By Dr. Soumen Kanjilal

1. Presented by: Dr. Soumen Kanjilal
PGT, 2nd year
Moderated by: Dr. A.S. Baishya
Assoc. Prof., Deptt. Of Surgery, SMCH

2. 
 Introduction
 Epidemiology
 Etiology
 Pathophysiology
 Clinical features
 Management
 Prognosis
Presentation overview

3. 
 Short-bowel syndrome is a disorder clinically
defined by
 Malabsorption
 Diarrhea
 Steatorrhea
 Fluid and electrolyte disturbances
 Malnutrition
 Due to functional or anatomical loss of extensive
segments of small intestine so that absorptive
capacity is severely compromised
Introduction

4. 
Extensive segment
Length of small gut* Neonate 250 cm
Adult Average 600 cm (Range 260- 800 cm)
Short Bowel Syndrome 200 cm viable (Adult)
>50 %-80% lost
Lifelong TPN Dependence
(Minimal gut length required for life)
Adult With intact colon 60 cm
Without colon 100 cm
Infant With ileocecal valve 11 cm
Without ileoceal valve 12-25 cm
*As a consequence, the infant and the young child have a favorable long-term
prognosis compared to an adult in regards to potential intestinal growth after
intestinal resection

5. 
Physiology
Jejunum Ileum
Villi Long Shorter
Absorptive surface area Large Less
Tight Junction Relatively large
• epithelium more porous to
larger molecules
• free and rapid flux of water
and electrolytes
Tighter
• permitting less flux of
water and electrolytes from
the vascular space into the
intestinal lumen
Water absorption Less effective More efficient
Absorption Carbohydrates, proteins, fat,
vitamins (iron-duodenum)
Bile acids, vitamin B-12
GI hormones that affect
intestinal motility
Enteroglucagon and
peptide YY
Etymology:
• Duodenum (Latin: duodēnum Twelve ) (duodenum is 12 fingerbreadth long)
• Jejunum (Latin: jejunus  fasting )(because it was usually found to be empty after death).
• Ileum (Greek: eilein  to twist up tightly)

6. 
SBS is an intestinal failure associated with
the inability to maintain protein-energy,
fluid, electrolytes or micronutrient balances
while on conventionally accepted normal
diet.
Short bowel syndrome and intestinal failure: consensus definitions and overview.
O'Keefe SJ1, Buchman AL, Fishbein TM, Jeejeebhoy KN, Jeppesen PB, Shaffer J.
Definition

7. 
 Prevalence is not identified worldwide
 United Kingdom, the incidence of short-bowel
syndrome which requires home TPN was 2 patients
per million population
 United States, approximately 10,000-20,000 patients
receive home-delivered TPN
 Prevalence in Spain 1.8 patients per 1 million
population
Epidemiology

9. 
Classification
Congenital versus Acquired
CONGENITAL ACQUIRED
Congenital Short Small Bowel * NEONATAL PERIOD ADULT
Necrotizing enterocolitis Crohn’s disease
Intestinal atresia Radiation enteritis
Intestinal volvulus Mesenteric vascular
accidents
OLDER INFANTS and
CHILDREN
Trauma
Intussusception with
ischemic small-intestinal
injury
Recurrent intestinal
obstruction
* Also associated with gastroschisis ,omphalocele and meconium peritonitis.

10. 
Classification
Structural versus functional
STRUCTURAL FUNCTIONAL
Any insult leading to 
< 200 cm or loss of ≥ 50% of viable small
bowel 
Increased risk of developing SBS ( NOT
ALWAYS)
Length maintained BUT function is lost.
For example:
Radiation Enteritis
Cloacal Exstrophy

12. 
Pathophysiology
Underlying
Disease
extensive
bowel
resection
affects
normal
intestinal
physiology
alteration of
intestinal
digestion
and
absorption
nutritional,
metabolic,
and infectious
consequences

13. 
Role of Ileo-caecal valve
 Loss of ileocecal valve
 transit time is faster, and loss of fluid and nutrients is
greater
 Colonic bacteria colonize the small bowel, worsening
diarrhea and nutrient loss.

14. 
Colon in Continuity
MERITS DEMERITS
Colonic water absorption could be
increased to as much as five times its
normal capacity following small bowel
resection
Increase incidence of urinary calcium
oxalate stone formation
(Oxalate is normally bound by calcium
in the small bowel and thus is insoluble
when it reaches the colon.
After massive enterectomy, much of this
calcium is bound by free intraluminal
fats)
Colonic bacteria  metabolize
undigested carbohydrates(starch &
fibres) into short-chain fatty acids, such
as butyrate, propionate, and acetate.
(up to 500 kcal/day)
Small intestinal bacterial overgrowth.
( in absence of ileocecal valve)

15. 
 Premorbid length of small bowel
 The segment of intestine that is lost
 The age of the patient at the time of bowel loss
 The remaining length of small bowel and colon,
 The presence or absence of the ileo-cecal valve.
Other factors which affect outcome

16. 
Proximal jejunal resection is better
tolerated than distal ileum resection

17. 
 The physiologic changes and adaptation of patients
with short-bowel syndrome can be viewed in three
phases.
1. Acute phase
2. Adaptation phase
3. Maintenance phase
Sundaram A, Koutkia P, Apovian CM. Nutritional management of short bowel
syndrome in adults. J Clin Gastroenterol. Mar 2002;34(3):207-20.
Adaptations to live without small bowel

18. 
 The acute phase occurs immediately after massive
bowel resection and may last up to 3-4 months.
 It is associated with malnutrition and fluid and
electrolyte loss through the GI tract.
 Enteral feedings may also be initiated, but it should
be relatively slow. Patients with less than 100 cm of
small intestine will require TPN.
Sundaram A, Koutkia P, Apovian CM. Nutritional management of short bowel
syndrome in adults. J Clin Gastroenterol. Mar 2002;34(3):207-20.
Acute phase

19. 
 The adaptation phase generally begins 2-4 days after
bowel resection and may last up to 12-18 months.
 During this second phase, up to 90% of the bowel
adaptation may occur.
 Villous hyperplasia
 Increased crypt depth
 Intestinal dilatation occur.
 Early continuous feedings with a high viscosity
elemental diet may reduce the duration of TPN.
Adaptation phase

20. 
 The absorptive capacity of the GI tract is at its
maximum.
 Some patients may still require TPN.
 In other patients, nutritional and metabolic
homeostasis can be achieved by small meals and
supplemental nutritional support for life.
Maintenance phase

22. 
 Weight loss, fatigue, malaise, and lethargy
 Vitamin A - night blindness and xerophthalmia
 Vitamin D - paresthesias and tetany
 Vitamin E - paresthesias, ataxic gait, and retinopathy
 Vitamin K depletion - easy bruisability or prolonged
bleeding
 Vitamin B12, folic acid - Anemia
 Calcium and magnesium - paresthesias and tetany
 Low zinc levels - anorexia and diarrhea
Clinical features

23. 
 Temporal wasting
 Loss of digital muscle mass
 Peripheral edema
 Dry and flaky skin
 Prominent ridges in nail
 Lingual papillae are blunted or atrophic
Physical signs

24. 
 TPN related issues
 Line sepsis and fulminant liver failure
 Enteral Feeding related issues
 gastrostomy or nasogastric tube issues

26. 
Work up
 Hematological and Biochemical investigations
 Radiological investigations
 Microbiological investigations
 Histopathological investigations
 Miscellaneous

27. 
Work up
Hematological and Biochemical investigations
CBC • Anemia (MCH, MCHC, MCV)
• Thrombocytosis/thrombocytopenia
• Hyper segmented neutrophils
Albumin (half life 21 days) • good indicator of hepatic protein synthesis
• indicator of overall nutritional status
Prealbumin ( 3-5 days) • indicator of acute nutritional status
• monitor the efficacy of nutrition support regimens
AST/ALT • TPN induced liver failure
Electrolytes
(Na, K, Cl, Zn, Ca, Mg,Cr, Se, PO4
-3)
• TPN monitoring
BUN • Renal reserve
• Dehydration(>20:1)
• Overfed with protein
Vitamins Level
Coagulation Profile • Deranged Liver function

28. 
Workup
Hematological and Biochemical investigations Frequency*
Electrolytes, BUN, creatinine, calcium, magnesium,
phosphorous
Twice weekly
Comprehensive metabolic panel, CBC, triglycerides,
cholesterol
Weekly
Folate, vitamin B-12, vitamin E, copper, zinc, selenium Monthly
*both in initial phase and the late period or at the time of presentation for
instability

29. 
Work up
Radiological investigations
Plain Chest X-ray Post CV line insertion
Plain Abdominal X-ray Suspected bowel obstruction
Barium imaging of the bowel
Abdominal USG fungal balls in the kidney (sepsis)
Renal Stones and related problems
Gall stones and related problems
Liver failure (spleen, ascites, liver texture, Portal
vein flow-Duplex)
CT-Abdomen identify persistent sepsis
Potential Liver/Bowel transplantAngiography

30. 
Work up
Microbiological investigations
 Blood cultures (both central and peripheral sites)
 Urinalysis and blood culture (specifically to search for fungal
infection)
 CV line tip
SOURCE of SEPSIS:
• Line Sepsis,
• Gut mucosal atrophy bacterial translocation
• Skin flora penetration

31. 
Work up
Histo-pathological investigation
Liver biopsy(rare)
TPN related liver problems

32. 
Work up
Miscellaneous
 Upper GI endoscopy
 to assess for peptic ulcer disease and possible signs of
liver disease e.g. esophageal varices, hypertensive
gastropathy
 Dual radiographic absorptiometry
 Bone density estimation in Metabolic Bone Disease

34. 
 Management of SBS is progressed through several
phases
 Management goals varies depending on phases
 Initial phase
 To stabilize critically ill patient
 Controlling sepsis
 Fluid and electrolyte balance
 Initiation of nutrtional support
Goals to be achieved

35. 
 As patient is recovered from acute stage primary
goal of management is to maintain nutritional status
 To maximize the absorptive capacity
 Prevent complications of PN and short bowel
syndrome

36. 
 Preserving the intestinal remnant
 Improve the function of remnant bowel
 Augmenting the intestinal length
 Intestinal transplantation
Management options

37. 
Treatment
(aggressive MULTI DISCPLINARY APPROACH)
MEDICAL CARE
 Nutrition
 Early aggressive enteral feeding
 Parenteral nutrition
 Aggressive Hydration
 Electrolytes replacement
 Acid reducing agents(PPI, H2
blocker)
 Antibiotics for bacterial
overgrowth
 Bile salt chelators (Cholestyramine)
 Psychosocial support
SURGICAL CARE
 Nontransplant surgery
 Small bowel ± liver
transplantation

38. Nutrition
Early aggressive
enteral nutrition*
as soon as patient
can tolerate
most important
stimulus for
intestinal
adaptation
Early
discontinuation
of parenteral
therapy.
Prolonged
NPO
Gut MUCOSAL
ATROPHY
BACTERIAL
TRANSLOCATION
SEPSIS

39. 
 PN support in the early post operative period
 Provision of energy substrate, protein, fluid,
electrolytes, minerals, vitamins and micronutrients
 25-30 kcal/kg per day
 1 to 1.5 g of proteins per day
Maintain nutritional status

41.  Should started as early as possible when ileus is
settled
 Help to maximize absorptive capacity and to reduce
the complications related to PN
 Patients with small bowel more than 180 cm will not
require PN
 Patients with small bowel more than 90 cm with
colon require PN less than 1 year duration
 Less than 60cm of small bowel might require
permanent PN depending on colon length
Long-term survival and parenteral nutrition dependence in adult patients
with the short bowel syndrome.Messing B1, Crenn P, Beau P, Boutron-Ruault
MC, Rambaud JC, Matuchansky C.
Enteral feeding following surgery

42. 
 Continuous enteral feeding may permit greater
absorption of nutrients than intermittent enteral
feeding
Continuous enteral nutrition during the early adaptive stage of the short
bowel syndrome. Levy E1, Frileux P, Sandrucci S, Ollivier JM, Masini
JP, Cosnes J, Hannoun L, Parc R.

43. 
Factors predictive of achieving
independence from TPN
 Residual Bowel length
 Intact colon
 Intact ileocecal valve
 Healthy (versus diseased) residual
small gut
 Jejunal resection (versus ileal resection)
 Bacterial overgrowth
 Dysmotility
SERUM
CITRULLINE
LEVEL

44. 
Serum Citrulline* in SBS
 α-amino acid
 key intermediate in the urea cycle.
 Citrullinemia is correlated to small bowel length and to net
digestive absorption of fat
 Cuff-off value of 20 µmol/l classified short bowel patients with
permanent intestinal failure with high sensitivity (92%),
specificity (90%), positive predictive value (95%), and negative
value (86%);
 more reliable indicator than anatomic variables to separate transient as opposed
to permanent intestinal failure.
__________________________________________________________________
*Etymology: Latin citrullus = watermelon (from which it was first isolated)

45. 
 surrogate marker for bowel length and function.
 past the 2-year adaptive period, a powerful
independent indicator allowing distinction of
transient from permanent intestinal failure.
Post-absorptive plasma citrulline concentration

46. 
 Hypo-osmolar diets are started initially to reduce the intestinal
fluid loss
 High protein, high carbohydrate diets are recommended for
maximum absorption
 Providing nutrient as their simplest form improves absorption
 Di and tri peptide sugars
 Medium chain tri glycerides
 Addition of pectin increase transit time and reduce water loss
Maximize absorptive capacity

47. 
 Early enteral nutrition
 Provision of long chain fatty acid and fiber
 Glutamine – trophic to the gut as well as act as fuel for
enterocytes
 Meal itself act as endocrine stimulation for adaptation
via various hormones and growth factors
Glutamine and the preservation of gut integrity. van der Hulst RR1, van Kreel
BK, von Meyenfeldt MF, Brummer RJ, Arends JW, Deutz NE, Soeters PB.
Maximize adaptive capacity

48. 
 To minimize diarrhoea and GI secretion
 Narcotics – Codeine, Diphenoxylate and Loperamide
 Diminished action over time
 Progressive dosage
 Drug holidays
Antimotility and Antisecretory drugs

49. 
 PPI and H2 receptor blockers reduce gastrointestinal
secretion
 Clonidine also reduce fluid loss (alpha 2 receptor
agonist)
 Prebiotics and probiotics also proven to improve
absorption.
Potential benefits of pro- and prebiotics on intestinal mucosal immunity and
intestinal barrier in short bowel syndrome.Stoidis CN1, Misiakos EP2, Patapis P2,
Fotiadis CI2, Spyropoulos BG3.

50. 
 GLP – 2 (Teduglutide)
 Increase intestinal absorption and adaptation
 Produce by entero-endocrine cells in small intestine
 Shown to increase absorption and increase villous height and
crypt depth
 Still undergoing further studies
Short Bowel Patients Treated for Two Years with Glucagon-Like Peptide 2 (GLP-2):
Compliance, Safety, and Effects on Quality of Life P. B. Jeppesen,1,* P. Lund,1 I. B.
Gottschalck,1 H. B. Nielsen,2 J. J. Holst,3 J. Mortensen,4 S. S. Poulsen,3 B. Quistorff,3
and P. B. Mortensen1
Newer therapies

51. 
 Complications of short bowel syndrome
 Therapy related
 Diarrhea and steatorrhea
 Metabolic abnormalities
 Nutritional deficiencies
 Infectious complications
 Liver disease
 Physiologic
 Cholelithaisis
 Nephrolithiasis
 Gastric hypersectretion
 Bacterial overgrowth
Prevent complications

52. 
 Supplementation of vitamin D calcium and
magnesium
 Treat bacterial over growth in small bowel which can
cause metabolic acidosis
 Prevent catheter related sepsis
 PN related liver disease – multifactorial
 Maximizing enteral calories
 Avoid over feeding
 Prevent specific nutrient deficiencies
Measures to prevent complications

53. 
 Occur in 1/3rd of patients
 Due to increase bile stasis, and reduction of bile salt
absorption which leads to cholesterol stones
 Early enteral feeding reduce the stasis and
occurrence of bile stones
 Intermittent CCK injections prevent stasis
 Consider prophylactic cholecystectomy when
laparotomy is being performed for other reasons.
Cholelithiasis

54. 
 Increase risk in colon preserved patients
 Binding of non absorbed FFA with calcium releases
free oxalate which are soluble and absorbed in colon
 Free oxalate bind with calcium and form stones in
urine
 To prevent
 Low oxalate diet
 Reduce intraluminal fat
 Oral calcium supplement
 Cholestyramine binds with oxalic acid in colon
Nephrolithiasis

55. 
 Due to loss of inhibiting factors from the small
bowel
 Exacerbate malabsorption and diarrhea
 Causes peptic ulcer disease
 Prevention by PPI and H2 receptor blockers, which
continue up to 1 year postop
Gastric hyper secretion

56. 
Bacterial overgrowth
 Predisposing factor
 Absence of ileocaecal valve
 Dysmotility of residual gut
 Manifestations
 Deconjugation of bile salts and depletion of bile salt stores
 Vitamin B12 deficiency  Pernicious anemia
 Carbohydrate malabsorption  worsening of osmotic diarrhea
 Metabolic lactic acidosis  CNS disturbances
 Dehydration
 Treatment
 Metronidazole alternating with either Kanamycin or oral
Gentamicin

58. 
 Re operation surgery is required in half of the patients
 Aim is to preserve the intestinal remnant length
 Avoid resection as much as possible
 Surgical options available
 Intestinal tapering for dilated segments
 Strictureplasty
 Serosal patching
 Recruitment of isolated or by-passed bowel segment
Surgical therapy

59. 
 Half of the patients can maintain nutrition only on
enteral nutrition and doesn’t require surgery
 But surgery should be consider if they are having
following
 worsening malabsorption
 Increased requirement for parenteral nutrition
 Disabling symptoms related to malabsorption
 Other half who is stable on TPN can undergo
surgery in the aim of weaning off from PN
When to consider surgical treatment

60. 
 Intestinal remnant length
 Intestinal function
 Diameter of the intestinal remnant
Type of surgery depend on

61. 
Preserving existing Intestine
Adults with remnant more than 120cm
Initial conservative management
But when dilatation occurs – due to
obstruction caused by adhesions or stricture at
anastomotic site, surgery is done for
adhesiolysis and strictureplasty
Serosal patching for chronic perforation

62. 
Tapering Enteroplasty
 Imbrication of redundant bowel
 Longitudinal transection & removal of part of the
circumference along the anti- mesenteric border.
Improving Intestinal Motility

63. 
Patients with marginal remnant, 60 -120cm.
They have rapid transit.
Reversing 10 – 15 cm segment yielded good
results
Other options
 Creation of artificial valves – not successful
 Retrograde intestinal pacing with electrodes
 Colonic transposition
Improving Intestinal Motility

64. 
 Patients with short remnant length < 60 cm with
dilated bowel
 Goal is to preserve the functional length and luminal
diameter
 When the dilatation is progressive in the absence of
obstruction – adaptive dilatation and attempted
medical management are unsuccessful surgical
intervention is indicated.
Intestinal Lengthening

65. 
LILT (Bianchi1980) Procedure

66. 
STEP
Serial applications of an intestinal
stapling device, with firings
oriented perpendicular to long axis
of intestine
By 2013, amongst 111 patients
operated 47 % cases had achieved
enteral autonomy by 21 months.
1st performed in 2003 on 2-year-
old baby who had been born with
gastroschisis

67. 
Intestinal Transplantation
 Indications
 Life threatening complications due to intestinal failure
or long term TPN
 Impending or overt liver failure
 Thrombosis of major central veins
 Frequent episodes of catheter-related sepsis
 Frequent episodes of severe dehydration.

68. 
 2000 of transplants done in US by 2012
 75% of patients are younger than 18 years
 1 year graft survival is 89% in adults
 But children less than 1 year of age it is 69%
 Patients survival rates are similar at 1 and 5 year
after transplant
 After one year of surgery 90 % of patients are
independent from PN
Intestine Transplantation in the United States, 1999–2008 Mazariegos, G. V.; Steffick, D.
E.; Horslen, S.; Farmer, D.; Fryer, J.; Grant, D.; Langnas, A.; Magee, J. C. [less] 2010-04

69. 
Yang feng suffering SBS following resection of small
bowel due to diverticulosis, 1st Chinese to survive
successfully following Small bowel transplantation

70. 
Yang Feng, the first Chinese alive who
received a small intestine transplant
holds his bride at the wedding