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Respiratory Disorders 1(or Pulmonary Potpourri)
Thomas Lahiri MDProfessor of Pediatrics
Larner College of MedicineUniversity of Vermont
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Disclosure I have no relevant financial relationships with the
manufacturers(s) of any commercial products(s) and/or provider of commercial services discussed in this CME activity.
I do not intend to discuss an unapproved/investigative use of a commercial product/device in my presentation.
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Objectives Identify causes of stridor in children Formulate a differential diagnosis for noisy
breathing Understand indications for further
evaluation of children with suspected airway or pulmonary malformations
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Lecture Content
Noisy Breathing & Stridor Laryngeal Disorders Vascular Airway Compression Pulmonary Malformations
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Noisy Breathing Stridor
Inspiratory, often high pitched noise Heard best over face, neck
Wheeze Primarily expiratory Monophonic and transmitted
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Stridor- By Age Neonatal: congenital and acquired Toddlers: infectious vs. non-infectious
Croup Laryngeal foreign body Vascular compression
School Aged/Adolescent Vocal cord dysfunction Supraglottitis
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Neonatal Stridor Laryngomalacia Vocal cord paresis Subglottic stenosis (congenital or
acquired) Subglottic/tracheal cysts/web Hemangiomas Vascular compression of the airways
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Laryngeal Anatomy
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The Floppy Airway Laryngomalacia—usually inspiratory
stridor heard best over the face/neck Tracheobronchomalacia—primarily
expiratory monophonic wheeze Noise may be accentuated in the supine
position, with activity or illness
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The Floppy Airway
“Raspy” breathing Often relieved with position changes No response to bronchodilator Resolution by 18-24 months in most cases
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Severe Laryngomalacia
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Laryngomalacia
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Vocal Cord Paresis• Unilateral• Hoarse cry• Inspiratory stridor• More common on left
(recurrent laryngeal)• Birth trauma• Surgical- PDA ligation
• Bilateral• Weak cry• May have stridor• Aspiration• Traumatic intubation• Chiari II malformation• Prolonged NGT
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Anterior Commissure Web
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Interarytenoid Web
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Severe Subglottic Stenosis
Congenital
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Post-intubation Stenosis
Grade III Stenosis
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Neonatal Stridor: Who Needs a Scope?
Feeding difficulties Poor weight gain Apnea Cyanosis Progressively increasing work of breathing Small minority of cases require surgery
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Vascular Rings Double aortic arch
Encircles trachea and esophagus
Right sided arch With aberrant lt. subclavian or mirror image
branching
Aberrant right subclavian artery Anomalous innominate artery Pulmonary artery sling
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Vascular Ring: Presentation Persistent or increasing stridor/wheeze Feeding intolerance (choking/gagging) Also may present with
Apnea/cyanotic spells Tachypnea/respiratory distress/exertional sx Failure to thrive Recurrent pneumonia Dysphagia
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Double Aortic Arch
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Innominate Artery Compression
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Right Aortic Arch
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Double Aortic Arch
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Double Aortic Arch
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Congenital Pulmonary Malformations
CPAMs are often detected by prenatal ultrasound
Presence of large CPAM and fetal hydropsis ominous
Even regressing lesions should be evaluated after birth
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CPAM Presenting Symptoms Unexplained neonatal respiratory distress Noisy breathing
Persistent wheeze/stridor Recurrent pneumonia/infections Swallowing problems Persistent infiltrate on radiographs
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Congenital Thoracic Malformations
Bronchogenic cyst Foregut cyst Duplication cyst
Lobar overinflation Cystic adenomatoid malformation Bronchopulmonary sequestration
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Bronchogenic Cyst Abnormal budding that occurs at various
points during gestation Early= mediastinal cysts Late = pulmonary cysts Size of cyst often related to
presentation
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Bronchogenic Cyst Mediastinal cysts may cause severe
respiratory distress May contain multiple cell types
(esophageal and respiratory origin) Secondary infection may develop Surgery is usually indicated
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DOL #1 5 months
Fetal ultrasound showed left CLLNoisy breathing since birth
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Cyst
Contrast study repeated during bronchoscopy without ETT in place
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Large Hyperlucent Lung Lobar “emphysema”- actually overinflation LUL>RML>RUL>lower lobes Ball valve mechanism due to abnormal
bronchus Occurs following airway development Surgery often indicated
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Lobar Overinflation
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Lobar Overinflation
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Lobar Overinflation
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Congenital Cystic Adenomatoid Malformation
Communication with bronchial tree
Usually unilobar (no particular location)
Mix of cysts with adenomatous tissue
5 pathologic types
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CCAMs
Surgical resection indicated
Potential for malignant transformation
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S12‐6664
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S12‐6664
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Sequestrations Non-functioning lung tissue Supplied by systemic circulation Diagnose by CT/MRI (feeding vessel) Lower lobes most common Two types:
Intralobar Extralobar
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Sequestrations Intralobar
More common Supplied by pulmonary
& systemic circulation Late presentation
(30% are < 10yrs) Recurrent pneumonia
Extralobar Rare More common in
males Systemic arterial and
venous supply Neonatal presentation Other anomalies
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1st day of life Age 3 weeks
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