876 REVIEW ARTICLE

Short Bowel Syndrome in Adults

REVIEW ARTICLE
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Jamie Bering, MD1 and John K. DiBaise, MD, FACG1

Short bowel syndrome (SBS) is a rare disorder characterized by severe intestinal dysfunction leading to malabsorption of
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macronutrients and micronutrients that often results in permanent need of parenteral nutrition support. Patients can
develop SBS because of massive intestinal resection or loss of intestinal function and consequently experience
significant morbidity and increased healthcare utilization. The remaining anatomy and length of bowel after intestinal
resection have important prognostic and therapeutic implications. Because patients with SBS constitute a heterogenous
group, management is complex and multifaceted, involving nutrition support, fluid and electrolyte management, and
pharmacologic therapies in particular to control diarrhea. Surgical interventions including intestinal transplantation may
be considered in selected individuals. Successful care of these patients is best accomplished by a multidisciplinary team
that is experienced in the management of this syndrome.
Am J Gastroenterol 2022;117:876–883. https://doi.org/10.14309/ajg.0000000000001763

INTRODUCTION prior abdominal surgery is a leading cause of SBS in adults and

Short bowel syndrome (SBS) is the rarest and least known organ accounts for as many as 50% of patients who develop SBS (6).
failure syndrome and is characterized by malabsorption of mac- Mesenteric ischemia remains an important predisposing factor as
ronutrients and micronutrients. Intestinal failure (IF), a related does Crohn’s disease, although the rates of intestinal resection in
condition, is defined as the reduction of gut function below the Crohn’s disease seem to be declining presumably because of
minimum necessary for the absorption of macronutrients and/or improved efficacy of medical therapy and the development of
water and electrolytes such that parenteral support, either fluid and/ alternative surgical treatment options (7,8).
or nutrition, is required to maintain health and/or growth (1,2). IF
may be classified as type I acute IF, type II prolonged acute IF, and
type III chronic IF (Table 1). SBS is the most common cause of
DEFINITION AND PATHOPHYSIOLOGY
Because of the limited facilities in clinical practice that can per-
chronic IF (SBS-IF), accounting for approximately 60% of adult
form nutrient absorption ‟balance” studies to better define SBS-IF,
cases. In contrast to IF, intestinal insufficiency refers to patients who
the remaining bowel length (when known) is generally used (9).
do not permanently require parenteral support because they are able
Importantly, although ,200 cm of small intestine remaining is
to compensate for the missing bowel length by hyperphagia, dietary
often used to define adult SBS (the Center for Medicare and
and fluid modifications, pharmacologic and surgical interventions,
Medicaid Services uses , 153 cm), the development of this con-
and/or enteral nutrition support.
dition is not solely dependent on the length of the remaining bowel
SBS in adults generally occurs because of massive intestinal re-
(2,10). Recall that small bowel length is highly variable in adults
section and carries significant morbidity and mortality and high
(between 3 and 8 m) and the small intestine can generally com-
utilization of healthcare resources, which substantially impairs
pensate for resections of up to nearly 75% of its total length,
quality of life (2,3). Potential clinical manifestations include chronic
depending on the section of the small intestine remaining and the
diarrhea, malabsorption with weight loss, fluid and electrolyte im-
presence of any disease in the residual bowel. Therefore, when
balance, micronutrient deficiencies, nephrolithiasis, and liver and
caring for an SBS patient, it is more important to know the length
kidney disease. Much of this relates to the loss of intestinal absorptive
and section (i.e., jejunum or ileum) of the remaining small bowel
surface and rapid intestinal transit present in these patients. Specific
rather than the length of the section that was removed (6). Opti-
clinical consequences tend to be related to the regions of the gut that
mally, the length of residual bowel is measured at the time of
are missing. These consequences provide important clinical clues
surgery along the antimesenteric border of unstretched bowel,
that can help refine SBS management, which can be complex and
from the duodenojejunal flexure to the ileocecal junction, the site of
whose outcome is strongly dependent on the care and support re-
any small bowel-colon anastomosis, or to the end-ostomy.
ceived from an experienced multidisciplinary team (4). In this nar-
As alluded to earlier, factors that affect the outcome of a pa-
rative review, we will focus on adult patients with SBS.
tient with SBS include not only the length but also the region of
the remaining small intestine and the presence of the colon. Fluid,
ETIOLOGY electrolyte, and nutrient absorption vary among the segments of
There are several disorders that may lead to SBS (Table 2) (3,5). intestine. Water absorption is a passive process resulting from the
Repeated surgical intervention to address complications from a active transport of nutrients and electrolytes. Because of leaky

1
Division of Gastroenterology and Hepatology, Mayo Clinic in Arizona, Scottsdale, Arizona, USA. Correspondence: John K. DiBaise, MD, FACG.
E-mail: Dibaise.john@mayo.edu.
Received December 31, 2021; accepted April 1, 2022; published online April 5, 2022

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Table 1. Types and causes of intestinal failure (1)
Type Characteristics
1 • Acute IF
• Self-limited, lasting days to weeks
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• Generally, require only short-term parenteral support
2 • Prolonged IF
• Requiring parenteral support over weeks to months
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• Eventually regain full enteral autonomy
3 • Chronic IF
• May evolve from type II IF
• Require long-term parental nutrition support
IF, intestinal failure.
intercellular junctions, the jejunal mucosa is more permeable to
water, sodium, and chloride compared with the ileal and colonic
mucosa (11). In fact, the ileum plays an important role in sodium
and water conservation when the body becomes deplete because it
can increase its absorption in response to hormonal signals in the
body such as aldosterone (12). Sodium absorption in the jejunum
is dependent on water fluxes and is coupled to the absorption of
glucose, factors that are particularly important in patients with
SBS who only have the jejunum remaining (13). Carbohydrate,
protein, and water-soluble vitamin absorption occurs pre-
dominantly in the jejunum, while the ileum is the primary site for
bile salt absorption, vitamin B12 absorption, and active sodium
chloride transport, allowing for fluid reabsorption. In addition, L
cells in the ileum and proximal colon are the sites of production of
several hormones, including glucagon-like peptides 1 and 2
(GLP-1 and GLP-2) and peptide YY (PYY) that have important
transit-modulating and intestinotrophic properties. Fat absorp-
tion, including fat-soluble vitamins, occurs over a larger length of
small bowel. The colon participates in the absorption of water,
electrolytes, minerals, amino acids, and medium-chain triglyc-
erides. In SBS, the colon plays a vital role in fluid and electrolyte
reabsorption, given the additional fluid that enters the colon with
a capacity to absorb up to 6 L daily (14). In addition, it scavenges
energy (up to 1,000 kcals/d in SBS) by participating in the process
of complex carbohydrate fermentation to short chain fatty acids
with subsequent absorption (15).
Based on the remaining bowel anatomy, SBS may be classified
into 3 anatomical groups: group 1—end-jejunostomy; group
2—jejunocolic, who retain a portion of the jejunum anastomosed
to a portion of the colon; and group 3—jejunoileocolic, who
generally retain the entire colon and ileocecal valve along with a
portion of terminal ileum and jejunum (Figure 1) (6). Those adult
patients with SBS and a jejunocolic anastomosis who have ,50
cm of jejunum attached to the colon are at higher risk to require
long-term parenteral nutrition (PN) as are those without a colon
and ,100 cm of jejunum (16,17). Regarding the need for PN, the
presence of at least half of the colon has been suggested to be
equivalent to approximately 50 cm of small bowel (17).
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Short Bowel Syndrome in Adults 877
Examples
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• Critical illness
• Ileus
• Intestinal obstruction
• Flare of inflammatory bowel disease
• Abdominal catastrophe (e.g., mesenteric ischemia,
volvulus, or abdominal trauma)
• Enterocutaneous fistulae
• Massive enterectomy
• Refractory inflammatory bowel disease
• Short bowel syndrome
• Chronic intestinal pseudo-obstruction
• Frozen abdomen or carcinomatosis with inoperable
intestinal obstruction
• Extensive small bowel mucosal disease
Group 1 tends to have the most consequential malabsorptive
issues and represents the most unfavorable phenotype of SBS.
Often, these patients present with high ostomy output related, in
part, to accelerated gastric and intestinal transit due to reduced
secretion of gut-derived gut transit-slowing hormones (i.e., GLP-
1 and PYY) noted previously. Because group 1 patients no longer
have an ileum or colon, synthesis of these hormones is markedly
diminished or absent. Without these enteric mechanisms in
place, patients are more susceptible to dehydration, electrolyte
disturbance, hemodynamic instability, and renal injury and
typically require long-term parenteral support (6).
Clinical manifestations of individuals with group 2 anatomy
relate to the length of the jejunum remaining. They often experi-
ence diarrhea, steatorrhea, and vitamin B12 and fat-soluble vitamin
deficiencies primarily because the loss of the ileum and portions of
the jejunum and colon affects absorption of water, vitamin B12,
and bile salts (12). In contrast to those with an end-jejunostomy,
these patients may have normal or near-normal intestinal transit
times because the colon helps to compensate by upregulating the
synthesis of PYY and GLP-1. GLP-2 synthesis is also upregulated in
group 2 patients and contributes to the adaptation process within
the small intestine (18).
Patients with group 3 anatomy have the most favorable
anatomy and often do not require additional nutritional support
because the ileum has a greater ability to adapt both structurally
Table 2. Causes of short bowel syndrome in adults
• Mesenteric ischemia
• Abdominal trauma
• Crohn’s disease
• Malignancy
• Radiation enteritis
• Postoperative obstructive or vascular catastrophes
• Repeated surgical intervention to address abdominal surgery
complications
The American Journal of GASTROENTEROLOGY
 878 Bering and DiBaise
and functionally (see below) (12,19). In addition, the colon has a
tremendous capacity to absorb excess fluid and calories as de-
scribed previously. Thus, these patients often do not present with
nutrient deficiency, electrolyte disturbance, or dehydration.
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INTESTINAL ADAPTATION
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After massive intestinal resection, the remaining bowel undergoes
the process of adaptation, including both structural and functional
processes, to improve the efficiency of nutrient absorption. Struc-
tural changes include villous hypertrophy, increased colonic crypt
depth, faster proliferation rate of epithelial crypts, and increased
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intestinal length and diameter (20–23). Functional changes include
increased gut hormone secretion, reduced gut motility, and
microbiome alterations. Hyperphagia, a spontaneous behavioral
change whereby oral calorie consumption is increased, has also been
described as a functional adaptive response (24). These macroscopic
and microscopic changes occur in response to a variety of internal
and external stimuli including nutrients, gastrointestinal secretions,
hormones, growth factors, and other genetic/biochemical factors
and typically occur during the first 1–2 years after resection (25).
The ileum has a greater ability to adapt than the jejunum and is
another reason why those SBS patients with an ileal remnant tend to
have a better prognosis (26,27). By contrast, the jejunum seems to
adapt with only functional changes. Consequently, patients with an
end-jejunostomy demonstrate the least amount of intestinal adap-
tation and more often require permanent parenteral support.
CLINICAL COMPLICATIONS
A variety of clinical complications may occur in patients with SBS
and may result from the underlying disease, the altered bowel
anatomy and physiology, or its treatment including the need for PN
and its associated central venous catheter (Table 3). Knowledge of
Figure 1. Bowel anatomy types in short bowel syndrome. Figure created by the Mayo Clinic.
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these complications is critical for those caring for these patients to be
able to not only identify and treat them when they occur but also
prevent their occurrence whenever possible. Here, we will focus our
overview on the more common bowel-related complications.
Diarrhea
Diarrhea tends to be the most common and bothersome symptom
affecting patients with SBS and imparts a considerable negative
impact on their quality of life (28). The diarrhea that is experienced
does not occur solely from the loss of gut surface area. Gastric
hypersecretion occurs during the early months after massive in-
testinal resection and adds a significant volume of secretions to the
upper gut, and the acidity can denature and destabilize pancreatic
enzymes and bile salts, respectively, which may contribute to
maldigestion and malabsorption. A diminished bile salt pool
resulting from distal ileum resection and impaired enterohepatic
circulation further aggravates fat malabsorption. Owing to re-
section of their sites of production, reduced gut hormone (e.g., PYY
and GLP-1) feedback mechanisms permit accelerated dumping
into the upper gut with rapid intestinal transit, causing poor mixing
of pancreaticobiliary secretions with food. Active bowel disease
(e.g., Crohn’s disease and radiation enteritis), medications, Clos-
tridioides difficile infection, and small intestinal bacterial over-
growth may also contribute to the diarrhea seen in SBS.
Management of diarrhea can be challenging and may require
multiple therapeutic approaches, described later in this review.
Fluid and electrolyte disturbances
Fluid and electrolyte problems not only tend to predominate the
clinical course early after massive intestinal resection but may also
complicate the subsequent stages of SBS and contribute to neph-
rolithiasis and acute and chronic kidney injury. This is particularly
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 Short Bowel Syndrome in Adults 879

Table 3. Selected complications of short bowel syndrome

Selected

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Complication Risk factors Potential treatments references
Protein calorie • Malabsorption • Mineral and electrolyte supplementation (25,29,30,45)
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malnutrition, • Chronic diarrhea • Parenteral nutrition

dehydration, and • High ostomy output • Intestinotrophic factor
nutrient deficiencies
Chronic diarrhea • Reduction of absorptive surface area • Dietary/oral fluid modifications (28)
• Decreased intestinal transit time • Oral rehydration solution
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• Gastric hypersecretion • Antidiarrheal agents

• Humorally mediated rapid gastrointestinal transit • Antisecretory agents (PPI and H2RA)
• Small intestinal bacterial overgrowth • Somatostatin analogs
• Intestinal trophic factor
Nephrolithiasis • Fat malabsorption with colon in continuity • Maintain adequate urine output with increased fluid intake (17,32)
• Volume depletion • Low oxalate diet
• Prolonged duration of SBS • Potassium citrate
• Decreased Oxalobacter formigenes in the colon • Calcium carbonate
Cholelithiasis • Altered enterohepatic circulation with lithogenic bile • Prophylactic cholecystectomy when abdominal surgery is (17,32–34)
• Gallbladder stasis being undertaken for other reasons
• Chronic PN • Consider ursodeoxycholic acid in nonsurgical patients
Metabolic bone disease • Effects of long-term PN • Periodic assessment of bone mineral density (32,36–38)
• Malabsorption of micronutrients and • Calcium, magnesium, and vitamin supplementation
macronutrients including vitamin D • Metabolic acidosis correction
• Electrolyte alterations • Specific osteoporosis treatments
• Chronic metabolic acidosis
• Underlying patient factors including medications
• Insufficient sun exposure
Intestinal failure- • Altered bowel anatomy (small bowel ,50 cm • Avoid excesses and deficiencies in the PN formula (39–42)
associated liver disease and lack of colon in continuity) • Limit intravenous lipid dose to ,1 g/kg/d
• PN-related factors • Reduce/eliminate soybean-based intravenous
• Underlying systemic and/or liver-related conditions lipid emulsion
• Recurrent sepsis • Use non-soybean-based intravenous lipid emulsions
• Lack of oral/enteral intake • Cycle PN
• Small intestinal bacterial overgrowth • Increase oral/enteral intake
• Identify/treat sepsis and/or small intestinal bacterial
overgrowth
Catheter-related • Type of catheter • Aseptic technique during placement and dressing changes (43,44)
bloodstream infection • No. of lumen • Appropriate catheter and insertion site
• Anatomic location • Proper catheter care
• Remove catheter when no longer needed
H2RA, histamine-2 receptor antagonist; PN, parenteral nutrition; PPI, proton pump inhibitor; SBS, short bowel syndrome.

so for patients with SBS and an end-jejunostomy, where de-
hydration and electrolyte derangements are a common source of
morbidity and hospitalization. Together with chronic dehydration,
sodium deficiency contributes to the other electrolyte disturbances
seen in SBS including sometimes-difficult-to-replete hypokalemia,
hypomagnesemia, and hypocalcemia (29). Depending on how
much the jejunum is remaining, patients with end-jejunostomies
may lose more water and sodium than what they take in orally and,
hence, be described as a net secretor. These patients will often
require additional parenteral fluid and electrolyte support (19).
Micronutrient deficiencies
Micronutrient deficiencies are common in patients with SBS,
albeit usually subclinical in presentation (30). Water-soluble
vitamin deficiencies (e.g., B vitamins and vitamin C) are unusual
in SBS because they are absorbed in the proximal small bowel,
which typically remains intact. By contrast, fat-soluble vitamin
(e.g., vitamins A, D, E, and K) and essential fatty acid deficiencies
are more common, and large doses of supplementation may be
needed. Regarding trace element deficiencies, zinc deficiency
occurs relatively commonly, particularly in those with increased
stool output, and requires supplementation. Copper and sele-
nium deficiencies are also occasionally encountered. Because iron
is absorbed in the duodenum, supplementation is not typically
needed except in circumstances where the patient has decreased
oral intake or chronic gastrointestinal bleeding. In patients who
have undergone distal ileal resection of more than 50–60 cm,
lifelong vitamin B12 supplementation, typically administered

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 880 Bering and DiBaise
parenterally, will be needed (31). Periodic micronutrient moni-
toring and supplementation are important in all SBS patients
whether they be on or off PN.
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Cholelithiasis, nephrolithiasis, and metabolic bone disease
Cholelithiasis, oxalate nephropathy, and osteoporosis are also
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common in SBS (10). Gallstone formation, which usually consists
of cholesterol stones, is incompletely understood but believed to
develop because of altered bile composition and bile stasis caused
by changes in intestinal hormone secretion and enterohepatic
circulation (32,33). Because patients with SBS are at higher risk of
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complication from gallstones, it has been recommended that in
those undergoing another abdominal surgery, prophylactic
cholecystectomy should also be considered (34). Although oral or
enteral intake stimulates cholecystokinin production, which acts
to improve gallbladder motility and reduce bile stasis, there is no
convincing evidence supporting the role of cholecystokinin
supplementation in preventing gallstones (33,35). Ursodeox-
ycholic acid may reduce lithogenic bile, especially in patients on
long-term PN (33). Although there are no studies of its use in SBS,
based on its US FDA-approved indication, it may be considered
for use to dissolve symptomatic gallstones when present in the
nonoperative candidate.
Oxalate nephropathy is a risk for patients with underlying fat
malabsorption who have their colon in continuity (32,36). In this
situation, calcium favorably binds to free fatty acids, leaving ox-
alate to be reabsorbed in the colon where it is subsequently filtered
by the kidneys. Multiple other mechanisms also contribute to
stone development. Over time, oxalate stones may develop and
contribute to progressive renal dysfunction. Dietary restriction of
fat and oxalate‐containing foods, correction of dehydration, and
calcium citrate supplementation are important maneuvers to
reduce the risk of stone development (32).
Multiple factors also contribute to the development of meta-
bolic bone disease in SBS, particularly low body mass index and
vitamin D deficiency. Prior exposure to glucocorticoids, such as
for patients who have underlying inflammatory bowel disease,
also increases the risk of developing osteopenia or osteoporosis in
this patient population (37). Periodic monitoring of bone density
in patients with SBS is recommended as is exercise; sunlight ex-
posure; calcium, magnesium, and vitamin D replacement; and
correction of metabolic acidosis when present. Given their poor
bioavailability, parenteral forms of bisphosphonates are preferred
in SBS when needed (38).
IF-associated liver disease
IF-associated liver disease (IFALD) is the preferred term for he-
patic complications (e.g., steatosis, cholestasis, and cirrhosis)
occurring in patients with IF. IF-associated liver disease is di-
agnosed in the presence of abnormal liver enzymes, abnormal
radiography, or abnormal histology in the absence of other causes
(39). The risk of IFALD is greater in those with a small bowel
segment of ,50 cm, lack of colon in continuity, absence of oral/
enteral intake, presence of intra-abdominal inflammation, re-
current central venous catheter-related sepsis, small intestinal
bacterial overgrowth, and PN-related factors (40). Steatosis is
more commonly seen in adults, and the progression is slow and
mortality is low (,5%). By contrast, cholestasis occurs more
commonly in infants and young children, and progression to
fibrosis and end-stage liver disease is more common and can be
rapid with mortality in up to 60% (41). The provision of .1 g/kg/d of
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soy oil-based parenteral lipids for .6 months and the presence of
chronic cholestasis have been associated with the development of
complicated liver disease (42). Table 4 lists several management
options to prevent and treat IFALD.
MANAGEMENT
The management of SBS is multifaceted (Table 4) and best served
by an experienced multidisciplinary team.
Dietary considerations
Diet and fluid modifications are important aspects in the care of
SBS patients. Indeed, oral/enteral intake in the early stages assists
with intestinal adaptation. As such, all patients with SBS should
undergo a comprehensive nutritional assessment. The goal of
these modifications is to maximize fluid and nutrient absorption,
and they should be considered complementary to the pharma-
cologic approaches discussed below. Dietary recommendations
should be tailored to the individual patient, and amounts of di-
etary carbohydrates, fat, oxalate, and fiber will vary based on the
patient’s remaining intestinal anatomy, particularly whether
there is colon in continuity (e.g., modest restriction of fat and
oxalate in those with colon in continuity) (25,45). The intake of
complex carbohydrates and high-quality protein with sodium
supplementation and avoidance of concentrated/simple sugars
should be encouraged in all patients with SBS. Despite these
recommendations, given limited evidence to support and limited
consensus on the importance of an optimized oral diet in the
management of SBS, the major emphasis is placed on maintaining
compensatory hyperphagia. Because most stable adult SBS pa-
tients absorb only about one-half to two-thirds as much energy as
normal, thus, dietary intake must be increased by at least 50%
from their estimated needs (i.e., hyperphagic diet).
Fluid and electrolytes
Fluid and electrolyte management is important in the manage-
ment of SBS because these patients are at higher risk for de-
hydration, electrolyte disturbances, nephrolithiasis, and kidney
injury. Adequate hydration is based on a goal urine output of .1
L/d (some suggest .800 mL/d is sufficient) and a urinary sodium
concentration of .20 mEq/L (46). The use of a glucose-
electrolyte solution, also known as an oral rehydration solution
(ORS), can enhance jejunal absorption and reduce gastrointes-
tinal secretion by using the sodium-glucose-coupled transport
system (29). This is particularly helpful in those patients with SBS
and an end-jejunostomy and may also be helpful in those with a
section of the colon remaining. Commercially prepared ORS
products are available as are recipes for inexpensive, homemade
ORS. Importantly, ORS differs from commercial sports drinks
because the sodium content is higher and carbohydrate content
lower. Occasionally, parenteral fluids (without nutrition) with
oral or parenteral electrolyte and/or bicarbonate supplementa-
tion may be necessary. Routine monitoring of urine output,
electrolytes, and renal function should be instituted to ensure
proper maintenance.
Conventional medications
Several classes of medications are available for use in the man-
agement of diarrhea in patients with SBS (47). Importantly, just as
with nutrition and fluids, medications may not be readily
absorbed; however, most medications are absorbed within the
proximal jejunum and can be used in patients with SBS. Delayed-
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 Short Bowel Syndrome in Adults 881

Table 4. Summary of management recommendations for short bowel syndrome

Treatment

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(references) Recommendations Comments
Diet (25,45) • Dietary assessment for all by a dietitian familiar with SBS • Encourage hyperphagia.
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• Consume complex carbohydrates and high-quality • Tailor to the individual depending on the presence/absence
protein with sodium supplementation and avoidance of the colon.
of concentrated/simple sugars
Hydration (29,46) • Monitor urine output and renal function/electrolytes regularly • Oral rehydration solutions particularly useful with an end-jejunostomy.
• Generally, avoid hypertonic and hypotonic oral fluids • Parenteral fluids and electrolytes sometimes needed.
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Antisecretory • Proton pump inhibitors • Decrease gastric hypersecretion.

medications (47) • Histamine-2 receptor antagonists • Particularly useful in the acute phase after massive resection.
Somatostatin • Octreotide • Decrease GI secretions and motility but can inhibit intestinal
analogs (47,50) adaptation.
• Mostly used in those with high output stomas (.2 L/d)
requiring IV fluids.
Antimotility • Loperamide • Administer 30–60 min before meals and bedtime.
medications (48) • Diphenoxylate-atropine • Use of codeine and opium limited by CNS-acting effects.
• Codeine phosphate
• Tincture of opium
Other medications • Clonidine • a2-adrenergic antagonist action can slow intestinal transit and
(40,47,51–54) reduce stool volume.
• Long-term benefit unproven.
• Antimicrobials to treat small intestinal bacterial overgrowth • Consider for those with persistent flatulence, bloating, and diarrhea.
• Bile acid sequestrants • Can worsen steatorrhea in SBS.
• Should generally be avoided in SBS.
• Pancreatic enzyme replacement • No current evidence to support use in SBS.
• Ursodeoxycholic acid • May reduce lithogenic bile and cholelithiasis, especially in the
PN-dependent.
Trophic factors • Glucagon-like peptide-2 analogs • Used to wean PN support after optimization of diet, fluids, and
(55,56) • Recombinant growth hormone conventional medications.
• Growth hormone use largely abandoned because of concerns
of risks and long-term efficacy.
Surgical • Restoration of intestinal continuity • Several surgical interventions available in various clinical scenarios.
interventions • Intestinal tapering • Intestinal transplantation reserved for patients on lifelong PN who
(58,59) • Stricturoplasty suffer serious complications.
• Serosal patching for chronic fistulae
• Autologous GI reconstruction
• Intestinal transplantation
CNS, central nervous system; IV, intravenous; GI, gastrointestinal; PN, parenteral nutrition; SBS, short bowel syndrome.

release medications should be avoided. When applicable, alter-
native drug delivery methods (e.g., liquids and topical) should be
considered as should the monitoring of medication levels in the
blood (47). Antimotility and antisecretory agents are a mainstay
in SBS treatment. The use of antidiarrheals should be guided by
the measurement of its effect on stool output. Loperamide and
diphenoxylate-atropine are first-line antimotility agents because
of their low incidence of systemic effects (48). Because loperamide
enters the enterohepatic circulation, which is disrupted in those
patients without an ileum, high doses are frequently needed
(i.e., up to 4 tablets taken 4 times/d). In the setting of SBS, these
agents seem to be most effective when administered about 30
minutes before meals and at bedtime. The use of codeine and
tincture of opium is limited by their sedating effect and potential
for addiction when used for a long term. Proton pump inhibitors,
typically administered twice daily, are routinely used in the first 6
months after resection to offset the gastric acid hypersecretion but
may be needed indefinitely in some patients who continue to be
bothered by dyspeptic symptoms (49). Histamine-2 receptor
antagonists may also be used as second-line agents. Other anti-
secretory and antimotility agents such as somatostatin analogs
and clonidine are sometimes used if additional therapies are
needed; however, high-quality evidence supporting their benefit
in SBS is lacking (50,51). Although bile acid sequestrants are often
used in patients with suspected bile acid malabsorption who have
less than 100 cm of distal ileum resected and colon in continuity,
this is an uncommon situation in SBS, where more extensive ileal
resection leads to a diminished bile acid pool (52). The use of bile
acid sequestrants in this situation may worsen steatorrhea and
fat-soluble vitamin losses, and as such, we recommend that bile
acid sequestrants generally be avoided in SBS. Notably, there is no
role for a bile acid binder in those patients without colon in

© 2022 by The American College of Gastroenterology The American Journal of GASTROENTEROLOGY

Copyright © 2022 by The American College of Gastroenterology. Unauthorized reproduction of this article is prohibited.
 882 Bering and DiBaise
continuity, regardless of the length of distal ileum resected (53).
There is no evidence yet supporting the usefulness of pancreatic
enzyme supplementation in SBS. Because SBS patients are also at
risk for the development of small intestinal bacterial overgrowth,
REVIEW ARTICLE
antibiotic therapy may also be cautiously tried as part of the
diarrhea management strategy (54).
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Trophic factors
There is great interest in the use of growth factors in patients with
SBS who have been unable to achieve enteral independence, de-
YQp/IlQrHD3i3D0OdRyi7TvSFl4Cf3VC4/OAVpDDa8K2+Ya6H515kE= on 07/16/2023
spite optimization of diet, fluids, and conventional medications.
Although still available, the use of recombinant human growth
hormone (Zorbtive, Serono Pharmaceuticals) has largely been
discontinued because of worrisome adverse effects and ques-
tionable long-term efficacy (55). Teduglutide (Gattex, Takeda
Pharmaceuticals), a recombinant, degradation-resistant, longer
acting GLP-2 analog, was studied in 2 phase III multinational,
randomized, double-blind, placebo-controlled trials and shown
to improve intestinal absorptive function and allow PN-weaning
in patients with SBS-IF, even allowing some patients to achieve
enteral autonomy (56,57). Longer acting GLP-2 analogs, such as
glepaglutide and apraglutide, with the potential advantage of less
frequent administration are currently under development. Be-
cause teduglutide is a growth factor and can enhance the growth
of colonic and other gastrointestinal polyps as well as accelerate
cancer growth, it is contraindicated in patients with active ma-
lignancies, and patients should be screened by colonoscopy be-
fore initiating treatment. The potential side effects of teduglutide
and its cost mandate that teduglutide is considered only after
optimizing routine treatment strategies described and after pa-
tients are carefully informed of the potential risks and benefits
associated with trophic factor use focusing on the probability of
reducing/eliminating the need for parenteral support, improving
the quality of life, expected duration of treatment, expected
consequences after cessation, the need for regular monitoring,
and the costs of the treatment.
Surgical management options
In patients with SBS, restoration of intestinal continuity and re-
cruitment of any available distal bowel should be accomplished as
soon as safely possible to improve bowel function and reduce the
risk of or decrease PN dependency. Restoring bowel continuity
and eliminating a stoma may also improve a SBS patient’s quality
of life and reduce the risk of catheter-related infections. Intestinal
tapering to improve the function of dilated bowel, stricturoplasty
for benign strictures, and serosal patching for chronic fistulae
may prevent the need for resection. Autologous gastrointestinal
reconstruction operations (e.g., lengthening procedures and re-
versed segment) designed to enhance the mucosal surface area for
absorption and/or slow intestinal transit to facilitate absorption
or correct stasis and SIBO may reduce gastrointestinal symptoms
and reduce or eliminate malabsorption (58). Nonetheless, these
techniques should only be considered in a stable SBS patient after
the initial adaptive period and after medical and dietary man-
agement have been maximized. Owing to its relatively high long-
term morbidity and mortality, intestinal transplantation remains
reserved primarily for patients with SBS and a lifelong need for
PN when complications of PN such as liver disease, loss of venous
access sites, or recurrent episodes of life-threatening central ve-
nous catheter sepsis occur (59).
The American Journal of GASTROENTEROLOGY
Copyright © 2022 by The American College of Gastroenterology. Unauthorized reproduction of this article is prohibited.
CONCLUSION
SBS can be a significant consequence of intestinal resection
leading to decreased patient quality of life, increased morbidity,
and increased healthcare utilization. Management of patients
with SBS is complex and multifaceted, involving nutritional
support, hydration management, pharmacologic therapies, and
sometimes surgery. Successful management is best accomplished
by an experienced multidisciplinary team. Owing to the physical,
psychosocial, and financial burdens confronting those with SBS,
clinicians should encourage ongoing education for patients and
caregivers and their participation in sources of psychosocial
support.
CONFLICTS OF INTEREST
Guarantor of the article: John K. DiBaise, MD, FACG.
Specific author contributions: Both authors contributed to the
conception, drafting, revision, and approval of the final draft.
Financial support: None to report.
Potential competing interests: J.K.D. is a research support for
Zealand Pharmaceuticals; he has served on advisory board for
Takeda Pharmaceuticals.
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