ERS Annual Congress Milan

09–13 September 2017

Challenging clinical cases

CC5 Paediatric non-invasive ventilation

Monday, 11 September 2017

07:00-08:15

Blue 2 (North) MICO

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Challenging clinical cases : CC5

Paediatric non-invasive ventilation

Aims : This session will focus on two challenging cases that involve paediatric non-invasive ventilation.

It will address disease-specific challenges, including the hurdles in implementation and risk

management.

Tracks: Acute and chronic respiratory failure/sleep

Tags: Paediatric

Target audience: Clinician - Intensivist/critical care physician - Junior member - Nurse - Paediatrician -

Physiologist - Physiotherapist - Researcher - Resident - Respiratory physician - Sleep

specialist/technologist - Student – Trainee

Chairs : Hui Leng Tan (London, United Kingdom)

Non-invasive ventilation in a paediatric patient with a craniofacial disorder Brigitte Fauroux (Paris, France)

Non-invasive ventilation in a paediatric patient with a neuromuscular disorder Francois Abel (London, United Kingdom)

Th e ERS Practical Handbook of Noninvasive Ventilation provides a concise ‘why and how to’ guide to NIV from the basics of equipment and patient selection to discharge planning and community care.

Editor Anita K. Simonds has brought together leading clinicians and researchers in the fi eld to provide an easy-to-read guide to all aspects of NIV. Topics covered include: equipment, patient selection, paediatric indications, airway clearance and physiotherapy, acute NIV monitoring, NIV in the ICU, long-term NIV, indications for tracheostomy ventilation, symptom palliation, discharge planning and community care, and setting up an NIV service.

Th is Practical Handbook is a valuable reference and training resource for all NIV practitioners.

• User-friendly format with key point summaries• Focused on practical aspects and problem solving• Multiple choice questions to enable self-assessment

To buy printed copies, visit the ERS Bookshop in the World Village at the ERS International Congress 2017.

Electronic: WWW.ERSPUBLICATIONS.COMPrint: WWW.ERSBOOKSHOP.COM

ERS Practical Handbook of Noninvasive VentilationEdited by Anita K. Simonds

ISBN (print) 978-1-84984-075-0ISBN (ebook) 978-1-84984-076-7

€30 (ERS members)€40 (non-members)

NONINVASIVE VENTILATION...how to do it, why to do it,

when to do it and when to stop!

Thank you for viewing these presentations.

We would like to remind you that these

materials are the property of the authors.

It is provided to you by the ERS for your

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authors.

2017 by the authors

NIV in a pediatric patient

with craniofacial disorder

Brigitte Fauroux

Pediatric noninvasive ventilation and sleep unit

Research unit INSERM U 955

Necker university Hospital, Paris, France

Inserm

Institut national

de la santé et de la recherche médicale

Conflict of interest disclosure

I have no real or perceived conflicts of interest that relate to this presentation.

I have the following real or perceived conflicts of interest that relate to this presentation:

This event is accredited for CME credits by EBAP and EACCME and speakers are required to disclose their potential conflict of interest. The intent ofthis disclosure is not to prevent a speaker with a conflict of interest (any significant financial relationship a speaker has with manufacturers or providersof any commercial products or services relevant to the talk) from making a presentation, but rather to provide listeners with information on which theycan make their own judgments. It remains for audience members to determine whether the speaker’s interests, or relationships may influence thepresentation. The ERS does not view the existence of these interests or commitments as necessarily implying bias or decreasing the value of thespeaker’s presentation. Drug or device advertisement is forbidden.

Affiliation / Financial interest Commercial Company

Grants/research support:

Honoraria or consultation fees:

Participation in a company

sponsored bureau:

Stock shareholder:

Spouse / partner:

Other support / potential conflict of

interest:

Mainly caused by a mutation in the TCOF1 gene on chr 5q32: introduction of a

premature termination codon and the induction of a nonsense-mediated mRNA

degradation leading to reduced levels of TCOF1

Decreased ribosome biogenesisEnhanced p 53 expression

Decreased protein synthesis,

cell cycle arrest and apoptosis

Incidence 1/50.000

Treacher Collins

Boy with Treacher Collins syndrome

• Bilaterally symmetrical features

– hypoplasia of the facial bones

• mandibule (lower jaw)

• zygomatic complex (cheek bones)

– coloboma of the lower eyelids

– microtia or atresia of the external ears

– downward slanting of the palpebral fissures

–±cleft or high arched palate

Boy with Treacher Collins syndrome

• Tracheotomy at the age of 3 weeks

• Decanulation at the age of 11 months

• Follow up by the maxillofacial and ENT

team

• At the age of 9 years:

– no symptoms of sleep-disordered breathing

Q 1: What is your attitude ?

1. Clinical follow up: sleep study if symptoms

2. Sleep study if hypertrophy of the adenoids

and/or tonsils

3. Systematic sleep study

OSAS and congenital anomalies

• Analysis of OSAS cases in Washington state between 1987 and 2003 (CIM-9)

• 1203 OSAS cases matched with cases without OSAS (1/5)

• OSAS is associated with

– any cranio-facial anomaly RR 38

– facial cleft RR 40

– Down syndrome RR 51

– any other malformation RR 4.1

Lam et al. Laryngoscope 2010;120:2098

OSAS in children with

craniofacial anomalies

• 44 children (Crouzon, Apert, Goldenhar, Treacher

Collins, Pierre Robin), mean age 5 yrs

• Pediatric Sleep Questionnaire

– symptoms of airway obstruction 82%

• snoring 64%

• apneas 33%

• Polygraphy• mild OSAS 20%

• moderate OSAS 9%

• severe OSAS 15%

Luna-Paredes et al. Int J Pediatr Otorhinolaryngol 2012:76:1767

575 children 2-8 yrs,

followed at a craniofacial

center between

2007-2011, USA

Sleep-related breathing disturbance scale of the Pediatric Sleep Questionnaire

OSAS in Treacher Collins

syndrome

Akre et al. Eur Arch Otorhinolaryngol 2012;269:331

PSGChildren

n=8

Adults

n=11

Normal PSG

Mild OSAS (AHI 1-5/5-15)

Moderate OSAS (AHI 5-10/15-30)

Severe OSAS (AHI >10/>30)

1 (12.5%)

4 (50%

2 (25%)

1 (12.5%)

0 (0%)

3 (27%)

4 (36%)

4 (36%)

No corrélation between phenotypic severity, symptoms (snoring) and the AHI

patients with Treacher Collins syndrome MUST undergo a PSG

Norwegian National Register

Patients No OSASMild

OSAS

Moderate

OSAS

Severe

OSAS

Children

(n=13)6 1 5 1

Adults

(n=22)13 3 0 6

Boy with Treacher Collins syndrome

• Tracheotomy at the age of 3 weeks

• Decanulation at the age of 11 months

• Follow up by the maxillofacial and ENT

team

• At the age of 9 years:

– no symptoms of sleep-disordered breathing

– no hypertrophy of the adenoids/tonsils

– systematic PG: AHI 9 events/h

Q 2: What is your attitude ?

1. ENT surgery

2. Maxillar/mandibular distraction

osteogenesis

3. CPAP

When to start CPAP ?

• No validated criteria: lack of validated markers of

OSA-end-organ morbidity in children

• Recommendations:

Acute group

n=15

Subacute group

n=18

Chronic group

n=43

Age, years 1.2±3.4 6.4±7.2 5.9±7.1

Female/male 7/8 8/10 22/21

DiagnosisPierre Robin syndrome

Laryngomalacia

Polymalformative sd

Kabuki syndrome

Cystic fibrosis

BPD

Neuromuscular disorder

6

3

2

1

1

1

1

Laryngomalacia

Prader Willi syndrome

Pierre Robin syndrome

BDP

Craniostenosis

Treacher Collins sd

Vocal cord palsy

Down syndrome

Mucopolysaccaridosis

Duchenne MD

Laryngeal mass

Craniofacial malform.

Generalised dystonia

Achondroplasia

Tracheomalacia

4

1

1

1

1

1

1

1

1

1

1

1

1

1

1

Pierre Robin syndrome

Down syndrome

Mucopolysaccaridosis

Charge syndrome

Laryngomalacia

Neuromuscular disorders

Polymalformative syndrome

Treacher Collins syndrome

Achondroplasia

Prader Willi syndrome

BPD

Myhre syndrome

Spinal muscular atrophy

Rett syndrome

Goldenhar syndrome

Idiopathic OSAS

Hanhart syndrome

Beckwith Wiedemann sd

Loeys Dietz syndrome

Ossificant fibrodysplasia

5

5

4

3

3

3

3

2

2

2

2

1

1

1

1

1

1

1

1

1

76 children started on NIV (Necker 2013-2014)

Q 3: Which equipment & settings ?

Interface

1. Nasal interface

2. Nasobuccal interface

3. Nasal canula

CPAP

1. Constant CPAP

2. Autotitrated CPAP

3. Bilevel PAP

Interfaces for children

Nasobuccal mask

Devices able to deliver CPAP

No difference between CPAP and BiPAP

Marcus et al. Pediatrics 2006;117:e442

29 children with OSAS: CPAP or Bilevel PAP

1/3 not compliant at 6 months, mean use/night: 5.3 ± 2.5 hours

13 yrs: internal maxillar

distraction osteogenesis

Post-operative PG: AHI 2/h allowing CPAP weaning

Male / Female 38/20

Age at CPAP/NIV initiation (year), median (range) 1.4 (0.01-16.2)

Ventilatory mode, n (%)

CPAP NIV

50 (86%)

8 (14%)

Duration of CPAP/NIV (year), median (range)

CPAP NIV

1.12 (0.16-8.85)

0.98 (0.16-7.49)

3.96 (0.27-8.85)

Primary diagnosis, n (%) Laryngeal obstruction*

Pierre Robin syndrome

Prader Willi syndrome

Treacher Collins syndrome

Bronchopulmonary dysplasia

Achondroplasia

Idiopathic OSA

Craniofaciostenosis (Crouzon, Apert)

Pycnodysostosis

Mucopolysaccharidosis

Polymalformative syndrome

Mandibular hypoplasia

Lung sequelae of viral infection/ARDS

Other#

9 (16%)

6 (10%)

6 (10%)

6 (10%)

4 (7%)

3 (5%)

2 (3%)

2 (3%)

2 (3%)

2 (3%)

2 (3%)

2 (3%)

2 (3%)

10 (21%)

Reason of CPAP/NIV weaning, n (%) Spontaneous improvement: 33 (57%)

Switch to oxygen therapy: 2 (3%)

After specific intervention:

ENT surgery 14 (24%)

Maxillofacial surgery 6 (11%)

Neurosurgery 1 (2%)

ENT and neurosurgery 2 (3%)

2 years after MDO

• Pubertal spurt: + 15 cm in one year

• Snoring, frequent awakenings, daytime

sleepiness

• Decrease of school performances

Obstructive sleep apnea: AHI 24/h

Q 4: How would you start CPAP ?

1. Which interface ?

2. Which CPAP device ?

3. Which CPAP settings ?

4. How to monitor CPAP efficacy ?

1. Ventilator: Resmed S10

2. Interface: nasobuccal mask (mouth

breathing)

3. Pressure: highest tolerated pressure

during adaptation when awake

4. Monitoring: polygraphy with CPAP

Our choice

PG with CPAP 10 cmH2OPosition

Body mov.

Snoring

Apnea

Hypop

CPAP

Pression

Leaks

SpO2

HR

Hour

PWA

Heart

rate

SpO2

Pressio

n

Flow

Thoracic

belt

Abdomi

nal belt

Position

Left side

PG with CPAP 10 cmH2O

PG with CPAP 10 cmH2O

PWA

Heart

rate

SpO2

Pressio

n

Flow

Thoracic

belt

Abdomi

nal belt

Position

Dorsal

PG with CPAP 10 cmH2O

Overnight gas exchange

TcCO2

SpO2

Heart

rate

Q5: What is your diagnosis ?

Explanation….!

Explanation

PG with CPAP 10 cmH2O

Position

Body mov.

Snoring

Apnea

Hypop

OSAS became positional

with CPAP therapy

Persistant positional OA during

CPAP 10 cmH2O

Q 6: What is your attitude ?

1. Keep the same pressure and check

2. Increase CPAP pressure to 12 cmH2O

if tolerated

3. Change for a bilevel PAP

4. Change for an autotitrated CPAP

5. Avoid sleeping on the back

• Correction of residual apneas with a auto-

titrated CPAP 8-12 cmH2O

• Second maxillar distraction after

completion of puberty:

– AHI 4/h

– allowing a definitive weaning from CPAP

Evolution

Conclusion

• A systematic PG is recommended in all

patients with any craniofacial abnormality

• Long term follow up is mandatory

(adulthood)

• CPAP

– may avoid a tracheotomy

– allows the optimal timing of corrective surgery

• Management by a multidisciplinary team

having an expertise in sleep and CPAP/NIV