CASE DISCUSSION Short Bowel Syndrome

(B/o Sonawane S)

Dr Sunil Deshmukh KEM Hospital Pune

Antenatal History Age -30 yrs G1P0 Antenatal period –spotting since 4th

month Antenatal USG –normal

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

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

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

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)

On days 16th – persistant oozing of fecal material from Surgical site

Planned re-exploration

Baby refer to KEM hospital for further management.

on admission – Active Weight 1.4 kg Pulses , perfusion –normal No organomegaly Discharge from surgical site ++

Feed started OGT EBM, Graded up

gradually

Again developed S/o intestinal obstruction

On day 30th Second Exploratory Laparotomy ---- Adhenolysis + Jejuno-Ascending Colon anastomosis

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

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

Current status Nutrition & weight gain- Anemia - Dyselectrolytemia - High fistula output - Hypoalbuminemia - Acidosis

THE NUTRITIONAL MANAGEMENT OFSHORT BOWEL SYNDROME

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

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

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

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

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.

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

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.

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

Predictors of mortality Cholestasis (parenteral nutrition-

associated),(conjugated bilirubin >2

mg/dL) % of remaining small bowel length Presence of ICV (ileocecal valve)

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

“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.

Nutritional proceduresThe nutritional management of SBS

involves 3 phases, namely1. Acute phase 2. Adaptation phase3. Maintenance phase

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

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.

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.

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

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.

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.

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.

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.

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

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.

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

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)

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.

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

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

Complications of SBS Small bowel bacterial overgrowth (SBBO) Diarrhoea and malabsorption Dehydration Micronutrient deficiencies Nutrient malabsorptions Complications related to TPN-

cholestasis, sepsis

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

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

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.

“Most infants and children with SBS have a

good prognosis, if effective nutritional

and medical therapy is provided for

intestinal adaptation.”

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.

Thank You