Review article of Pediatric Gastroenterology Hepatology & Nutrition

Short Bowel Syndrome as the Leading

Cause of Intestinal Failure in Early Life:
Some Insights into the Management
Olivier Goulet , Elie Abi Nader , Bénédicte Pigneur , and Cécile Lambe

Febrina

Supervisor:
Prof. Dr. dr. B. Soebagyo, Sp. A (K)
dr. Evi Rokhayati, Sp. A (K), M. Kes.
Dr. Rekno Widati, Sp. A

Department of Child Health

Faculty of Medicine Sebelas Maret University / Moewardi Hospital
 01.
INTRODUCTION
● Intestinal failure (IF) is the critical reduction of the gut mass or its function below
the minimum needed to absorb nutrients and fluids required for adequate growth
in children

● Most commonly due to congenital/neonatal intestinal diseases/malformations

divided into 3 groups:

1) Reduced intestinal length and consequently reduced absorptive surface,

such as in short bowel syndrome (SBS) or extensive aganglionosis;

2) Abnormal development of the intestinal mucosa such as congenital diseases

of enterocyte development;

3) Extensive motility dysfunction such as chronic intestinal pseudo-obstruction

syndromes.
● The short bowel syndrome (SBS) is the leading cause of IF.

● IF may be reversible or irreversible, depending on a number of factors such as

1. The underlying cause of SBS

2. The length of the remaining intestine

3. The treatment used to develop or restore intestinal capacity.

● Parenteral nutrition (PN), has become a safe and efficient feeding technique for IF
for normal or catch up growth.

● IF that requires long-term PN may be associated with various complications

including infections, growth failure, metabolic disorders, and bone disease.

● Cholestatic liver disease (CLD) was rapidly identified as one of the limitations in the
of long-term management of SBS-IF while CLD related factors are mostly related to
IF rather PN supporting the wording “IF Associated Liver Disease” (IFALD).

● Severe liver disease may lead to the so-called “nutritional failure” which is
considered as a major indication for intestinal transplantation (ITx) or combined
liver-ITx
 02.
DEFINITION & ETIOLOGY
OF THE SBS
● SBS is, usually following extensive surgical resection leaving the small bowel (SB)
length below a critical value for adequate nutritional supply that results in
malabsorption with subsequent diarrhea, water-electrolytes imbalance, and
malnutrition.
● term-neonates : SB length of approximately 250 cm and their intestines lengthen
substantially during the first year of life
● Preterm infants have a greater potential for bowel growth since their intestines
lengthen substantially during the last trimester of gestation
● Minimum SBL for Autonomous Enteral Function : 40 cm with ICV
● A residual bowel length of only 15–40 cm has been associated with bowel
adaptation, intestinal autonomy and PN weaning, but, most of the time with few
information regarding the long-term growth
● Total/Near Total intestinal aganglionosis (TIA/NTIA) is the most uncommon and
lifethreatening form of Hirschsprung disease (HD). Most of the time the neonate is
left with less than 50 cm normally innervated small intestine or even shorter length
below the ligament of Treitz. It may be considered as a SBS type 1
 03.
MANAGEMENT OF SBS-IF
 04.
CONCLUSION
● IF requires specialized and individualized medical therapy that includes surgery,
medical equipment, nutritional products, and standard nursing care.

● Tx registry report at the XV SB Transplant Symposium, New York, June 2017 showed
early evidence of a world-wide trend of reduction in the number of pediatric ITx.
This might be explained by at least the following factors:
○ Provision of guidelines and training
○ Development of IRC with increasing IF expertise
○ Enlarged use of non-transplant surgery
○ Better prevention of IFALD, with fish oil-based lipid emulsions playing a role
○ Improved prevention of CRBSIs by using taurolidine or ethanol locks
○ Onset of hormonal treatment in SBS-IF by using GLP-2 analogs

● Major efforts are needed to improve the outcome of ITx that will likely remain part
of the armamentarium required to prolong the survival of children with life-
threatening complications of IF.
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