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Pediatric Intestinal Failure: Nutrition, Pharmacologic, and Surgical Approaches
Y. Avery Ching, MD,
Kathleen Gura, PharmD,
Biren Modi, MD and
Tom Jaksic, MD, PhD
Children's Hospital of Boston, Boston, Massachusetts
Correspondence: Y. Avery Ching, Children's Hospital of Boston, Department of
Surgery, Fegan 3, 300 Longwood Avenue, Boston, MA 02115. Electronic mail may
be sent to
yching{at}bidmc.harvard.edu.
Intestinal failure (IF) is a condition where there is insufficient
functional bowel to allow for adequate nutrient and fluid absorption to
sustain adequate growth in children. Several etiologies can predispose to IF,
including necrotizing enterocolitis, gastroschisis, and intestinal atresias.
Intestinal rehabilitation can be seen as a 3-pronged strategy merging
nutrition, pharmacologic, and surgical approaches to achieve the ultimate goal
of enteral nutrition. Nutrition approaches should seek to facilitate
transition from parenteral nutrition (PN) to enteral nutrition because
prolonged use of PN is associated with severe morbidity and mortality. Enteral
nutrition, on the other hand, promotes and enhances an adaptive response in
the intestine. Medications used in the treatment of IF may help alleviate
symptoms of diarrhea, bacterial overgrowth, and gastrointestinal dysmotility.
Surgical procedures, such as longitudinal intestinal lengthening and tapering
(LILT) or serial transverse enteroplasty (STEP), can increase mucosal surface
area and may enhance intestinal adaptation. IF is a difficult disease process
with a complex patient population and is best guided through this 3-pronged
approach by a multidisciplinary team featuring surgeons, gastroenterologists,
dietitians, pharmacists, and nurses.
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Intestinal Failure
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Various definitions of intestinal failure (IF) have been proposed. Perhaps
the simplest is that IF is a condition of malabsorption of fluid and
nutrients, necessitating parenteral nutrition (PN) to sustain adequate growth
in children. Etiologies of IF are detailed in
Table 1. Many of these
conditions do not intrinsically cause IF, but their natural histories often
mandate surgical resection of bowel.
This article reviews the 3 primary modalities in managing IF in pediatric
patients: nutrition, pharmacologic, and surgical approaches. A multimodality
treatment program, using all 3 approaches in conjunction, enhances and
facilitates overall intestinal rehabilitation.
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Nutrition Approaches to IF
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PN
Arguably, there has been no greater advancement in managing IF than the use
of PN in patients with enteral intolerance. IF patients are susceptible to
significant fluid losses and electrolyte imbalances. Emesis, stomal output,
and diarrhea are several symptoms that alter fluid balance. Care must be taken
to adjust the PN content, and replenish the patient with additional hydration
fluid. Typically, PN should be maintained as a standard formula, whereas
additional losses are replaced on an individual basis. Measurement of
electrolytes in urine and stomal output is helpful in assessing electrolyte
supplementation. Acute electrolyte deficiencies can be supplemented in
replacement fluids as an adjunct to PN. When the patient's fluid and
electrolyte status has stabilized, these supplements can be incorporated into
the PN components.
Management of PN-Associated Liver Disease (PNALD)
Although PN has allowed for the provision of nutrients and calories,
pediatric patients are susceptible to PN-associated cholestasis and subsequent
liver damage. Ironically, the very therapy that sustains patients with short
bowel syndrome (SBS) may also be hepatotoxic. Between 40% and 60% of IF
patients who require long-term PN develop liver-associated disease. Infants
and children often present with cholestasis, whereas adolescents present with
steatosis. Ursodeoxycholic acid and sincalide have been used to prevent or
treat PNALD, but with limited
effectiveness.1
Ursodeoxycholic acid is a hydrophilic bile acid that improves bile acid flow
and displaces toxic acids. It reduces the clinical signs and symptoms of
cholestasis, but does not prevent disease progression. To prevent interactions
with cholestyramine, ursodeoxycholic acid should be taken 1 hour before or
4–6 hours after cholestyramine administration.
It has been demonstrated that lipids are metabolized differently depending
on their route of
administration.2
Enteral lipids are absorbed by the enterocyte in the form of a micelle and
packaged into chylomicrons for ultimate disposal in the liver. In the
bloodstream, these particles rapidly acquire apolipoproteins from circulating
high-density lipoproteins and subsequently can be metabolized by the liver.
The emulsified particles of commercially made and IV-administered lipid
emulsions, such as Intralipid (Kabi Pharmacia, Clayton, NC), mimic the size
and structure of chylomicrons but differ in their content. In contrast to
chylomicrons, artificial lipid particles primarily contain essential fatty
acids, such as -6 fatty acids and triglycerides, but are devoid of
cholesterol or
protein.3 Recent
studies have suggested that these -6 fatty acid–containing
emulsions are dependent on lipoprotein lipase, apolipoprotein E, and
low-density-lipoprotein receptors for clearance, and are metabolized with less
lipolysis and release of essential fatty acids than chylomicrons. In fact, it
appears that they may be cleared as whole particles by tissues other than the
liver.4 These
factors may account for the increased incidence of steatosis associated with
IV administration of IV lipid emulsion products.
There are several methods that clinicians can use to reduce the risk of
PN-associated cholestasis. In neonates and infants, PN caloric intake should
be limited to 90–100 kcal/kg to avoid overfeeding. In addition, attempts
should be made to cycle PN 2–6 hours a day, which promotes the cyclic
release of gastrointestinal (GI) hormones. Patients that exhibit signs and
symptoms of infections, bacterial overgrowth, or line sepsis should be
aggressively treated. Last, efforts should be made to encourage and advance
enteral
nutrition.5
An exciting and recently published case report discusses the potential role
of IV fat emulsion to treat infants receiving prolonged courses of PN
complicated by
PNALD.6 Clinicians
should consider stopping all conventional lipid emulsions and instead
providing 1 g/kg/d as parenteral fish oil. Lower doses of Omegaven may not be
effective and may predispose patients to essential fatty acid deficiency,
which has also been linked to the development of fatty liver.
Enteral Nutrition
Despite the benefits of PN, however, the ultimate goal for all patients
should be to achieve enteral tolerance. Enteral feeding, regardless of whether
it is oral or via a feeding tube (eg, nasogastric tube or
gastrostomy), promotes physiologic responses that may enhance the intestine's
adaptation process. Mucosal hyperplasia, for example, is stimulated through
direct contact with epithelial cells; stimulation of gastric, biliary, and
pancreatic secretions; and enhanced production of trophic
hormones.7 Early
enteral feeding (ie, within 6 weeks after intestinal resection) reduces the
duration of PN, as well as the associated risk of PN-associated
cholestasis.8
Oral feedings of infants can prevent feeding aversion, as a result of
learning how to suck and swallow. Continuous enteral feedings achieve total
and constant saturation of intestinal transporters, thus using the full extent
of the remaining absorptive surface
area.9 Bolus
feedings are another method of providing enteral nutrition in older children,
but are poorly tolerated in infants.
Multiple administration routes can be used for enteral nutrition. Patients
who receive bolus or continuous feedings should also be encouraged to eat
orally. Patients should be engaged in oral motor stimulation therapy if
needed. Early referral to occupational and speech therapists is important,
especially given the difficulty in treating feeding aversion in SBS
patients.10
Patients may initially require PN support until they tolerate goal enteral
nutrition. Enteral feedings should be advanced steadily as clinical status
permits. Parenteral calories are simultaneously decreased by rate or number of
hours to ensure nutrition status and fluid balance. It is not uncommon to have
some GI intolerance as feedings are advanced. Typically, however, fecal
samples should have reducing substances <1% and a fecal pH above
5.5.5 Severe
carbohydrate malabsorption is identified if fecal reducing substances are
>1% and fecal pH is
<5.5.5
Transition to Enteral Nutrition: Factors for Consideration
Once patients with SBS are successfully transitioned from PN to enteral
nutrition, care must be taken to avoid nutrient deficiencies. As previously
noted, fat malabsorption predisposes patients to deficiencies in the
fat-soluble vitamins, as well as in zinc, calcium, and magnesium. In patients
with ileal resection, cyanocobalamin (vitamin B12) deficiency may
occur. Some centers will administer pancreatic enzymes with fat malabsorption
to improve nutrient
absorption.11
Dietary Modifications
Only a small number of evidence-based studies exist regarding diet
modifications for
IF.12–14
These studies do not allow for a consensus regarding the efficacy of
peptide-based diets. One study has shown an improvement in nitrogen
absorption,15
whereas 2 others have failed to demonstrate a significant
impact.16,17
The benefits of an amino acid–based formula over a protein hydrolysate
is also unclear.18
It is true, however, that IF patients are predisposed to intestinal mucosal
barrier breakdown, bowel dilation, and bacterial overgrowth, all of which may
increase the risk of food allergies. As a result, amino acid–based
formulas may reduce the risk of the development of food
allergies.19,20
Although protein provides little osmotic load, simple carbohydrates, such
as sucrose and fructose, can lead to osmotic diarrhea. GI bacteria break down
these simple sugars in osmotically active acids that increase the osmotic
load.5 In
contradistinction, complex carbohydrates found in pasta, potatoes, and breads
are well tolerated and, if possible, should comprise the majority of energy
intake (eg,
50%–60%).21
Fat can be a significant source of energy intake for IF patients.
Unfortunately, fat is not well tolerated due to bile salt malabsorption, which
leads to decreased micelle formation and fat digestion. Medium-chain
triglycerides (MCTs) compared with long-chain triglycerides (LCTs) do not
require micelles for absorption and thus are better tolerated in patients with
bile acid or pancreatic insufficiency. MCTs, however, also increase the
osmotic load in the intestine and provide fewer calories than LCTs. In
addition, LCTs may stimulate intestinal adaptation after intestinal
resection.7 In SBS
patients with a colon in continuity, it has been shown that a mixture of an
MCT and LCT diet can improve energy and fat
absorption.22
The addition of fiber to the diet can decrease overall intestinal transit
time. In patients with a colon in continuity, the metabolism of fiber can
serve as an energy
source,23 as well
as lead to the production of short-chain fatty acids (SCFAs). SCFAs, such as
butyrate, are considered a fuel source for
colonocytes24 and
also enhance sodium and water absorption, thereby reducing stool output and
sodium
losses.25
Of the formulas available, Alimentum (Ross, Columbus, OH), Pregestimil, and
Nutramigen (Mead Johnson, Evansville, IN) are "semielemental"
hypoallergenic formulas with hydrolyzed casein as the protein source, not
amino acids. Nutramigen is the most nutritious choice for babies with moderate
protein allergies. The most elemental formulas are Elecare (Ross) and Neocate
(SHS, Gaithersburg, MD). Both have amino acids as a protein source. Elecare
has 33% MCT and Neocate has only about 5% MCT as a fat source. Babies with
severe protein allergies or multiple food allergies should be given Elecare or
Neocate. In babies with severe fat malabsorption and SBS, Elecare may be
preferable.
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Pharmacologic Approaches to IF
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Patients with SBS often require unique therapies to assist in bowel
adaptation (Table 2). Depending
on the patient, they may require prokinetic agents or antidiarrheal agents or
a combination of both. Other therapies such as the use of IV fish oil have
been used to treat PN-associated liver injury in these
patients.6 Due to
the complexities of SBS, dosage recommendations are often quite different from
established norms.
Antimotility Agents
In SBS, gut motor activity is typified by a normal feeding pattern, along
with more frequent interdigestive motor complexes and a marked reduction in
phase 2 activity. Loperamide is often used in these patients to reduce transit
rate and enhance absorption. By reducing intestinal motility, water and sodium
output from an ileostomy is reduced by approximately
20%–30%.26 It
acts to enhance the general muscle tone of the small intestine, thus
increasing water and nutrient
absorption.27 The
drug works by way of intestinal opioid receptors, acting directly on
intestinal muscles to inhibit
peristalsis.28
Loperamide is preferred over opiates such as codeine because it is not a
sedative or addictive. Because it undergoes enterohepatic circulation (which
is altered in patients with SBS), higher doses are often needed; doses as high
as 0.8 mg/kg/d to a maximum of 24 mg/d may be required. It should not be used
in patients with slow transit times or those with refractory small bowel
bacterial overgrowth. The liquid formulation of loperamide should not be used
due to its sorbitol and alcohol content.
Prokinetic Agents
Intolerance to enteral nutrition continues to be a problem with SBS.
Nausea, vomiting, and abdominal distention are common signs and symptoms.
Prokinetic agents such as metoclopramide, erythromycin, and cisapride have
been shown to promote gastric motility in these
patients.29 Side
effects, however, limit their usefulness. Metoclopramide, a central and
peripheral dopamine type 2 receptor antagonist, has a variety of central
nervous system side effects, including dystonic reactions, extrapyramidal side
effects, and tardive dyskinesia. Erythromycin has been used as a motilin
receptor agonist. All are associated with a wide number of potentially serious
drug interactions.
Cisapride, a 5-HT3 agonist, is associated with serious cardiac
arrhythmias and sudden death, limiting its usefulness. Cisapride has been part
of an FDA-mandated limited-access protocol in the United States since May 2000
due to concerns of adverse cardiac events. The prescribing physician
participating in the limited-access program must be board eligible or
certified in 1 or more of the following areas: internal medicine (including
gastroenterology and cardiology), family practice, pediatrics (including
neonatology), or surgery. As part of this program, institutional review board
(IRB) approval completion of a Form FDA 1572 and signed informed consent are
required.30
Tegaserod (Zelnorm; Novartis, East Hanover, NJ), a selective
5-HT4 receptor agonist, stimulates peristalsis and accelerates
colonictransit.29,31
Originally approved for irritable bowel syndrome (constipation type) in women,
doses of up to 12 mg/d have been used to accelerate small bowel and colonic
transit. It modulates both normal and altered motility throughout the GI
tract. Evidence supporting its use is limited to case series in critically ill
adults to advance enteral feedings in the presence of impaired gastric
motility.32 Because
its main metabolic pathway is presystemic, no clinically relevant drug-drug
interactions have been identified. It does not cross the blood-brain barrier,
nor does it have cardiac repolarization effects or QTc-interval
prolongation.33 In
March 2007, however, tegaserod was withdrawn from the market over concerns of
increased risks of cardiovascular events, such as heart attacks and
strokes.
Antisecretory Drugs
Patients with SBS, especially children, often develop gastroesophageal
reflux. Contributing factors include altered gut secretions, dysmotility, and
bacterial overgrowth. Gastric acid hypersecretion may occur during the early
weeks after a small bowel resection. H2 antagonists and proton-pump
inhibitors have both been used for this purpose because acid blockade can
reduce jejunostomy output. Parenteral administration may be preferred,
especially in those patients with extremely short bowel, because drug
absorption is less than optimal. Octreotide, a somatostatin analog, has been
shown to have an inhibitory effect on gut
motility.34 It has
been shown that long-acting octreotide may be useful in prolonging small bowel
transit time in adults with
SBS.35 It reduces
ileostomy output and large-volume jejunostomy output. Experience with these
drugs in children is limited.
In patients with ileal resection and loose, watery stools, the bile acid
binder cholestyramine may be useful in reducing secretory diarrhea. In
patients with fat malabsorption due to bile salt insufficiency, however, it
may actually worsen diarrhea and increase the risk of deficiency of
fat-soluble
vitamins.36
Antimicrobials (Table 3)
Intestinal bacterial translocation often predisposes patients with SBS to
sepsis. Reflux of bacteria from the colon up into the small intestine, in
tandem with poor motility, which prevents flushing of gut bacteria in an
antegrade fashion, bowel dilation, and stasis of the small bowel all promote
bacterial overgrowth. This results in inflammation of the mucosal surface and
impairs both adaptation and transport of
nutrients.37
Bacterial overgrowth may also cause deconjugation of bile acids, resulting in
bile acid deficiency. Symptoms include abdominal distention, cramping,
diarrhea, and weight loss. Mental status changes can occur due to accumulation
of
D-lactate.38
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Table 3 Comparison of agents used for treatment of bacterial overgrowth
(typical course 7-10 days; all meds are to be given orally)
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Short courses of oral antimicrobials are the main-stay of therapy in the
management of bacterial
overgrowth.39 Most
protocols include metronidazole with or without trimethoprim/sulfamethoxazole,
aminoglycosides, extended-spectrum penicillins/cephalosporins, or vancomycin.
Some centers also include a week of antifungals such as amphotericin B. Due to
product design limitations, oral administration of injectable products may be
necessary. Typically, antimicrobials are given for 1 week per month, although
some patients may require continuous administration. Concerns about the
development of resistance may require rotating the agents in the protocol.
In addition to antimicrobials, other therapies have been used to reduce
bacterial overgrowth. Periodic flushes with oral polyethylene glycol
electrolyte solutions or other cathartics have been
used.40 Probiotics,
such as Lactobacillus rhamnosus GG, have been used, although
additional studies are
needed.41,42
Mineral Supplementation
Magnesium deficiency is often seen in patients with a jejunocolic
anastomosis or those with jejunostomies as a result of hyperaldosteronism
secondary to dehydration and sodium
depletion.43,44
Once water and sodium depletion is corrected, magnesium supplementation is
often necessary. Most oral magnesium products have poor bioavailability in
this patient population, making IV administration the preferred route of
delivery. If oral products must be used, magnesium oxide salts are
preferred.
Vitamin Deficiencies
Children with IF, especially those with hepatic disease, are at risk for
developing a variety of fat-soluble vitamin deficiencies because of fat
malabsorption and inadequate dietary intake.
Table 4 contains dosing
recommendations for children with malabsorption syndrome, as well as
cholestasis. Even with supplementation, children with mild to moderate
cholestatic liver disease are at risk for developing phylloquinone (vitamin K)
deficiency. Traditional assessment of vitamin K status (ie, prothrombin time)
may be inadequate, and recently, it has been suggested that elevations in
PIVKA-II (protein induced by vitamin K absence-II) concentrations may be a
more sensitive marker of vitamin K
stores.45 Vitamin K
concentrations are directly correlated with the severity of liver disease.
Traditional doses of supplemental oral vitamin K (2.5–5 mg, 2–7
times a week) seem to be inadequate to meet the needs in >50% of the
children studied.
Similarly, low vitamin A concentrations are present in children with
cholestatic liver disease receiving routine vitamin
supplementation.46
Altered hepatic synthetic function, however, may also decrease retinol binding
protein synthesis, suggesting that unbound vitamin A concentrations could
actually be elevated in liver failure. Interestingly, vitamin E levels remain
normal in this population, although unsupplemented infants and children
continue to demonstrate
deficiencies.46
Nutritional Supplements: Growth Hormone and Glutamine
Enhancement of intestinal adaptation continues to be an attractive
alternative in the management of patients with SBS. Rodent studies have
described improved intestinal adaptation with the use of growth hormone (GH)
or insulin-like growth factor-1 (IGF-1). IGF-1 has been shown to decrease
PN-associated mucosal
atrophy47 and in
combination with glutamine has increased villous growth and intestinal
DNA.48,49
GH, through IGF-1 signaling, has been proposed as a potential treatment option
for patients with IF. In animals receiving PN, mucosal atrophy with glutamine
deficiency can occur, and glutamine supplementation reverses the loss of
mucosal
thickness.50
Further, glutamine may improve gut immunity because it has been shown to
prevent PN-associated depletion of immunoglobulin A, which produces gut lamina
propria plasma cells.
To date, there is insufficient evidence to support the use of GH,
glutamine, or a combination of the 2 as standard therapy in IF patients. There
have been several small studies that evaluated the use of GH, or a combination
of GH and glutamine, that have demonstrated an improved nutrient absorption
and ability to wean off
PN.51–53
Increased lean body mass was shown in 2 studies, using GH and
glutamine.54,55
Other studies, however, have failed to demonstrate clinical improvement in
fecal volume or intestinal
absorption.56–58
None of these studies, including those with positive findings, have shown
statistically significant increases in fat absorption.
Other Considerations
Because many drugs tend to be incompletely absorbed by patients with SBS,
high doses may be
required.59 Drugs
such as digoxin and levothyroxine may be given parenterally to ensure
consistent therapeutic blood concentrations. Similarly, medications and
vitamins that undergo enterohepatic recirculation such as loperamide and
vitamin A may require higher than normal doses. Because patients with SBS are
prone to dumping syndrome, practitioners should also be careful to avoid
hypertonic liquid medications or those products containing sorbitol.
Emerging Therapies
Glucagon-like peptide 2 (GLP-2) has generated recent attention as a
potential agent in the management of patients with IF. There have been several
studies describing the various gastrointestinal properties of GLP-2, such as
enhanced epithelial and mucosal
proliferation,60,61
improved gastric
motility,62 and
decreased gastric
secretion.63 The
initial studies using GLP-2, or its protease-resistant analog Teduglutide,
have demonstrated improvements in intestinal absorption and decreases in fecal
volumes.64,65
Studies with large sample sizes are ongoing.
Pharmaceutical care of the patient with SBS is complex and ever changing as
intestinal function improves or complications develop. Pharmacologic therapy
is directed at controlling gut motility, enhancing intestinal adaptation,
minimizing small bowel overgrowth, and preventing nutrient deficiencies and
hepatic complications. No single approach is effective for all patients with
SBS, and care must be directed toward specific patient conditions, using an
integrated team approach.
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Surgical Approaches to IF
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In addition to medical and pharmacologic treatments for IF, some patients
may require surgical management. Surgical indications for IF can be divided
into 2 categories: (1) procedures aimed at correcting the etiology of IF, and
(2) procedures addressing the numerous complications from IF.
Initial surgeries focus on the correction of anatomic or mechanical
abnormalities that threaten the patient's bowel or life. Diseases such as
necrotizing enterocolitis, gastroschisis, volvulus, and intestinal atresias
all can result in the resection of a large percentage of small and large
bowel. The major principles of management are bowel conservation (especially
small intestine) and the prompt reestablishment of bowel
continuity.8
Surgical management of IF should not be viewed as a last resort but rather
an integral aspect of intestinal rehabilitation. Although not fully
understood, the small intestine undergoes an adaptation process that may
consist of histologic and enzymatic changes, enhancing nutrient absorption and
improving bowel motility. Surgeons have used various techniques to manage the
symptoms of IF. In 1980, a method of longitudinal intestinal lengthening and
tailoring (LILT) was
developed.66 This
method takes advantage of the 2 leaves of the mesenteric blood supply to the
intestines by dividing the bowel longitudinally, at the 12 o'clock position
(with the mesentery at the 6 o'clock position). Next, these hemiloops are
anastomosed isoperistaltically, resulting in bowel that is smaller in diameter
and longer in length. Isolated bowel segments, in which there is an imported
blood supply to the antimesenteric border of a dilated bowel loop by
attachment to the abdominal wall, have also been
attempted.67 A
useful adjunct to bowel-lengthening procedures is the creation of a nipple
valve.68 This
valve, tailored to cause proximal bowel dilation without obstruction, can
facilitate a bowel-lengthening operation.

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Figure 1. A schematic representation of the serial transverse enteroplasty procedure.
Stapling devices are applied in alternating directions (arrows). Reprinted
from Journal of Pediatric Surgery, Vol 38, Kim HB, et al, Serial transverse
enteroplasty (STEP): a novel bowel lengthening procedure, pages 425–429,
© 2003, with permission from Elsevier.
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Outcomes With LILT
Reported survival after LILT ranges from 30% to
100%.69,70
Survivors that were successfully weaned off PN ranged from 28% to
100%.69,70
The wide disparity in mortality and reduction of PN dependence can likely be
attributed to multiple variables among institutions, including preoperative
bowel length and degree of liver failure. Furthermore, many of the reported
series have been limited by small sample sizes, with the largest including 49
patients.70 Bowel
necrosis with LILT has also been
reported.70
Serial Transverse Enteroplasty
The technique of serial transverse enteroplasty (STEP) was described more
recently.71,72
In this operation, a stapler is applied across the dilated bowel in an
alternating fashion, leaving a zigzag-shaped bowel
(Figure 1). This approach both
lengthens and tapers the dilated bowel without damaging the mesenteric blood
supply or diminishing mucosal surface area.
A salient advantage of the STEP procedure is that it is simple to perform.
Because of the adaptation process, there can be redilation of the bowel after
a LILT operation. Further, bowel segments of variable dilation can be
lengthened with the maintenance of a uniform channel. Multiple STEP procedures
are feasible by repeating the operation after the process of adaptation and
dilation has
occurred.73,74
The STEP procedure is also suitable for patients with neonatal atresias and
limited bowel length, as well as patients with refractory D-lactic
acidosis.71,75–77
Initial studies demonstrated that after the STEP procedure, there are
improvements in enteral tolerance, nutrition indices, and ability to wean off
PN.74,78,79
Outcomes With STEP
Recent data from the International STEP Registry, examining a total of 38
patients, demonstrated a substantial increase in intestinal length and
improvement of enteral
tolerance.74 In the
SBS cohort, nearly 50% of patients were successfully weaned off PN.
Transplantation
Commonly cited indications for transplantation include failure to wean off
of PN, PN-associated liver disease, recurrent central catheter sepsis, and
loss of vascular access to provide
PN.80 Similar to
the LILT and STEP procedures, intestinal transplantation should be viewed as
an adjunct to intestinal rehabilitation.
Historically, intestinal transplantation was complicated by significant
morbidity and mortality. With recent advances in immunosuppression,
specifically the use of tacrolimus, outcomes such as mortality,
cost-effectiveness, and quality of life have improved over the last
decade.81
Currently, patients undergoing intestinal transplants experience 1-year graft
survivals of 80% and survival of
80%.82
Three different types of transplants can be offered to patients depending
on the nature and extent of their IF: isolated intestinal, liver-intestinal,
and multivisceral (eg, stomach, duodenum, pancreas, intestine, and liver).
Recent data show that of pediatric intestinal transplantations, 50% are
liver-intestinal, 37% are isolated, and 13% are
multivisceral.82
The decision regarding the type of transplantation should be based on each
patient's specific disease and condition.
 |
Conclusion
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Intestinal rehabilitation for pediatric patients with IF is best approached
in a multidisciplinary fashion. With specialized nutritionists, pharmacists,
gastroenterologists, nurses, and surgeons, patients can be provided with
complex medical care tailored to their specific needs. The overriding goal
remains the use of the GI tract for oral/enteral nutrition.
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Accessed June 30, 2007.
Nutrition in Clinical Practice, Vol. 22, No. 6,
653-663 (2007)
DOI: 10.1177/0115426507022006653

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