Short Bowel Syndrome
by Dr. Shahanur RahmanDefinition: Short bowel syndrome (SBS) is a devastating
condition in which small intestinal length is inadequate and
characterized clinically by inability to absorb adequate enteral
nutrition to sustain normal growth and development.
For adult patients SBS has been arbitrarily defined as the
presence of less than 200 cm of residual small bowel following
massive bowel resection.
For pediatric (neonate) patients SBS can be defined when
small bowel length is <25 cm in presence of ileocaecal valve
and <40 cm without ileocaecal valve.In
Vv
adults, the most common etiologies of SBS are
Acute mesenteric ischemia,
Crohn’s disease,
Radiation enteritis,
Trauma,
Recurrent intestinal obstruction
Malignancy.
75% of cases result from resection of a large amount of
small bowel at a single operation (eg. acute mesenteric
ischemia).
25% of cases result from the cumulative effects of multiple
operations during which small intestine is resected (eg.
Crohn’s disease).Risk factors for development of SBS after
massive small bowel resection in adult
patients:
Small bowel length <200 cm
Absence of ileocecal valve
Absence of colon
Diseased remaining bowel (e.g., Crohn’s
disease)
lleal resectionIn pediatric patients, the most common
etiologies of SBS are:
Necrotizing enterocolitis, NEC (35%),
Intestinal atresia (25%).
Gastroschisis (18%).
Midgut volvulus (14%).
Long segment Hirschsprung’s disease (2%).
Meconium ileus.
True congenital short gut.based on etiology
Acquired Short Gut— Functional Short Gut —
Mesentric vascular * Lymphangiectasis.
ischaemia/accidents. * Pseudo-obstruction.
Trauma — loss of * Radiation enteritis.
intestinal vascularity.
Post-traumatic mesentric
thrombosis.
Regional enteritis.
Proximal ostomy.
Malignancy.Absorption in small intestine
* The jejunum is involved in magnesium
absorption.
* The absorption of nutrients from the food takes
place in Meum are:
amino acids (the end products of protein digestion),
fatty acids (the end products of fat
digestion),cholesterol,
sodium, potassium
alcohol, and
fat-soluble vitamins (A, D, E, and K),
Vitamin B12. (The terminal ileum is an important part
as this is where vitamin B12 is absorbed into the
blood capillaries.)Clinical Sequale of SBS
Malnutrition- pH of proximal small intestine is reduced > inactivation of
digestive enzyme > Grossly impaired absorption of all nutrients
(malabsorption).
Diarrhea- Malabsorbed fats in colon > fermented by colonic bacteria into
short chain fatty acid > diarrhoea.
Rapid transit- The lowering of intraduodenal pH stimulates peristalsis.
This phenomenon seen with proximal and midgut resection.
Loss of cholecystokinin and secretin- secondary to loss of entero-
endocrine cell mass > impaired gall bladder motility > decreased bile
and pancreatic secretion.1. Malabsorption
Decreased output of brush border disaccharidases > carbohydrate
malabsorption.
lleal resection — bile salt malabsorption > diarrhea, steatorrhea, loss of
fat soluble vitamins.
Malabsorbed fats — in colon — fermented by colonic bacteria into short
chain fatty acid —> diarrhoea.
Decreased bile acid pool which cannot be compensated with increased
hepatic synthesis.2. Bacterial overgrowth
Loss of Ileo-caecal valve — reflux of colonic bacteria
into small bowel.
Adaptive process — dilated dysfunctional loops >
bacterial overgrowth.
Bacterial overgrowth > cause of diarrhoea.
The endotoxins of the bacteria > damage mucosa,
affect motility, doconjugation of bile salts, aggravate
stasis > bacterial translocation.3. Cholelithiasis
* decreased bile salt pool.
* Poor contractility of GB.
4. Renal stone
* Hyperoxaluria , oxalate renal stones.
* Metabolism of increased carbohydrate — generates excess oxalate.
* Unabsorbed fatty acid binds calcium more than oxalate.5. D-lactic acidosis
Colonisation of bowel with lactobacilli > converts excess carbohydrate to
D-lactate via enzyme D-lactate dehydrogenase.
Metabolic acidosis > altered sensorium > coma
Mx: IV sodium bicarbonate , antibiotic therapy, interruption of enteric
feeding.Adaptation
Crypt cell proliferation: Increased villus height and overall
increase in functioning surface area for nutrient absorption.
The diameter of the bowel increases significantly.
Slow down in peristalsis or movement of food through small
intestine.
Intestine lengthens: More if proximal small bowel is resected
than distal.
After resection of jejunum, ileum adopts the morphologic
features of jejunum (Ileum adapts more than jejunum).Neonates have better adaptation than adults.
The adaptive process is effective enough to allow full enteral
nutrition even 70-80 % small bowel mass has been removed.
No survival is possible if small intestestine is < 15 cm.
When ileocaecal valve is present minimum bowel length for
survival is 15 cm with TPN support and 30 cm without TPN
support.
When ileocaecal valve is fost, for survival 40 cm of small gut
length is compatible with TPN support and 50cm without
TPN.Medical Management
Acute phase management:
Replacement of fluid and electrolytes — Ringer lacate is
preferred.
Risk of dehydration, sodium imbalance, metabolic acidosis.
Nutritional support:
After sepsis is cleared — central venous lines are put — TPN
started.
Ongoing losses calculated and added to TPN solution.
TPN given in cycles — so patient has off time from TPN.
This reduces the incidence of cholestasis.
Adequate micronutrients, fat soluble vitamins — added to TPN
solution.The use of TPN permits the survival of children who would
have otherwise died of the complications of malnutrition.
In addition, it gives the remaining bowel time to undergo
adaptation (mucosal hyperplasia, which results in increased
mucosal surface area).
However, this also is accompanied frequently by bowel
dilatation.
4 years survival rate for patients on TPN is 70%.
Complications: ran out of venous access, severe sepsis, liver
failure > death.Patient groups for whom parenteral nutrition (TPN) has been used :
A) Adult patients with SBS secondary to massive small-bowel
resection
— <60 cm with intact ileocecal valve and colon.
— <120 cm without ileocecal valve or colon .
B)Neonates with SBS
— <15 cm with intact ileocecal valve and colon.
— <40 cm without ileocecal valve or colon .Enteral Feeding:
Enteral feeding started as soon as bowel function returns.
NEC may take 3 weeks to resume normal bowel function.
Enteral feeding stimulates adaptation and decreases cholestasis.
Gradual enteral feeding started — as rapid introduction may
increase secretory diarrhoea.
Elemental formulas are started as it has easy absorption and
digestion.* Hypoallergenic formulas — hydrolysed extensively through
enzymatic process and have low antigenecity.
* Well tolerated by most children with SBS.
* Infants in comparison to adults tolerate more fat based formulas,
while
adults tolerate more carbohydrate based formulas.
* So pediatric formulas have 40-50 % calories as fats.
* Long-chain fatty acids higher calorie density and better stimulators
of adaptation but medium-chain are more easily absorbed.The carbohydrates are well absorbed except for lactose.
Ratio of carbohydrate to fat determines the osmotic load
presented to the intestine.
Carbohydrate produce high osmotic load, so formula with
modest carbohydrate content is preferred.
Small children have more difficulty with carbohydrate rich
formulas — as they have greater propensity for bacterial
overgrowth.
In older children carbohydrates are converted to short chain
fatty acids in colon which are then absorbed providing
additional calories.Protein absorption is not a problem in SBS.
But protein is provided in hydrolysed form for easier
absorption.
Feeds are initiated as dilute infusion in a continuous tube
feeding.
Reaching full concentration 0.67 cal/ml for small children and
1 cal / ml for bigger children.
Once concentration is reached — amount is increased and
parenteral fluids are decreased isocalorically every 1-3 days.Tolerance estimated — by reducing substance in stools and monitoring volume
and consistency of stool.
As fat absorption is not osmotically significant and protein malabsorption is
rarely seen - advancement feeding monitor carbohydrate malabsorption
only.
Vitamin A,D,E,K and heavy metal deficiency such as zinc, calcium, magnesium
— should be replaced.
When short term enteral feeds required or child does not require continuous
infusion — N/G feeding preferred.
Gastrostomy feed — long term feed and continuous drip feed.
Blockage of the tube is a continuous problem.
Nowadays infusion pump for bolus feeds.Control of diarrhoea
Acute phase — gastric hypersecretion — Mx by PPI.
Medications to slow intestinal transit — Loperamide (dose 1-2 mg
TDS).
If a second agent is required — Codeine (dose of 0.5 -1mg/kg/
dose TDS/QID) is added.
Use of anti-motility agents , in children without ileo-caecal valve
increases the risk of bacterial overgrowth(pain , fever, diarrhea with
foul smelling stool).
When suspected, stool and gastric aspirate taken for culture. Oral
feeding stopped. Anti-motility agents stopped. IV antibiotics ,
Metronidazole started.Management of complications
CVP lines has more incidence of sepsis compared to other conditions by
Enteric organisms.
Regular dressing in aseptic manner, flushing with urokinase and antibiotic
solution — antibiotic lock technique.
Sepsis — gram negative organisms.
After ileal resection — deficiency of Vit B12. > given parenterally every 1-3
months.
Zn, Ca, Mg — given .
Mg - given parenteral as it causes osmotic diarrhoea.
Cholelithiasis seen in 10 % of children with SBS > Use of ursodeoxy colic acid
minimises TPN associated cholestasis.uu PWN
Medical enhancement of intestinal
function
Epidermal growth factor (EGF)
. Growth hormone (GH)
. Glucagon-Like Peptide 2 (GLP-2)
. Insulin-like Growth Factor-1 (IGF-1)
. Hepatocyte Growth-Factor (HGF)1. Epidermal growth factor (EGF)
* EGF enhances cellular proliferation, differentiation and
survival.
* EGF improves carbohydrate absorption and intestinal
permeability and reduces weight loss.
* Possible Mechanisms of action include
> a reduction in apoptosis of intestinal cells,
> increased expression of the anti-apoptotic gene bcl-w, and
> decreased expression of the pro-apoptotic gene bax .
* Other factors further enhance the adaptive action of EGF such
as
> interleukin-11,
> bombesin and neurotensin.
* EGF is given orally with foods 100 ug/kg/day for six weeks.2. Growth hormone (GH)
Receptors of GH have been found throughout
the intestine.
Laboratory studies have shown that GH causes
mucosal hyperplasia and increases the
adaptive capacity after small resection.
enhancement of the villus height and crypt
depth, positive nitrogen balance and bowel
growth.
The results in adults are controversial.3.
Glucagon-Like Peptide 2 (GLP-2)
secreted by enteroendocrine L-cells of the
terminal ileum and colon.
Release of GLP-2 is stimulated by food input,
directly or indirectly promotes intestinal
growth and nutrient absorption.
Teleglutide is an analog of GLP-2 with a longer
half-life which encourages villus height and
increases crypt depth, improving nutrient
absorption, gastric emptying and body weight.. Insulin-like Growth Factor-1 (IGF-1)
secreted primarily by hepatocytes, and activated by GH.
mediates growth effects of GH and GLP-2 on the intestine.
may reinforce further growth if given shortly after bowel
resection.
may prevent mucosal atrophy, enhance gut metabolism, and
protect the intestinal barrier against sepsis.
. Hepatocyte Growth-Factor (HGF)
secreted by mesenchymal cells and carries trophic properties.
enhances small intestine growth and absorptive function
regarding carbohydrates and amino-acids.Surgical therapy
* The goals of bowel surgery in pediatric short
bowel syndrome are :
> (1) the small bowel remnant is lengthened to
potentially increase the mucosal surface area,
slow luminal transit time, and allow improved
nutrient absorption; and
> (2) the dilated small bowel is tapered so that
bowel motility is optimized and the risk for
bacterial overgrowth is reduced.Two main problems of SBS:
* the loss in absorptive surface and
* dysmotility of the residual bowel.
Surgical procedures categorized into two
subgroups:
* |) lengthening of the residual short bowel to
provide satisfactory nutrient amount, and
* Il) slowing of the intestinal transit .Small bowel lengthening procedure
A. Bianchi’s procedure,
B. STEP (Serial Sransverse EnteroPlasty) or Kim’s
procedure.
C. Kimura procedure.Patients were divided into two groups depending upon
the predominant reason for surgical lengthening
(a) to increase the enteral caloric intake
(decrease/wean TPN requirement) in patients with
poor enteral progression/adaptation and had dilated
small bowel loops on endoscopy or imaging studies
(preferably >4 cm in diameter).
(b) Intractable symptoms of bacterial overgrowth in the
setting of SBS not controlled with antibiotics and had
dilated small bowel loops. Patients who had anatomical
causes of bowel obstruction were corrected at the time
of bowel lengthening.A. Bianchi’s procedure
In 1980, Bianchi was the first to apply the
longitudinal intestinal lengthening and tailoring
(LILT) technique.
Based on the principle that
the mesenteric blood supply to the bowel “splits
as it enters the bowel wall so that the bowel and
mesentery can be divided longitudinally while
maintaining half of the blood supply to each half
of the bowel.
”* This technique consists of a construction from a
segment of small intestine of two isoperistaltic
hemiloops of half the original diameter.
* The two hemiloops are then positioned in a
circular manner, and an end-to-end anastomosis
is performed between them.
* Subsequently, the new segment is reconnected
with the remaining bowel.Besnefits of Bianchi’s procedure
¢ This allows the doubling of the entire length of
the original segment to be performed.
* The reduction of bowel diameter removes the
problems of ineffective peristalsis and stasis.
* Increased length of bowel prolongs transit and
intestinal contact time.
* Division of the circular muscle fibers —
prolongs transit time.Disadvantages —
Not recommended in conditions of inflammatory bowel disease, chronic
vascular occlusion.
Segments like duodenum have essentially no mesentry and not suitable
for longitudinal lengthening.
Successful outcome is achieved when the following anatomical conditions
are met:
a) an intestinal diameter > 3 cm;
b) a residual small bowel length > 40cm,
c) a dilated bowel length > 20 cm,
d) presence of ileocaecal valve,
e) presence of colon.
Complications — stenosis due to inadequate vascularity.B. STEP (Serial Sransverse EnteroPlasty)
or Kim’s procedure.
STEP was introduced by Kim et al in 2003.
The STEP procedure is easy to perform,
Does not require any bowel anastomoses,
Could almost double the length of the residual
intestine,
Can be performed either primarily or after a
prior Bianchi procedure.
Carries a low risk of intestinal ischemia,
Can be used repeatedly.Methods:
Lengthening of the dilated bowel is performed
by serial transverse applications of a GIA
stapler sequentially, from alternating and
opposite directions, in transverse, partially
overlapping fashion to create a zig zag like
channel of approximately 2 to 2.5 cm in
diameter .
This required the creation of a mesenteric
defect at each staple line.
The staplers are placed from the 90° and 270°
positions (0° being the mesenteric border).tection of insertion of the GIA stapler
‘and the sites of the mesenteric de-
fects. The staplers are placed in the
‘90° and 270° orientations using the
‘mesentery as the O* reference point.ECE Che scrial transverse enteroplasty (STEP) procedure
Muttiple fires of an anastomotic stapling device to aleernating sides of
the bowel wall result in decreased bowel caliber with an increased
length of enteral nutrient trans!Fig 2. Bowel after the STEP procedure. Note the dotted line
marki the antimesenteric border. This keeps the bowel from twist-
ing during placement of the stapler.L= Length of Bowel
'S= Length of Each Cut
N=# of cuts
New Length =L + (Sx N)
‘Channel Size
Fig 4. Schematic of the STEP procedure. The formula for the
theoretic new length of bowel is calculated based on measurement
along one side of the lengthened bowel. The channel size is deter-
mined by the length of the staple line and the distance between
staple lines. We try to keep the channel size constant along the
length of the bowel.the STEP procedure has several theoretic advantages over these other methods.
First, the procedure is quite easy to perform. There are no anastomoses, the bowel
is never opened, and the mesentery is never jeopardized.
Second, the total theoretical increase in length depends on the degree of bowel
dilatation and the size of the channel created. With massively dilated segments,
one could easily more than double the length of bowel.
Third, the degree of tapering is customizable. With a Bianchi procedure, the
circumference must be reduced by 50%. With the STEP, the channel size and thus
the diameter is determined by the surgeon. The bowel after the STEP procedure
tends to grow, so that the channel size can be made smaller than the control distal
bowel and thus allow even more lengthening and tapering effect.
Lastly, the STEP procedure can be performed in sequence after a successful
Bianchi procedure. Because the blood supply to the bowel after a Bianchi
procedure remains perpendicular to the long axis of the bowel, staple lines that
remain perpendicular to the long axis of the bowel should not cause ischemia. This
would theoretically allow one to first double the length of the bowel with a
Bianchi, followed several months later with a STEP, resulting in an increase in
bowel length exceeding 2-fold and possibly reaching 3 to 4 fold or greater,
depending on the degree of bowel dilatation.Article on Intestinal Lengthening in Adult Patients with Short Bowel Syndrome.
(Accepted: 5 August 2010 / Published online: 24 August 2010]
This is the first study specifically describing surgical lengthening in adult patients.
Indications were
> (a) to increase the enteral caloric intake thereby reduce or wean parenteral nu
> (b) to decrease bacterial overgrowth.
n (PN)
Median remnant bowel length prior to surgery, length gained and final bowel
length was 60, 20, and 80 cm, respectively.
Survival was 90% with mean follow-up of 4.1 years (range=1-7.9 years).
Overall, 59% patients achieved enteral autonomy and were off PN. 43% patients
showed significant improvement in enteral caloric intake.
Conclusions: Bowel lengthening is technically feasible and effectively leads to
weaning from PN in more than half of the adult patients. Lengthening procedures
may be an underutilized treatment for adults with short bowel syndrome.C. Kimura procedure
* described by Kimura and Soper.
* based on allowing time for the antimesenteric
border of the bowel to “parasitize” a new
blood supply from the liver and abdominal
wall musculature before dividing it from the
mesenteric half and creating a new tube.Kimura procedureSurgical techniques to slowing the
intestinal transit
A) Anti-peristaltic segments,
B) Colon interposition,
C) Intestinal valves,
D) Tapering enteroplastyA) Anti-peristaltic segments:
* Surgical reconstruction of the residual small
bowel using antiperistaltic jejunal segments has
been recommended for patients with a resected
ileum and ICV.
Method: the technique includes excision of a
small segment (10-15cm in length for adults and
3cm for children) of the distal intestine with its
mesenteric blood supply rotation over 180
degree of the distal intestine, and an end-to-end
anastomosis between the reversed intestinal
segment and the proximal jejunum and distally to
the remaining colon.Reversed intestinal segment desigr
intestinal wa A small segment of distal intestine is excised with
the mesenteric blood supply intact. It is then flipped 180 degrees and
reanastomosed in continuity with the remaining gastrointestinal tract.
In theory, the direction of peristalsis in the reversed segment will
contract against the wave of peristalsis in the native intestine to act
as a physiologic valv
d to slow
thus slowing transitClinical improvement seen in 80 % of patients.
A reasonable survival rate of 50% and weaning off total PN.
Optimal length of anti-peristaltic segment
> 10 cm in adults and
> 3-4 cmin children.
In children the anti-peristaltic segment grows along with the rest of
intestine so consistent result cannot be achieved.
Complications —
> transient obstructive symptoms.
> Eosinophilic colitis — with bleeding can occur in the interposed colonic
segment , may necessitate excision.B) Colon interposition.
used as an adjuvant method in cases of medical
management failure.
Studies in adults have shown that after interposition,
> will retard intestinal motility due to inherent slow
peristaltic activity of large bowel.
The technique may be performed iso-or
antiperistatically.
The colon behaves in a similar way to the reversed
small intestinal segement > Induces retrograde
peristalsis by disrupting the myo-electrical activity.C) Intestinal valves.
The simplest techniques include the installation of sutures or external
Teflon around the circumference of the bowel.
Another option involves the creation of small intussusceptions by everting
a segment of small bowel.
Intussuscepted nipple valves — purpose is to simulate ileocaecal valve
function. To slow intestinal transit, to prevent bacterial back wash into
proximal bowel.
Valve construction at ileocolonic junction — with 8 cm of intestine.
PTFE valve uses 3 cm of native intestine.
Problem — obstruction due to construction of too tight a valve.
No benefit —with loose valve.
Complication: Erosion of valve into the lumen of the intestine.B
Figure 86-3 [i
‘stinal valves constructed to slow intestinal transit.
A valve may be constructed in cither an iso- (A) or anti- (B) peristaltic
limb and is then intussuscepted into the other limb.D) Tapering enteroplasty.
another option in the management of PSBS
involves reducing the caliber of the dilated intestinal segment in
patients with PSBS. Usually, this portion exhibits low contraction
resulting in stasis, malabsorption and bacterial overgrowth.
As patients with PSBS have a short residual intestine, excision of the
dilated intestine may be not reasonable.
The reduction could be performed either by excising the
antimesenteric portion of the dilated segment or by the folding and
placation of the intestine. A drawback to this method is the possible
breakdown of the sutures lines with recurrence of dilation and
functional obstruction of the bowel.Tapering enteroplasty. A stapling devise is used
to longitudinally excise the dilated antimescnteric portion of the
intestine. The remaining bowel is therefore smaller in calibe
demonstrates improved motility
andIntestinal transplantation
Indicated
* in patients with failure of intestinal improvement
after various surgical techniques,
* jin those with no possible feeding tolerance,
* irreversible hepato-intestinal disease,
* recurrent sepsis, and
* failure of central venous sites.= Post-operative immunosuppression is done with
tacrolimus, mycofenolate, azathioprine,
cyclophosphamide.
= No useful marker to see rejection — like creatinine
in renal transplant, albumin in liver transplant.
= During follow up > Stool frequency, volume ,
consistency, repeated endoscopic mucosal biopsy.= Complications include
> acute cellular rejection (fever, nausea, vomiting,
abdominal distention), Rejection is common due
to gut associated lymphoid tissue.
> graft vs. host disease,
> post-transplant lymphoproliferative disorder,
» Sepsis is common complication.
= Post operative Mortality up to 30%.
» Most common causes — sepsis and hepatic failure.
= 1 year survival is 90% and 4 years survival is
60%Tissue Engineering Small Intestine
(TES!)
In order to build a viable and functional small intestine, the
armamentarium of TES! includes
> sophisticated materials,
> human pluripotential stem cells, and
> biopharmaceutical means.
Anumber of proteins or nucleic acid, known as biopharmaceuticals, have
been used either in clinical trials or in vitro .
Available biopharmaceuticals products include IGF-I, IGF-II, EGF, TNF-a,
etc.