Management of Short bowel syndrome in neonates & infants.........................by
Dr Sunil B Deshmukh, MBBS MD Paediatrics, Fellow in Neonatology(KEM Hospital ,Pune)
4. ī¨ Baby cried well after birth required no
resuscitation.
ī¨ Apgar score â not known
ī¨ Was shifted to Sahyadri hospital NICU in view
of Respiratory Distress at birth
5. Post natal course
ī¨ Baby had RDS at birth managed with surfactant
and Ventilatory support.
ī¨ HS PDA treated with Ibugesic.
ī¨ Baby extubated to CPAP at 42 hrs of life, weaned
off over next 4 days.
ī¨ OGT feeds started on 1st day , gradually graded
up to full feed on day 6th of life
6. ī¨ Day 7 of life â baby developed feed
intolerence
ī¨ Baby kept NBM, septic work up sent,
antibiotics started.
ī¨ Later Culture- Klebisella
ī¨ On day 10th - baby had Pneumoperitoneum.
ī¨ Peritoneal drain inserted- had fecal matter.
ī¨ On day 11th â Exploratory laparotomy with
resection anastomosis of gangrenous bowel
(jejuno-colic)
7.
8.
9.
10. ī¨ On days 16th â persistant oozing of fecal
material from Surgical site
ī¨ Planned re-exploration
ī¨ Baby refer to KEM hospital for further
management.
11. on admission â
ī¨ Active
ī¨ Weight 1.4 kg
ī¨ Pulses , perfusion ânormal
ī¨ No organomegaly
ī¨ Discharge from surgical site ++
12. ī¨ Feed started OGT EBM, Graded up gradually
ī¨ Again developed S/o intestinal obstruction
ī¨ On day 30th Second Exploratory Laparotomy -
--- Adhenolysis + Jejuno-Ascending Colon
anastomosis
13. ī¨ On day 36th Feeding restarted with EBM -----
Graded up gradually to reach full feed in next 7
days
ī¨ On day 48th developed feed intolerance with
excessive leakage from surgical wound site (>400
ml/day)
ī¨ Started of 1/2 OGT, 1/2 Fluid EBM------ taken on
full feed
14. ī¨ During hospital stay baby had multiple
episodes of feed intolerance i.e. excessive
stoma output, electrolytic imbalance,
dehydration, anemiaâĻ...... Treated accordingly
ī¨ Gastroenterologist opinion taken for feed
intolerance
ī¨ Finally baby started on elemental formula i.e.
Neocate
15. Current status
ī¨ Nutrition & weight gain-
ī¨ Anemia -
ī¨ Dyselectrolytemia -
ī¨ High fistula output -
ī¨ Hypoalbuminemia -
ī¨ Acidosis
20. Definition
Short bowel syndrome (SBS) is a state of malabsorption,
and parenteral nutrition (PN) is needed for a prolonged
period of time.
ī¨ Amin et al. define the need for PN as a minimum of 3
months
ī¨ Canadian Association of Pediatric Surgeons --PN as
>42 days
It is particularly more severe when there is resection of the
ileocecal valve and colon.
SBS is most common cause of intestinal failure in NICU
21. SBS may occur when
> 50% of small bowel resected or
< 100cm of small bowel is left.
Ultra-short bowel has been defined by
ī¨ Diamanti et al. as10 cm;
ī¨ by Gambarara et al. as 20 cm; and
ī¨ by De Greef et al. as 40 cm
22. Incidence
True incidence of SBS is unknown.
ī¨ 0.7% of VLBW infants by the National Institute of
Child centers
ī¨ Inverse relationship with birth weight and
gestation.
Common causes of SBS in children include:
Necrotising enterocolitis (35%) â most
common
Intestinal atresia (25%)
Gastroschisis (18%)
Malrotation with volvulus (14%)
Hirschsprungs disease- less common
23. Development of GIT
ī¨ The small intestine is completely formed by 20
weeks gestation age.
ī¨ It grows 142Âą22 cm at 19-27 weeks
217Âą24 cm at 27-35 weeks
304Âą44 cm at term
ī¨ The mucosal surface area in infants is 950
cm²
24. The large intestine is
important in
īŽ Absorption of fluids and electrolytes.
īŽ Microflora plays a role in the fermentation of
carbohydrates to short chain fatty acids, which
then can be use as an energy
īŽ Delayed gastric emptying.
It is recommended that any significant portion of
colon remaining in SBS patients be re-
anastomosed to the small intestine, either a
primary or staged procedure.
25. Patients can be grouped into 2 subgroups;
ī¨ without a colon and
ī¨ with an intact colon in continuity.
Clinical presentation and outcome of SBS depends on-
Length & health of remaining bowel
Age of patient
GIT region(s) resected
Presence of ICV
Associated co-morbidities- Prematurity,
CLD,MBD,EUGR,CHD
26. Intestinal Rehabilitation
ī¨ Intestinal adaptation is the best option for
patients with SBS.
ī¨ In humans, intestinal adaptation begins
within 24-48 hours of resection may take
1 to 2 years to evolve.
ī¨ It includes
morphological and
functional changes of the remaining
bowel.
27. The length of the remaining bowel
required to prevent dependence on TPN
is
īą with 15 cm of remaining small intestine with an
intact ICV, or
ī¨ 40 cm of small intestine without an ICV
28. Predictors of mortality
ī¨ Cholestasis (parenteral nutrition-associated),
(conjugated bilirubin >2 mg/dL)
ī¨ % of remaining small bowel length
ī¨ Presence of ICV (ileocecal valve)
29. Nutritional Management of Short
Bowel Syndrome
The main goals and objectives are to:
ī¨ Ensure sufficient nutrients ie. energy & proteins
ī¨ To monitor fluid and electrolyte imbalance
ī¨ Prevent dehydration and provide the appropriate
fluid replacements.
ī¨ To prevent any vitamin or trace element
deficiencies
30. âA, B, C, Dâ approach
ī¨ Anthropometry â Wt, Length, HC
ī¨ Biochemistryâ SE, RTF, BSL, LFT, CBC
ī¨ Clinical
ī§ Amount of bowel resected
ī§ Area / site of resection
ī§ Presence of ICV
ī§ Presence of colon
ī¨ Dietary,
To provide a step by step reference as to how to
approach nutrition support.
32. Acute phase
ī¨ This occurs after the resection of the small bowel.
ī¨ Lasts less than 4 weeks
ī¨ This phase serves for patient stabilization
ī¨ Fluid, Electrolytes & Metabolic âmanagement
PN is started and serve as the sole source of
energy needs for the growth of the infant or child.
Associated with gastric hypersecretion-
H2 blocker/PPI may become necessary for 6-12
months
33. Adaptation phase (Recovery
phase)
ī¨ Depending on each individual, a trial of enteral nutrition can
be started on day 4 or 5 postoperatively.
ī¨ The patient should be hemodynamically stable and has
passed stool or a functional stoma.
ī¨ This phase could last up to 1 â 2 years.
ī¨ This is the time that maximal absorption capacity needs to be
achieved.
ī¨ Enteral nutrition is given gradually at small volumes to
determine the level of tolerance of the gut.
34. Maintenance phase
ī¨ Having established intestinal adaptation and
successfully weaned off TPN,
ī¨ EN must ideally continue for 12 months to achieve
intestinal adaptation.
ī¨ Slowly progress to bolus feeding, by giving small 2
hourly oral feeds.
ī¨ Pass NGT if oral feeds poorly tolerated. First attempt
orally, then pass the remaining feed through NGT.
ī¨ Refer to speech therapist if there is poor oral skills.
35. Parenteral nutrition strategies
ī¨ Promote growth,
ī¨ Bone mineralization, and
ī¨ Neurodevelopment
Ideal PN macronutrient needs for VLBW
provides
90-100 kcal/kg/day,
4 g amino acids/kg/day, and
2.5-3 g fat/kg/day
36. Enteral nutrition strategies
ī¨ Starting with trophic volumes of
~10 mL/kg/day given as 1-2 mL Q 3-6 h for 24-48
hrs
ī¨ advanced by 20-30 mL/kg/day
ī¨ Once the infant is tolerating at least 50-60
mL/kg/day, the advancement of feeds may be
able to quicken, again, depending on the infantâs
clinical picture.
37. ī¨ Feed advancement depends on the tolerance of
feed determined by monitoring the ostomy or stool
output and what is present in the stool.
ī¨ Ideal ostomy output volume should be
<40mL/kg/day
ī¨ The volume of the enteral feeds is gradually
increased as parenteral feedings are decreased in
an isocaloric fashion.
38. ī¨ Continuous enteral nutrition is preferred
over bolus administration to assist with better
absorption as the release of nutrients are
much slower.
ī¨ Bolus or intermittent feeding- a more
physiological hormonal response, improved
motility, but result in increased feeding
intolerance
ī¨ Oral feeding should be attempted, at the
appropriate age, to prevent any food aversion.
39. Factors to monitor tolerance of
enteral nutrition:
ī¨ Stool output (i.e via rectum)
ī¨ Iliostomy output
ī¨ Stool reducing substances
ī¨ Signs of dehydration
ī¨ Fecal Osmolar Gap (FOG): only in those with
intact colon
ī¨ Gastric aspirates- no longer evidence based.
40.
41. Choice of enteral feed
a) Small bowel of < 100cm, only jejenum + no ICV + no colon
Breastmilk OR Elemental feed
b) Small bowel of < 100cm + with ICV + no colon
Breastmilk OR Semi elemental casein dominant feed
c) Small bowel of < 100cm + with ICV + colon (but not in
continuity)
Breastmilk OR Semi elemental casein dominant feed
d) Small bowel < 100cm + with ICV in continuity with colon
Breastmilk OR Polymeric feed
42. Enteral Nutrition Composition
Protein
īŽ Hypoallergenic formulas are preferred
īŽ Hydrolyzed or elemental diets would be the
preferred choice if no breast milk is available
īŽ Gradually progress to a more complex formula to
intact protein formula once over the age of one
Fats
Choice depend on whether there is fat mal-
absorption and a colon present
Fat mal-absorption---MCT & LCT
Otherwise---- LCT predominant over MCT.
43. MCT LCT
âĸwater soluble, absorb in colon
âĸsignificant absorptive advantage
âĸMucosal adaptation occurs to a lesser
degree
âĸonly of benefit if the colon is in
continuity with the small bowel
âĸ increase the output of
ostomy losses due to the high
osmolality
âĸshown to decrease the
absorption of other nutrients such as
protein and carbohydrate
âĸexcess intake of MCTs may result in
nausea, vomiting and ketosis
âĸLipophilic
âĸMucosal adaptation occurs to a MORE
âĸimprovement in the mucosal function
âĸimprovement in weight gain
âĸoverall improvement in the absorption
of the nutrients
44. Carbohydrates
ī¨ As a result of gut resections a lack of mucosal
disaccharides.
ī¨ Sucrose and lactose are commonly poorly
tolerated
ī¨ This is associated with osmotic diarrhoea.
ī¨ Carbohydrate mal-absorption can occur in SBS,
but would be of limited importance if the colon is
intact,
(as the bacteria present helps to ferment the 80% of
the carbohydrate)
45. Micronutrients
ī¨ fat soluble vitamins, such as, vitamin A, D and E
ī¨ vitamin B12 if the whole or > 60% of the terminal
ileum as been resected
ī¨ Folate supplementation would be required if
proximal jejunum has been resected
ī¨ Patients with SBS lose a significant amount of
zinc and selenium in their feces
ī¨ Magnesium (Mg) can be lost in jejenal or ileal
effluent
ī¨ Calcium deficiency can occur as unabsorbed
fatty acids in the small bowel
ī¨ Iron supplementation in microcytic anemia.
46.
47. Type of Enteral Nutrition
ī¨ Breastmilk
īŽ superior choice
īŽ Immumnological benefit
īŽ Improved mucosal adapatation
īŽ Protective colonic bacterial flora- Lactoferrin
ī¨ Semi elemental formulas
īŽ contains protein hydrolysed to peptides
īŽ LCTs and MCTs
īŽ casein dominant , completely lactose free
īŽ Ex- Alimentum (Abbot), Peptamen Junior
ī¨ Elemental formulas
īŽ the protein hydrolyzed to amino acids
īŽ predominantly made up LCTs, Lactose free
īŽ Ex- Neocate
48. Laboratory monitoring
ī¨ Complete blood counts
ī¨ Electrolytes & renal function
ī¨ Blood glucose
ī¨ Serum triglycerides & Liver function test
ī¨ Serum Zinc, Copper, and Selenium monthly
ī¨ Vitamins A, D, E, and K every 1, 3, or 6
months
ī¨ Serum Citrulline levels as a marker for
small bowel length, absorption capabilities,
and prognosis for weaning from PN
49. Complications of SBS
ī¨ Small bowel bacterial overgrowth (SBBO)
ī¨ Diarrhoea and malabsorption
ī¨ Dehydration
ī¨ Micronutrient deficiencies
ī¨ Nutrient malabsorptions
ī¨ Complications related to TPN- cholestasis,
sepsis
50. Medical Therapy
ī¨ H2 blocker / PPI- first 6-12 months
ī¨ Loperamide and codeine- anti-motility, slow
intestinal transit
ī¨ Octreotide- secretory diarrhea
ī¨ Cholestyramine- cholerheic diarrhoea -binds bile
acids
ī¨ Broad spectrum antibiotics-small bowel
bacterial overgrowth
ī¨ Clonidine- to reduce excess fluid/ostomy losses
51. Growth factors for intestinal
adaptation
ī¨ Glutamine- main fuel for enterocytes and is
also a substrate for the synthesis of nucleic
acids
ī¨ Growth hormone (GH)- shown to increase
colonic mass, enhance sodium and water
absorption, and promote mucosal
hyperplasia.
ī¨ Probiotics- capable of stimulating growth of
the mucosa in the lower gastrointestinal tract.
ī¨ Fibre and Short chain fatty acids (Butyrate)-
causes bulking of the stool and leads to a
decrease in the whole transit time
52. Follow up / Discharge
Procedures
ī¨ Follow up on a monthly basis.
ī¨ Diet history: Lactose and sucrose free diet.
ī¨ Ensure Calcium supplement prescribed.
ī¨ Appropriate feed given
ī¨ Necessary micronutrient supplementation eg.
Vitamin B12
ī¨ Ensure that all medications and supplements
are sucrose free.
53. âMost infants and children with SBS have a good
prognosis, if effective nutritional and medical
therapy is provided for intestinal adaptation.â
54. References
ī¨ Management of short bowel syndrome in
postoperative very low birth weight infants Olivia
Mayer, 50 J.A. Kerner / Seminars in Fetal & Neonatal
Medicine 22 (2017) 49-56.
ī¨ âThe Nutritional Management of Short Bowel
Syndrome of Infants and Childrenâ, Pediatric Working
Group Western Cape Red Cross Childrenâs Hospital,
2009
ī¨ Short Bowel Syndrome in the Nicu, Sachin C. Amin,
MD, Cleo Pappas, MLIS, and Akhil Maheshwari, MD
as:Clin Perinatol. 2013 Mar; 40(1):
10.1016/j.clp.2012.12.003.