Anaesthetic issues for children with Down’s syndrome - PowerPoint Presentation

1. Anaesthetic issues for children with Down’s syndromeLiam BrennanAddenbrooke’s Hospital Cambridge

2. Addenbrooke’s Hospital founded 1766 Moved to current site late 1970’s Serve population of 1.5 million < 16 years old 6500 anaesthetics per year for < 16 years old >200 neonatal surgical procedures All surgical specialties (except cardiac) Paediatric trauma centre for East of England 12 consultant paediatric anaesthetists

3. About me Trained in London including year at GOSH Appointed consultant in Cambridge 1994 Mixed adult and paediatric practice RCoA lead paediatric adviser Co-author paediatric specialty guidelines Editor, RCoA CPD Journal RCoA Council Member & Vice-President Married with three sons Eldest son, Patrick has Angelman’s syndrome

4. DemographicsClinical features relevant to the anaesthetistPreoperative assessment & preparationAirway problemsCervical spine issues Where should DS children be treated?Transition to adult services

5. Anaesthetist’s knowledge of DS Explicitly referred to in the CCT curriculum Knowledge assessed in FRCA exams  Recalls/explains the implications of paediatric medical and surgical problems including major congenital abnormalities (eg tracheoesophageal fistula, diaphragmatic hernia,) congenital heart disease and syndromes e.g. Down’s for anaesthesia A 9 year-old child with Down’s syndrome is scheduled for adenotonsillectomya. List the cardiovascular (10%), airway/respiratory (25%) and neurological (15%) problems associated with this syndrome which are relevant to the anaesthetist.b. How may Down’s syndrome influence your preoperative (15%), intraoperative (20%) and postoperative (15%) management of this patient Included in many CPD articles http://ceaccp.oxfordjournals.org/content/3/3/83.full.pdf+html?sid=b89dc6bf-60a8-49bb-a05c-3ffe92df4459

6. Some facts and figures ~ 750 babies born with DS in UK each year ~65,000 people with DS in the UK Life expectancy has increased dramatically:↑antenatal diagnosis & terminationsMedical developments NB Cardiac surgery↑ equality of access to medical care Less institutionalised care ?Trisomy 21 protects against some cancersIncreased demand for surgery, anaesthesia & critical care 1990-2010; DS adults >40 yrs old has ↑by 75%

7. Surgery for DS patientsENT T&A’s Grommets Airway stenoses - ∆ & RxMax-fax Exodontia Conservation Craniofacial procedures Eyes Cataract removal Strabismus correctionCardiac Diagnostic procedures Palliative surgery Corrective surgeryPlastics Cleft surgeryNeuro Epilepsy surgery C-spine stabilisation* General GI endoscopy Anti-reflux surgery Atresia correctionsOther Vascular access LP/BM for leukaemia Rx CT/MRI* May also be managed by orthopaedics

8. Clinical features of interest to the anaesthetistGeneral Low birth weight Obesity Short statureRespiratory ↑ RTI& Airway OSA Airway narrowing Airway care tricky ↑Cleft lip/palate Cardiovascular Congenital heart disease Pulmonary hypertension Valvular heart disease ↑ Bradyarrythmias Difficult vascular accessNeurological Neonatal hypotonia Intellectual impairment Epilepsy A-A instability ↑ Autism ↑ Dementia at younger age ↑ Post-op agitationGastrointestinal GI reflux Gut atresiasEndocrine Hypothyroidism Diabetes (type 1 & 2)Haematological Polycythaemia ↑ Acute leukaemia Immunological ↓ IgA, ↓T-cell function ↑ Hepatitis B

9. Troublesome anatomical features of the DS airwayMacroglossiaNarrow midfaceOropharyngeal hypotoniaMicrognathiaSmall mouthShort neckIncreased incidence airway stenosis

10. Airway stenosis in DSAirways smaller in DS childrenDiscrete airway stenoses commoner in DS childrenChoanalSubglottic 2-6%TrachealCongenital subglottic stenosisOccurs in 1% DS childrenMay only become apparent with RTI, at intubation or during sedationSymptoms may be attributed to co-existing cardiac diseaseHistory of stridor, wheezing, high ventilatory pressures or previous difficult intubation raises suspicion 23% DS children require ET tube 1-2 mm smaller than predicted Post extubation stridor common; 35% following cardiac surgery v <2% for normal children

11. Paediatric OSA – the dilemma~ 12% of children snore0.7-1.8% have OSA; incidence in DS much higherDistinction may be difficult clinicallyImplications for safe perioperative care~ 25% paed T&As for obstructive symptomsFewer T&As performed; ?Large numbers OSA cases undiagnosed

12. Which children get OSA?Peak age 3-6 yearsAdenotonsillar hypertrophy- commonestDown’s syndromeNeuromuscular disease e.g. cerebral palsyCraniofacial abnormalitiesAchondroplasiaMucopolysaccharidosesObesity

13. Which children get OSA?

14. Symptoms & signsDaytimeNeurobehavioural disorders Failure to thriveCor pulmonale – rare Night timeSnoringRestless sleepApnoeasHead extensionSecondary enuresisExcessive sweatingAll may be features of DS or associated co-morbidities

15. Overnight oximetry trace.

16. Examination & investigationENT examination - assess nasal airflowOvernight oximetry home v hospital Useful screening testHigh positive predictive value (97%) but low negative predictive value (53%)Full sleep study Gold standard, but scarce resourceAdditional investigations ECG/echo – right heart impairment, but late sign

17. Who should be managed in specialist centres?Age < 2 yearsWeight < 15 kgsFailure to thriveObesityHypotonia/neuromuscular discorder/CPSerious craniofacial abnormalityMucopolysaccharidosesSerious co-morbidity e.g. congenital heart diseaseClinical/ECG/Echo findings of right heart dysfunctionSevere OSA on sleep study/oximetry ; SpO2 nadir <80%DS children may have many of these problems

18. Cervical spine issues10-20% DS patients have AAIAlmost all asymptomatic Perioperative spinal cord injury very rare1 possible case in ASA closed claims database (6,500 cases spinal injury)8 case reports 1966-2003; laryngoscopy implicated in two casesValue of routine radiological screening unclearClinical symptoms currently the most useful predictor of risk‘Cervical spine x-rays have no predictive validity for subsequent acute dislocation/subluxation at the A-A joint ’‘Anaesthetists should be alerted to the possibility of C-spine injurywhilst manipulating the head & neck in unconscious patients’ DSMIG guidelines

19. Screening questionsDoes your child have any known problems with their neck?Have they developed any new difficulties with walking?Have you noticed that they are more clumsy than usual?Has your child developed any new bowel or urinary incontinence?Has you child complained of neck pain?Does you child refuse or seem unable to turn their head? Positive responses should prompt further assessment Consider deferring elective surgery particularly if difficult intubation predicted and/or surgery will involve neck being positioned beyond neutral (NB ENT and cleft surgery)

20. Intraoperative management Avoid extreme neck movements during anaesthesia and surgery whenever possible; mandatory if known AAI Ensure surgeon aware of potential problems Discuss alternative positioning for ENT/cleft procedures Use laryngeal mask airways if possible If intubation required use airway adjuncts to minimise neck extension during laryngoscopy If symptomatic AAI consider perioperative spinal cord monitoring

21. Where should DS children be treated?Children should be treated as close to home as possibleBUT Specialist children’s surgical services increasingly centralised Specialist paediatric anaesthetic services co-located Paediatric anaesthetic skills (≥ 1 year advanced training) relatively rare in DGH Children with DS frequently require anaesthetists with advanced training Experienced support services (inc PICU/HDU) sparse outside specialist centresAll under 1 year oldSignificant OSA and/or airway stenosisUncorrected CHD other than uncomplicated AVSDCorrected CHD if requiring on-going medical therapyKnown or suspected AAI Anticipated need for PICU/HDU Needs exceeds local surgical, anaesthetic, nursing capacity

22. Transition to adult services Increasing numbers of DS patients accessing adult servicesTransitional arrangements well developed for some areas of medical practice e.g. cystic fibrosisTransitional policies for perioperative care lackingAustralia (2002) - Surgical admissions to tertiary paediatric hospital for > 18 year olds doubled in 10 year period; no transitional plan for 50% of adolescent patientsUK (2011) - 5% hospitals have transitional arrangements in place Adult trained staff lack expertise in managing DS patients with complex medical and psychosocial needs

23. 19 year old man with severe CP and developmental delay Body weight 35 kgListed for femoral osteotomyPaediatric bed arranged but no space available so admitted to adult orthopaedic wardSurgery by adult-trained surgeon and anaesthetistSevere pain in recovery room Adult morphine PCA regimen commencedReturned to side room on adult ward with mum in attendance6 hours post op – profound respiratory depressionMum had been activating the PCA device on son’s behalfTransferred to paediatric HDU – uneventful recovery

24. Some thoughts……Start transfer discussions early – age 14 suggestedDon’t forget surgical & anaesthetic needsEncourage close liaison between paediatric and adult surgical/anaesthetic colleagues Remember care may need to be transferred from a tertiary paediatric to an adult centre

25. Thank you!