gabe h, Lyn Gillanders i, Mette Holst j,
`Palle Bekker Jeppesen k, Francisca Joly l, Darlene Kelly m, Stanislaw Klek n, Øivind Irtun o,
`SW Olde Damink p, Marina Panisic q, Henrik Højgaard Rasmussen j, Michael Staun k,
`Kinga Szczepanek n, Andre Van Gossum r, Geert Wanten s, Stephane Michel Schneider t,
`Jon Shaffer u, the Home Artificial Nutrition & Chronic Intestinal Failure and the Acute
`Intestinal Failure Special Interest Groups of ESPEN
`a Center for Chronic Intestinal Failure, St. Orsola-Malpighi Hospital, University of Bologna, Bologna, Italy
`b Tumor Biology Center, Albert-Ludwigs-University, Freiburg, Germany
`c Tayside Nutrition Managed Clinical Network, Dundee, UK
`d Faculty of Medicine, University of Milan, Milan, Italy
`e Nutritional Support Unit, University Hospital Vall d'Hebron, Barcelona, Spain
`f Nutrition Unit, Hospital General Universitario Gregorio Mara~non, Madrid, Spain
`g University of East Anglia, Norwich Research Park, Norwich, UK
`h The Lennard-Jones Intestinal Failure Unit, St Mark's Hospital and Academic Institute, Harrow, UK
`i National Intestinal Failure Service, Auckland City Hospital (AuSPEN), Auckland, New Zealand
`j Centre for Nutrition and Bowel Disease, Department of Gastroenterology, Aalborg University Hospital, Aalborg, Denmark
`k Rigshospitalet, Department of Gastroenterology, Copenhagen, Denmark
`l Centre for Intestinal Failure, Department of Gastroenterology and Nutritional Support, H^opital Beaujon, Clichy, France
`m Division of Gastroenterology and Hepatology, Mayo Clinic College of Medicine, Rochester, Minnesota and Oley Foundation for Home Parenteral
`and Enteral Nutrition, Albany, NY, USA
`n General and Oncology Surgery Unit, Stanley Dudrick's Memorial Hospital, Skawina, Poland
`o Dept. of Gastroenterologic Surgery, University Hospital North-Norway, Tromso, Norway
`p Department of Surgery, Maastricht University Medical Centre, Maastricht, The Netherlands
`q Department for Perioperative Nutrition, Clinic for General Surgery, Military Medica Academy, Belgrade, Serbia
`r Medico-Surgical Department of Gastroenterology, H^opital Erasme, Free University of Brussels, Belgium
`s Intestinal Failure Unit, Department of Gastroenterology and Hepatology, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands
`t Gastroenterology and Clinical Nutrition, CHU of Nice, University of Nice Sophia Antipolis, Nice, France
`u Intestinal failure Unit, Salford Royal Hospital, Salford, UK
`
`a r t i c l e i n f o
`
`s u m m a r y
`
`Article history:
`Received 5 July 2014
`Accepted 23 August 2014
`
`Keywords:
`Intestinal failure
`Short bowel syndrome
`Chronic intestinal pseudo-obstruction
`Enterocutaneous fistulas
`Home parenteral nutrition
`Intestinal transplantation
`
`Background & aims: Intestinal failure (IF) is not included in the list of PubMed Mesh terms, as failure is
`the term describing a state of non functioning of other organs, and as such is not well recognized. No
`scientific society has yet devised a formal definition and classification of IF. The European Society for
`Clinical Nutrition and Metabolism guideline committee endorsed its “home artificial nutrition and
`chronic IF” and “acute IF” special interest groups to write recommendations on these issues.
`Methods: After a Medline Search, in December 2013, for “intestinal failure” and “review”[Publication
`Type], the project was developed using the Delphi round methodology. The final consensus was reached
`on March 2014, after 5 Delphi rounds and two live meetings.
`Results: The recommendations comprise the definition of IF, a functional and a pathophysiological
`classification for both acute and chronic IF and a clinical classification of chronic IF. IF was defined as “the
`reduction of gut function below the minimum necessary for the absorption of macronutrients and/or
`
`* Corresponding author. Center for Chronic Intestinal Failure, Department of Gastroenterology, St. Orsola-Malpighi Hospital, University of Bologna, Via Massarenti, 9, 40138
`Bologna, Italy. Tel./fax: þ39 051 6363073.
`E-mail address: loris.pironi@unibo.it (L. Pironi).
`
`http://dx.doi.org/10.1016/j.clnu.2014.08.017
`0261-5614/© 2014 The Authors. Published by Elsevier Ltd and European Society for Clinical Nutrition and Metabolism. This is an open access article under the CC BY-NC-SA
`license (http://creativecommons.org/licenses/by-nc-sa/3.0/).
`
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`172
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`L. Pironi et al. / Clinical Nutrition 34 (2015) 171e180
`
`water and electrolytes, such that intravenous supplementation is required to maintain health and/or
`growth”.
`Conclusions: This formal definition and classification of IF, will facilitate communication and cooperation
`among professionals in clinical practice, organization and management, and research.
`© 2014 The Authors. Published by Elsevier Ltd and European Society for Clinical Nutrition and
`Metabolism. This is an open access article under the CC BY-NC-SA license (http://creativecommons.org/
`licenses/by-nc-sa/3.0/).
`
`Abbreviations
`
`acute intestinal failure
`AIF
`chronic intestinal failure
`CIF
`chronic idiopathic pseudo-obstruction
`CIPO
`enterocutaneous
`EC
`ESPEN European Society for Clinical Nutrition and
`Metabolism
`intestinal failure
`IF
`intestinal transplantation
`ITx
`HAN&CIFhome artificial nutrition and chronic intestinal
`failure
`home parenteral nutrition
`short bowel syndrome
`
`HPN
`SBS
`
`1. Introduction
`
`Intestinal failure (IF) was first defined in 1981 by Fleming and
`Remington as “a reduction in the functioning gut mass below the
`minimal amount necessary for adequate digestion and absorption
`of food” [1]. IF may be due to acquired or congenital, gastrointes-
`tinal or systemic, benign or malignant diseases, which may affect all
`age categories [2,3]. It may have an abrupt onset, or may be the
`slow, progressive evolution of a chronic illness, and may be a self-
`limiting short-term or a long-lasting condition (chronic intestinal
`failure, CIF). Treatment of CIF relies on intestinal rehabilitation
`programs that aim to restore bowel function through nutrition,
`pharmacological and/or surgical therapy [4]. Patients with irre-
`versible CIF are destined to need life-long home parenteral nutri-
`tion (HPN) or to undergo intestinal transplantation (ITx) [5].
`The definition of IF by Fleming and Remington has been revised
`by other experts [2e6], but no scientific society has yet devised a
`formal definition and classification of IF. Indeed, IF is not included
`in the list of PubMed Mesh terms, as failure is the term describing a
`state of non functioning of organs. A PubMed search on March 15th
`2014, using “intestinal
`failure” as general
`term, nonetheless
`generated a total of 981 items, and showed that the number of
`publications has rapidly grown in the past decades, indicating an
`increased awareness of this condition (Table 1).
`
`Table 1
`PubMed search on March 15th, 2014.
`
`The European Society for Clinical Nutrition and Metabolism
`(ESPEN) has two “special interest groups” devoted to IF, “the home
`artificial nutrition and chronic intestinal failure group (HAN&CIF)”
`established in 1992 and the “acute intestinal failure group (AIF)”
`established in 2010 [7]. The Guideline Committee of ESPEN
`committed the two groups to develop the ESPEN guidelines on IF
`[8] and endorsed them to support the Guidelines with recom-
`mendations on the definition and classification of IF.
`
`2. Material and methods
`
`The project of writing “recommendations on definition and
`classification of IF in adults” was agreed on March 14th 2013, with a
`member of the ESPEN Guideline committee to assist the develop-
`ment of the guidelines on IF, and was formally approved by the AIF
`and the HAN&CIF special interest groups at their meetings held at
`the ESPEN Congress in Leipzig, September 2013. All the members of
`the two groups were invited to be part of the expert panel.
`The work was carried out between December 2013 and February
`2014, using Delphi round methodology [9]. The results of the Del-
`phi rounds were also discussed during the face-to-face winter
`meetings of the two groups.
`Each Delphi round consisted of a proposal, to which each expert
`replied as “agree”, “agree, with suggested minor changes”, or
`“disagree, with suggested major changes”. The first proposal was
`based on a MedLine Search, performed on 10/12/2013, for “intes-
`tinal failure” AND “review”[Publication Type], which resulted in a
`total of 298 articles. Only publications in English specifically dedi-
`cated to the definition and classification of IF were selected. Any
`pertinent publications retrieved from the references of the selected
`papers were also considered. In order to avoid duplicates, only
`those articles with an “original” definition and classification were
`chosen. These initially selected papers, used as starting point for
`the first round are reported in Table 2. The subsequent proposals
`were based on the collected comments as well as on any further
`publications found non systematically but suggested by the ex-
`perts. All the proposals were prepared and circulated by LP. The
`final consensus was reached on March 1st 2014, after 5 Delphi
`rounds (on 16/12/14, 27/12/14, 19/01/14, 25/02/14 and 01/03/14)
`and two live meetings (AIF 11/01/14, HAN&CIF 22/02/14). For the
`purpose of the paper, the following terms were used: “oral
`feeding”, to indicate the ingestion of food, “oral supplementation”
`
`1946e1959
`
`1960e1969
`
`1970e1979
`
`1980e1989
`
`1990e1999
`
`2000e2009
`
`2010e2014 March
`
`Total
`
`Intestinal failure
`[general term]
`Kidney failure
`[MeSH Term]
`Heart failure
`[MeSH Term]
`Liver failure
`[MeSH Term]
`Respiratory failure
`[MeSH Term]
`
`0
`
`393
`
`2184
`
`153
`
`4
`
`0
`
`4226
`
`5141
`
`833
`
`3942
`
`0
`
`13012
`
`7372
`
`1572
`
`9154
`
`20
`
`18086
`
`9420
`
`1594
`
`7892
`
`118
`
`24268
`
`13218
`
`3719
`
`10633
`
`450
`
`39768
`
`30803
`
`6627
`
`13216
`
`399
`
`21790
`
`16811
`
`3382
`
`5805
`
`981
`
`120939
`
`84385
`
`17788
`
`50433
`
`Page 2
`
`

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`L. Pironi et al. / Clinical Nutrition 34 (2015) 171e180
`
`173
`
`Table 2
`Main original definitions and classifications of Intestinal Failure reported in the literature prior to March 15th 2014, in order of publication date. Bold characters indicate the
`original contribution of each paper.
`
`Author, date (ref)
`
`Definition and classification of intestinal failure
`
`Fleming CR and Remington M. 1981 [1]
`
`Irving M. 1995 [10]
`
`Irving M. 2000 [11]
`
`Jeppesen PB and Mortensen PB. 2000 [12]
`
`Nightingale J. 2001 [13]
`
`Shaffer J. 2002 [14]
`
`Buchman AL et al., 2003 [15]
`
`Ding LA and Li jS. 2004 [16]
`
`Goulet O et al., 2004 [17]
`
`Kocoshis SA, 2004 [18]
`
`Jeejeebhoy KN. 2005 [19]
`
`O'Keefe SJD. 2006 [3]
`
`Lal S. (2006) [20]
`
`Messing B and Joly F [21]
`
`Nightingale J and Woodward JM (2006) [22]
`
`Beath S et al., 2008 [23]
`
`Gillanders L. et al., 2008 [24]
`
`NHS National Commissioning Group for
`Highly Specialised Services. 2008 [6]
`
`A reduction in the functioning gut mass below the minimum amount necessary for adequate digestion and
`absorption of food
`The spectrum of intestinal failure covers a wide range of diseases but essentially they can be placed in four major
`categories:
`short bowel syndrome, motility disorders of the bowel (chronic pseudoobstruction), small bowel parenchymaI disease,
`intestinal fistula
`Intestinal failure can be complete or partial, the former typically following total small bowel enterectomy, whilst the
`latter is seen following partial resection.
`The condition can be acute and temporary, as seen with recoverable motility disorders such as ileus and obstruction, or
`chronic and permanent.
`Although a wide spectrum of conditions can be associated with IF, four major underlying causes can be identified.
`These are: (i) the short bowel syndrome; (ii) total parenchymal bowel disease (e.g. Crohn's disease); (iii) motility
`disorders, such as visceral myopathy and chronic intestinal obstruction; and (iv) small bowel fistulation causing
`premature loss of enteric content.
`The principal resulting nutritional disorders are starvation and dehydration, but loss of body mass is frequently
`made worse by catabolism from associated sepsis.
`Treatment is complicated, but has at its core the provision of nutritional support, principally through the
`intravenous route.
`Resolution of IF can occur spontaneously by the process of intestinal adaptation.
`Intestinal failure may be defined by the minimum energy and wet weight absorption required to avoid home
`parenteral nutrition
`Patients with intestinal insufficiency who maintained intestinal autonomy and did not depend on parenteral
`supplements
`Involuntary ingestion below the minimal amount necessary to maintain nutrient and fluid balance, frequently termed
`oral failure, is seen in patients with pseudoobstruction and dysmotility syndromes.
`Intestinal failure occurs ‘when there is reduced intestinal absorption so that macronutrient and/or water and
`electrolyte supplements are needed to maintain health and/or growth’.
`A novel classification of intestinal failure was recently devised to reflect this:
`Type I intestinal failure is classified as self-limiting intestinal failure as occurs following abdominal surgery;
`Type II is intestinal failure in severely ill patients with major resections of the bowel and septic, metabolic and
`nutritional complications requiring multidisciplinary intervention with metabolic and nutritional support to permit
`recovery;
`Type III is chronic intestinal failure requiring long-term nutritional support.
`It has been suggested that intestinal failure is better defined in terms of fecal energy loss rather than residual bowel
`length.
`However, fecal energy loss is a function of both energy intake and energy absorption. Patients who are unable to
`increase their oral intake sufficiently or are unable to absorb sufficient energy despite significantly increased
`intake, are defined as patients with intestinal failure and require parenteral nutrition support.
`Staging of intestinal failure: Acute intestinal failure, Chronic intestinal failure
`Grading of intestinal failure: severe, moderate, mild
`Intestinal failure can be defined as the reduction of functional gut mass below the minimum needed for digestion and
`absorption of nutrient and fluids required for maintenance in adults or growth in children. It has been suggested that IF
`is better defined in terms of fecal energy loss rather than residual bowel length in patients with short bowel syndrome.
`Another approach is to define the degree of IF according to the amount of PN required for maintenance in adults and
`growth in children
`Although intestinal failure can be defined by excessive fecal energy loss, a more widely accepted definition is “the
`inability of the gastrointestinal tract to sustain life autonomously”.
`Gastrointestinal function is inadequate to maintain the nutrition and hydration of the individual without supplements
`given orally or intravenously
`Intestinal failure ‘results from obstruction, dysmotility, surgical resection, congenital defect or diseaseeassociated
`loss of absorption and is characterized by the inability to maintain protein-energy, fluid, electrolyte or micronutrient
`balance’
`Causes of intestinal failure are varied, with self-limiting or ‘Type 1’ intestinal failure occurring relatively commonly
`following abdominal surgery, necessitating short-term fluid or nutritional support. The rarer, ‘Type 2’ intestinal failure,
`is associated with septic, metabolic and complex nutritional complications, usually following surgical resection in
`patients with Crohn's or mesenteric vascular disease.
`In broad terms, intestinal failure can result from intestinal resection, inflammation or fistulization, from mechanical
`or functional intestinal obstruction, or indirectly from the effects of sepsis on the gastrointestinal tract
`The recognized definition of chronic intestinal failure is a nonfunctioning small bowel either removed after severe
`disease leading to very short bowel syndrome, or present but impossible to use by enteral support even accessed
`through jejunostomy (eg, chronic intestinal pseudo-obstruction or extensive villous atrophy diseases).
`IF may be defined and quantified by balance study techniques; however, only few centres have the facilities for these
`difficult metabolic studies, and therefore nutrient/fluid requirements determine whether IF is termed severe,
`moderate, or mild. Severe is when parenteral, moderate when enteral, and mild when oral nutritional fluid
`supplements are needed.
`Intestinal failure is defined as the inability of the alimentary tract to digest and absorb sufficient nutrients to maintain
`normal fluid balance, growth and health.
`Intestinal failure occurs when there is reduced intestinal absorption so that intravenous nutrients and/or water and
`electrolyte supplements are needed to maintain health and/or growth. IF can be short (<1 y) or long term.
`Intestinal Failure comprises a group of disorders with many different causes, all of which are characterised by an
`inability to maintain adequate nutrition via the intestines.
`It results from obstruction, abnormal motility, major surgical resection, congenital defect or diseaseeassociated
`(continued on next page)
`
`Page 3
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`

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`174
`
`Table 2 (continued )
`
`Author, date (ref)
`
`Fishbein TM. 2009 [25]
`
`Staun M et al., 2009 [26]
`
`Rudolph A and Squires R. 2010 [27]
`
`Gardiner KR. 2011 [28]
`
`Krawinkel MB. 2012 [29]
`
`Murray JS and Mahoney JM. 2012 [30]
`
`Pironi L. et al., 2012 [5]
`
`Squires RH et al., 2012 [31]
`
`L. Pironi et al. / Clinical Nutrition 34 (2015) 171e180
`
`Definition and classification of intestinal failure
`
`loss of absorption. It is characterised not only by the inability to maintain protein-energy, but also often in difficulties in
`maintaining water, electrolyte or micronutrient balance, particularly when there has been a major loss of length of the
`small bowel. If it persists for more than a few days it demands treatment with the intravenous delivery of nutrients
`and watereparenteral nutrition.
`Type I e this type of Intestinal Failure is short-term, self limiting and often peri-operative in nature. Type I Intestinal
`Failure is common and these patients are managed successfully in a multitude of healthcare settings, especially surgical
`wards, including all units which perform major, particularly abdominal surgery. Some patients on high dependency
`units (HDU) and intensive care units (ICU) will also fall into this category Care location: Wards, (HDU, ITU)
`Type II e Type II IF occurs in metabolically unstable patients in hospital and requires prolonged parenteral nutrition over
`periods of weeks or months. It is often associated with sepsis, and may be associated with renal impairment. These
`patients often need the facilities of an Intensive Care or High Dependency Unit for some or much of their stay in hospital.
`This type of IF is rarer and needs to be managed by a multi-professional specialist intestinal failure team. Effective
`management of Type II IF can reduce the likelihood of the development of Type III Intestinal Failure. Care location: HDU,
`ITU (Wards)
`Type III e Type III is a chronic condition requiring long term parenteral feeding. The patient is characteristically
`metabolically stable but cannot maintain his or her nutrition adequately by absorbing food or nutrients via the intestinal
`tract. These are, in the main, the group of patients for which Home Parenteral Nutrition (HPN) is indicated. Care location:
`Wards to home
`Intestinal failure refers to actual or impending loss of nutritional autonomy due to gut dysfunction. The condition is
`initially managed by parenteral delivery of nutrition.
`Intestinal failure is defined as a condition with reduced intestinal absorption to the extent that macronutrient and/or
`water and electrolyte supplements are needed to maintain health and/or growth. Intestinal failure is severe when
`parenteral nutrition and or additional parenteral electrolytes and water are required. The condition may be
`transient if gut function can be restored, but HPN is indicated for patients with chronic intestinal failure.
`Intestinal failure, defined as an inability of a child to achieve adequate weight and growth without intravenous
`nutritional support, has two principal components: the intestine is too short as a consequence of surgical resection
`and the intestine is dysfunctional despite adequate length.
`The term intestinal failure was introduced by Fleming and Remington(1) and defined as a ‘reduction in functioning gut
`mass below the minimum necessary for adequate digestion and absorption of nutrients’.
`Initially, this definition was used interchangeably with the need for parenteral nutrition.
`Since that time the definition has been broadened and is now recognised to occur when ‘gastrointestinal function is
`inadequate to maintain the nutrition and hydration of the individual without supplements given orally or
`intravenously.
`IF has been sub-classified into three types on the basis of duration and irreversibility.
`The term “chronic intestinal failure” (CIF) refers to the body's inability to meet its energy and nutritional needs
`through the gastrointestinal tract
`IF is defined as the inability of the gastrointestinal system to properly function for the adequate digestion and
`absorption of necessary nutrients and fluids for growth and development
`Other experts describe this illness as a state in which gastrointestinal function is not adequate to support sufficient
`growth and physiological balance in children
`Intestinal failure results from reduction in the functioning gut mass characterized by the inability to maintain protein-
`energy, fluid, electrolyte and/or micronutrient balance.
`Intestinal failure in infants and children is a devastating condition that can be broadly defined as the inability of the
`gastrointestinal tract to sustain life without supplemental parenteral nutrition
`
`to indicate the ingestion of nutritional supplements, “enteral
`nutrition” to indicate enteral tube feeding and “parenteral nutri-
`tion” to indicate the intravenous infusion of nutritional admixtures
`or of water and electrolyte solutions.
`The definitive recommendations consist in the “definition of IF”,
`a “functional classification of IF”, a “pathophysiological classifica-
`tion of IF” and a “clinical classification of chronic IF”.
`As there were no published data available to serve as a starting
`point for a “clinical classification”, this was developed on the basis
`of the common experience of the panel experts. The applicability of
`the devised “clinical classification” was verified on two samples of
`randomly selected patients, currently on HPN for CIF due to either
`benign or active malignant disease. This consisted in a cross-
`sectional investigation of the energy content and volume of the
`parenteral nutrition admixture of 114 patients cared for at the
`Center for Benign Chronic Intestinal Failure of the S. Orsola-
`Malpighi University Hospital, Bologna (Italy) and of 50 patients
`with active cancer cared for at the Tumor Biology Center, Albert-
`Ludwigs-University, Freiburg (Germany).
`
`3. Results
`
`The definition and classification of IF are reported and discussed
`below and are summarized in Table 3. Table 4 summarizes the
`
`pathophysiological mechanisms of
`determine an IF are listed in Table 5.
`
`IF. The diseases that may
`
`3.1. Definition of intestinal failure
`
`Intestinal failure is defined as the reduction of gut function below
`the minimum necessary for the absorption of macronutrients and/or
`water and electrolytes, such that intravenous supplementation is
`required to maintain health and/or growth.
`The reduction of gut absorptive function that doesn't require
`intravenous supplementation to maintain health and/or growth, can
`be considered as “intestinal insufficiency” (or “intestinal deficiency” for
`those languages where “insufficiency” and “failure” have the same
`meaning).
`The panel identified IF as a “state of non-functioning”, where the
`gut function referred to was the “absorption of proteins, lipids,
`carbohydrates, water and electrolytes” [12,13,15,24,29,30], and the
`“threshold for loss of function” was the “need for intravenous
`supplementation”
`to maintain
`health
`and/or
`growth
`[6,12e14,21,24,31]. For this purpose, the original definition by
`Fleming and Remington was modified by deleting the term “mass”,
`identifying “absorption” as the key gut function, replacing the term
`“food” with “macronutrients and/or water and electrolytes”, and by
`specifying the “need for intravenous supplementation to maintain
`
`Page 4
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`175
`
`Table 3
`ESPEN recommendations: definition and classification of intestinal failure.
`
`Definition
`Intestinal failure is defined as the reduction of gut function below the
`minimum necessary for the absorption of macronutrients and/or water
`and electrolytes, such that intravenous supplementation is required to
`maintain health and/or growth.
`The reduction of gut absorptive function that doesn't require intravenous
`supplementation to maintain health and/or growth, can be considered as
`“intestinal insufficiency” (or “intestinal deficiency” for those languages where
`“insufficiency” and “failure” have the same meaning).
`Functional classification
`On the basis of onset, metabolic and expected outcome criteria, intestinal failure
`is classified as:
` Type I e acute, short-term and usually self limiting condition
` Type II e prolonged acute condition, often in metabolically unstable
`patients, requiring complex multi-disciplinary care and intravenous
`supplementation over periods of weeks or months
` Type III e chronic condition, in metabolically stable patients, requiring
`intravenous supplementation over months or years. It may be reversible or
`irreversible.
`Pathophysiological classification
`Intestinal failure can be classified into five major pathophysiological conditions,
`which may originate from various gastrointestinal or systemic diseases:
` short bowel
` intestinal fistula
` intestinal dysmotility
` mechanical obstruction
` extensive small bowel mucosal disease
`Clinical classification of chronic intestinal failure
`On the basis of the requirements for energy and the volume of the intravenous
`supplementation (IV), chronic intestinal failure is categorized into 16 subtypes
`
`IV energy
`supplementationb
`(kcal/kg Body
`Weight)
`
`Volume of the IV supplementationa
`(ml)
`1000
`[1]
`
`1001e2000
`[2]
`
`2001e3000
`[3]
`
`>3000
`[4]
`
`0 (A)
`A1
`A2
`A3
`A4
`1e10 (B)
`B1
`B2
`B3
`B4
`11e20 (C)
`C1
`C2
`C3
`C4
`> 20 (D)
`D1
`D2
`D3
`D4
`a calculated as daily mean of the total volume infused per week ¼ (volume per
`day of infusion x number of infusions per week)/7.
`b calculated as daily mean of the total energy infused per week ¼ (energy per
`day of infusion x number of infusions per week)/7.
`
`health and growth”. The panel was aware that balance study
`techniques, comparing nutrient requirement with nutrient ab-
`sorption, would be the optimal way to identify and quantify IF in
`the individual patient [12]. However, considering that very few
`centres have the facilities for these difficult metabolic studies, the
`requirement of intravenous nutrient/fluid supplementation was
`used as a “surrogate diagnostic criterion”. The exclusive need for
`intravenous supplementation was the most debated issue, because
`some previous definitions of IF included also oral supplementation
`and enteral nutrition [2,5,6,11,18,19,22,23,25,26,28]. Micronutrients
`were not mentioned in the definition in order to avoid any
`misunderstanding about impaired gut absorption resulting in
`micronutrient deficiency alone, as this would not be considered as
`IF [2,5,6].
`The proposed definition indicates that for the diagnosis of IF
`two criteria must be simultaneously present: a “decreased ab-
`sorption of macronutrients and/or water and electrolytes due to a
`loss of gut function” and the “need for intravenous supplemen-
`tation”. This facilitates an understanding of which conditions
`cannot be considered IF because only one criterion is present:
`patients with reduced food intake but normal gut function, like
`those with disease-related hypophagia, or with anorexia nervosa
`or any other psychiatric disorders; patients with altered gut
`function but conserved intestinal absorption, like neurological or
`cancer patients with impaired swallowing or dysphagia; patients,
`
`especially children, with active Crohn's disease treated by enteral
`nutrition; patients treated by parenteral nutrition because of
`refusal of otherwise effective enteral nutrition; patients with a
`reduction of gut function impairing intestinal absorption but in
`whom health and growth can be maintained by oral supple-
`mentation, enteral nutrition, re-feeding enteroclysis (reinfusion of
`chyme to the distal limb of a high output small bowel fistula), or
`those who require only vitamins and trace element supplemen-
`tation. For these last conditions, the panel proposes that the term
`“intestinal
`insufficiency or
`intestinal deficiency”
`could be
`considered [12]. The alternative between “insufficiency” and
`“deficiency” has been included to allow an appropriate translation
`in those languages where “insufficiency” and “failure” have the
`same meaning, such as in French,
`Italian and other Latin
`languages.
`
`3.2. Functional classification
`
`On the basis of onset, metabolic and expected outcome criteria, IF is
`classified as
`
`▪ Type I e acute, short-term and usually self limiting condition
`▪ Type II e prolonged acute condition, often in metabolically un-
`stable patients, requiring complex multi-disciplinary care and
`intravenous supplementation over periods of weeks or months
`▪ Type III e chronic condition, in metabolically stable patients,
`requiring intravenous supplementation over months or years. It
`may be reversible or irreversible
`
`This classification, termed “functional”, was also used in the UK
`project “A Strategic Framework for Intestinal Failure and Home
`Parenteral Nutrition Services for Adults in England” [6], and was
`first described in 2002 [14]. It aims to categorize the medical care,
`the professional expertise, the management, the treatment setting
`as well as the organization,
`logistic and administrative issues
`required for the treatment of IF.
`Acute type I and type II IF have been extensively reviewed
`[20,28]. Type I IF is a common, short and often self limiting, con-
`dition, estimated to occur in about 15% patients in the peri-
`operative setting after abdominal surgery or in association with
`critical
`illnesses such as head injury, pneumonia and acute
`pancreatitis. While intestinal function recovers, short-term paren-
`teral fluid and nutrition support can be required. Post-operative
`ileus usually spontaneously resolves within a few days. This dura-
`tion can be shortened by multimodal enhanced recovery tech-
`niques aiming to promote early mobilization and early introduction
`of oral nutrition [32]. Such patients are usually managed in surgical
`wards, although some patients in critical care environments also fit
`into this category.
`Type II IF is an uncommon condition, most often seen in the
`setting of an intra-abdominal catastrophe (like peritonitis due to
`visceral injury) and is almost always associated with septic, meta-
`bolic and complex nutritional complications. Renal impairment
`may be present. It is originally an acute event, often occurring in a
`previously healthy subject (mesenteric ischaemia, volvulus or
`abdominal trauma) or complicating intestinal surgery (anastomotic
`leak; inadvertent and unrecognized intestinal injury) and necessi-
`tating massive enterectomy and/or resulting in one or more
`enterocutaneous fistulae, with or without a proximal stoma. Less
`frequently, it may be the complication of a type III chronic IF, rep-
`resenting a condition of “acute on chronic” IF. Type II IF requires
`prolonged parenteral nutrition over periods of weeks or months.
`These patients often initially need the facilities of an intensive care
`or high dependency unit and to be managed by a multi-professional
`specialist IF team for part or most of their stay in hospital. Using a
`
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`
`Table 4
`Pathophysiological classification of intestinal failure.
`
`Condition
`
`Short bowel
`
`Primary mechanism of intestinal failure
`
`Reduced absorptive mucosal surface
`
`Intestinal fistula
`
`By pass of large areas of absorptive mucosal surface
`
`Intestinal dysmotility
`
`Restricted oral/enteral nutrition or total fasting from intolerance
`due to feeding-related exacerbation of digestive symptoms or to
`episodes of non-mechanical intestinal obstruction
`
`Mechanical obstruction
`
`Incomplete or total fasting (bowel rest)
`
`Extens