Short Bowel Syndrome

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Short Bowel Syndrome

• Dr John Puntis
• The General Infirmary at Leeds

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Short Bowel Syndrome

• definition
• aetiology intestinal failure
• management
• complications
• prognosis
• surgery
• liver disease

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Short Bowel Syndrome

• malabsorption resulting from loss of part of the
  small intestine inadequate absorptive area, and
  the physiological consequences
• a reduction in the functioning intestinal mass
  below the amount necessary for adequate
  absorption to allow for growth

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Short Bowel Syndrome

• Common causes
• premature newborn NEC resection
• newborn - congenital anomalies (atresias,
  volvulus, etc.)
• older children - Crohns vascular
  malignancy
• most frequently ileum and proximal colon involved

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• Necrotising enterocolitis (NEC) BPSU Survey
  1993/94
• 300 cases
• 65 lt 1500g
• 30 required surgical intervention
• 22 overall mortality
• 0.23/1000 live births
• 2.1/1000 NICU admissions

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Severe malnutrition in ileal atresia pre-PN
era. Successful long term PN in such a patient
first described in 1968 by Wilmore and Dudrick
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Short Bowel Syndrome

• pathophysiology
• smaller surface area
• more rapid transit
• hypergastrinaemia
• neurohumoral changes

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Short Bowel Syndrome

• Consequences of loss of jejunum
• carbohydrate malabsorption
• osmotic diarrhoea
• iron, calcium, magnesium poorly absorbed
• hyperoxaluria (malabsorbed fat complexes with
  dietary calcium oxalate absorbed because calcium
  oxalate cannot form)

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Short Bowel Syndrome

• loss of ileum
• vitamin B12 deficiency (can present many years
  later, therefore need monitoring)
• bile salts not reabsorbed (may then cause fluid
  secretion in colon)
• vitamin D deficiency
• steatorrhoea fat soluble vitamin deficiency
  (A,D,E,K)

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Short Bowel Syndrome

• adaptation
• begins 24-48 hours after resection
• increase in bowel length, circumference, villus
  height, crypt depth
• enteral nutrition necessary for mucosal
  hyperplasia
• 4 x increase in absorptive capacity possible

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The effect of extensive resections of the small
intestineFlint JM Bull Johns Hopkins Hosp 1912
23127-143

• Dog No 4
• Escaped from the dog house in good condition

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Short Bowel Syndrome

• intestinal length increases x 1.7 during third
  trimester
• 200 cm small bowel in newborn
• 350 - 800cm in adult

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Short Bowel Syndrome

• initial management
• parenteral nutrition
• replacement of fluid/electrolytes
• continuous NG enteral feeding (1ml/hr)
• some units would feed orally rather than NG to
  minimise risk of later feeding disorder

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Short Bowel Syndrome

• Enteral nutritional management
• glucose polymers
• oligopeptides
• medium chain triglycerides
• fat soluble vitamins
• copper and zinc supplementation

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Eating problems are common after prolonged
PN encourage oral feeds/stimulation involve SLT
and OT
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Short Bowel Syndrome

• further management
• speech therapist oral stimulation programme
• gastrostomy
• cyclical parenteral nutrition
• psycho-social stimulation
• OT/physio assessment

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Short Bowel Syndrome

• medical management
• H2 blocker (for hypergastrinaemia)
• Loperamide (up to 0.2mg/kg qds)
• Cholestyramine (0.1 g/kg bd)
• treatment of small bowel overgrowth (usually
  metronidazole trimethoprim nystatin, but
  other regimens also used)

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Short Bowel Syndrome

• bacterial overgrowth
• results from
• hypomotile bowel causing stasis
• loss of ileo-caecal valve
• may be exacerbated by pharmacological acid
  suppression

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Short Bowel Syndrome

• bacterial overgrowth
• consequences include
• hydroxy fatty acids impair colonic water
  absorption
• ileal mucosal inflammation/colitis
• deconjugation of bile salts, further impairing
  fat digestion
• D-lactic acidosis

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Short Bowel Syndrome

• possible therapeutic interventions
• pectin
• glutamine
• trophic factors (EGF insulin)
• Saccharomyces boulardii
• (none of proven benefit)

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Surgery for Short Bowel Syndrome

• indications poorly defined
• To get off PN
• Bianchi (bowel lengthening)
• STEP (serial transverse enteroplasty)
• To improve motility, and hopefully enteral
  tolerance
• plication
• tapering (but some absorptive surface area lost)

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Bianchi bowel lengthening procedure
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STEP procedure
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Alex Malo gastroschisis. First patient to have
STEP procedure, Boston Childrens Hospital.
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Short Bowel Syndrome

• late complications
• parenteral nutrition associated cholestasis
• central venous catheter related
• specific nutritional deficiencies
• ileo-colic anastomotic ulceration
• bacterial overgrowth (D-lactic acidosis colitis)

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Short Bowel Syndrome

• survival (neonatal)
• lt 40 cm (n 35) 67
• 40 - 80 cm (n 51) 92
• (Goulet et al, 1991)

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Short Bowel Syndrome

• prognosis
• newborn, full enteral feeding established
• lt40 cm mean 27 months (3 - 84)
• 40 - 80 cm mean 14 months (1 - 70)
• (Goulet et al, 1991)

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Short Bowel Syndrome

• Prognosis not always simple
• 4 year old girl, 12cm jejunum following midgut
  volvulus survived without TPN! (Surana et al.
  JPGN 199419246-249)
• survival 8/9 patients without ileo-caecal valve
  and lt 40 cm small bowel
• (Chaet et al. JPGN 199419295-298)

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PN dependency difficult to predict simply on
basis of residual bowel length long term
survival in part depends upon avoiding major PN
related complications. Also, quality of
remaining bowel v. critical.
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Children referred to Birmingham ? Tx SBS 102
dysmotility 31 mucosal disorders 19
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IFALD

• IFALD aka PNAC , PNALD
• persistent (gt6 wk) elevation of liver function
  tests (ALP, ?GT) 1.5 x the upper reference range
  in patient receiving PN

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IFALD

• persistent (gt6 wk) elevation of liver function
  tests (ALP, ?GT) 1.5 x the upper reference range
  in patient receiving PN
• other causes of liver disease excluded

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IFALD

• persistent (gt6 wk) elevation of liver function
  tests (ALP, ?GT) 1.5 x the upper reference range
  in patient receiving PN
• other causes of liver disease excluded
• 3 types
• early (type 1)
• established (type 2)
• late (type 3)

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IFALD

• clinical presentation most commonly as jaundice
  (type 2)

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IFALD

• clinical presentation most commonly as jaundice
  (type 2)
• conjugated bilirubin gt50 µmol/L is a
  manifestation of significant liver disease

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IFALD

• clinical presentation most commonly as jaundice
  (type 2)
• conjugated bilirubin gt50 µmol/L is a
  manifestation of significant liver disease
• urgent review by NST with access to specialist
  hepatology advice

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IFALD

• clinical presentation most commonly as jaundice
  (type 2)
• conjugated bilirubin gt50 µmol/L is a
  manifestation of significant liver disease
• urgent review by NST with access to specialist
  hepatology advice
• diagnosis of IFALD should prompt
  multi-professional review of enteral and
  parenteral nutrition

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IFALD

• infection?
• other causes of liver disease?
• HPN and early hospital discharge?

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IFALD

• protein energy malnutrition as a feature of
  severe liver disease is masked by PN

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IFALD

• protein energy malnutrition as a feature of
  severe liver disease is masked by PN
• type 2 can progress to type 3 in a matter of
  weeks decompensation can be very rapid (high
  mortality rate on transplant list)

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IFALD

• protein energy malnutrition as a feature of
  severe liver disease is masked by PN
• type 2 can progress to type 3 in a matter of
  weeks decompensation can be very rapid (high
  mortality rate on transplant list)
• Working Group Xth International Symposium for
  small bowel transplantation (2007)

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IFALD

• Type 1
• 50 of infants on PN 4 12 wk
• total bilirubin lt 50 µmol/L
• USS echogenic
• Bx 50 portal tracts fibrotic changes
• potentially fully reversible

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IFALD

• Type 2
• bilirubin 50-100 µmol/L
• USS markedly echogenic liver, enlarged spleen,
  biliary sludge
• Bx fibrosis affecting gt50 of portal tracts
• potentially fully reversible

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IFALD

• Type 3
• features of liver failure
• low platelets coagulopathy
• severe fibrosis
• usually irreversible
• urgent transplantation

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IFALD

• Risk factors
• prematurity
• IUGR
• co-morbidity (mucosal disease BPD ischaemia)
• dysmotiity
• short bowel
• early (lt4wk) CVC infection
• CVC infection (gt3/yr)

• nutrient excess (lipid)
• poor aseptic technique
• delayed hospital discharge
• lack of enteral stimulation
• Mn Al bacterial overgrowth glyco-lithocholic
  acid choline

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IFALD

• Risk factors
• prematurity
• IUGR
• co-morbidity (mucosal disease BPD ischaemia)
• dysmotiity
• short bowel
• early (lt4wk) CVC infection
• CVC infection (gt3/yr)

• nutrient excess (lipid)
• poor aseptic technique
• delayed hospital discharge
• lack of enteral stimulation
• Mn Al bacterial overgrowth glyco-lithocholic
  acid choline

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Intestinal failure

• IF in West Yorkshire (2001/2002)
• 93 children received PN for gt28 days

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Intestinal failure

• IF in West Yorkshire (2001/2002)
• 93 children received PN for gt28 days
• 61 preterm
• 37 immature GIT
• 23 NEC
• 1 pseudo-obstruction

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Intestinal failure

• IF in West Yorkshire (2001/2002)
• 23 NEC
• 15 surgery
• 6/15 SBS as result
• 20 term infants
• 11 gastroschisis
• 3 malrotation volvulus
• 6 other

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Intestinal failure

• IF in West Yorkshire (2001/2002)
• 6/93 patients died
• 5 premature/NEC (3 care withdrawn 2 sepsis)
• 1 gastroschisis (died on Tx waiting list)

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Intestinal failure

• IF in West Yorkshire (2001/2002)
• 5 discharged on HPN
• 3 weaned to full enteral feeding
• 1 Tx for IFALD
• 1 remained on HPN at 2 years

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UK paediatric population is 11.6 million
Child with IFx lasting more than 28 days 1300
children per year
1200 15 new pts per year weaned off PN
discharged home
55 new pts per year stable and well on home PN
1200 per year Medical surgical attempts to
improve intestinal function
70 per year Train in Home PN
Successful - wean off PN
45 complications 30 improve with adjustments to
Rx
15 fulfil criteria
for small bowel Tx (isolated, or combined with
liver)
J Köglmeier, C Day and J W L Puntis. Clinical
outcome in patients from a single region who were
dependent on parenteral nutrition for 28 days or
more. Arch. Dis. Child. 200893300-302.
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Intestinal failure

• IFALD in Leeds
• 24/51 (47) long term PN (gt3/12)
• 8 mild IFALD (bili 50 100 µmol/L)
• all recovered normal liver function

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Intestinal failure

• IFALD in Leeds
• 24/51 (47) long term PN (gt3/12)
• 16 severe IFALD (bili gt100 µmol/L)
• 8 referred to Birmingham for assessment
• 6 listed for Tx

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Intestinal failure

• IFALD in Leeds
• of 6 listed for Tx
• 3 died on waiting list
• 2 transplanted
• 1 spontaneous improvement (off list)

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Intestinal failure

• IFALD in Leeds
• will fish oil emulsion change things?

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IFALD

• Fish oil
• improvement in cholestasis in a 17 yr boy with
  Soy allergy given Omegaven to
• correct EFAD

Mark Puder, Boston
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Pediatrics, Jul 2006 118 e197 - e201
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IFALD

• Mechanisms?
• phytosterols in soy emulsions
• ?-6 pro-inflammatory oxidative damage
• ( sepsis, surgery, drugs)
• ?-3 anti-inflammatory

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Graph demonstrating changes in serum bilirubin
over time. Week 0 represents start of Omegaven
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IFALD

• minimise sepsis
• maximise enteral nutrition
• bacterial overgrowth (inflammation)
• metronidazole
• reduction in lipid intake
• cessation of PN
• fish oil
• ceruletide

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Outcome of referrals for intestinal
transplantation
. . . . early referral
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. . . prevention