Respiratory Concerns in Children with Down Syndrome …Respiratory Concerns in Children with Down...
Transcript of Respiratory Concerns in Children with Down Syndrome …Respiratory Concerns in Children with Down...
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Respiratory Concerns in
Children with Down Syndrome
Paul E. Moore, M.D.Associate Professor of Pediatrics and Pharmacology
Director, Pediatric Allergy, Immunology, and Pulmonary Medicine
Vanderbilt University School of Medicine
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Respiratory Concerns in Children
with Down Syndrome
• Overview
• Respiratory infections
• Airway issues
• Sleep issues
• Pulmonary vascular issues
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Respiratory Concerns in Children
with Down Syndrome: Overview
• Most common reason for children to be admitted to the hospital.
• Respiratory infections can be more severe, and hospitalization often results in admission to the intensive care unit.
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Anatomical features in DS that
contribute to respiratory concerns
• Craniofacial features
– Narrowed nasopharynx
– Flattened mid-face
– Macroglossia
• Adenotonsillar hypertrophy
• Airway size
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Other features of DS that contribute
to respiratory concerns
• Low tone (hypotonia)
– Upper airway muscles: dysphagia
– Airway: malacia
• Gastroesophageal reflux
– Contribution to adenotonsillar hypertrophy
– Contribution to airway inflammation
• Cardiac disease
• Obesity
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Pearl #1: Anatomical features and
physiologic contributors specific to
Down Syndrome result in malacia
• Definition: softening or loss of consistency in any of the organs or tissues
• Origin: Greek malakía
softness, tenderness, weakness
• Laryngomalacia, tracheomalacia, bronchomalacia, airway malacia
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Respiratory Concerns in Children
with DS: Respiratory Infections
• Respiratory infection is a significant burden.
– Bronchiolitis
– URI/LRTI: upper respiratory infection and
lower respiratory tract infection
– Pneumonia
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Respiratory Concerns in Children
with DS: Respiratory Infections
• The specific defect in the immune system is not clear, although a number of studies suggest developmental delay.
• Chronic aspiration is a significant contributor.
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Pearl #2: Developmental delay in
Down Syndrome can refer to more
than the nervous system.
• Coordination of swallow
• Immune development
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Pearl #3: Water (reflux) damage can
be significant in children with DS.
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Symptoms of chronic aspiration
• Chronic cough
• Wheezing not controlled by asthma medications
• Pneumonia
• Radiographic findings that suggest chronicity
– Right middle lobe syndrome
– Bronchiectasis
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Evaluation for recurrent pneumonia
• Swallowing evaluation to look for aspiration
• Studies looking for reflux
• Chest radiograph when well
• CT scan
• Bronchoscopy and bronchoalveolar lavage
• Immune evaluation
• Allergy evaluation
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Respiratory Concerns in Children
with Down Syndrome: Airway Issues
Symptoms that reflect airway issues:
• Stridor: Inspiratory wheeze that suggests upper airway obstruction.
• Wheeze: Expiratory wheeze that suggests lower airway disease, including asthma.
• Dyspnea: Shortness of breath
• Retractions: Use of accessory muscles
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Upper airway abnormalities
• Narrowing of the nasopharyngeal passages
• Macroglossia
• Adenotonsillar hypertrophy
• Laryngomalacia, worsened by reflux
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Lower airway abnormalities
• Subglottic stenosis
– May be more likely following intubation, as
tracheal diameter is smaller
• Tracheobronchomalacia
• Tracheal stenosis
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Definition of asthma
• Chronic inflammation
• Airway reactivity to specific triggers
• Reversible airway obstruction
• Manifest as symptoms that can include cough, wheeze, and dyspnea
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Asthma in Down Syndrome
• Studies have been equivocal about whether there is increased risk of asthma in children with DS.
• Infants with DS who have bronchiolitis, including RSV, are more likely to require hospitalization.
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Evaluation for airway issues
• Careful history
• Physical exam
• Chest radiograph
• Bronchoscopy
• Sleep study
• Echocardiogram
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Respiratory Concerns in Children
with Down Syndrome: Sleep Issues
• Impact on other development
• Obstructive sleep apnea
• Review of anatomic features
• Increased BMI associated with OSA
• AAP recommendation: Screening at age 1
• Importance of sleep study
• Complication: pulmonary hypertension
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Respiratory Concerns in Children
with Down Syndrome: Pulmonary
vascular issues
• Heart disease, with increased pulmonary blood flow
• Hypoxia
• Airway obstruction
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Pulmonary hypertension in
newborns with DS
• Persistent pulmonary hypertension
• Can occur with or without heart disease
• “Delay” in vascular remodeling
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Review of Pearls
• Anatomical features and physiologic contributors specific to DS result in malacia.
• Developmental delay in DS can refer to more than the nervous system.
• Water damage can be significant.
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What Health-Care Providers Can
Do to Reduce Respiratory Disease
• Look for aspiration.
• Vaccinate against influenza and pneumococcus.
• Address sleep issues.
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What Parents Can do to Reduce
Respiratory Disease
• Reduce exposure to viral respiratory infections.
• Maintain a healthy lifestyle to boost the immune system and to reduce obesity.
• Reduce exposure to environmental tobacco smoke.
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Suggested Reading
Pulmonary Complications of Down Syndrome during Childhood
Karen M. McDowell and Daniel Craven, Cincinnati Children’s Hospital Medical Center
Journal of Pediatrics, 2011