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Abstract
Most intestinal failure in children is due to short bowel syndrome (SBS) where congenital or acquired lesions have led to an extensive
loss of intestinal mass. The vast majority of morbidity and mortality of patients with SBS is due to complications secondary to their long
term dependence on parenteral nutrition. In response to SBS, the intestine undergoes a process of remodeling termed adaptation. Principles
guiding the medical management of SBS include providing adequate parenteral nutrition, fluids and electrolytes for growth and normal
development, promoting small bowel adaptation, and preventing and treating complications related to the patients underlying disease and
their parenteral nutrition. Catheter associated blood stream infection (CABSI) is major source of morbidity and mortality in patients with
intestinal failure from SBS. Intestinal failure associated liver disease (IFALD)is another major source of morbidity and mortality in patients
with SBS. IFALD is the most consistent negative predictor of outcome including death and continued parenteral nutrition dependence. Enteral
nutrition is critical for intestinal adaptation and preventing IFALD. Patients with SBS who develop dilated dysmotile segments may benefit from
autologous intestinal reconstruction surgery (AIRS) with the goal of decreasing stasis and disordered motility through intestinal narrowing
and lengthening. Patients with SBS should be referred for transplantation if they have failed intestinal rehabilitation including AIRS, have
no reasonable chance for enteral feeding tolerance, develop irreversible IFALD, have recurrent sepsis, or have exhausted their central venous
access sites. With improvements in medical and surgical care, overall survival of patients with SBS now exceeds 90%.
2013 Elsevier Ireland Ltd. All rights reserved.
Keywords: Short bowel syndrome; intestinal failure; intestinal failure associated liver disease; IFALD; autologous intestinal reconstructive surgery
1. Introduction
Intestinal failure refers to all states where the intestine
has inadequate absorptive capacity to meet the nutritional,
fluid and electrolyte needs to sustain the life and growth
requirements of a child [1,2]. Intestinal failure leads to
chronic dependence on parenteral nutrition to maintain adequate growth, hydration, and micronutrient balance. There
are three major categories of intestinal failure including short
bowel syndrome with the loss of intestinal length leading to
decreased absorptive capacity, malabsorptive states, such as
microvillus inclusion disease, and motility disorders, such as
intestinal pseudo-obstruction [1].
Corresponding author at: Center for Surgical Outcomes Research, The
Research Institute at Nationwide Childrens Hospital, 700 Childrens Drive,
JW4914, Columbus, OH 43205, United States. Tel.: +1 614 722 3066;
fax: +1 614 722 3903.
E-mail address: peter.minneci@nationwidechildrens.org
(P.C. Minneci).
0928-4680/$ see front matter 2013 Elsevier Ireland Ltd. All rights reserved.
http://dx.doi.org/10.1016/j.pathophys.2013.11.013
112
Table 1
Common causes of pediatric intestinal failure in 689 patients
[7,26,28,29,84,85].
Cause
Percent
Necrotizing enterocolitis
Congenital small bowel atresia
Gastroschisis
Midgut volvulus
Intestinal pseudo-obstruction
Other
26
14
21
12
8
18
2. Intestinal adaptation
In response to SBS, the intestine undergoes a process
of remodeling termed adaptation. This process attempts to
compensate for the loss of absorptive and digestive capacity in SBS by increasing intestinal mass and surface area
and improving the absorptive capacity of the enterocytes.
Adaptation is an ongoing process that begins 2448 h after
surgical resection and can take years to complete [5]. During adaptation, the intestine undergoes mucosal hypertrophy
with villus hyperplasia, increased crypt depth and muscle
thickness; grossly, the bowel will dilate, lengthen and thicken
[1114]. Since longitudinal growth of the intestine occurs
in proportion to somatic growth, adaptation usually leads to
dilation of the shortened bowel in order to increase the absorptive surface area; however, continued dilation of the bowel
can become detrimental leading to dysmotility. Bowel dilation and dysmotility promote stasis leading to small bowel
bacterial overgrowth which can contribute to malabsorption,
bacterial translocation, systemic infection, and intestinal failure associated liver disease (IFALD) [15]. These detrimental
effects can negatively impact further bowel adaptation and the
ability of the child to wean from parenteral nutrition [16,17].
Several prognostic factors have been associated with successful adaptation including younger age, limited extent of
resection, segment of intestine resected (jejunum vs. ileum),
presence of the ileocecal valve, re-establishment of bowel
continuity, and maintenance of adequate nutritional status to
sustain growth.
113
114
115
116
Table 2
Factors affecting outcomes in patients with short bowel syndrome [5,6,15,18,45,8183].
Factors associated with improved outcomes
9. Conclusions
Patients with IF secondary to SBS require multidisciplinary care involving nutritional, pharmacologic,
gastrointestinal and surgical interventions. Medical efforts
should focus on maintaining bowel length, promoting
intestinal adaptation, maximizing parenteral nutrition, and
preventing or minimizing central venous catheter complications and IFALD. Recent advances in the care of patients
with SBS include centralized multidisciplinary intestinal failure care teams, strategies of lipid restriction and cycling of
parenteral nutrition, use of omega-3 rich lipid emulsions
in patients with IFALD, use of PICCs and ethanol lock
therapy, and advances in autologous intestinal reconstructive surgery and transplantation [1,5,15,18,45,8183]. With
these improvements, survival rates of children with SBS now
exceed than 90%.
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