Gastro- oesophageal Reflux in
Cerebral Palsy- medical and surgical
management
Professor Antonino Morabito
Dr Lisa Kauffmann
Feb 2015
Why is it a problem?
• GORD is a motility disorder.
• The Lower Oesophageal Sphincter is the
critical area.
• 2 main mechanisms:
– Mechanical GOR
– Allergic Reflux
Lower Oesophageal Sphincter
• Lower oesophageal sphincter not a brilliant design.
• Straddles the chest and abdominal cavities.
• Normally relaxes to gastric pressure in response to
swallow.
• What matters to the pathology is:
– the mechanisms for clearing the refluxate
– the mucosal defences
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•
•
•
•
LOS - function
Allows passage of food from mouth to
stomach
Prevents reflux of gastric juices
Relaxes in response to swallow
Transient relaxation allows release of air
Increase lower oesophageal pressure in
response to raised intra-abdominal pressure –
abolished by atropine, and reduced after
vagotomy or in reflux.
Mechanical factors contributing to GOR
• Intrinsic to LOS:
resting LOS pressure
LOS length
Length of intra-abdominal segment
Vagal response to increase IAP
• Extrinsic to LOS: gravity and posture
Angle of His
Crural sling
IAP
Ratio IAP to intra-thoracic pressure
(increased in respiratory pathology).
Presentation in childhood
• Expected symptoms
– Regurgitation
– Vomiting
– Pain
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•
•
•
•
•
Faltering growth
Anaemia
Irritability
Stricture related symptoms
Food refusal and food aversion
Chronic chest problems
Respiratory effects
• Can get overt aspiration, or chronic microaspiration
• Can produce pneumonia, wheezing and
asthma, ALTEs, apnoea and cyanotic episodes.
Also cough, stridor and hoarseness, and sore
throat.
Other presentations
• Neurobehavioural
– infant spells, Sandifer’s syndrome,
• Glue ear
– Pepsin found in effusion in glue ear! (Newcastle) and
another group found helicobacter in the middle ear.
– Pepsin has also been found in broncho-alveolar lavage
fluid
• ?role of reflux in laryngomalacia and sinusitis
• Dental erosion
– Especially back teeth.
GORD occurs in 15-75% of children with
neurodisability
• Why is not clear from literature
– abnormal foregut motility
– physical deformity of the GOJ
– high intra-abdominal pressure
– chronic lung disease
– brain related
NICE guideline on GORD in infants an children
(Jan 2015)
• Research recommendation:
– What are the symptoms of GORD in infants, children and
young people with a neurodisability
– In infants, children and young people with overt or occult
reflux, is fundoplication effective in reducing acid reflux as
determined by oesophageal pH monitoring?
NICE 2015 continued
• 4-week trial of a PPI or H2RA for children with neurodisability
who have overt regurgitation with 1 or more of the following:
– unexplained feeding difficulties (for example, refusing feeds, gagging or
choking)
– distressed behaviour
– faltering growth.
• Assess the response and consider referral to a specialist for
possible endoscopy if the symptoms
– do not resolve or
– recur after stopping the treatment.
• Do not offer metoclopramide, domperidone or erythromycin to
treat GORD without seeking specialist advice and taking into
account their potential to cause adverse events.
Medical Management - evidence
• Thickeners - ↓episodes of vomiting, no change in
reflux
• Gaviscon - ↓ height of refluxate on impedance
• Domperidone – no evidence
• Omeprazole - ↓ acid in children with NI, no effect on
motility (Is study!)
• CMP free diet – No change in measured reflux,
↓symptoms
Enteral tube feeding for GORD
• Only consider enteral tube feeding to promote weight gain in
infants and children with overt regurgitation and faltering
growth if:
– other explanations for poor weight gain have been explored and/or
– recommended feeding and medical management of overt regurgitation is
unsuccessful.
• Consider jejunal feeding for infants, children and young
people:
– who need enteral tube feeding but who cannot tolerate intragastric feeds
because of regurgitation or
– if reflux-related pulmonary aspiration is a concern.
• AMOR and LK say: enteral feeding is NOT a
treatment for GORD
So what is the right answer?
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•
•
•
Of course there isn’t one
Investigations can be helpful, but often aren’t
Gaviscon has evidence and is easy and safe
PPI or H2RAs are easy to give and often help and
NICE agrees
• For children with CP benefits of motility stimulants
(domperidone, erythromycin) usually outweigh risks .
• We usually recommend all 3
• Enteral tube feeding is part of solution for poor
intake, but NOT for GORD
NICE: Surgery for GORD
• Offer an upper GI endoscopy with oesophageal biopsies
before deciding whether to offer fundoplication for
presumed GORD.
• Consider oesophageal pH study (or combined
oesophageal pH and impedance if available) and an
upper GI contrast study before deciding whether to offer
fundoplication.
• Consider fundoplication in infants, children and young
people with severe, intractable GORD if:
– appropriate medical treatment has been unsuccessful or
– feeding regimens to manage GORD prove impractical, for
example, in the case of long-term, continuous, thickened enteral
tube feeding.
So what is the right surgery?
• Fundoplication can help, but results are very
poor:
• This surgery has a higher failure rate than anything else we
do” “But it is the best we can do”
• In 2006 GOSH reported 850 fundoplications
• In infants after fundoplication
– 50% continue to have weight problems
– 20% need redo (50% wrap disruption, 44% intact wrap but herniation)
– 70% stopped having apnoeas
• Outcome better if uncomplicated reflux (ie no other
abnormalities)
• Quoted 15% failure rate after fundo in NI – but what is
failure?
Fundoplication
Failure of fundoplication
Is in the region of
50%
Failure includes:
herniation and/or slippage with crural
disruption
Islam S, Taitelbaum DH, Butain W, Hirscl RB:
Esophagogastric separation for failed fundoplication in
neurologically impaired children. J Pediatr Surg,
39,3,287-291,2004
Martinez DA, Ginn-Pease ME, Caniano DA: Sequelae of
antireflux surgery in profoundly disabled children. J
Pediatr Surg. Vol27, No2,267-273, 1992
Fundoplication Failure
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•
•
•
•
•
•
•
Prolonged supine position
Tension on wrap
Kyphoscoliosis
Spasticity of abdominal musculature- pressure on
GOJ
Vomiting & Retching
Abnormal movement of the GOJ
Fundo may trigger the development of gastric
dysrhythmia that might facilitate postoperative retching
? Seizures ?
GOR IN N.I. CHILDREN
71% return within 1 yr of operation with clinical
complaints that pre-op had been associated with
GORD
Islam S, Taitelbaum DH, Butain W, Hirscl RB: Esophagogastric separation for failed fundoplication in
neurologically impaired children. J Pediatr Surg, 39,3,287-291,2004
Pearl RH, Robie DK, Ein SH, et al: Complications of gastroesophageal antireflux surgery in neurologically
impaired versus neurologically normal children. J Pediatr Surg 25:1169-1173, 1990
GOR IN N.I. CHILDREN
.....with a subsequent potential need
for more complex & potentially risky redosurgery
Great Ormond Street : Redo Fundo
Neurologically
Total: 71
Normal
1994-2004
Neurologically
Impaired
Persistent 29 (41%)
vomiting
9 ( 31%)
20 (48%)
Retching 47 (66%)
Gas bloat
Dysphagia
Dumping
18 (62%)
29 (69%)
GOR IN CHILDREN :
GOS Experience. A A P, Washington DC Oct 2005
Redo-Fundoplication is associated with
high rate of persistent GI symptoms
• This operation does not control vomiting in half
of the children with neurodisability, and & one
third of normally developing children
GOR IN CHILDREN :
GOS Experience. A A P, Washington DC Oct 2005
Other surgical strategies should be considered
in children with neurodisability and G O R D
&
recurrent G O R D
TOGD
Does relief of symptoms for an average duration
of 1 yr justify an antireflux operation?
Can a “perfect” antireflux procedure be developed ?
Should we concentrate in feeding difficulties?
Is quality of life important?
Martinez DA et al: Sequelae of Antireflux Surgery in Profoundly Disabled Children.
JPS, Vol 27, No 2, 1992
TOGD
“ an alternative approach is needed in some situations,
either as a primary procedure
or following failure of previous management ”
Bianchi - 1 9 9 7
Bianchi A: Total esophagogastric dissociation: an alternative approach. J Pediatr Surg, 32,9,1291-94, 1997
“Esophagogastric Dissociation” in Gastroesophageal Reflux in Infants and Children. Esposito C, Montupet P, Rothenberg S Chapter 31
Bianchi’s procedure: TOGD + Non-Refluxing Gastrostomy
Oesophagus
: detached from stomach.
: connected to an
Isoperistaltic Jejunal Roux loop
Bowel continuity
: end-to-side
jejunojejunostomy at 40cm
Non-refluxing Gastrostomy
: Vascularized gastric tube
on right gastroepiploic pedicle
Makes reflux less likely to happen
Patient Selection
• Severe neurodisability (GMFCS5)
• ?Severe hypotonia
• Failure of other surgical therapies
32 TOGD
• 25 primary
• 7 rescue
TOGD
mean weight standard deviation score
1
.5
0.0
0
-.5
-1
-1.0
-2
-1.5
-3
-2.0
-4
-2.5
-5
N=
-3.0
6
N=
WTZPRE
6
WTZPOST
Pre-op : -2.63 (-6.1 – 0)
Post-op: -0.96 (-6.32 – 1.25)
(p = 0.005)
Local study
59 lapaoscopic
funoplication
35 not GMFCS 5
26 TOGD
24 GMFCS 5
3 not GMFCS 5
23 GMFCS 5
Laparoscopic fundoplication
TOGD
p
Subsequent anti-reflux surgery
3
0
>0.05
Subsequent PICU admissions
9
7
>0.05
Acid reducing medications at last review
13
4
0.0349
Domperidone at last review
10
6
>0.05
Domperidone alone
0
4
Median length of clinic follow-up
(Range)
5.8 years
(2.1-8.5 years)
6.25 years
(2.8-10 years)
>0.05
Deceased
9
5
>0.5
1 year
LF
2 years
TOGD
LF
Currently
TOGD
LF
TOGD
How easy is it to feed your child?
1.75
1.5
1.75
1.5
1.70
2.25
Does your child get uncomfortable during feeding?
2.25
1.75
2.5
1.75
2.7
2.25
Does your child choke/gag during feeding?
1.75
1.75
1.75
1.5
2.2
1.75
Does your child vomit?
2
1.25
2
1
2.2
1
Is retching a problem?
1.75
2.25
1.75
2.25
2.2
2.5
Does your child experience constipation?
3
3.5
3.25
3
3.2
2.75
Does your child experience wind or bloating after feeding?
2.5
2
2.5
1.75
2.8
1.5
Does your child have difficulty swallowing e.g. saliva?
3.25
2.5
3.25
2.5
2.83
2.75
Does your child get frequent chest infections requiring antibiotics?
2.5
2.25
2.5
2.25
2.7
1.75
How comfortable is your child generally?
2.5
1.25
2.25
1.25
2.3
2
How able is your child to enjoy life?
3.5
2
3
1.75
3
1.5
TOGD
best treatment for patients
with special needs
‘ To F U N D O
or
N O T to F U N D O ‘
That is NOT the question !
TOGD: Individually
Planned Surgery
- Fundoplication/
- TOGD
: +/- Pyloroplasty,
: Gastrostomy,
NG feed / Feeding jejunostomy
for high-risk patients (reflux not resolved)
TOGD
We advocate TOGD as a
Primary Definitive Procedure
- in selected NI children
Rescue TOGD : carries greater morbidity
: difficult surgery
: poor oesophageal tissue
: vagal nerve injury
Conclusions
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•
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Definitions are not clear
High quality research does not exist
GORD is important
Eliminating it is impossible
Our aim should always be best possible control for symptoms
and quality of life
• Drug treatment is of limited benefit – but we do it
• Fundoplication is sometimes helpful but is not the answer and
often unhelpful
• Other surgical options can also be considered