Anatomical findings: upper and lower airways · 2019. 4. 30. · Ernst Eber, MD, ATSF, FERS Division of Paediatric Pulmonology and Allergology, Dept of Paediatrics and Adolescent

Ernst Eber, MD, ATSF, FERS

Division of Paediatric Pulmonology and Allergology, Dept of Paediatrics

and Adolescent Medicine, Medical University of Graz, Austria

Anatomical findings: upper and lower airways

(normal/abnormal)

Cairo, 28 April 2019

• Nasal cavity

• Pharynx

• Larynx

• (Extrathoracic) trachea

Upper airway

Upper airway

Wallis C. Normal anatomy. In: Priftis KN, Anthracopoulos MB, Eber E, Koumbourlis AC, Wood RE (eds). Paediatric Bronchoscopy. Prog Respir Res. Basel, Karger 2010

• Choanal atresia and stenosis

• Cysts, mass lesions

• Adenoids

Nasal cavity/nasopharynx

• Defective formation of definitive choanae (uni- vs.

bilateral; bony vs. membranous occlusion – 9:1)

• 2/3 with associated anomalies (e.g. CHARGE syndrome)

• Bilateral atresia – respiratory distress at birth

Unilateral lesions – detected later in childhood

• Diagnosis: Flexible endoscopy / CT scan

• Treatment: Establishment of nasal airway

Choanal atresia and stenosis

Lowe et al. Radiographics 2000

Choanal atresia and stenosis

Normal choanae of a 33 weeks newborn

Bilateral choanal atresia, post surgery in a 37 weeks newborn

Eber. Pädiatrische Pneumologie. Berlin Heidelberg, Springer, 2014

Choanal atresia and stenosis

Cysts, mass lesions

Dinwiddie Paediatr Respir Rev 2004

Adenoids

Courtesy of P. Pohunek

• Pierre Robin sequence

• Dysmorphic syndromes with maxillary or mandibular

hypoplasia, e.g. Apert, Crouzon, Goldenhar, Pfeiffer,

Treacher Collins syndromes

Often associated with other malformations, e.g. cleft palate

• Neuromuscular diseases/neurologic impairment with loss of

pharyngeal muscular stability pharyngeal collapse

• Base of tongue mass

Oropharynx

• Mandibular micrognathia, glossoptosis, and resultant (pharyngeal) airway obstruction

• Incidence: ~1:8,500-14,000; >50% with associated anomalies (>40; e.g. Stickler syndrome or 22q11.2 deletion syndrome); frequently cleft palate

• Variable severity of airway obstruction

• Treatment: In severe cases intubation; nasopharyngeal airway; noninvasive respiratory support; surgical procedures (mandibular distraction osteogenesis, tracheostomy); palatal plates (e.g. preepiglottic baton plate with velar extension)

(Pierre) Robin sequence

Evans et al. Pediatrics 2011

(Pierre) Robin sequence

Evans et al. Pediatrics 2011

(Pierre) Robin sequence

• Thyroglossal duct cyst

• Mucus retention cyst

• Lingual thyroid

Symptoms: Stridor, respiratory distress,

feeding difficulties/aspiration

May be intermittent!

Base of tongue mass

Base of tongue mass

Larynx

• Malacia (infantile larynx)

• Subglottic stenosis (congenital,

acquired)

• Vocal cord paralysis (uni-, bilateral)

• Haemangioma, lymphangioma

• Saccular cyst, laryngocele

• Web

• Cleft

• Papillomatosis

• Foreign body

Larynx

Stridor

• Often the most prominent symptom of UAO

• Heard predominantly during inspiration

• Indicative of substantial narrowing or obstruction

of the larynx or extrathoracic trachea

• increased velocity and turbulence of airflow,

vibration of the aryepiglottic folds or vocal cords

• Patients with more than 50% obstruction may be

asymptomatic!

Wood Pediatr Clin North Am 1984

... is visible

Eber et al. Wien Klin Wochenschr 1995

Indikationen

De Blic et al. Eur Respir J 2002

Persistent stridor

In 14-26% of patients with persistent stridor,

significant additional lower airway

abnormalities, or two or more synchronous

airway lesions may be detected.

Wood Pediatr Clin North Am 1984

Gonzalez Ann Otol Rhinol Laryngol 1987

Eber Monatsschr Kinderheilkd 1996

• Most common congenital laryngeal anomaly and

most common cause of persistent stridor in infancy

• Specific disease state with ill-defined pathogenesis

(specific aetiology still obscure)

• Anatomical abnormality or delayed development in

neuromuscular control?

• Worsened by application of lidocaine

Nielson Am J Respir Crit Care Med 2000

Laryngomalacia

• Frequently associated with other airway lesions and GER

• Onset of stridor within 4-6 weeks of life; cry & cough normal

• Stridor varies with posture & airflow, loudest with increased ventilation (e.g. crying, agitation, feeding)

• Stridor tends to resolve with time

• Diagnosis: History & physical examination, confirmed by flexible airway endoscopy

• Treatment: In most cases, no specific therapeutic measures needed. In severe cases (failure to thrive and/or obstructive apnoeas), surgical treatment (supraglottoplasty, rarely tracheostomy)

Laryngomalacia

Laryngomalacia – Types

Eber et al. ERS Handbook Paediatric Respiratory Medicine 2013

Laryngomalacia – Types

van der Heijden et al. Pediatr Pulmonol 2015

Laryngomalacia

Grading

Myer Ann Otol Rhinol Laryngol 1994

Symptoms:

Stridor, respiratory distress

Recurrent / atypical croup

Prognosis:

Depending on grade of stenosis

Stenosis may improve with

airway growth

Subglottic stenosis

Subglottic stenosis

Types

a) congenital

- membraneous (thickening of the soft tissues in the

subglottic area - symmetrical narrowing; common)

(endoscopic appearance may resemble subglottic edema)

- cartilagineous (malformation of the cricoid

cartilage - circumferential stenosis/variable

appearance; rare)

Types

b) acquired

- Majority of subglottic stenoses

- Etiology: Trauma (tracheal intubation pressure necrosis; tracheostomy)

- Ulcerations, granulation tissue, subglottic cysts

- Endoscopic appearance is usually irregular, not symmetrical

Subglottic stenosis

Subglottic stenosis

Subglottic oedema

Causes

Infection: croup, bacterial tracheitis

Allergy

Trauma: laryngeal foreign body

thermal injury

iatrogenic: tracheal intubation,

bronchoscopy (most common complication of

rigid bronchoscopy)

• Subglottic space swollen, symmetrically narrowed

• Airway sometimes slit-like

• Mucosa may appear inflamed

• Glottic shape preserved

Subglottic oedema

Types

• Isolated – associated with malformations

• Unilateral – bilateral

• Adductor – abductor paralysis

• Symptoms: Aphonia, feeding

difficulties/aspiration; hoarseness,

stridor, respiratory distress

Vocal cord paralysis

• Most common tumor in infancy

• Supraglottic - subglottic

• Asymmetric mass, endoscopic recognition

may be difficult

• Often rapid growth until 6-10 months

• Involution usually begins at ~18 months

• Symptoms: Stridor, respiratory distress

Recurrent/atypical croup

Haemangioma

Eber Paediatr Respir Rev 2004

Haemangioma

• Usually supraglottic

• 2 types: anterior, lateral

• Symptoms: Stridor, respiratory distress

Dysphagia/aspiration

Weak cry or aphonia

Laryngeal cyst

Types

• Supraglottic - glottic - subglottic

• Thick - membranous

• Complete - incomplete

Symptoms: Hoarseness, aphonia,

stridor, respiratory distress

Laryngeal web

Eber. Pädiatrische Pneumologie. Berlin Heidelberg, Springer, 2014

Laryngeal web

• May extend inferiorly into the trachea

• May be associated with other anomalies

of larynx, trachea, or oesophagus

Symptoms: Stridor, feeding difficulties/

aspiration

Laryngeal cleft

Laryngeal cleft

Eber et al. ERS Handbook Paediatric Respiratory Medicine 2013

Laryngeal papillomatosis

• Multiple irregular masses, most

commonly on the vocal cords

• May extend into the trachea and bronchi

Symptoms: Hoarseness, stridor,

respiratory distress

Atypical croup

Age less than 6 months

Prolonged symptoms

No response to treatment

Vocal Cord Dysfunction (VCD) or Inducible Laryngeal Obstruction (ILO)

• Inappropriate vocal (and false)

cord adduction during inspiration

or during both inspiration and

expiration

• Gastro-esophageal reflux may play

a causative role

Courtesy of. P Pohunek

• (Intrathoracic) Trachea

• Bronchial tree

Lower airway

Murray JF. The Normal Lung. 1986

Lower airway

Lower airway

Holinger LD, Lusk RP, Green CG (eds). Pediatric Laryngology & Bronchoesophagology. Lippincott-Raven 1997

Lower airway

Wallis C. Normal anatomy. In: Priftis KN, Anthracopoulos MB, Eber E, Koumbourlis AC, Wood RE (eds). Paediatric Bronchoscopy. Prog Respir Res. Basel, Karger 2010

Wallis C. Normal anatomy. In: Priftis KN, Anthracopoulos MB, Eber E, Koumbourlis AC, Wood RE (eds). Paediatric Bronchoscopy. Prog Respir Res. Basel, Karger 2010

• Trachea

Lower airway

Courtesy of P. Pohunek

• Right mainstem bronchus

Lower airway

Courtesy of P. Pohunek

• Right upper lobe bronchus

Lower airway

Courtesy of P. Pohunek

• Right intermediate bronchus

Lower airway

Courtesy of P. Pohunek

• Right lower lobe bronchus

Lower airway

Courtesy of P. Pohunek

• Left mainstem bronchus

Lower airway

Courtesy of P. Pohunek

• Left upper lobe bronchus

Lower airway

Courtesy of P. Pohunek

• Left lower lobe bronchus

Lower airway

Courtesy of P. Pohunek

• Incidence: ~1 in 2,000

• Weakness of the airway wall, due to reduction and/or

atrophy of the longitudinal elastic fibres of the pars

membranacea, or impaired cartilage integrity („immaturity“)

Airway more susceptible to collapse

Retention of secretions from the lower airways

Bronchopulmonary infections

• No generally accepted definition of degree of collapse to be

taken as abnormal

Tracheomalacia

Congenital airway malacia

Holinger et al. Pediatric Laryngology & Bronchoesophagology 1997

Rohde et al. Acta Paediatr 2006

Inspiration Expiration

Eber. Pädiatrische Pneumologie. Berlin Heidelberg, Springer, 2014

Intrathoracic tracheomalacia

Bronchomalacia

• congenital - acquired

• localised - generalised

• primary - secondary

Tracheomalacia and bronchomalacia

Types

• Congenital type

• Extrinsic compression type

• Acquired type

Severity

• Mild airway collapse < 70%

• Moderate airway collapse 70-90%

• Severe airway collapse > 90%

Major airway collapse

Mair and Parsons Ann Otol Rhinol Laryngol 1992

F.B., male, 7 yrs, primary tracheomalacia

"Persistent wheezing"

Flow-volume curve in a patient with tracheomalacia

Airways and vascular anomalies

Holinger LD, Lusk RP, Green CG (eds). Pediatric Laryngology & Bronchoesophagology. Lippincott-Raven 1997

Secondary tracheomalacia - double aortic arch

M.A., male, 7.8 yrs, persistent wheezing

Secondary tracheomalacia – pulmonary artery sling

L.K., female, 15.1 yrs, recurrent wheezing

Secondary tracheomalacia – innominate artery

• Incidence: 1 in 3,000 (-4,000)

• ~30% born prematurely

• ~50% with associated anomalies (e.g. VACTERL association)

• >50% with structural anomalies of the tracheal wall (i.e. tracheomalacia)

Wailoo and Emery. Histopathology 1979: In the majority of patients reduced circumference of tracheal cartilage and widened membranous part (normal 4-5:1)

Tracheo-oesophageal fistula

• Various types; most common type (~85%) with

upper blind pouch and fistula between the lower trachea and the lower oesophagus

Tracheo-oesophageal fistula / oesophageal atresia

Laberge and Puligandla. Congenital malformations of the lungs and airways.

In: Taussig, Landau, (eds.) Pediatric Respiratory Medicine. Mosby Elsevier, 2008

• Incidence: 1 in 80,000 (-90,000)

• Commonly not associated with other anomalies

• Usually, fistula located in the extrathoracic trachea

• Diagnosis: (Rigid) endoscopy, contrast injection

Isolated tracheo-oesophageal fistula (H-type)

• Short-segment stenosis (incl. tracheal webs and cysts)

hourglass-shape

• Long-segment stenosis

“stovepipe trachea”

funnel- or carrot-shape (“rat-tail trachea”)

• Frequently associated with other anomalies (pulmonary artery sling, abnormal bronchial arborisation, single right or left lung)

Congenital tracheal stenosis

Speggiorin et al. Ann Thorac Surg 2012

Congenital tracheal stenosis

Eber et al. ERS Handbook Paediatric Respiratory Medicine 2013

Eber. Pädiatrische Pneumologie. Berlin Heidelberg, Springer, 2014

Congenital tracheal stenosis

Etiology

• Endotracheal tube

• Tracheotomy tube

• Improper tracheotomy

Prevention!

Acquired tracheal stenosis

Tracheal bronchus

Intraluminal mass lesions

Courtesy of P. Pohunek

Tracheal wall granulomas, suction trauma

Suprastomal granulation tissue

Tumors – airway compression

Tracheobronchomegaly

Benesch et al. Pediatr Pulmonol 2000

Know normal to recognize abnormal

Thank you for your attention!