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BC 2019 R.Wiest 1

Klinik für Viszerale Chirurgie und Medizin

Short-Bowel-Syndrome

Reiner Wiest M.D.

Michele Leuenberger, M.D.

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Content – Outline – Topics - Highlights

Definition

Epidemiology

Etiology, Physiology

Classification-systems

Clinical, diagnostic Issues

Therapy

…………..

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Small intestine: normal length ?

Definition of short bowel syndrome – SBS

Small intestine: 275 cm – 850 cm normal range

shorter in women and hence SBS more frequent in women (67%)

< 200 cm remaining functional short bowel

usually results in short bowel syndrome:

Bryant 1924 ESPEN Clinical Nutrition 2015

SBS is a malabsorptive condition characterized by the inability

to maintain protein-energy, fluid, electrolyte or micronutrient balances

when on a conventionally accepted, normal diet.

SBS is the main mechanism (accounting for 75%) of chronic intestinal failure (IF)

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Definition of intestinal failure ?

reduction in gastrointestinal function

below the minimum necessary for the absorption of

macornutrients a/o water and electrolytes such that

intravenous supplementation is required

to maintain health/growth

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Epidemiology, incidence, prevalence of SBS

True incidence is not known (in europe, switzerland)

Neonatal incidence overall 24.5 per 100.000 live birth

Adults: 9/1.ooo.ooo/year 1

:

Prevalence HPE: 5-80/1Mio; Incidence 7.7-15 /1Mio/year

50-70% can be weaned off from HPE long-term, but

Case fatality rate up to 30% as well1Lal: Aliment Pharmocol Therapeut 20062Clin Nutr 2018;37:1798-1809.

DiBaise JK et al. Am J Gastroenterol 2004;99:1386-95

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Most common causes of short bowel syndrome

pathophysiological groups –

Short-bowel:

Parenchymal disease

Fistula

Motility disorder

Mechanical Obstruction

Malabsorption due to

=reduced absorptive

/functional mucosal surface

Feed related exacerbation of symptoms

=restriction of oral/enteral nutrition

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examples for parenchymal/ SBS

Short-bowel: 75% in adults Parenchymal disease (5%):

Extensive surgical resection for:

Mesenteric infarction/thrombosis

Crohn's disease

Surgical complications

Intestinal volvulus, Intussusception

Familial polyposis

Abdominal trauma

…….

Radiation enteritis

Coeliac disease

Immunodeficiency

Crohn's disease

Lymphangectasia

Radiation enteritis

Tufting enteropathy

Autoimmune enteropathy

Microvillus atrophy

Others

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examples for motility/obstruction-group

Motility disorder (20%): Mechanical obstruction:

Acute: critical illness due to post-operative,

systemic inflammatory, neurological reactions…..

acute colonic non-mechanical obstruction

Chronic Intestinal Pseudo-Obstruction

Primary/idiopathic (with no underlying disorder)

Neuropathic: inflammatory or degenerative

Myopathic: damage of the smooth muscle

familial visceral myopathy mostly type 3

Endocrine: diabetes, HPT, ….

Medications…

«frozen» abdomen

«bowel rest» due to

Obturation

Tumors, intussusception,

Gallstones, foreign bodies, …

Intrinsic bowel lesions

stenosis or strictures:

- neoplastic,

- inflammatory bowel disease

- chemical,

- anastomotic

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High-output fistula is defined as ?

> 500ml /day in fasted condition via fistula

Fistula (2-5%):

Exogenous aetiology: (75-85%)

Post-surgically

Endogenous etiology (15-25%)

crohn^s disease, irradiation, malignancy……

Fistula – groups and examples ?

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Pediatric/children special causes, e.g. congenital….

Congenital defects of glycosylation, primary bile acid malabsorption,

Glucose-galactose malabsorption,

Gastroschisis

Nectrotizing enteritis

Midgut volvolus

Intestinal atresia

Microvillous inclusion disease and intestinal

Epithelial dysplasia

………………………………………

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FKl 11

GI-Function:

Secretion +

Absorption

how much +

where ? 78%

21%

1%Secretion

9000ml/dResorption

8700ml/d

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Knowledge test on where and what……is absorbed

Fe, Mg, Zn, P

Ca, Folic acid

Glucose

Fructose

Galactose

Min

era

ls

Carb

oh

yd

rate

s

Electrolytes (Na,K),

Amino-acids, glucose,

watersoluble vitamins,

Trace elements,

Micronutrients

Vitamin B12

Bile acids

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Absorptive Functions

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Ileum: different from Jejunum also as for ………. ?

Inter-cellular junctions:

Water + sodium flux:

Fluid reabsorption:

Capacity for adaptation:

Tighter than in upper GI-tract

less para-cellular permeability

Less flux than in upper GI-tract

Substantially higher – concentrating content

also due to active NaCl-transport

Greater than in upper GI-tract

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What about prognosis: dependency on

remaining length small intestine –

5y survival for < 50 and > 150 cm

124 adult patients with non-malignant SBS

93%

79%

57%

Messing B. Gastroenterology 1999;117:1043–1050

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Factors other than remaining length small intestine and

site affected determining need for parenteral nutrition ?

Secretion:

Bile acid, gastric,

duodenal, pancreatic

secretion

Presence

of colon

Ileal valve

brake ?

State of other

digestive organs

Age/BMI of the

patient

Efficacy of nutrition

support

Pharmacologic

therapy

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What is the «ileal brake» and

how does it work ?

Peptide YY (and Enteroglukagon, Neurotensin)

released from L-cells in distal ileum

do slow gastric emptying and tansit-time

Loss of distal ileum

= loss of those hormones

= loss of feedback inhibition

-> transit-time faster, loss of fluids and nutrients bigger

-> colonic bacteria can colonize small intestine,

worsening diarrhoe and nutrient loss

Intact valve:

Chyme more time in

Small intestine =

Contact with

aborptive mucosa

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Role and function of colon – compared to small

intestine ?

Colon main function:

absorption of H2O, electrolytes (Na, Mg, Ca, K), MCT

Compared to jejunum and ileum

Slowest transit

tightest intercelluar junctions

Greatest efficiency of

water + sodium absorption

capable of absorbing

fermented carbohydrates

In healthy adults

1-1.5 L of fluid

enters colon

each day, all but

150 ml is

reabsorbed

helps to slow transit and

stimulate intestinal adaption

(increase in GLP-1 and peptide YY)

How much fluid and energy

via colon absorbable ?

If the colon is

present, it can

absorb up to 6 L of

excess fluid each

day in patients with

SBS

In patients with SBS on

a high-carbohydrate

diet, the colon can

absorb up to 50% of

energy requirements

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Increasing colonic water

absorption as much as 5-

times ist normal capacity

Resident bacteria capacity

to metabolize undigested

CHO into SCFA- which are

preferred fuel source for

coloncytes & body

Increasing incidence of

urinary calcium oxalate stone

formation

Small intestinal

bacterial overgrowth

Po

sit

ive

Ne

ga

tive

Positive/negative effects if colon is preserved ?

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Uncommon sites of resection in SBS

Resection typically results in

–Hypergastrinemia and

–Gastric hypersecretion

–Decreased pancreatic and biliary secretions

–This causes

» Deficiencies in certain micronutrients

(including iron and folate)

» Peptic complications

» Impairment in the function of

digestive enzymes (low pH)

e.g. pancreas-lipase

e.g. bile-acid deconjug.

worsening

malabsorption

Negative feedback for

inhibiting gastrin secretion

and reducing gastric acid

production is removed

Loss of CCK synthesis

(CCK stimulates

pancreatic and biliary

secretions)

Consequences of duodenal resection ?

Lack of mineral-

absorption -> ?

Anemia

Osteoporosis

Absorption adequate if < 75% resected

Normal absorption Vitamin B12 + bile acids

Transit-time normal, Ileum adapts well

Usually no home parenteral nutrition needed

Jeejeebhoy KN. CMAJ 2002;166:1297-1302

Consequences of jejunal resection ?

Sekretion Cholecytokinin, Sekretin

Kontraktion der Gallenblase = Cholelithiasis

Pankreassekretion

Gastrin , Magensäure, pH Dünndarm

Peptische Ulcera; Verdauungsleistung

Lactase-deficiency,……………..

Jejunum less capacity to adapt

Resection > 25cm: chologenic diarrhoe

Resection > 50 cm Vitamin B12-Malabsorption with

inadequate energy- and fluid-absorption

> 100cm: Steatorrhoe (Vitamines A,D,E,K!)

Bacterial overgrowth

Messing B. Gastroenterol. 1999; 117:1043-1059

Consequences of

ileum resection ?

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Kruis W. Dt Ärzteblatt 1982

Compensated vs. Decompensated BA-loss-syndrome

3-10-times increased

Hepatic BA-synthesis

Speeds motility

Hyperpermeability

Mucus/Na-Cl-secretion…

BA-loss cannot be compensated

By the liver, luminal BA-conc

Degrades ->

Fat-malabsorption-> steatorrhoe

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How do bile acides cause diarrhoe ?

Colonic microbiome deconjugates BA to secondary BA e.g. DCA, LCA

Particularly dihydroxylated BA chenodeoxycholate and deoxycholate

stimulate electrolyte and water secretion

Cholestyramine: when to use and/or not ?

Good response in compensated BA-loss-syndrome

Contraindicated in decompensated syndrome and

No effect/do-not-do it if no colon is preserved

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Clinical consequences of interrupted enterohepatic

circulation ……

Cholagenic Diarrhoe

Bile-acid-deficit

Fat-malabsorption ↓

Steatorrhoe

Lithogenic bile

Gallstones

ADEK ↓

Hypocalcemia

Osteoporosis

Bleeding

Kidney stones

Malnutrition !!!

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What is «functional classification» of intestinal

failure ?

On basis of onset, metabolic and expected outcome criteria

Type I acute, short-term and usually self limiting condition

Type II prolonged acute condition, often in metabolically unstable

patients, requiring complex multi-disciplinary care and

intravenous supplementation over periods of weeks or months

Type III chronic condition, in metabolically stable patients,

requiring intravenous supplementation over months or years.

It may be reversible or irreversible

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Clinical classification of short-bowel ?

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Three types of short bowel syndrome ?

Critical length of remaining segment ?

Jejuno-ileal

-anastomosisJejuno-colic

-anastomosis

End-Jejunos-

tomy

no colon in continuity

resection entire ileum +

colon or

colon present but

disconnected

ileo-cecal valve + entire

colon in continuity

no ileo-cecal valve

part of colon in continuity

Critical

length

< 35 cm

Critical

length

< 60 cm

Critical

length

< 115 cm

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Three types of short bowel syndrome ?

Rank according to severity, loss, level….?

Jejuno-ileal

-anastomosisJejuno-colic

-anastomosis

End-Jejunos-

tomyCritical

length

< 35 cm

Critical

length

< 60 cm

Critical

length

< 115 cm

Transit-time

Adaptation-

capacity

Post-

prandial

…GLP-2

….PYY

Prognosis

Transit-time - + +++

Adaptation-

capacity

Post-

prandial

…GLP-2

….PYY

Prognosis


Adaptation-

capacity

+++ + -

Post-

prandial

…GLP-2

….PYY

Prognosis


Adaptation-

capacity

+++ + -

Post-

prandial

…GLP-2

+++ + 0

….PYY

Prognosis


Adaptation-

capacity

+++ + -

Post-

prandial

…GLP-2

+++ + 0

….PYY +++ + -

Prognosis Good Fair/depends worst

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Prognosis according to anatomy/type of SBS

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Differences in Jejunocolic vs. End-jejunostomy as

for ?

Jejunum-Colon Jejunostomy

Water, Na, Mg-depletion Uncommon Common, frequent*

D-Lactacidosis Occasionally None

Renal Stones (Ca-Oxalate) 25% None

Gall Stones (pigment) 45% 45%

*: < 100 cm jejunum: losses > oral intake

Rapid sodium fluxes occur in jejunum:

if water/solutions of < 90 mmol/L Na are drunk a net

efflux of Na into the bowel lumen occurs until 100mmol/L is reached

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D-Lactat-acidosis: when ? why ? Clinical features ?

Present colon: overflow of non-absorbable, undigested carbohydrates

from the small intestine (lack of digestion) into the colon: ->

anaerobic microbial fermentation-> lowering pH->

overgrowth of acid resistant bacteria such as Lactobacill acidophilus...

D-lactate not metabolizable (vs. L-lactat->LDH/liver: pyruvat) and accumulates

Diagnostic: serum level > 3 mmol/L; high anion gap, normal L-lactate

Effects of D-lactat:

aggravation of osmotic diarrhoe, metabolic acidosis

neurologic symptoms: encephalopathy, confusion, ataxia, visual defect

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Treatment of D-lactacidosis ?

Pediatrics. 2018;

142(3):e20180337

Oral antibiotics

(vancomycin, metronidazol)

to lower anaerobic bacteria

producing D-Lactate

OR:

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Nephrolithiasis in SBS: occurs due to ?

No-nabsorbed bile acids increase colonic permeability to small molecules e.g. oxalate

Normally oxalate in diet

binds to dietary calcium

and is excreted in feces

increased fat-> FFA in colon

-> binds calcium to lime soap

-> lowering calcium conc.

-> enhanced luminal free oxalate

-> more oxalate absorbed colonic

Oxalat in kidney

is filtered

binds to calcium

-> nephrocalcinosis

-> nephrolithiasis

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Bones in short-bowel-syndrome ?

High risk of osteomalacy and osteoporosis

due to

- Immobilisation

- Acidosis

- Malabsorption (Ca,Mg)

- Vit-D-Deficit

Bone-densitometry

every 2 years

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Below which Mg-level symptomatic ? Clinical picture ?

Mostly when high-output stoma/fistula (End-Jejunostomy), but also

Chelation of Mg with unabsorbed fatty acids reduces absorption

Increased renal excretion*, e.g. secondary hyperaldosteronism

Low Mg reduces 1,25 Vit-D-synthesis-> less Mg-absorption....

PPIs are often associated with hypomagnesaemia

*: low Mg per se reduces secretion/function of PTH, -> impact on kidney

Mechanisms – Role of – Magnesium-Deficiency

< 0.6 mmol/L serum-concentration->

Depression, muscle weakness, arrythmia, convulsions…..

Treatment: hydration, low-fat-diet (when colon is preserved); Mg

supplementation (oral difficult-> i.v.), evtl. Vit-D3, -B6

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Post-resection adaptations are …..?

Structural adaptations:

macroscopic

-elongation and dilatation

microscopic

-villus: increase height, diameter

-crypt elongation

-epithelial proliferation increased

-decreased apoptosis

Functional adaptations:

slowing gastrointestinal transit

Upregulation sodium-glucose transporter

Induced / influenced by ?

GI-regulatory peptides

luminal content, nutrients,

-pancreatico-biliary secretions

Systemic factors

- growth factors, hormones,

- Cytokines

Tissue factors

- immune system, mesenchymal factors

- Mesenteric blood flow, neural influences

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Phases after surgical resection are divided in……

Stadium I Stadium II Stadium III

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THERAPY

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