Long-term parenteral nutritional support and intestinal adaptation in children with short bowel syndrome: A 25-year experience

doi:10.1016/j.jpeds.2004.02.030

The Journal of Pediatrics

Volume 145, Issue 2, August 2004, Pages 157-163

https://doi.org/10.1016/j.jpeds.2004.02.030 Get rights and content

Abstract

Objective

To analyze the outcome of children with short bowel syndrome (SBS) who required long-term parenteral nutrition (PN).

Study design

Retrospective analysis of children (n = 78) with SBS who required PN >3 months from 1975 to 2000. Statistics: univariate analysis, Kaplan-Meier method, and Cox proportional regression model were used.

Results

We identified 78 patients. Survival was better with small bowel length (SBL) >38 cm, intact ileocecal valve (ICV), intact colon, takedown surgery after ostomy (all P < .01), and primary anastomosis (P < .001). PN-associated early persistent cholestatic jaundice (P < .001) and SBL of <15cm (P < .01) were associated with a higher mortality. Intestinal adaptation was less likely if SBL <15 cm (P < .05), ICV was removed, colonic resection was done (both P < .001), >50% of colon was resected (P < .05), and primary anastomosis could not be accomplished (P < .01). Survival was 73% (57), and 77% (44) of survivors had intestinal adaptation.

Conclusions

SBL, intact ICV, intestinal continuity, and preservation of the colon are important factors for survival and adaptation. Adaptation usually occurred within the first 3 years. Need for long-term PN does not preclude achieving productive adulthood. Patients with ICV even with <15 cm of SBL and patients with SBL >15 cm without ICV have a chance of intestinal adaptation.

Section snippets

Methods

We analyzed the complete charts of all children who required PN support at UCLA for more than 3 months from January 1975 to April 2000 (n = 875). We defined SBS as a residual jejunoileal segment of ≤75 cm secondary to a surgical resection or congenital malformation.⁴ Children were subdivided into three groups according to their jejunoileal segment: (1) Short small bowel (SSB), >38 cm; (2) Very short small bowel (VSSB), 15 to 38 cm; and (3) Ultra short small bowel (USSB), <15cm. The cited small

Results

We identified 78 patients. Forty-nine were male. PN was started at the median age of 0.6 months (0.1-152 months). In Table I we compared the characteristics of survivors versus nonsurvivors. We found better survival with SBL >38 cm, intact ICV, whole colon left, primary anastomosis, and takedown surgery after an initial ostomy. Early persistent cholestatic jaundice and SBL <15cm were associated with higher mortality. These results were duplicated when all binary variables were analyzed by

Discussion

The main goal of treatment in SBS is intestinal adaptation while optimizing weight gain and linear growth while trying to maximize enteral nutrition while minimizing parenteral support. This is as much an art as it is a science. Since the late 1960s, PN has had an enormous impact on the accomplishment of these goals. After small bowel resection, the residual intestine becomes dilated, and crypt depth along with villus height increases by mucosal hyperplasia. The overall outcome is an increase

References (38)

D.W. Wilmore
Factors correlating with a successful outcome following extensive intestinal resection in newborn infants
J Pediatr
(1972)
A. Cooper et al.
Morbidity and mortality of short-bowel syndrome acquired in infancy: an update
J Pediatr Surg
(1984)
E.W. Fonkalsrud et al.
Long-term hyperalimentation in children through saphenous central venous catheterization
Am J Surg
(1982)
R.A. Cannon et al.
Home parenteral nutrition in infants
J Pediatr
(1980)
S.A. Dorney et al.
Improved survival in very short small bowel of infancy with use of long-term parenteral nutrition
J Pediatr
(1985)
D.L. Christie et al.
Dilute elemental diet and continuous infusion technique for management of short bowel syndrome
J Pediatr
(1975)
R.C. Williamson et al.
Proximal enterectomy stimulates distal hyperplasia more than bypass or pancreaticobiliary diversion
Gastroenterology
(1978)
D.J. Andorsky et al.
Nutritional and other postoperative management of neonates with short bowel syndrome correlates with clinical outcomes
J Pediatr
(2001)
J.M. Sondheimer et al.
Predicting the duration of dependence on parenteral nutrition after neonatal intestinal resection
J Pediatr
(1998)
O.J. Goulet et al.
Neonatal short bowel syndrome
J Pediatr
(1991)

B. Messing et al.

Long-term survival and parenteral nutrition dependence in adult patients with the short bowel syndrome

Gastroenterology

(1999)

W.R. Hanson et al.

Compensation by the residual intestine after intestinal resection in the rat, I: influence of amount of tissue removed

Gastroenterology

(1977)

R.W. Summers et al.

Effects of drugs, ileal obstruction, and irradiation on rat gastrointestinal propulsion

Gastroenterology

(1970)

L.R. Jacobs et al.

Response of plasma and tissue levels of enteroglucagon immunoreactivity to intestinal resection, lactation and hyperphagia

Life Sci

(1981)

S. Nundy et al.

Onset of cell proliferation in the shortened gut: colonic hyperplasia after ileal resection

Gastroenterology

(1977)

T.R. Weber et al.

Adverse effects of liver dysfunction and portal hypertension on intestinal adaptation in short bowel syndrome in children

Am J Surg

(2002)

E.M. Quigley et al.

Motility of the terminal ileum and ileocecal sphincter in healthy humans

Gastroenterology

(1984)

H.A. Pitt et al.

Increased risk of cholelithiasis with prolonged total parenteral nutrition

Am J Surg

(1983)

A.A. Moukarzel et al.

230 patient years of experience with home long-term parenteral nutrition in childhood: natural history and life of central venous catheters

J Pediatr Surg

(1994)

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A growing proportion of children with short bowel syndrome (SBS) remain dependent on long-term parenteral nutrition (PN). Teduglutide offers the potential for more children to decrease PN support and achieve enteral autonomy (EA), but at a significant expense. This study aims to assess the incremental costs of teduglutide plus standard of care compared to standard of care alone in weaning PN support per quality-adjusted life year (QALY) gained in children with SBS.
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Although teduglutide was not cost-effective in weaning PN support in children with SBS, starting teduglutide once natural intestinal adaptation is reduced and adjusting its monthly cost to reflect cost by volume as dictated by weight-based dosing rendered the intervention cost-effective relative to standard of care. These results indicate the potential for clinicians to re-assess optimal time for initiation of teduglutide after intestinal resection, drug manufacturers to consider the use of multi-dose or paediatric-dose vials, and the opportunity for decision-makers to re-evaluate teduglutide funding.
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Increased intestinal absorption and PN dependency reduction were observed with teduglutide treatment in children with SBS-IF.
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Original ArticleLong-term parenteral nutritional support and intestinal adaptation in children with short bowel syndrome: A 25-year experience

Abstract

Objective

Study design

Results

Conclusions

Section snippets

Methods

Results

Discussion

J Pediatr

J Pediatr Surg

Am J Surg

J Pediatr

J Pediatr

J Pediatr

Gastroenterology

J Pediatr

J Pediatr

J Pediatr

Gastroenterology

Gastroenterology

Gastroenterology

Life Sci

Gastroenterology

Am J Surg

Gastroenterology

Am J Surg

J Pediatr Surg

Original Article
Long-term parenteral nutritional support and intestinal adaptation in children with short bowel syndrome: A 25-year experience