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Professor Antonino Morabito Dr Lisa Kauffmann Feb 2015

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1 Professor Antonino Morabito Dr Lisa Kauffmann Feb 2015
Gastro- oesophageal Reflux in Cerebral Palsy- medical and surgical management Professor Antonino Morabito Dr Lisa Kauffmann Feb 2015

2 GORD is a motility disorder.
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

3 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 Talk given by Professor Evans from Barts – very keen on his topic! LOS forms a barrier between the oesophagus and the stomach. The resting pressure is around mmHg (range of normal 10-30) in adults and older children. Lower in newborn, rises gradually. It straddles the chest and the abdomen with huge pressure differences between them anyway. Normal abdominal pressure is 0-5 mmHg. Normal intra-thoracic pressure is x x x x x Normally relaxes to gastric pressure in response to swallow. Can be a problem if gastric pressure v high (e.g. in obesity)

4 Allows passage of food from mouth to stomach
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. There is a physiological increase lower oesophageal pressure in response to raised intra-abdominal pressure - this can be abolished by atropine, and reduced after vagotomy or in reflux. TLOSR is a normal physiological phenomenon and occurs in response to gastric distension (to allow burping)! This becomes difficult after fundo. In healthy adults, it is suppressed by lying down. There is suppression in patients with reflux too – but not enough. Occurs very rarely in sleep. If it occurs at other times, it can cause problems.

5 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). LOS factors: Non-LOS factor: I mentioned earlier that anatomy contributes, and the angle of His is important. This is changed in gastrostomy, and may be one of the reasons that GORD increases after gastrostomy. Crural sling is the diaphragm bit. IAP can be increased in obesity, but also in severe scoliosis? and constipation?

6 Presentation in childhood
Expected symptoms Regurgitation Vomiting Pain Faltering growth Anaemia Irritability Stricture related symptoms Food refusal and food aversion Chronic chest problems None specific. complex relationship

7 Respiratory effects Can get overt aspiration, or chronic micro-aspiration Can produce pneumonia, wheezing and asthma, ALTEs, apnoea and cyanotic episodes. Also cough, stridor and hoarseness, and sore throat. The relationship between GOR and cough is filling several journals single handedly, Interesting paper form Australia where the respiratory society’s statement and the Gastroenterology society’s statement on this are different. Now that applies to children and adults, and not particularly to NI, but still interesting.

8 Other presentations Neurobehavioural Glue ear
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. Interestingly it may be the pepsin in refluxate that does damage rather than gastric acid.

9 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

10 Why does it matter? Children with severe cerebral palsy have markedly decreased life expectancy BUT will survive longer if FREE of GORD (probably) GORD contributes to a poor quality of life for child and family. Die from chronic malnutrition , or chest disease.

11 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?

12 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.

13 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

14 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

15 So what is the right answer?
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

16 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.

17 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?

18 Fundoplication Failure of fundoplication 5 0 % Failure includes:
Is in the region of 5 0 % 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, ,2004 Martinez DA, Ginn-Pease ME, Caniano DA: Sequelae of antireflux surgery in profoundly disabled children. J Pediatr Surg. Vol27, No2, , 1992

19 Fundoplication Failure
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 ?

20 GOR IN N.I. CHILDREN 71% return within 1 yr of operation with clinical complaints that pre-op had been associated with G O R D Islam S, Taitelbaum DH, Butain W, Hirscl RB: Esophagogastric separation for failed fundoplication in neurologically impaired children. J Pediatr Surg, 39,3, ,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: , 1990

21 .....with a subsequent potential need
GOR IN N.I. CHILDREN .....with a subsequent potential need for more complex & potentially risky redo-surgery

22 Great Ormond Street : Redo Fundo
Total: 71 Neurologically Normal Neurologically Impaired Persistent vomiting 29 (41%) 9 ( 31%) 20 (48%) Retching Gas bloat DysphagiaDumping 47 (66%) 18 (62%) 29 (69%)

23 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

24 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

25 Does relief of symptoms for an average duration
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

26 TOGD “ an alternative approach is needed in some situations, either as a primary procedure or following failure of previous management ” Bianchi Bianchi A: Total esophagogastric dissociation: an alternative approach. J Pediatr Surg, 32,9, , 1997 “Esophagogastric Dissociation” in Gastroesophageal Reflux in Infants and Children. Esposito C, Montupet P, Rothenberg S Chapter 31

27 Makes reflux less likely to happen
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

28 Patient Selection Severe neurodisability (GMFCS5) ?Severe hypotonia
Failure of other surgical therapies

29 Nutrition status Pre-operative and follow-up weights were available on 16 of the 24 children Malnutrition defined as weight for age standard deviation score (z-score) of -2 or less

30 32 TOGD 25 primary 7 rescue

31 TOGD mean weight standard deviation score
Pre-op : (-6.1 – 0) Post-op: (-6.32 – 1.25) (p = 0.005)

32 59 lapaoscopic funoplication
Local study 59 lapaoscopic funoplication 35 not GMFCS 5 24 GMFCS 5 26 TOGD 3 not GMFCS 5 23 GMFCS 5

33 Laparoscopic fundoplication TOGD p Subsequent anti-reflux surgery 3
Laparoscopic fundoplication TOGD p Subsequent anti-reflux surgery 3 >0.05 Subsequent PICU admissions 9 7 Acid reducing medications at last review 13 4 0.0349 Domperidone at last review Domperidone alone 10 6 Median length of clinic follow-up (Range) 5.8 years ( years) 6.25 years ( years) Deceased 5 >0.5

34 How easy is it to feed your child? 1.75 1.5 1.70 2.25
1 year 2 years Currently LF TOGD How easy is it to feed your child? 1.75 1.5 1.70 2.25 Does your child get uncomfortable during feeding? 2.5 2.7 Does your child choke/gag during feeding? 2.2 Does your child vomit? 2 1.25 1 Is retching a problem? Does your child experience constipation? 3 3.5 3.25 3.2 2.75 Does your child experience wind or bloating after feeding? 2.8 Does your child have difficulty swallowing e.g. saliva? 2.83 Does your child get frequent chest infections requiring antibiotics? How comfortable is your child generally? 2.3 How able is your child to enjoy life?

35 ‘ To F U N D O That is NOT the question ! or N O T to F U N D O ‘
best treatment for patients with special needs TOGD ‘ To F U N D O or N O T to F U N D O ‘ That is NOT the question !

36 TOGD: Individually Planned Surgery
- Fundoplication/ - TOGD : +/- Pyloroplasty, : Gastrostomy, NG feed / Feeding jejunostomy for high-risk patients (reflux not resolved)

37 We advocate TOGD as a Rescue TOGD : carries greater morbidity
Primary Definitive Procedure - in selected NI children Rescue TOGD : carries greater morbidity : difficult surgery : poor oesophageal tissue : vagal nerve injury

38 Conclusions 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

39 Conclusions Total oesophago-gastric dissociation has huge positive impact on the physical well being of the children with severe neuro disability suffering from gastro-oesophageal reflux. In the eyes of the carers the overall view of the procedure is one of success, surpassing all expectations TOGD also has a positive impact on the quality of life and attitudes of the families


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