Presentation on theme: "Professor Antonino Morabito Dr Lisa Kauffmann Feb 2015"— Presentation transcript:
1 Professor Antonino Morabito Dr Lisa Kauffmann Feb 2015 Gastro- oesophageal Reflux in Cerebral Palsy- medical and surgical managementProfessor Antonino MorabitoDr Lisa KauffmannFeb 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 GORAllergic 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 refluxatethe mucosal defencesTalk 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 xNormally 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 - functionAllows passage of food from mouth to stomachPrevents reflux of gastric juicesRelaxes in response to swallowTransient relaxation allows release of airIncrease 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 pressureLOS lengthLength of intra-abdominal segmentVagal response to increase IAPExtrinsic to LOS: gravity and postureAngle of HisCrural slingIAPRatio 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 symptomsRegurgitationVomitingPainFaltering growthAnaemiaIrritabilityStricture related symptomsFood refusal and food aversionChronic chest problemsNone specific. complex relationship
7 Respiratory effectsCan get overt aspiration, or chronic micro-aspirationCan 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 earPepsin 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 sinusitisDental erosionEspecially 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 literatureabnormal foregut motilityphysical deformity of the GOJhigh intra-abdominal pressurechronic lung diseasebrain related
10 Why does it matter?Children with severe cerebral palsy have markedly decreased life expectancyBUTwill 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 neurodisabilityIn 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 continued4‑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 behaviourfaltering growth.Assess the response and consider referral to a specialist for possible endoscopy if the symptomsdo not resolve orrecur 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 refluxGaviscon - ↓ height of refluxate on impedanceDomperidone – no evidenceOmeprazole - ↓ 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/orrecommended 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 orif 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 oneInvestigations can be helpful, but often aren’tGaviscon has evidence and is easy and safePPI or H2RAs are easy to give and often help and NICE agreesFor children with CP benefits of motility stimulants (domperidone, erythromycin) usually outweigh risks .We usually recommend all 3Enteral tube feeding is part of solution for poor intake, but NOT for GORD
16 NICE: Surgery for GORDOffer 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 orfeeding 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 fundoplicationsIn infants after fundoplication50% continue to have weight problems20% need redo (50% wrap disruption, 44% intact wrap but herniation)70% stopped having apnoeasOutcome 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 of5 0 %Failure includes:herniation and/or slippage with crural disruptionIslam S, Taitelbaum DH, Butain W, Hirscl RB: Esophagogastric separation for failed fundoplication in neurologically impaired children. J Pediatr Surg, 39,3, ,2004Martinez 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 positionTension on wrapKyphoscoliosisSpasticity of abdominal musculature- pressure on GOJVomiting & RetchingAbnormal movement of the GOJFundo may trigger the development of gastric dysrhythmia that might facilitate postoperative retching? Seizures ?
20 GOR IN N.I. CHILDREN71% return within 1 yr of operation with clinical complaints that pre-op had been associated with G O R DIslam S, Taitelbaum DH, Butain W, Hirscl RB: Esophagogastric separation for failed fundoplication in neurologically impaired children. J Pediatr Surg, 39,3, ,2004Pearl 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 needfor more complex & potentially risky redo-surgery
22 Great Ormond Street : Redo Fundo Total: 71Neurologically NormalNeurologically ImpairedPersistent vomiting29 (41%)9 ( 31%)20 (48%)Retching Gas bloat DysphagiaDumping47 (66%)18 (62%)29 (69%)
23 GOR IN CHILDREN : GOS Experience. A A P, Washington DC Oct 2005 Redo-Fundoplication is associated withhigh rate of persistent GI symptomsThis 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 TOGDDoes relief of symptoms for an average durationof 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 procedureor following failure of previous management ”BianchiBianchi 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 GastrostomyOesophagus: detached from stomach.: connected to anIsoperistaltic Jejunal Roux loopBowel continuity: end-to-sidejejunojejunostomy at 40cmNon-refluxing Gastrostomy: Vascularized gastric tubeon right gastroepiploic pedicleMakes reflux less likely to happen
28 Patient Selection Severe neurodisability (GMFCS5) ?Severe hypotonia Failure of other surgical therapies
29 Nutrition statusPre-operative and follow-up weights were available on 16 of the 24 childrenMalnutrition defined as weight for age standard deviation score (z-score) of -2 or less
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 study59 lapaoscopic funoplication35 not GMFCS 524 GMFCS 526 TOGD3 not GMFCS 523 GMFCS 5
33 Laparoscopic fundoplication TOGD p Subsequent anti-reflux surgery 3 Laparoscopic fundoplicationTOGDpSubsequent anti-reflux surgery3>0.05Subsequent PICU admissions97Acid reducing medications at last review1340.0349Domperidone at last reviewDomperidone alone106Median length of clinic follow-up(Range)5.8 years( years)6.25 years( years)Deceased5>0.5
34 How easy is it to feed your child? 1.75 1.5 1.70 2.25 1 year2 yearsCurrentlyLFTOGDHow easy is it to feed your child?1.751.51.702.25Does your child get uncomfortable during feeding?2.52.7Does your child choke/gag during feeding?2.2Does your child vomit?21.251Is retching a problem?Does your child experience constipation?220.127.116.11.75Does your child experience wind or bloating after feeding?2.8Does your child have difficulty swallowing e.g. saliva?2.83Does your child get frequent chest infections requiring antibiotics?How comfortable is your child generally?2.3How 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 patientswith special needsTOGD‘ To F U N D OorN 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 jejunostomyfor high-risk patients (reflux not resolved)
37 We advocate TOGD as a Rescue TOGD : carries greater morbidity Primary Definitive Procedure- in selected NI childrenRescue TOGD : carries greater morbidity: difficult surgery: poor oesophageal tissue: vagal nerve injury
38 Conclusions Definitions are not clear High quality research does not existGORD is importantEliminating it is impossibleOur aim should always be best possible control for symptoms and quality of lifeDrug treatment is of limited benefit – but we do itFundoplication is sometimes helpful but is not the answer and often unhelpfulOther surgical options can also be considered
39 ConclusionsTotal 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 expectationsTOGD also has a positive impact on the quality of life and attitudes of the families