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Pathophysiology 21 (2014) 111118

Management of short bowel syndrome


Jason P. Sulkowski, Peter C. Minneci
Center for Surgical Outcomes Research, The Research Institute at Nationwide Childrens Hospital and Department of Surgery, Nationwide Childrens
Hospital, Columbus, OH, United States

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

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 (Table 1) [24].
The earliest descriptions of SBS describe midgut volvulus
and small bowel atresia as the most common causes of SBS.
However, more recently, necrotizing enterocolitis (NEC) has
become the most common cause of intestinal failure due to
SBS [57]. NEC is expected to continue as a predominant
cause of SBS as more neonates at high risk for developing NEC survive including extremely premature infants and
infant with complex congenital cardiac anomalies [8].
The reported incidence of SBS varies based on the presence of prematurity and low birth weight. In one report form
Canada, the overall incidence of SBS was 24.5 cases per
100,000 live births; however, the incidence in premature
infants (<37 weeks gestation) was 353 cases per 100,000,
while it was only 3.5 per 100,000 in term neonates [9]. The
higher reported incidence with prematurity underscores the
physiology that can lead to prematurity including intrauterine
volvulus and polyhydramnios and the increased risk of NEC

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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

in premature neonates [1]. In another study, the incidence


of surgical SBS was 0.7% in very low birth weight infants
and 1.1% in extremely low birth weight infants with NEC
responsible for 96% of the cases of SBS [10]. These studies
highlight the increasing contribution of NEC as a cause of
SBS now and in the future.
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 particular catheter
associated blood stream infections and intestinal failure associated liver disease [8]. This review focuses on the medical
and surgical management of intestinal failure due to SBS to
optimize growth and minimize parenteral nutrition associated
complications.

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.

Residual bowel length is the most consistently reported


predictor of developing intestinal failure in patients with
SBS [18]. The residual length of small intestine is predictive of both the ability to wean and time to wean from
parenteral nutrition [18]. Inaccuracies in bowel length measurement techniques have led to significant variation in the
reported length of small intestine necessary to predict successful weaning form parenteral nutrition. However, 35 cm
appears to be an inflection point above which most neonates
will be able to successfully wean from parenteral nutrition
[1]. In terms of remaining bowel length, it may be better to express it as a percent of expected intestinal length
based on age or size of the child because of periods of
rapid bowel growth [19]. The intestine increases in length
by 150% between 30 and 40 weeks gestation and will
nearly double in length again in first 18 months of life
[2022]. Therefore, a residual bowel length of 25 cm has
vastly different implications for successful weaning from parenteral nutrition in a 2 day old 27 week gestation premature
neonate compared to a 2 day old 40 week gestation term
neonate.
In addition to the length of remaining intestine, the
segment of remaining intestine can have implications for
successful adaptation. Although jejunal resections will lead
to at least temporary losses of absorptive capacity, massive
jejunal resections are better tolerated than massive ileal resections [23,24]. The ileum is responsible for net water and salt
absorption, therefore ileal resections can lead to the inability to resorb both dietary fluid and the fluid secreted by
the jejunum. In addition, the ileum absorbs vitamin B12 ,
bile salts and fatty acids. The presence of unabsorbed bile
salts and fatty acids in the colon will stimulate colonic
secretions and promote motility leading to worse malabsorption.
The presence of remaining colon is another important predictor of successful adaptation because it allows for improved
water and electrolyte absorption. In addition, previous reports
have cited the importance of preservation of the ileocecal
valve. However, with improvements in our understanding of
bowel adaptation and the importance of the ileum, it is now
considered that the ileocecal valve itself may not be crucial; it
is more likely that the ileocecal valve is a marker for the presence of remaining terminal ileum and colon [6,19,2528].

3. Medical management of intestinal failure due to


SBS
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. These patients are best cared for by a
specialized multidisciplinary team including pediatric gastroenterologists and surgeons, nutritionists, pharmacists and

J.P. Sulkowski, P.C. Minneci / Pathophysiology 21 (2014) 111118

social workers [29]. This multidisciplinary team approach


to caring for intestinal failure patients has been associated
with improved survival with several groups now reporting
survival and parenteral nutrition weaning rates over 90%
[7,30].
The goals of treatment of SBS are to provide optimal
nutrition to sustain growth and development initially with parenteral nutrition and to stimulate intestinal rehabilitation and
adaptation through the initiation and advancement of enteral
feeding. Patients with SBS should receive hepatic sparing
parenteral nutrition to decrease the chances of developing
IFALD and need careful central venous access management to maintain long-term central access and minimize
the risks of catheter-associated infections and thrombosis. In
addition, these patients are in a hyper-gastrin state because
gastrin degradation primarily occurs in the small intestine;
therefore, these patients should be treated with a proton
pump inhibitor [31,32]. Strategies for enteral feeding to promote intestinal adaptation include initiating feedings as soon
as bowel function recovers, early restoration of continuity of the bowel, use of continuous gastric tube feedings
with elemental formulas to optimize enterocyte absorption,
antibiotic therapy for bacterial overgrowth, and the use of
autologous intestinal reconstructive surgery when appropriate [1]. In addition, the developmental and social needs of
the child and their caregivers must be addressed and supported.
3.1. Parenteral nutrition considerations in SBS
Children with SBS are dependent on parenteral nutrition
for provision of adequate calories, macronutrients, micronutrients, vitamins, and electrolytes. Unique considerations
when prescribing parenteral nutrition for patients with SBS
are their bicarbonate and sodium needs. These patients can
have significant losses of bicarbonate in their stool or stoma
output which must be closely monitored and replaced. In
addition, patients with SBS are susceptible to sodium depletion from both intestinal loss and inadequate absorption.
Sodium depletion can be detected prior to developing low
serum sodium levels by monitoring urinary sodium. The
sodium content in the parenteral nutrition should be titrated
to keep urine sodium >30 mequiv./L and to maintain a urine
sodium to potassium ratio of 1:1 [2].
3.2. Enteral nutrition considerations in SBS
Enteral nutrition is critical for intestinal adaptation and
preventing IFALD. Because luminal substrates and nutrients are essential for adaptation, attempts should be made
to establish enteral nutrition no matter how small an amount
[33]. Once intestinal function has returned, attempts at enteral
feeding should be initiated regardless of length of remaining
bowel. Enteral feedings lead to intestinal growth, enhancement of enterocyte enzyme activity, and bowel motility [33].

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In addition, tolerance of any amount of enteral feeding can


dramatically decrease the risk profile of patients with SBS.
Initial enteral feedings in patients with SBS are usually
administered continuously to maximize the function of the
residual intestinal mucosa by allowing for a relatively uniform saturation of intestinal mucosa. Use of breast milk
or elemental formulas, which are more easily absorbed,
can decrease duration of parenteral nutrition dependence in
neonates with SBS [18]. Feeding advancement is limited to
keep stool or stoma output to <50 ml/kg/day. Enteral absorption of fats and carbohydrates can be monitored by following
levels of fecal fats or fecal reducing substances. Patients who
have lost their terminal ileum are at risk for malabsorption of
fat soluble vitamins (vitamins A, D, E, and K), vitamin B12 ,
and zinc and should be monitored for these deficiencies.
Adjunct therapies that can improve enteral nutrition tolerance include re-feeding of stoma output into a mucous fistula
when present and the appropriate use of indicated pharmacologic therapies. Soluble dietary fiber (pectin or guar gum)
can slow gastrointestinal transit time allowing for improved
absorption [2,34,35]. The soluble fiber in the colon is fermented to short chain fatty acids, including butyrate which
is an energy source for colonocytes. In addition, the butyrate
regulates colonocyte proliferation and improves water and
sodium absorption by up-regulating the sodium-hydrogen
exchangers [35]. However, excess pectin (>3%) can lead
to an osmotic diarrhea which can counteract its benefits.
Patients with SBS with high stool or stoma output limiting enteral intake and no mechanical or intestinal cause may
benefit from the use of loperamide to decrease stool output.
Patients with SBS who have poor motility will develop vomiting, abdominal distention, or high gastric residuals; these
patients may have improved enteral tolerance with the use
pro-motility agents including erythromycin, azithromycin, or
augmentin.
Small bowel bacterial overgrowth can occur in up to 50%
of patients with SBS [16]. As part of adaptation, segments
of bowel may dilate and develop disordered motility leading
to stasis with the potential for bacterial overgrowth. Consequently, bacterial overgrowth leads to inflammation with
mucosal injury that may allow for bacterial translocation
or gastrointestinal bleeding. Bacterial overgrowth can also
worsen absorption through the deconjugation of bile acids
and can produce toxic by-products such as d-lactic acid
which can lead to ataxia, mental status changes, and acidosis
[16,36]. Clinically, small bowel bacterial overgrowth can
present as new feeding difficulties, worsening of motility,
abdominal distention or pain, or gastrointestinal bleeding.
Treatment is empirically initiated in the appropriate clinical
setting with a dilated segment of bowel and consists of
cycles of enteral antibiotics with anaerobic spectrum, such
as metronidazole or augmentin if there are also motility
issues, for 710 days of therapy following 710 days off
therapy [2]. Recurrent episodes of bacterial overgrowth
are an indication for surgical intervention to narrow of the
dilated segment of intestine.

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4. Vascular access and catheter associated blood


stream infection
Patients with SBS require long term central venous
access to receive their parenteral nutrition. This traditionally required surgically placed tunneled cuffed central venous
catheters (Broviac or Hickmann catheters); however, many
centers are now using peripherally inserted central catheters
(PICCs) for long term parenteral nutrition. Use of percutaneous placement techniques and peripheral lines can decrease
loss of central venous access [37]. Advantages of PICCs are
that they can be placed without general anesthesia, they do not
require ligating a central vein, and they may carry a lower risk
of thrombosis of the central veins. In one recent report, the
incidence of PICC related catheter associated blood stream
infection (CABSI) was 5.3 per 1000 catheter days and PICC
related venous thrombosis was 2.0 per 1000 catheter days.
These rates are similar or lower than reported rates with surgical central lines, therefore, the authors recommend using
PICCs for central access in patients with intestinal failure
until 1 year of age when a tunneled central catheter should
be placed [38]. In long term intestinal failure patients, central venous access can be exhausted over time secondary to
thrombosis or occlusion. In these cases, alternative sites such
as transhepatic, translumbar, or internal mammary venous
lines may need to be considered. Referral for transplantation
becomes the only option when central venous access can no
longer be obtained accounting for up to 50% of transplant
referrals [39,40].
Catheter associated blood stream infection (CABSI) is
major source of morbidity and mortality in patients with
intestinal failure from SBS. CABSI rates in patients with
intestinal failure range from 4 to 10 infections per 1000
catheter days [1,41]. Circumstances leading to higher rates
of CABSI in this population include a long duration of time
with the catheter with a willingness to treat through infections to maintain central access instead of removing the
catheter, long term infusion of high concentration dextrose
solutions, and the presence of dilated intestinal segments
with the potential for bacterial overgrowth. Risk factors for
developing CABSI in patients with SBS include prematurity,
low birth weight, and shorter length of remaining intestine.
In patients with SBS, fever, lethargy or ileus should raise
concerns for CABSI. These patients should have blood cultures drawn and be treated empirically with broad spectrum
antibiotics for 48 h. The catheter should be removed if the
blood cultures are repeatedly positive for 3 days, there are
signs of systemic instability, or if fungus is isolated in
the cultures. Several methods to reduce CABSI have been
reported including using checklists for insertion and dressing changes, using chlorhexidine based cleansing solutions,
and using ethanol locks [4244]. In particular, 70% ethanol
locks have been reported to decreased CABSI in intestinal
failure patients from 9.9 cases per 1000 catheter days to 2.1
cases per 1000 catheter days [42].

5. Intestinal failure associated liver disease


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 [5,4547]. It occurs in up to 60% of
children with SBS with 16.6% progressing to end stage
liver disease [48,49]. The mechanism of IFALD is thought
to be multifactorial with contributions from episodes of
infection/sepsis, bowel stasis, altered enterohepatic bile acid
circulation, susceptibility of the neonatal liver to cholestatic
injury, and parenteral nutrition toxicity, particularly from
the lipid infusions [15,4852]. Clinically, IFALD presents
with increased serum transaminases and direct hyperbilirubinemia. Histologically, there is cholestasis, bile duct
proliferation, periportal inflammation and fibrosis [53]. Of
note, the level of cholestasis may not reflect the presence
and degree of liver damage [54]. If not reversed, ongoing
liver injury leads to cirrhosis with coagulopathy and eventually thrombocytopenia and portal hypertension. However,
the histopathologic changes and cholestasis can be reversed
if enteral nutrition can be initiated and advanced [18].
Several parenteral nutrition strategies that focus on cycling
parenteral nutrition and limiting or changing lipid emulsions
have been developed to try to prevent IFALD. One strategy
is to cycle parenteral nutrition to provide the total caloric
amount over less than 24 h. Cycling of parenteral nutrition allows for disconnection of the central venous catheter,
improves visceral protein stores, and can reduce the incidence of hyperinsulinemia. Cycling is recommended when
the expected duration of parenteral nutrition is expected
to be longer than 30 days [55,56]. In one small study of
patients with SBS secondary to gastroschisis, patients who
had cycling of parenteral nutrition were three times less likely
to develop IFALD compared to patients who received continuous parenteral nutrition [57].
Lipid limitation is another strategy that is now commonly
being applied. The lipid emulsions used in the United States
are soybean and/or safflower oil based which are rich in
omega-6 fatty acids, including arachidonic acid, which lead
to the production of pro-inflammatory cytokines. The proinflammatory properties of these lipid emulsions may play a
role in IFALD. In order to prevent or delay the development
of IFALD, many groups are now limiting lipid administration to 1 g/kg/day or lower [5861]. This strategy limits the
hepatotoxicity of parenteral nutrition and has been associated with a decreased incidence of IFALD [59,60]. However,
lipid limitation should be used cautiously in neonates as
extended periods of limitation may lead to growth retardation and the decrease in calories must be compensated for
by increasing the amount of dextrose in the parenteral nutrition.
Another area of investigation to prevent IFALD involves
using fish oil based lipid emulsions that are rich in omega-3
fatty acids which have anti-inflammatory properties. Studies

J.P. Sulkowski, P.C. Minneci / Pathophysiology 21 (2014) 111118

of omegaven, a fish oil based lipid emulsion rich in omega-3


fatty acids, demonstrated higher and faster rates of reversal
of cholestasis compared to historical controls that received
soybean oil based lipid emulsions [6265]. Other groups
have reported chemical reversal of IFALD with the use of
lipid emulsions rich in omega-3 fatty acids [62,63]. There
is an ongoing clinical trial evaluating omegaven; however,
for now, omegaven is only available for compassionate use
in the U.S. for patients with significant cholestatic disease
with little chance of enteral feeding advancement in hopes of
allowing for reversal of cholestasis to permit more time for
intestinal adaptation.

6. Surgical therapy for SBS


6.1. Initial surgical management
Initial surgical management of patients at risk for SBS
requires conservation of every centimeter of intestine possible. In patients with NEC or volvulus, this may involve an
initial abdominal exploration with resection of only frankly
necrotic areas of bowel with planned serial second look
operations to evaluate marginal areas of intestine for viability.
It may also involve using temporary abdominal silos which
allow for constant visualization of the remaining bowel. At
the end of initial surgical management, most patients with
SBS from NEC will have a stoma and mucous fistula created.
The child can be started on enteral nutrition once bowel function has returned and they have recovered from their episode
of NEC. The stoma and mucous fistula are usually reversed
to restore bowel continuity once the neonate has grown and
recovered from their underlying medical conditions. Whenever possible, early stoma closure is recommended because
it can hasten the ability to wean the patient from parenteral
nutrition [18]. In patients with SBS, placement of a surgical gastrostomy tube during the stoma closure procedure is
recommended to allow for long term enteral access for tube
feedings. In children with SBS with poor gastric motility, the
gastrostomy tube can later be converted to a gastro-jejunal
tube which allows for post-pyloric feedings while draining
the poorly motile stomach.
6.2. Autologous intestinal reconstruction surgery (AIRS)
As intestinal adaptation occurs, the small bowel may dilate
and loose its normal peristaltic activity. The increased diameter of the dilated segment prevents proper and complete
approximation of the bowel walls which can lead to disorganized antegrade progression of luminal contents [6668].
These dilated dysmotile segments can become sites of stasis and bacterial overgrowth [66]. Patients with SBS who
develop dilated dysmotile segments may benefit from autologous intestinal reconstruction surgery (AIRS).
The goals of AIRS are to correct stasis and disordered
motility through intestinal narrowing and lengthening [66].

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These procedures should be considered in patients with SBS


with dilated intestinal segments that have reached a plateau
in enteral tolerance despite maximal medical management.
There are three relatively common AIRS procedures that can
be considered in patients with SBS. The oldest and simplest
procedure is a tapering enteroplasty in which the dilated intestine is narrowed by excising a wedge of its anti-mesenteric
border. The tapering enteroplasty improves motility by narrowing the intestinal lumen, but is does so at the expense of
excising a segment of viable intestine. This procedure should
be reserved for children who are thought to have enough
intestinal length to successfully wean from parenteral nutrition once the dysmotility and stasis have improved. The other
two AIRS procedures, the longitudinal intestinal lengthening
and tapering (LILT) and the serial transverse enteroplasty
(STEP), combine the benefit of tapering with bowel lengthening and preservation of all viable intestinal tissue. Both the
LILT and the STEP procedures have been reported to improve
enteral autonomy in patients with SBS.
The LILT procedure was developed in 1980 by Bianchi
and is often referred to as the Bianchi procedure. During
LILT, the dilated segment of bowel is longitudinally divided
along its length into two smaller equal diameter segments
which are then anastomosed end-to-end to increase the length
of the intestine. This requires a difficult and meticulous dissection of the mesentery supplying the dilated segment into
two separate leaves, one to supply each half of the dilated
segment when it is divided. The overall effect of the LILT is
to double the length of the dilated segment and to halve its
diameter. In patients with SBS undergoing a LILT procedure,
overall reported rates of survival are 30100% with rates of
successfully weaning from parenteral nutrition ranging from
28 to 100% [68]. Recurrent dilation of the bowel can occur
after a LILT. Disadvantages of the LILT include its technical
difficulty, the need for the dilated segment of intestine to be
uniformly dilated, and it can only be performed once.
The STEP procedure was first reported in 2003 [69,70].
The STEP uses sequential alternating lateral stapling of
the dilated intestinal segment along its long axis to narrow
the lumen to approximately 2 cm and increase its length
[69,70]. Advantages of the STEP procedure are that it is
easily reproducible, can be used to create a uniform bowel
diameter without uniform dilation preoperatively, carries
minimal risk of intestinal ischemia, and can be performed
primarily, repeated or performed in patients who have recurrent dilation of their bowel after a LILT procedure. The
STEP has been shown to decrease dependence on parenteral
nutrition in patients with SBS [7173]. It has also been
used as a primary procedure in neonates with dilated atresia and marginal length and to treat problematic bacterial
overgrowth including d-lactic acidosis [69,7477]. A recent
report of the International STEP Registry on 97 patients
reported a mortality rate of 11% with 47% of patients reaching enteral autonomy; the median time to enteral autonomy
post-STEP procedure was 21 months [78]. Recurrent dilation of the bowel can occur after a STEP procedure if

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Table 2
Factors affecting outcomes in patients with short bowel syndrome [5,6,15,18,45,8183].
Factors associated with improved outcomes

Factors associated with worse outcomes

Residual bowel length >35 cm


Older infant/child at initial bowel loss
Jejunal resection
Presence of terminal ileum/ileocecal valve
Presence of colon
Earlier re-establishment of bowel continuity

Residual bowel length <20 cm or <10% of age expected length


Prematurity/younger infant at initial bowel loss
Ileal resection
Presence of IFALD
Bacterial overgrowth
Gastroschisis as underlying cause of bowel loss
Recurrent catheter associated infections

patients cannot be weaned from parenteral nutrition; these


patients may undergo a repeat STEP procedure if clinically
indicated.
7. Transplantation
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 [1,40]. In neonates
and young children, liverintestine or multi-visceral transplantation are the only options. In older children, isolated
intestinal transplantation is an additional option. Recently,
the transplant center at the University of Pittsburgh reported
results with liver-small bowel transplantation including a 5
year actuarial survival of 81% with graft survival of 76%;
with isolated small bowel transplantation alone, the 3 year
graft survival was 73% with a 5 year survival of 56% [79].
Post-transplantation complications include infections, acute
and chronic rejection, and post-transplant lymphoproliferative disorder.
8. Outcomes of children with SBS
Overall survival of patients with SBS has improved over
time. The development of complete intravenous nutrition in
1960s converted SBS from a fatal disease to a survivable
one. Today, most mortality from SBS is due to complications
of long term parenteral nutrition, mainly catheter associated sepsis and IFALD [80]. Improvements in survival of
patients with SBS plateaued around 75% until recently when
improvements in medical care and surgical care have led to
reported survival rates as high as 9095% [1,15,18,19,26].
Reported predictive factors of outcomes of children with SBS
are shown in Table 2.

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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