short bowel syndrome in infants... dr sunil deshmukh
TRANSCRIPT
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CASE DISCUSSION Short Bowel Syndrome
(B/o Sonawane S)
Dr Sunil Deshmukh KEM Hospital Pune
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Antenatal History Age -30 yrs G1P0 Antenatal period –spotting since 4th
month Antenatal USG –normal
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Intrapartum Details Preterm Vaginal delivered (32weeks) Private Nursing Home, Pune. DOB -14-11-16 TOB- 3:45 PM SEX –Female GA- 32 Weeks BIRTH WEIGHT- 1.5 KG
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Baby cried well after birth required no resuscitation.
Apgar score – not known Was shifted to Sahyadri hospital NICU in
view of Respiratory Distress at birth
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Post natal course Baby had RDS at birth managed with
surfactant and Ventilatory support.
HS PDA treated with Ibugesic.
Baby extubated to CPAP at 42 hrs of life, weaned off over next 4 days.
OGT feeds started on 1st day , gradually graded up to full feed on day 6th of life
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Day 7 of life – baby developed feed intolerence
Baby kept NBM, septic work up sent, antibiotics started.
Later Culture- Klebisella On day 10th - baby had Pneumoperitoneum. Peritoneal drain inserted- had fecal matter. On day 11th – Exploratory laparotomy with
resection anastomosis of gangrenous bowel (jejuno-colic)
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On days 16th – persistant oozing of fecal material from Surgical site
Planned re-exploration
Baby refer to KEM hospital for further management.
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on admission – Active Weight 1.4 kg Pulses , perfusion –normal No organomegaly Discharge from surgical site ++
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Feed started OGT EBM, Graded up
gradually
Again developed S/o intestinal obstruction
On day 30th Second Exploratory Laparotomy ---- Adhenolysis + Jejuno-Ascending Colon anastomosis
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On day 36th Feeding restarted with EBM ----- Graded up gradually to reach full feed in next 7 days
On day 48th developed feed intolerance with excessive leakage from surgical wound site (>400 ml/day)
Started of 1/2 OGT, 1/2 Fluid EBM------ taken on full feed
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During hospital stay baby had multiple episodes of feed intolerance i.e. excessive stoma output, electrolytic imbalance, dehydration, anemia…...... Treated accordingly
Gastroenterologist opinion taken for feed intolerance
Finally baby started on elemental formula i.e. Neocate
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Current status Nutrition & weight gain- Anemia - Dyselectrolytemia - High fistula output - Hypoalbuminemia - Acidosis
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THE NUTRITIONAL MANAGEMENT OFSHORT BOWEL SYNDROME
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Definition
Short bowel syndrome (SBS) is a state of malabsorption, and parenteral nutrition (PN) is needed for a prolonged period of time.
Amin et al. define the need for PN as a minimum of 3 months
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
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SBS may occur when > 50% of small bowel resected or < 100cm of small bowel is left.
Ultra-short bowel has been defined by Diamanti et al. as10 cm; by Gambarara et al. as 20 cm; and by De Greef et al. as 40 cm
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Incidence
True incidence of SBS is unknown. 0.7% of VLBW infants by the National Institute of
Child centers Inverse relationship with birth weight and gestation.
Common causes of SBS in children include:Necrotising enterocolitis (35%) – most commonIntestinal atresia (25%)Gastroschisis (18%)Malrotation with volvulus (14%)Hirschsprungs disease- less common
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Development of GIT The small intestine is completely formed
by 20 weeks gestation age.
It grows 142±22 cm at 19-27 weeks217±24 cm at 27-35 weeks304±44 cm at term
The mucosal surface area in infants is 950 cm²
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The large intestine is important in
Absorption of fluids and electrolytes.
Microflora plays a role in the fermentation of carbohydrates to short chain fatty acids, which then can be use as an energy
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.
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Patients can be grouped into 2 subgroups; without a colon and with an intact colon in continuity.
Clinical presentation and outcome of SBS depends on-Length & health of remaining bowelAge of patientGIT region(s) resectedPresence of ICVAssociated co-morbidities- Prematurity, CLD,MBD,EUGR,CHD
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Intestinal Rehabilitation Intestinal adaptation is the best
option for patients with SBS. In humans, intestinal adaptation
begins within 24-48 hours of resection may take 1 to 2 years to evolve.
It includes morphological and
functional changes of the remaining bowel.
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The length of the remaining bowel required to prevent dependence on TPN is
with 15 cm of remaining small intestine with an intact ICV, or
40 cm of small intestine without an ICV
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Predictors of mortality Cholestasis (parenteral nutrition-
associated),(conjugated bilirubin >2
mg/dL) % of remaining small bowel length Presence of ICV (ileocecal valve)
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Nutritional Management of Short Bowel Syndrome
The main goals and objectives are to: Ensure sufficient nutrients ie. energy & proteins
To monitor fluid and electrolyte imbalance
Prevent dehydration and provide the appropriate fluid replacements.
To prevent any vitamin or trace element deficiencies
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“A, B, C, D” approach Anthropometry – Wt, Length, HC Biochemistry– SE, RTF, BSL, LFT, CBC Clinical
Amount of bowel resected Area / site of resection Presence of ICV Presence of colon
Dietary,
To provide a step by step reference as to how to approach nutrition support.
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Nutritional proceduresThe nutritional management of SBS
involves 3 phases, namely1. Acute phase 2. Adaptation phase3. Maintenance phase
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Acute phase This occurs after the resection of the small bowel. Lasts less than 4 weeks This phase serves for patient stabilization Fluid, Electrolytes & Metabolic –management
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
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Adaptation phase (Recovery phase)
Depending on each individual, a trial of enteral nutrition can be started on day 4 or 5 postoperatively.
The patient should be hemodynamically stable and has passed stool or a functional stoma.
This phase could last up to 1 – 2 years.
This is the time that maximal absorption capacity needs to be achieved.
Enteral nutrition is given gradually at small volumes to determine the level of tolerance of the gut.
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Maintenance phase Having established intestinal adaptation and
successfully weaned off TPN,
EN must ideally continue for 12 months to achieve intestinal adaptation.
Slowly progress to bolus feeding, by giving small 2 hourly oral feeds.
Pass NGT if oral feeds poorly tolerated. First attempt orally, then pass the remaining feed through NGT.
Refer to speech therapist if there is poor oral skills.
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Parenteral nutrition strategies Promote growth, Bone mineralization, and 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
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Enteral nutrition strategies Starting with trophic volumes of~10 mL/kg/day given as 1-2 mL Q 3-6 h for 24-
48 hrs advanced by 20-30 mL/kg/day
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 infant’s clinical picture.
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Feed advancement depends on the tolerance of feed determined by monitoring the ostomy or stool output and what is present in the stool.
Ideal ostomy output volume should be <40mL/kg/day
The volume of the enteral feeds is gradually increased as parenteral feedings are decreased in an isocaloric fashion.
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Continuous enteral nutrition is preferred over bolus administration to assist with better absorption as the release of nutrients are much slower.
Bolus or intermittent feeding- a more physiological hormonal response, improved motility, but result in increased feeding intolerance
Oral feeding should be attempted, at the appropriate age, to prevent any food aversion.
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Factors to monitor tolerance of enteral nutrition:
Stool output (i.e via rectum)
Iliostomy output
Stool reducing substances
Signs of dehydration
Fecal Osmolar Gap (FOG): only in those with intact colon
Gastric aspirates- no longer evidence based.
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Choice of enteral feeda) Small bowel of < 100cm, only jejenum + no ICV + no colonBreastmilk OR Elemental feed
b) Small bowel of < 100cm + with ICV + no colonBreastmilk 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 colonBreastmilk OR Polymeric feed
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Enteral Nutrition Composition
Protein Hypoallergenic formulas are preferred Hydrolyzed or elemental diets would be the
preferred choice if no breast milk is available 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 & LCTOtherwise---- LCT predominant over MCT.
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MCT LCT
•water soluble, absorb in colon
•significant absorptive advantage
•Mucosal adaptation occurs to a lesser degree
•only of benefit if the colon is in continuity with the small bowel
• increase the output ofostomy losses due to the high osmolality
•shown to decrease theabsorption of other nutrients such as protein and carbohydrate
•excess intake of MCTs may result in nausea, vomiting and ketosis
•Lipophilic
•Mucosal adaptation occurs to a MORE
•improvement in the mucosal function
•improvement in weight gain
•overall improvement in the absorption of the nutrients
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Carbohydrates As a result of gut resections a lack of mucosal
disaccharides. Sucrose and lactose are commonly poorly tolerated
This is associated with osmotic diarrhoea.
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)
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Micronutrients fat soluble vitamins, such as, vitamin A, D and E vitamin B12 if the whole or > 60% of the terminal
ileum as been resected Folate supplementation would be required if
proximal jejunum has been resected Patients with SBS lose a significant amount of zinc
and selenium in their feces Magnesium (Mg) can be lost in jejenal or ileal
effluent Calcium deficiency can occur as unabsorbed
fatty acids in the small bowel Iron supplementation in microcytic anemia.
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Type of Enteral Nutrition Breastmilk
superior choice Immumnological benefit Improved mucosal adapatation Protective colonic bacterial flora- Lactoferrin
Semi elemental formulas contains protein hydrolysed to peptides LCTs and MCTs casein dominant , completely lactose free Ex- Alimentum (Abbot), Peptamen Junior
Elemental formulas the protein hydrolyzed to amino acids predominantly made up LCTs, Lactose free Ex- Neocate
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Laboratory monitoring Complete blood counts Electrolytes & renal function Blood glucose Serum triglycerides & Liver function test Serum Zinc, Copper, and Selenium monthly Vitamins A, D, E, and K every 1, 3, or 6 months Serum Citrulline levels as a marker for
small bowel length, absorption capabilities, and prognosis for weaning from PN
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Complications of SBS Small bowel bacterial overgrowth (SBBO) Diarrhoea and malabsorption Dehydration Micronutrient deficiencies Nutrient malabsorptions Complications related to TPN-
cholestasis, sepsis
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Medical Therapy H2 blocker / PPI- first 6-12 months Loperamide and codeine- anti-motility,
slow intestinal transit Octreotide- secretory diarrhea Cholestyramine- cholerheic diarrhoea -
binds bile acids Broad spectrum antibiotics-small bowel
bacterial overgrowth Clonidine- to reduce excess fluid/ostomy
losses
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Growth factors for intestinal adaptation
Glutamine- main fuel for enterocytes and is also a substrate for the synthesis of nucleic acids
Growth hormone (GH)- shown to increase colonic mass, enhance sodium and water absorption, and promote mucosal hyperplasia.
Probiotics- capable of stimulating growth of the mucosa in the lower gastrointestinal tract.
Fibre and Short chain fatty acids (Butyrate)- causes bulking of the stool and leads to a decrease in the whole transit time
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Follow up / Discharge Procedures
Follow up on a monthly basis. Diet history: Lactose and sucrose free
diet. Ensure Calcium supplement prescribed. Appropriate feed given Necessary micronutrient
supplementation eg. Vitamin B12 Ensure that all medications and
supplements are sucrose free.
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“Most infants and children with SBS have a
good prognosis, if effective nutritional
and medical therapy is provided for
intestinal adaptation.”
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References 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.
“The Nutritional Management of Short Bowel Syndrome of Infants and Children”, Pediatric Working Group Western Cape Red Cross Children’s Hospital, 2009
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.
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Thank You