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Intestinal Failure in Children

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Current Concepts of Intestinal Failure

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Abstract

Intestinal failure (IF) is a condition in which severe intestinal malabsorption requires parenteral nutrition (PN). Causes of protracted intestinal failure include short bowel syndrome (SBS), congenital diseases of enterocyte development (CDED), and severe motility disorders (total or subtotal aganglionosis or chronic intestinal pseudo-obstruction syndrome). IF can result in “nutritional failure,” defined as the incapacity to continue to feed a child by using PN. Today, intestinal failure-associated liver disease (IFALD) is the most common cause of nutritional failure, but catheter-related sepsis and extensive vascular thrombosis may also jeopardize the use of long-term PN. For a child with nutritional failure, intestinal transplantation (ITx), often in the form of a composite visceral graft, offers the only option for long-term survival. The management of IF requires a multidisciplinary approach. There have been a number of recent advances in both medical and surgical treatments of IF. In particular, new intestinal lengthening techniques and the use of parenteral nutrition formula rich in fish oil have both resulted in decreased rates of severe complications of IF and its treatment. In addition, improved awareness of the risks and benefits of ITx have resulted in better patient selection, and ultimately in improved patient survival, leading to restrict the indication to ITx only to patients with nutritional failure with no other chance to survive.

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Abbreviations

AABF:

Amino acid-based formulas

CRS:

Catheter-related sepsis

CLD:

Cholestatic liver disease

CIPOS:

Chronic intestinal pseudo-obstruction syndrome

(CSD):

Congenital sodium diarrhea

CDED:

Congenital diseases of enterocyte development

CTF:

Continuous tube feeding

ETF:

Enteral tube feeding

EGF:

Epidermal growth factor

EFA:

Essential fatty acid

GI:

Gastrointestinal

GLP-2:

Glucagon-like peptide 2

HM:

Human milk

ICV:

Ileocecal valve

IGF-1:

Insulin-like growth factor-1

IF:

Intestinal failure

IFALD:

Intestinal failure-associated liver disease

ITx:

Intestinal transplantation

ILE:

Intravenous lipid emulsions

LOT:

Ligament of Treitz

LILT:

Longitudinal intestinal lengthening and tailoring

MCT:

Medium-chain triglyceride

MVID:

Microvillus inclusion disease

OF:

Oral feeding

PN:

Parenteral nutrition

PUFA:

Polyunsaturated fatty acid

rhGH:

Recombinant human growth hormone

STEP:

Serial transverse enteroplasty technique

SBS:

Short bowel syndrome

SCFA:

Short-chain fatty acid

SIBO:

Small intestinal bacterial overgrowth

TIA:

Total intestinal aganglionosis

TE:

Tufting enteropathy

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Authors and Affiliations

  1. Division of Pediatric Gastroenterology-Hepatology-Nutrition, National Reference Center for Rare Digestive Diseases, Pediatric Intestinal Failure Rehabilitation Center, Hôpital Necker-Enfants Malades, University Paris Descartes, Paris, France

    Olivier Goulet MD, PhD, Florence Lacaille MD & Cécile Lambe MD

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  1. Olivier Goulet MD, PhD
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  2. Florence Lacaille MD
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  3. Cécile Lambe MD
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Correspondence to Olivier Goulet MD, PhD .

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  1. Children's Hospital, Helsinki University Central Hospital, HUS Helsinki, Finland

    Risto J. Rintala

  2. Children's Hospital, Helsinki University Central Hospital, HUS Helsinki, Finland

    Mikko Pakarinen

  3. Department of Pediatric Surgery, Karolinska University Hospital, Stockholm, Sweden

    Tomas Wester

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Goulet, O., Lacaille, F., Lambe, C. (2016). Intestinal Failure in Children. In: Rintala, R., Pakarinen, M., Wester, T. (eds) Current Concepts of Intestinal Failure. Springer, Cham. https://doi.org/10.1007/978-3-319-42551-1_1

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