Short bowel syndrome is defined as malabsorption resulting from the anatomical or functional loss of a significant length of the small intestine. It can be caused by conditions that remove portions of the small intestine like necrotizing enterocolitis or Crohn's disease. Management involves nutritional support through parenteral nutrition or specialized diets. Surgical interventions may also be used to taper or lengthen remaining intestine to promote adaptation. Complications include liver disease and infections resulting from long-term nutritional support needs.

1. SHORT BOWEL SYNDROME
PRESENTOR:DR PASHI
MODERATORS: PROF MUNKONGE/
DR BVULANI

2. PRESENTATION LAY OUT
• INTRODUCTION
• EMBRYOLOGY
• AETIOLOGY
• PATHOPHYSIOLOGY
• CLINICAL PRESENTATION
• MANAGEMENT
• COMPLICATIONS

3. INTRODUCTION
Short bowel syndrome or short gut
• Defined as malabsorption resulting from
anatomical or functional loss of a significant
length of the small intestines
• Intestinal failure due to a loss of intestine
resulting in inadequate length of bowel for
maintaining the nutrition and hydration of the
individual without either intravenous or oral
supplementation.

4. • It is intrinsic bowel disease resulting in an
inability to sustain growth, hydration, or
electrolyte homeostasis
• Many causes in infants and newborns are
removal of half or more of the small intestine,
injuries or defects present at birth, NEC,
intussusception, congenital defects, Crohns
disease

5. Normal bowel lengths at birth
• The minimum length of small bowel required for
infant survival on enteral feeds is 25 cm in the
presence of an intact ileocaecal valve (ICV) and
colon
• 40 cm without an ICV and large bowel.
• Normal intestinal length vary in the range of 250
cm to 300 cm of small bowel at term.
• The estimated length in a preterm infant of 26 and
32 weeks gestation is 70 cm and 120 cm,
respectively.

6. INTESTINAL FAILURE
• SBS has been documented as been a subset of
intestinal failure
• Mucosal enteropathies and motility disorders are
other subsets described under intestinal failure
• But in practice intestinal failure and short gut are
often used interchangeably
• The management of SBS is resource-intense,
requiring the availability of intensive care, TPN, and
expert medical and surgical intervention.
• In most countries in Africa where TPN is not
available, the outcomes are poor.

7. • the incidence of SBS in neonates is around 3
per 100,000 births per year.
• In Africa, the incidence is unknown because
survival is close to zero.

8. EMBRYOLOGY
• Midgut is known to form the primary
intestinal loop
• Derivative of midgut include duodenum distal
to the entrance of the bile duct, and continues
to the junction of the proximal two-thirds of
the transverse colon with the distal third
• Vitelline duct provides a temporal connection
between the midgut and the yolk sac
• During the sixth week, the loop grows so
rapidly that it protrudes into the umbilical
cord

9. • It will return into the abdominal cavity in the 10th
week
• At the end of these processes the midgut have
undergone a total rotation of 270ᴼ
counterclockwise
• During this development the midgut can have a
number of abnormalities such as
– Remnants of the vitelline duct,
– Failure of the midgut to return to the abdominal
cavity,
– malrotation, stenosis,
– duplication of parts of the gut

10. AETIOLOGY
• Primary: (Abnormal anatomically)
Born with short bowel
Congenital anomalies
e.g. Multiple Artesia's, Gastroschisis
• Secondary:
NEC
Hirschsprung disease
Ischemia
Tumors
Crohns’s

11. PATHOPHYSIOLOGY
• Small intestines of a neonate 250cm in length.
• By adulthood the small intestines grows to
approximately 750cm
• The effects of loss of bowel length depend on
the type and length of bowel remaining.
• Small bowel motility is three times slower in
the ileum than in the jejunum.
• The ileo-caecal valve also slows transit.
• The colon has the slowest transit time

12. Jejunum
• Long villi →Large absorptive surface area
• High concentration of enzymes and transport
carrier/hormones cholecystokinin, serotonin,
gastric inhibitory peptide, and secretin
• Large tight junction →Porous to large molecule
• The ileum will take over in case of loss

13. Ileum
• Short villi →Less absorptive capacity
• Small tight junction →Less porous and
increased absorption for fluid & electrolyte
• Specific function of absorption of B12 and
bile salts (specific receptors)
• Synthesis of hormones e.g.Enteroglucagon &
negative gastrin feedback
• Complications of ileal loss include choleretic
diarrhoea, cholelithiasis, steatorrhoea

14. Ileocecal valve/colon
• Stops reflux of bacteria
• Regulate fluid and nutrient exit
• Loss decreases transit time and bacterial over
growth in small bowel
• Colon is important for driving adaptation of the
gut.

15. Gut adaptation
• Process by which the intestine adjusts to its
loss of length through hyperplasia of the
mucosal surface so as to increase its
absorptive capacity
• The bowel dilates, lengthens, and thickens to
increase the efficiency of absorption.
• There is an increase in the number of cells in
the proliferating zones of the crypts, and villus
height increases, resulting in an increased
surface area for absorption.

16. • Adaptation is driven by the increased load of
fatty acids, carbohydrates, and proteins on the
enteroglucagon-producing cells of ileum.
• Enteroglucagon stimulates ornithine
decarboxylase, which in turn stimulates crypt
cell proliferation

17. Clinical Presentation
• Babies with severe SBS tend to be intolerant
of full enteral feeding, and will present
vomiting, diarrhoea, or both.
• weight loss, fatigue, malaise, and lethargy.
• These symptoms are protean but consistent
with the diarrheic diathesis and resultant
dehydration, electrolyte imbalance, protein-
calorie malnutrition, and loss of critical
vitamins and minerals
• Vitamin and mineral deficiencies can lead to
some specific symptoms

18. Acute phase
• Starts immediately after bowel resection and
lasts 1-3 months
• Ostomy output of greater than 5 L/d
• Life-threatening dehydration and electrolyte
imbalances
• Extremely poor absorption of all nutrients
• Development of hypergastrinemia and
hyperbilirubinemia

19. Adaptation phase
• Begins within 48 hours of resection and lasts up
to 1-2 years
• Approximately 90% of the bowel adaptation takes
place during this phase.
• Enterocyte hyperplasia and villous hyperplasia
and increased crypt depth occur, resulting in
increased surface area. Intestinal dilatation and
lengthening also occur.
• Luminal nutrition is essential for adaptation and
should be initiated as early as possible. Parenteral
nutrition is also essential throughout this period.

20. Maintenance phase
• The absorptive capacity of the intestine is at
its maximum.
• Nutritional and metabolic homeostasis can be
achieved by oral feeding, or patients are
committed to receiving supplemental or
complete nutritional support for life.

21. Management

24. Principles
• Electrolyte and fluid balance first priority.
• H2 receptor antagonist or proton pump
inhibitor.
• NGT
• Catheter
• IV fluids
• Na and K cardinal ions (normal saline + KCl)
• Urine monitoring
• A urine sodium level >30 mmol/l should be
maintained.

25. • Nutritional therapy only when the patient is
haemodynamically and fluid stable.
• Nearly all patients with SBS will require
parenteral nutrition to survive the period
while the bowel adapts
• Oral feeds can be started at the same time as
parenteral feed and gradually increased as
tolerated by the patient.

26. Nutritional support
• In infants, breast milk with oral sodium and
vitamin supplements
• Older children > ORS then carbohydrates
• Diet high in calories
• Supplements of potassium, sodium, magnesium,
• calcium, fat-soluble vitamins (vitamins A, D, and
E), and zinc. Vitamin B12
• Sodium is vital because it stimulates the bowel to
absorb, promoting adaptation
• Loperamide

27. Medical Management
• Aimed at improving complications induced by
intestinal failure.
• Promoting bowel adaptation.

28. Supportive medication
• Loperamide slows bowel transit time.
• cholestyramine reduces the irritant effect of
bile salts on the colon
• ursodeoxycholic acid reduces cholestasis,
• oral antibiotics to reduce bacterial overgrowth

29. Surgical management
Aims
• correct mechanical obstruction
• maximise bowel length.
• intestinal transplant has become a reality
• Stomas should be closed as early as possible

30. Bowel conservation
In initial surgical operation preserve as much
intestine as possible.
• use of a “second-look” operation
• use of a temporary transparent plastic silo to
cover the bowel.

31. Autologous Intestinal
Reconstruction Surgery
Tapering
• This procedure involves excision of the
antimesenteric border of the dilated portion
of bowel.

32. longitudinal intestinal lengthening and tailoring
operation (LILT)
• Principle on the bifurcation of the mesenteric
vessels at the mesenteric border of the small
bowel.
• The bowel is divided longitudinally between
the mesenteric and antimesenteric borders
along its dual blood supply.
• Two limbs are then closed and anastomosed
end to end, thus doubling that length of
bowel.

34. Iowa I operation
• Principle on the formation of collaterals
between the antimesenteric portion of the
small intestine and the abdominal wall in the
first operation
• Transverse division, as well as isoperistaltic
anastomosis of the bowel at a second
operation
• Modifications were made

36. Serial transverse entroplasty (STEP)
• Steppling device is used transversely across
the dilated bowel from the antimesenteric
border, leaving 2 cm of the bowel diameter
uncut.
• The next cut is made distally from the
antimesenteric border via a small gap created
in the mesentery
• A zigzag lengthening and tapering of the
intestine ensues

37. Introduced in 2003 based on the following
indications
• Refractory SBS
• Neonatal atresia with limited distal bowel
• Severe bacterial overgrowth.
• A contraindication to surgery is end-stage IFALD
with portal hypertension
http://www.orlive.com/childrenshospitalboston/vi
deos/serial-transverse-enteroplastybowel-
lengthening-and-tapering.

39. Complications
• Intestinal failure–associated Liver disease
• Catheter-associated bloodstream Infections
• Decreased intestinal motility
• Bacterial overgrowth

40. References
• Langman’s Medical Book of Embyology 12th
edition
• Coran Pediatric Surgery 7th Edition
• Paediatric Surgery - A Comprensive Text for Africa –
volume 02.
• www.slideshare.com
• http://www.orlive.com/childrenshospitalboston/vi
deos/serial-transverse-enteroplastybowel-
lengthening-and-tapering