DOI: 10.1002/ncp.

10978

INVITED REVIEW

Short bowel syndrome: Complications and management

Jamie Bering MD | John K. DiBaise MD

Division of Gastroenterology and

Hepatology, Mayo Clinic in Arizona, Abstract
Scottsdale, Arizona, USA Short bowel syndrome (SBS) occurs when a patient loses bowel length or
Correspondence
function significantly enough to cause malabsorption, oftentimes requiring
Jamie Bering, MD, Division of lifelong parenteral support. In adults, this occurs most commonly in the
Gastroenterology and Hepatology, Mayo setting of massive intestinal resection, whereas congenital anomalies and
Clinic Arizona, 13400 East Shea Blvd,
Scottsdale, AZ 85259, USA. necrotizing enterocolitis predominate in children. Many patients with SBS
Email: [email protected] develop long‐term clinical complications over time related to their altered
intestinal anatomy and physiology or to various treatment interventions such
Funding information
Financial support for the publication of as parenteral nutrition and the central venous catheter through which it is
the Nutrition in Clinical Practice administered. Identifying, preventing, and treating these complications can
supplement in which this article appears
be challenging. This review will focus on the diagnosis, treatment, and
was provided by VectivBio AG
prevention of several complications that can occur in this patient population,
including diarrhea, fluid and electrolyte imbalance, vitamin and trace element
derangements, metabolic bone disease, biliary disorders, small intestinal
bacterial overgrowth, D‐lactic acidosis, and complications of central venous
catheters.

KEYWORDS
bacterial overgrowth, electrolytes, intravenous infusions, metabolic bone disease, minerals,
parenteral nutrition, short bowel syndrome, vitamins

Short bowel syndrome (SBS) is a complex and sometimes occurrence whenever possible. Here, we will focus our
debilitating malabsorptive condition that occurs in the overview on the more common bowel and CVC‐related
setting of an anatomical or functional reduction in small complications.
intestine length. Many patients with SBS go on to develop
intestinal failure, in which parenteral support of fluids
and/or nutrition is necessary to maintain health and/or DIARRHEA
growth.1 Because of the anatomic and physiologic changes
that occur with SBS, it is not surprising that these patients Diarrhea is the most common presenting symptom for
may experience a variety of clinical complications resulting patients with SBS and may be severe and difficult to
from the underlying disease, the altered bowel anatomy control, leading to a substantial reduction in quality of
and physiology, or its treatment, including parenteral life.2 Diarrhea in this patient population is a result of the
support and its associated central venous catheter (CVC). multiple physiologic changes that occur from having a
Knowledge of these complications is critical for those massively shortened bowel. Specific factors such as
caring for these patients not only to identify and treat the the remnant bowel anatomy and remaining intestinal
complications when they occur but also to prevent their absorptive surface area, increased gastric motility and

© 2023 American Society for Parenteral and Enteral Nutrition.

S46 | wileyonlinelibrary.com/journal/ncp Nutr. Clin. Pract. 2023;38:S46–S58.

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NUTRITION IN CLINICAL PRACTICE | S47

accelerated small bowel transit, gastric hypersecretion, however, data may be extrapolated from their use in
diminished bile salt pool, altered enterohormone feed- patients with high‐output ileostomies and fistulas.10
back mechanisms, medications, and presence of active Loperamide or diphenoxylate‐atropine, generally admi-
intestinal infection or inflammation can affect the degree nistered 30 min before meals and bedtime, are recom-
of diarrhea that a patient may experience.3,4 mended first‐line agents because of their low incidence of
In the early postoperative phase following massive systemic side effects.11 In the case of loperamide, because
intestinal resection, the stomach increases the production of its normal uptake in the enterohepatic circulation,
of gastric acid, resulting in a hypersecretory state that can those patients with SBS without the distal ileum may
last 6–12 months. This phenomenon, originally described require higher than conventionally recommended doses
in 1974, leads to a larger volume of intestinal secretions as (eg, up to four tablets four times per day) to achieve a
well as increased acidity, which can result in further therapeutic response.2
maldigestion and malabsorption by denaturing and More systemically available opioids such as codeine
destabilizing digestive enzymes.5,6 In patients with SBS and tincture of opium are alternatives for patients with
who have undergone distal ileal resections >100 cm diarrhea; however, their use is generally limited to those
(ie, most patients with SBS), the bile salt pool eventually with refractory diarrhea because of increased risk of
becomes diminished, further exacerbating diarrheal centrally acting adverse effects such as sedation and
symptoms due to fat maldigestion.7 In addition, several potential for addiction. In addition, a small percentage of
enterohormones act to maintain normal gut transit the population may have altered metabolism of codeine
and bile acid production in healthy patients, including either by being a “poor metabolizer” with inactive
glucagon‐like peptide 1 (GLP‐1) and glucagon‐like peptide CYP2D6 enzymes or by being an “ultrarapid metaboli-
2 (GLP‐2), fibroblast growth factor 19 (FGF19), and peptide zer” with extra functional copies of the CYP2D6 gene.12
YY (PYY). Because these hormones are produced in the Those patients who are “ultrarapid metabolizers” have
distal ileum and proximal colon, in those patients who increased risk of developing adverse effects even with
have had resection of these intestinal segments, hormone standard doses of codeine. In some cases of refractory
signaling and feedback mechanisms become impaired, diarrhea, providers may trial combination therapy
furthering the development of diarrhea.8,9 with medications that work by different mechanisms of
Diarrhea is often a therapeutic challenge and may action, although this increases the risk of toxicity without
require a multimodal approach that includes diet and clear proven benefit. It is important to recognize,
fluid modifications and different classes of medications however, that diphenoxylate‐atropine and codeine can
for effective management. First‐line medications availa- have more pronounced adverse effects in elderly patients
ble for use in the management of diarrhea in SBS (those over 65 years of age). Therefore, it is recom-
are antimotility and antisecretory agents (Table 1). There mended that the antidiarrheal regimen be individualized,
are no randomized controlled trials comparing the taking into consideration age and other underlying
effectiveness of these medications in patients with SBS; comorbidities.

TABLE 1 First‐line medications for treatment of diarrhea in short bowel syndrome.

Class Medication Mechanism of action Comments
Antisecretory PPIs, H2RAs Decrease gastric acid and Use during the first 6–12 months after
medications fluid secretion massive intestinal resection; may need
to use longer
H2RA can be added to PNS
PPIs cannot be added to PN, but IV
formulations are available if oral is
ineffective
Antimotility Loperamide, diphenoxylate‐atropine, Slow intestinal transit Administer 30–60 min before meals and
medications codeine phosphate, tincture of bedtime
opium Liquid formulations are availablea
Use of diphenoxylate‐atropine, codeine,
and opium limited by CNS‐acting
effects
Abbreviations: CNS, central nervous system; H2RA, histamine 2 receptor antagonist; IV, intravenous; PN, parenteral nutrition; PPI, proton pump inhibitor.
a
Liquid formulations should be screened for unabsorbable osmotically active sugars that are added for sweetening purposes because these additives can draw
water into the lumen of the bowel, which can paradoxically worsen diarrhea.
 19412452, 2023, S1, Downloaded from https://aspenjournals.onlinelibrary.wiley.com/doi/10.1002/ncp.10978 by Cochrane Romania, Wiley Online Library on [16/11/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S48 | BERING and DIBAISE

Antisecretory medications such as histamine 2 electrolyte disturbances both after initial intestinal
receptor antagonists (H2RAs) and proton pump inhibi- resection and chronically. If not monitored carefully
tors (PPIs) can help reduce the volume of gastric and managed appropriately, further complications can
secretions, especially during the period of greatest arise including dehydration, chronic fatigue, altered
hypersecretion.13 It is worth noting that H2RA therapy mental status, weight loss, nephrolithiasis, and chronic
can be added to parenteral nutrition (PN) to ease kidney injury.
administration and optimize absorption. Because PPIs Remnant anatomy is an important factor to con-
cannot be added to PN formulations, separate intra- sider, as both the length and location of the remaining
venous (IV) administration may also be considered, small intestine and presence of a colon‐in‐continuity
particularly in those with ultrashort bowel (ie, <30 cm of can impact intestinal nutrient and fluid absorption.
small bowel remaining). Given the important role of the colon in fluid absorp-
Somatostatin analogues and clonidine are occasion- tion, patients with an end‐jejunostomy are particularly
ally used when first‐line agents are ineffective or only prone to increased morbidity and hospitalization related
partially effective; however, high‐quality evidence to dehydration and electrolyte imbalance.6 Patients with
supporting their clinical benefit in SBS is lacking.10,14 an end‐jejunostomy have an increased risk of becoming
Bile acid sequestrants are not recommended in patients a “net secretor” whereby they lose more fluid than they
with SBS, as they may worsen steatorrhea and fat‐ take in by mouth. These patients also lose large
soluble vitamin malabsorption due to an already amounts of sodium and other electrolytes in the stool,
reduced bile acid pool.14 Importantly, there is no role and this, coupled with dehydration, contributes to
for bile acid sequestrants in those patients with an end‐ other electrolyte disturbances that may be difficult to
jejunostomy/ileostomy. Finally, there is currently no correct.20
evidence suggesting a role for pancreatic enzyme Hydration status can be objectively assessed using
replacement in SBS, as there is not a deficiency in urine output and urine sodium measurements, for which
pancreatic exocrine secretion in the vast majority of urine output of >1 L/day (some suggest >800 ml/day is
patients with SBS. A recent study by Sainath et al15 did sufficient) and a urinary sodium concentration >20 mEq/
evaluate the use of pancreatic enzymes in a small L suggest that a patient is achieving adequate hydra-
cohort of 11 patients (6 pediatric and 5 adult patients). tion.20 For those patients with SBS who have an end‐
Although an improvement in the coefficient of fat jejunostomy, average daily stoma outputs >1–1.2 L/day
absorption and coefficient of nitrogen absorption was have been associated with increased dehydration
seen in some patients, they did not reach statistical risk.21,22 Asking patients to weigh themselves regularly
significance. Perhaps, in a subset of patients with SBS can also be helpful in assessing whether they are
who have very rapid small bowel transit, poor mixing developing a fluid deficit. A patient's reported level of
of nutrients with pancreatic enzymes due to asyn- thirst can be another clinical indicator of volume status,
chrony of pancreatic juice secretion meeting up with as those who are volume depleted often have increased
food boluses may occur and contribute to diarrhea. feelings of thirst. This can occur even when they are
Further studies are needed. Although not specifically drinking a high volume of fluid, especially if the type of
indicated for treatment of diarrhea, longer‐acting GLP‐ fluids consumed are inappropriately hypotonic as this
1 and GLP‐2 agonists may have the desired effect of can increase stool output and create a negative fluid
reducing stool output by either slowing gut transit or balance. This is discussed in detail in another article in
enhancing absorption, respectively.16–18 These agents this supplement.
are discussed in detail in another article in this Several interventions may be needed to help patients
supplement. with SBS achieve adequate hydration. A common
misconception by patients with SBS is that they need to
drink large amounts of water to maintain adequate
FLUID AND ELECTROLYTE hydration. However, both hypotonic and hypertonic
IMBALANCE fluids can increase stool output and exacerbate diar-
rhea.11 Oral rehydration solutions (ORSs) leverage the
It is estimated that under normal conditions, the sodium‐glucose cotransport system of the jejunum to
gastrointestinal tract may handle 8–10 L of fluid per promote passive absorption of water and electrolytes
day from various sources, including oral intake and in the proximal intestine.23 Patients with an end‐
intestinal secretions.19 Intestinal resection can lead to jejunostomy may benefit most from the use of ORSs,
substantial challenges with fluid and electrolyte balance. with a goal sodium concentration of 90–120 mEq/L. For
Indeed, patients with SBS are at high risk for fluid and patients who are unable to maintain a euvolemic state
 19412452, 2023, S1, Downloaded from https://aspenjournals.onlinelibrary.wiley.com/doi/10.1002/ncp.10978 by Cochrane Romania, Wiley Online Library on [16/11/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
NUTRITION IN CLINICAL PRACTICE | S49

with oral intake, parenteral fluids with or without TABLE 2 Complications of vitamin and mineral deficiencies.
electrolyte additives may be needed. Deficiency Oral repletion
Hyponatremia, hypokalemia, and hypophosphate- Vitamin complications dosing
mia are common occurrences in the patient with SBS, Vitamin A Night blindness 5000–50,000 IU/day
and magnesium deficiency is a particularly common
Vitamin D Osteoporosis <12 ng/ml: 50,000 IU/
and challenging complication in the patient with SBS
Osteomalacia week × 6–8 weeks
and can affect several bodily systems, including Secondary followed by
neuromuscular and cardiovascular. Although magne- hyperparathyroidism maintenance
sium absorption occurs throughout the small intestine, Hypophosphatemia 12–20 ng/ml:
most absorption occurs in the distal small bowel.24 Hypocalcemia 800–1000 IU/day
Because of this, hypomagnesemia may be more 20–30 ng/ml:
difficult to manage in patients with distal small bowel 600–800 IU/day
resections, particularly in those patients with end‐ Vitamin E Neuromuscular 400 IU up to 3×/day
jejunostomies. In addition, free fatty acids, often a disorders
result of fat maldigestion and/or bacterial carbohy- Hemolytic anemia
drate fermentation, can bind magnesium and interfere Vitamin K Bruising 10‐mg single dose, can
with absorption.25 Oral supplementation may aggra- Mucosal bleeding be repeated
vate or cause diarrhea and may be insufficient to Hematuria 48–72 h later
achieve normal serum levels. There are some magne- Vitamin B12 Peripheral neuropathy 300–1000 mcg/month
sium preparations that may be better tolerated in this Spinal cord neuropathy Should be
patient population, such as magnesium bisglycinate or (loss of administered via
magnesium pidolate, but their availability is limited. proprioception, sublingual,
For patients whose serum magnesium levels are ataxic gait) subcutaneous, or
Dementia intramuscular
persistently <1.0 mg/dl or who are symptomatic,
Atrophic glossitis route
parenteral replacement may be needed. Of note,
patients with impaired kidney function are at risk for Copper Myeloneuropathy 2 mg of elemental
Skin depigmentation copper per day
developing hypermagnesemia when replacement is
Anemia Higher doses may be
provided in large doses, as magnesium is renally Neutropenia needed
cleared. These patients require close monitoring until Pancytopenia IV formulation
magnesium levels stabilize. Cognitive deficits available
Iron Anemia 100–200 mg/day or
every other day
V I T A M I N AN D T R A C E E L E M E N T IV formulation
DEFICIENCIES available
Selenium Skeletal muscle 100–200 mcg/day
Vitamin absorption in patients with SBS is largely dysfunction
influenced by remnant anatomy, although other Cardiomyopathy
factors such as medications, diet, use of PN, and Mood disorders
disease states such as inflammatory bowel disease with Impaired immune
active inflammation also contribute to malabsorption function
and vitamin deficiency. Because the proximal small Zinc Impotence 50‐mg elemental zing
bowel typically remains intact, water‐soluble vitamin Hypogonadism 1–2×/day
deficiencies are relatively uncommon. An exception to Alopecia Dietary sources such
this includes patients with terminal ileal resection Dysgeusia as oysters and
Immune dysfunction mussels can also
longer than 60 cm, as they will likely require lifelong
Impaired wound healing be considered
vitamin B12 supplementation.26 In contrast, deficien- Herpetiform rash
cies in fat‐soluble vitamins (ie, A, D, E, K) and
Abbreviation: IV, intravenous.
essential fatty acids are common in the patient with
SBS. These deficiencies can lead to night blindness,
osteomalacia, neurologic disorders, coagulopathy, poor Trace element deficiencies are also common in
wound healing, and dermatitis, thus making monitor- patients with SBS, although they are usually clinically
ing and replacement a key part of the care of these silent. In patients whose proximal small bowel is
patients (Table 2).27 bypassed, such as in patients with proximal resection,
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S50 | BERING and DIBAISE

fistula, or certain intestinal bypass anatomies, however, output promotes oxalate stone formation, and over
clinically symptomatic deficiencies may occur. Zinc, time, a large stone burden can lead to progressive
copper, and selenium deficiency occur commonly in renal dysfunction. Rarely, progression to a systemic
patients with increased gastrointestinal losses. Although process (ie, oxalosis) may occur, in which oxalate
iron is absorbed in the proximal small bowel, which crystals deposit in other tissues of the body, including
remains intact in most patients with SBS, iron deficiency the bone marrow, leading to further complications.30
is one of the most common deficiencies, particularly in Patients with SBS, including those without a colon,
those patients who require chronic PN, as it does not are also at risk of developing uric acid stones, mostly
contain iron, owing to its incompatibility with lipids and as a consequence of chronic volume depletion and
propensity to cause allergic reactions.28 bicarbonate loss.34
Periodic monitoring for vitamin and micronutrient Although nephrolithiasis may be asymptomatic,
deficiencies is recommended for patients with SBS. classical colicky flank pain, hematuria, nausea, and
Patients frequently require micronutrient supplemen- vomiting may occur. Urinalysis with crystal testing,
tation, and often with doses above the dietary specific gravity, pH, leukocyte measurements, and
reference intakes, to maintain normal serum levels.19 blood detection can also provide information regarding
Assessment of vitamin and mineral levels is typically the patient's hydration status, stone presence/type, and
accomplished via serum laboratory monitoring. 20 whether concomitant infection is present. Urine
Baseline vitamin and trace element levels— cultures that grow urease‐positive organisms may
including zinc, selenium, vitamins A/D/E, vitamin indicate the presence of staghorn calculi.35 Radiologic
B12 , folate, iron, and copper—should be established, studies can help confirm the diagnosis with noncon-
and deficiencies should be replaced with supplemen- trast computed tomography scanning as the preferred
tation. Note that chronic oral zinc supplementation test.36
can compete with absorption of copper, leading to Specific factors of stone size, type, location, and
deficiencies. Recommended monitoring intervals presence of symptoms or hydronephrosis can help
range from every 3 to 12 months and can be determine the optimal management of nephrolithiasis.
determined based on whether existing deficiencies Preventive measures are grounded in fluid and dietary
are present. 29 In patients who require repletion of fat‐ alterations. In general, increased fluid intake to the target
soluble vitamins, oral formulations should be taken daily urine volume of 2 L can help ensure adequate
with a meal or snack that includes dietary fat to hydration and prevent kidney stone formation.37 Dietary
facilitate absorption. Collaboration with a registered oxalate and fat restriction for patients who have their
dietitian is recommended for assistance with supple- colon‐in‐continuity is also recommended, although
ment dosing recommendations. adherence can be challenging. Calcium and/or potassium
citrate supplementation is also commonly used to
prevent recurrent stones.38 A 24 h urine test for stone
NE P HR OL I TH I A S I S risk factors is recommended by the American Urologic
Association both at time of initial stone presentation and
Both calcium oxalate and uric acid renal stones are for long‐term monitoring after therapies and stone
common in patients with SBS. Oxalate stones have prevention strategies are in place to confirm treatment
been reported to occur in up to 25% of patients with adherence.38
SBS and a retained colon at a median of 30 months Microbiome modification targeting Oxalobacter for-
after bowel resection. Those patients with colon and migenes to increase oxalate destruction is currently under
ileal resections are more prone to uric acid stone investigation.39 Limited data suggest that the use of bile
development. 30–32 Patients with SBS who have fat acid sequestrants to bind oxalate may reduce urinary
malabsorption and their colon‐in‐continuity are at oxalate excretion; however, additional investigation is
increased risk for developing oxalate stones, which, if needed before definitive recommendations can be
not controlled, can progress to oxalate nephropathy, made.40,41 Bile acid binders, however, are not recom-
including chronic kidney disease and occasionally mended in patients with SBS because they may worsen
dialysis‐requiring end‐stage renal disease. In these fat malabsorption. Ureteroscopic or surgical interven-
patients with steatorrhea, calcium that would nor- tions may be needed to manage urolithiasis, depending
mally bind to oxalate instead binds to free fatty acids, on the stone size and burden. Finally, combined kidney
leaving oxalate free to be absorbed in the colon.33 and intestinal transplantation has been described as a
Oxalate then enters the bloodstream and is filtered by limited treatment option for patients who have developed
the kidneys. Volume depletion and reduced urine enteric hyperoxaluria and renal failure.
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NUTRITION IN CLINICAL PRACTICE | S51

METABOLIC BON E DIS EAS E lead to supersaturation of cholesterol within the bile.
This, in conjunction with gallbladder stasis due to
Metabolic bone disease encompasses a range of disease reduced oral intake, may lead to the development of
states, including osteopenia, osteoporosis, and osteo- biliary sludge and stone formation.3 Hormonal changes,
malacia. Patients with SBS often have several risk factors including decreased production of cholecystokinin, may
for developing metabolic bone disease, such as low body also play a role in cholelithiasis development.49 In
mass index, vitamin and mineral deficiencies (calcium, addition, patients who require chronic PN or certain
magnesium, vitamin D, phosphorous), corticosteroid use, medications such as octreotide are also at increased risk
and chronic metabolic acidosis.42 In one study, the of biliary disease.50,51 It seems likely that a combination
prevalence of osteoporosis in patients with SBS was of factors contributes to the increased risk of gallstone
56.9% compared with 24.2% of patients in the control formation seen in patients with SBS, and providers need
group.42 Patients who develop decreased bone strength to have a high index of suspicion when patients present
are at increased risk for fractures and fracture‐related with symptoms that are suggestive of biliary disease.
complications such as thromboembolism, infection, Oral or enteric nutrition when feasible, even in those
mobility limitations, and in certain cases, increased patients who are PN‐dependent, may help to reduce the
mortality.43 development of sludge and gallstones,52 at least in part,
When evaluating patients for metabolic bone disease, by stimulating natural production of cholecystokinin,
the American Association for Endocrinology recom- which improves gallbladder motility and reduces bile
mends obtaining a detailed history, physical examina- stasis. Ursodeoxycholic acid may help prevent gallstones
tion, and clinical fracture risk with a fracture risk by reducing lithogenic bile, although this has not been
assessment tool.44 Bone mineral density testing should studied in patients with SBS.53 Because of the increased
be obtained with dual‐energy x‐ray absorptiometry. risk of cholelithiasis and subsequent complications, it has
Recommended laboratory studies include 25‐hydroxy been proposed that asymptomatic patients with SBS and
vitamin D, magnesium, phosphorous, calcium, para- gallstones should be considered for prophylactic chole-
thyroid hormone, and thyroid‐stimulating hormone. cystectomy, particularly when a surgical procedure is
Patients who meet criteria for osteoporosis, who have already being planned for another reason.48,54,55
osteopenia and a history of fragility fracture, or have high
risk of fracture based on their fracture risk assessment
tool scores should be considered for treatment.44 Several INTESTINAL FAILURE–ASSOCIATED
medications are approved for osteoporosis treatment, LIVER DISEASE
including IV formulations, which are the preferred route
in this patient population given the poor bioavailability of Intestinal failure–associated liver disease (IFALD)
the oral forms. Additionally, the PN formulation should reflects a wide range of pathology that can be considered
be optimized so as not to worsen metabolic bone disease under the IFALD umbrella, including minor transient
by ensuring that the calcium, phosphate, magnesium, liver test abnormalities, steatosis, cholestasis, and cirrho-
and acid‐base status are normalized.45 sis. It is a diagnosis of exclusion, with the greatest risk in
those with a small bowel segment <50 cm, lack of colon‐
in‐continuity, absence of oral/enteral intake, presence of
BIL I ARY DISEAS E intra‐abdominal inflammation, recurrent CVC‐related
sepsis, small intestinal bacterial overgrowth (SIBO), and
Patients with SBS have an increased risk of developing PN‐related factors (Table 3).56 Diagnosis includes a
biliary disease, including gallbladder sludge and choles- combination of biochemical, clinical, and in some cases,
terol gallstone formation.46 The reported prevalence of histologic findings and is considered after excluding
cholelithiasis in these patients varies widely with early other causes of liver dysfunction. It is important to
studies, suggesting that up to 100% of patients with SBS recognize that an elevation in liver tests is not a
will develop gallstones, especially in the setting of mandatory criterion for diagnosis, as some patients can
chronic PN.47 More recent studies with larger participant develop advanced fibrosis with normal or near normal
cohorts, however, estimate the prevalence to be 20%–30% liver enzymes.57
of patients with SBS.46,48 Whereas IFALD can rapidly develop in pediatric
Although the pathophysiology has not been com- patients, it is generally slowly progressive in adults.
pletely elucidated, several hypotheses have been pro- Several preventive and treatment strategies exist to help
posed. Altered bile composition and loss of bile salts in manage patients at risk or who have developed IFALD.
those patients who have undergone ileal resection may These include PN formula optimization, encouraging
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S52 | BERING and DIBAISE

TABLE 3 Risk factors for intestinal failure–associated liver disease.

Patient risk factors PN risk factors
• Small bowel segment <50 cm • Type and amount of IV lipid emulsion
• Colon left in discontinuity or • Overfeeding
resected • Duration of PN
• Absence of oral or enteral intake
• Intra‐abdominal inflammation
• Recurrent CRBSI or recurrent sepsis
• Small intestinal bacterial overgrowth
Abbreviations: CRBSI, catheter‐related bloodstream infection; IV, intravenous; PN, parenteral nutrition.

TABLE 4 Common intravenous lipid emulsions and their the development of SIBO, including slowed intestinal
content. transit and dilatation of the bowel. These changes may
Oil content Allergy result in ineffective removal of bacteria from the small
Lipid emulsion percentages cautions bowel.63 In addition, those patients with SBS who lack an
Intralipid SO 100% ileocecal valve may be at risk for retrograde movement of
colonic contents leading to small bowel colonization of
Omegaven FO 100% Fish, egg
colonic bacteria. Finally, the use of PPIs, often adminis-
Clinolipid SO 20% Egg, soybean, tered to patients with SBS to help control dyspeptic
OO 80% peanuta
symptoms and stool output, may also increase the risk of
SO, MCT, OO, SO 30% Coconut, egg, SIBO via reduction in gastric acid secretion.64
FO‐lipid MCTs 30% soybean, SIBO may cause a variety of upper and lower
OO 25% peanut,a fish gastrointestinal symptoms, leading to a reduction in oral
FO 15%
intake.62 In severe cases, vitamin B12 deficiency may
Abbreviations: FO, fish oil; MCT, medium‐chain triglyceride; OO, olive oil; develop. Although SIBO may have potential benefit to the
SO, soybean oil.
a
patient with SBS in terms of increasing energy extraction
There is potential for primary sensitization or cross‐reactivity between
from malabsorbed carbohydrate, it can also induce
peanuts and soybeans. Thus, peanut allergy is also included here as a
potential allergy precaution. inflammatory and atrophic changes in the gut, impairing
absorption; deconjugate bile acids, resulting in fat mal-
digestion; and potentially increase the risk of IFALD, CVC
oral intake/enteral stimulation, normalizing micronutri- infections, and chronic gastrointestinal bleeding.
ents, avoiding hepatotoxic medications, and cycling the The diagnosis of SIBO using breath testing in the patient
delivery of PN.58 In addition, using non–soy‐based lipid with SBS is problematic and not recommended because
emulsions and limiting the amount of IV lipid to rapid transit in the shortened bowel makes it difficult to
<1 g/kg/day can reduce or prevent hepatic injury differentiate small bowel vs colonic hydrogen produc-
(Table 4). Soybean oil, a lipid emulsion base that has tion.62,63 As a result, diagnosis using the small bowel
been used for decades, is high in plant phytosterol aspirate, whenever possible and despite its limitations, is
content thought to accumulate in the liver and interfere preferred.62,63 Because of the limitations of the diagnostic
with bile acid synthesis, driving IFALD.59 Use of lipid tests, empiric treatment is often provided. Antimicrobial
emulsions with a lower proportion of soybean oil may agents are generally used to treat SIBO62; a variety of oral
reduce the risk of developing hepatic injury related to PN broad‐spectrum antibiotics can be used, with success being
administration and, in some cases, reverse existing judged on improvement in symptoms, reduction in stool
IFALD.60,61 For patients who develop liver failure in output, and/or weight gain. The continuous use of low‐dose
the setting of SBS–intestinal failure, combined intestine‐ antibiotics in SBS may be necessary. To reduce the risk of
liver transplantation may be needed.57 antibiotic resistance, periodic rotation of the antibiotic used
or use of a poorly absorbable antibiotic has been advised;
however, evidence from controlled studies to support this
SIBO practice is lacking. Other strategies suggested to be useful in
some patients but lacking strong evidence include limiting
SIBO is a clinical syndrome characterized by excessive antisecretory and antimotility medication use, carbohydrate
numbers of bacteria in the small bowel.62 Patients with restriction, intermittent bowel flushing with a laxative, and
SBS may have several risk factors predisposing them to bowel‐tapering operations.63
 19412452, 2023, S1, Downloaded from https://aspenjournals.onlinelibrary.wiley.com/doi/10.1002/ncp.10978 by Cochrane Romania, Wiley Online Library on [16/11/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
NUTRITION IN CLINICAL PRACTICE | S53

D ‐ LACTIC AC IDOS IS Infection

D‐Lactic acidosis is a rare neurologic complication of SBS Infection is the most common complication of long‐term
that can lead to symptoms of altered mental status, CVCs, with the incidence of catheter‐related bloodstream
slurred speech, psychosis, and even coma.1 It appears to infection (CRBSI) estimated at 4.1 infections per 1000
be more common in children with SBS than in adults. central‐line days.67 PN administration is independently
Symptoms are often precipitated by intake of carbohy- associated with an increased risk of bacteremia when
drates, which are then fermented by colonic bacteria, compared with enteral or oral routes of nutrition.68,69
leading to the production of D‐lactate. D‐Lactate is then Proper catheter insertion and care are of crucial
absorbed into the blood, leading to the development of importance to prevent contamination of the catheter
metabolic acidosis and neurologic alterations.65 hub, as migration of a skin organism into the catheter
The diagnosis of D‐lactic acidosis can be challenging, tract can lead to the development of bacteremia.70 As
and a high index of suspicion is needed. Laboratory such, patients and their caregivers should receive
assays are designed to detect L‐lactate levels. As such, education and training on how to care for CVCs. Other
measurement of D‐lactate concentration requires a factors that have been implicated in increasing the
separate test that may not be readily available. Thus, infection risk include the catheter tip location, frequency
diagnosis requires a high index of clinical suspicion. of catheter access, larger catheter caliber, absence of a
Treatment involves administration of antibiotics with cuff, and increased number of catheter lumens.70 The
activity against D‐lactate production bacteria, including expertise of the care team and the use of standardized
metronidazole, vancomycin, neomycin, clindamycin, and protocols significantly reduce the incidence of infec-
tetracycline.1 Patients should also restrict carbohydrate tions.71 A CRBSI should be suspected when a patient
intake. Other supportive therapies including hydration, develops symptoms such as fever, chills, malaise, pain, or
IV bicarbonate for treatment of acidosis, and thiamin tachycardia, especially while using the CVC. Localizing
supplementation in the acute setting.65 symptoms may not be present at infection onset, but
rather, general symptoms of fever, malaise, and abnor-
mal laboratory findings such as new or worsening liver
C V C‐ RELATED C OMPLICATIONS test abnormalities and leukocytosis may be the initial
signs of infection in these patients.72 Early diagnosis is
Patients with SBS often require chronic home PN important to prevent more serious sequelae of infection.
support. Home PN necessitates long‐term central venous Diagnosis of a CRBSI requires simultaneous blood
access for administration. Central venous access devices cultures obtained from a peripheral site as well as from
carry their own risk of complications, including infec- the CVC. Blood cultures should be obtained before
tion, thrombosis, malfunction, breakage, and dis- starting empiric antimicrobial therapy whenever possible
lodgement66 (Table 5). so that accurate diagnosis and pathogen identification

TABLE 5 Central venous catheters.

Type of catheter Duration Pros Cons
Peripherally inserted Short‐term to • Ease of insertion and removal • May have an increased risk of thrombosis
central catheter midterm • Cost‐effectiveness and displacement
• Accessibility • Patient does not have both hands available
to help access/manage the line
Subcutaneous port Long‐term • Low risk of infection • Requires surgical placement and removal
• Easier site care • Requires a needle to access the port,
• Patient comfort limiting use in patients requiring daily line
access
Tunneled catheter Long‐term • Low risk of infection compared • Requires surgical insertion
with nontunneled
• Patient has both hands available
to help access/manage the line
Nontunneled catheter Short‐term • Ease of insertion (can be done at • Higher rate of infection
bedside) • Patient discomfort
Source: Adapted with permission from Bering and DiBaise.66
 19412452, 2023, S1, Downloaded from https://aspenjournals.onlinelibrary.wiley.com/doi/10.1002/ncp.10978 by Cochrane Romania, Wiley Online Library on [16/11/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
S54 | BERING and DIBAISE

can be achieved. If a CRBSI is confirmed, targeted staphylococci and are lowest for infections from Staphylo-
antibiotics should be initiated and consideration for coccus aureus and polymicrobial infections.76 Salvage
infectious disease consultation may be needed to help should not be attempted in the setting of fungal infections,
direct duration of therapy and guide whether the CVC most gram‐negative infections, or critically ill patients.73 If
should be removed. Figure 1 illustrates an approach to line salvage is pursued, infusion of targeted systemic
managing CRBSI.73 antibiotics should occur through the contaminated line as
Patients who require long‐term PN can develop part of the salvage strategy. Antibiotic lock therapy,
challenges to vascular access over time. With each catheter ethanol lock therapy, and taurolidine lock therapy, when
removal and insertion, the number of viable veins is available, may be viable options for catheter salvage and/or
threatened.74 As a consequence, CVC salvage therapy prevention of future CRBSI.77–79
should be attempted depending on the severity of the Other types of infectious complications that can
clinical presentation and the culprit organism and involve CVCs include catheter colonization, tunnel
therapeutic susceptibilities.75 Salvage success rates are infections, and exit‐site infections. Exit‐site infections
higher when treating infections due to coagulase‐negative typically present with erythema, pain, and drainage at

F I G U R E 1 Algorithmic approach to the management of catheter‐related bloodstream infection. AC, arterial catheter; CFU, colony‐
forming unit; CVC, central venous catheter; IDSA, Infectious Diseases Society of America. Adapted with permission of the Infectious
Diseases Society of America from Mermel et al.73
 19412452, 2023, S1, Downloaded from https://aspenjournals.onlinelibrary.wiley.com/doi/10.1002/ncp.10978 by Cochrane Romania, Wiley Online Library on [16/11/2023]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
NUTRITION IN CLINICAL PRACTICE | S55

the catheter exit site and may respond to topical approach has been shown to have an overall catheter
therapies. Culture of the exudate can be obtained via clearance of 90%.84
swab with subsequent tailoring of antimicrobial therapy.
CVC removal is generally not necessary. In contrast,
tunnel infections, which are characterized by pain and Thrombosis
erythema along the tunnel tract, are best treated with
line removal and systemic antibiotic therapy.80 The incidence of CVC‐associated deep vein thrombosis is
estimated to be 14%–18%.85 Risk factors for thrombus
formation are found in Table 6. If thrombus has occurred
Occlusion without an obvious precipitant, it is recommended that
patients be investigated for hypercoagulability. When
Catheter occlusion may be partial or complete, impacting thrombosis is suspected, duplex ultrasonography should
normal function of the catheter. Catheter occlusion may be employed for diagnostic evaluation. Venography is an
present with difficulty flushing or infusing, slow infusion alternative diagnostic option and may be best employed
rate, and/or inability to draw blood from the CVC. when suspicion remains high in the setting of a negative
Occlusion may be related to a thrombotic or nonthrom- ultrasound evaluation.
botic process, and management may differ depending on Initiation of anticoagulation is usually recommended
the origin of the blockage. for catheter‐associated thrombus and may require
Nonthrombotic sources of catheter occlusion include parenteral administration of low‐molecular‐weight hepa-
mechanical obstruction, drug precipitation, and lipid rin, as the bioavailability of oral anticoagulants in
residue deposits.81,82 Pinching of the catheter between a patients with SBS is unclear.72 Thrombus formation does
patient's clavicle and first rib may be suspected if CVC not necessitate CVC removal if there is no associated
occlusion is position‐dependent and can be investigated infection, the catheter is functional and well positioned,
with radiologic imaging. Various solutions may help to and ongoing use of the catheter is needed.86 Expert
clear deposits from the line, including 0.1 N hydrochloric consensus suggests that anticoagulation should be
acid for drug/mineral deposits, sodium bicarbonate for administered for a minimum of 3 months with consider-
acidic precipitants, or 70% ethanol for lipid deposits.72 ation for subsequent prophylaxis if the line remains
Proper flushing techniques are crucial in preventing in situ.85
occlusion.
Thrombotic complications related to CVCs may also
cause catheter occlusion. If occlusion of the line occurs Catheter damage
because of a thrombotic process, such as fibrin sheath or
intraluminal clot, alteplase can be instilled within the Damage or deterioration of the CVC may occur over time
catheter and left to dwell for up to 120 min. This can be with regular use. Catheter damage can also occur when
repeated if needed to attempt to salvage the line.83 This patients use high‐pressure flushing against an occluded

TABLE 6 Risk factors for catheter‐related thrombosis.

Risk factor Notes
Catheter type PICC carries greater risk than tunneled or implanted lines
Insertion site Femoral carries greater risk than other sites
Catheter tip location proximal to the superior vena cava —
Larger catheter diameter —
Catheter material Polyethylene chloride and polyvinyl chloride carry higher risk than
silicone or third‐generation polyurethane
Hypercoagulable state For example, malignancy, sepsis, critical illness, thrombophilia
Presence of a CVC infection —
Patient history of venous thromboembolism —
Concomitant use of certain medications For example, oral contraceptive or hormone replacement therapies
Abbreviations: —, none; CVC, central venous catheter; PICC, peripherally inserted central catheter.
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S56 | BERING and DIBAISE

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Jamie Bering and John K. DiBaise equally contributed to 15. Sainath NN, Bales C, Brownell JN, Pickett‐Blakely O, Sattar A,
the conception and design of the work and drafted the Stallings VA. Impact of pancreatic enzymes on enteral fat and
manuscript. All authors critically revised the manuscript, nitrogen absorption in short bowel syndrome. J Pediatr
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17. Jeppesen PB, Gilroy R, Pertkiewicz M, Allard JP, Messing B,
CONFLI CT OF I NTER EST STATEMENT
O'Keefe SJ. Randomised placebo‐controlled trial of teduglutide
The authors declare no conflict of interest. in reducing parenteral nutrition and/or intravenous fluid
requirements in patients with short bowel syndrome. Gut.
ORCID 2011;60(7):902‐914.
Jamie Bering http://orcid.org/0000-0001-6161-7088 18. Schwartz LK, O'Keefe SJD, Fujioka K, et al. Long‐term
John K. DiBaise http://orcid.org/0000-0001-6985-5953 teduglutide for the treatment of patients with intestinal
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