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A. F. Karas and J. W. Lee
Other Syndromes
• Beckwidth-Wiedemann syndrome
– AD, mostly sporadic
– Macroglossia, organomegaly, omphalocele
– Monitor for hypoglycemia in perioperative period
• Mobius syndrome
– Wide spectrum of anomalies secondary to central brainstem defects
– Bilateral or unilateral CN VI and VII paralysis
• Prader-Willi syndrome
– Chromosome 15q11
– Hyperphagia and morbid obesity → OSA
• Angelman syndrome
– Chromosome 15q11
– Microcephaly, coarse facial features
– Seizures, ataxia, severe developmental delay
• Kartagener’s syndrome
– Primary ciliary dyskinesia, decient outer dynein arm
– Triad: chronic rhinosinusitis, bronchiectasis, situs inversus
– Male infertility
• Kallman’s syndrome
– X-linked
– Most common form of hypogonadotropic hypogonadism
– Absence of olfactory bulb (anosmia)
Further Reading
Scott AR, Tibesar RJ, Sidman JD.Pierre Robin Sequence: evaluation, management, indications for
surgery, and pitfalls. Otolaryngol Clin N Am. 2012;45(3):695–710.
Solomon BD.VACTERL/VATER association. Orphanet J Rare Dis. 2011;6:56.
Rodman R, Pine H. The otolaryngologist’s approach to the patient with Down syndrome.
Otolaryngol Clin N Am. 2012;45:599–629.
Swibel Rosenthal LH, Caballero N, Drake AF.Otolaryngologic manifestations of craniofacial syn-
dromes. Otolaryngol Clin N Am. 2012;45(3):557–77.
Collins WO, Choi SS.Otolaryngologic manifestations of achondroplasia. Arch Otolaryngol Head
Neck Surg. 2007;133:237–44.
Marom T, Roth Y, Goldfarb A, Cinamon U.Head and neck manifestations of 22q11.2 deletion
syndromes. Eur Arch Otorhinolaryngol. 2012;269(2):381–7.

Chapter 4
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Pediatric Airway andEsophagology
AnatoliF.Karas andJanetWaiminLee
Pearls
• Recommend using formula (age+ 16)/4 for uncuffed endotracheal tube (ETT)
and ½ size smaller for cuffed ETT.
• Recommend using ½ size smaller ETT based on age formula for patients with
Down syndrome.
• Endotracheal tube cuff pressures >20mmHg will exceed the capillary lling
pressure and cause ischemic damage over time.
• More severe airway pathology is less likely to respond to endoscopic verses open
interventions.
• The history is essential to providing a complete and focused differential diagno-
sis to pediatric airway pathology.
• Voice dysfunction in children may become more of an issue as they are intro-
duced to more social environments; they may become withdrawn if they are not
able to be easily understood or communicate with others. The emotional/social
impact is not yet well elucidated but should not be ignored.
A. F. Karas
Department of Otorhinolaryngology, Head & Neck Surgery, Rush University Medical Center,
Chicago, IL, USA
Department of Pediatrics, Rush University Medical Center, Chicago, IL, USA
e-mail: anatoli_f_karas@rush.edu
J. W. Lee (*)
Department of Head and Neck Surgery & Communications Sciences, Duke University
Medical Center, Durham, NC, USA
e-mail: janet.w.lee@duke.edu
© Springer Nature Switzerland AG 2023
F. Y. Lin, Z. M. Patel (eds.), ENT Board Prep,
https://doi.org/10.1007/978-3-031-26048-3_4
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A. F. Karas and J. W. Lee
Differences Between Pediatric andAdult Airways
• Pediatric larynx higher in the neck (C2, descends to C6 with age).
• Epiglottis curved/omega shaped, tip in contact with soft palate.
• Thyroid cartilage oblique, no dening angle.
• Infant vocal cords shorter, 4–4.5mm long at birth, adults 14–23mm.
– True vocal cord in infants—50% composed of vocal process compared to
25–33% in adults
• Infant subglottis is narrowest part of airway (glottis narrowest part in adults):
4.5–7mm in full-term infant.
– 1 mm circumferential mucosal edema in infant narrows subglottis by >60%.
Clinical Evaluation
• Location of stridor by its pattern
– Inspiratory: supraglottic
– Biphasic: subglottic, glottic
– Expiratory: xed intrathoracic trachea
• SPECSR mnemonic
– Subjective—parent’s impressions
– Progression
– Eating/feeding difculties
– Cyanosis
– Sleep disordered breathing
– Radiography
• Pediatric airway abnormalities that may improve in prone position
– Laryngomalacia
– Pierre Robin sequence
– Vascular compression
– Mediastinal mass
• Common gastroesophageal reux disease (GERD)-related (or exacerbated)
laryngeal disorders
– Recurrent croup
– Chronic cough
– Laryngospasm
– Dysphonia
– Subglottic stenosis

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– Aspiration
– Laryngomalacia
• History
– Laryngomalacia most common cause of stridor in infants, may worsen gradu-
ally in the rst few weeks of life.
– Viral prodrome, fever, coryza → infection.
– Foreign body (FB) unlikely in non-ambulatory child (especially without older
siblings).
– Less than 6months old, new-onset biphasic stridor, no FB history → subglot-
tic hemangioma.
– If prior history of intubation, NICU, prematurity → subglottic stenosis, sub-
glottic cyst.
– Evaluate for vallecular cyst on bedside laryngoscopy.
– History of cardiothoracic procedure → recurrent laryngeal nerve (RLN) injury.
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Radiographic Examination
• “Steeple sign”—subglottic narrowing on anterior-posterior (AP) neck X-ray, can
include dilated hypopharynx → croup, can also be seen in subglottic hemangioma (usually asymmetric) and bacterial tracheitis
• Indistinct vocal cords → infectious process
• “Thumb sign”—thickening of epiglottis on lateral neck X-ray → epiglottitis/
supraglottitis
• “Candle-dripping sign”—linear soft tissue lling defect within the trachea, irregularity of the tracheal wall → exudative/bacterial tracheitis
Laryngomalacia
• Most common cause of inspiratory stridor, stridor worse with crying and
lying supine
• Uncertain pathophysiology, thought to be affected by:
– Anatomic factors: shortened aryepiglottic folds, anterior cuneiform cartilage
prolapse
– Immature neuromuscular control
– GERD
• Medical therapy
– Reux control
– Speech-language pathology (SLP) evaluation, feeding strategies, positioning

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• Indications for surgical intervention
– Severe stridor with failure to thrive or weight loss
– Obstructive sleep apnea
– Severe chest deformity
– Cyanotic episode
– Pulmonary hypertension, cor pulmonale
• Surgical approaches
– Supraglottoplasty—division of aryepiglottic folds, removal of redundant
mucosa and cuneiform and corniculate cartilages as needed
– Epiglottopexy
– Tracheostomy for severe cases
– Possible Nissen/g-tube adjunct for severe cases for recalcitrant reux
A. F. Karas and J. W. Lee
Laryngeal Cleft
• Can contribute to chronic cough, aspiration, chronic lung disease
• May be suspected on modied barium swallow
• Diagnosis requires microdirect laryngoscopy and palpation of the interarytenoid
space—cannot be ruled out on exible laryngoscopy
• Classication
– Type 1: supraglottic interarytenoid cleft above the level of the vocal cords
Treatment: observation, diet modication, injection laryngoplasty, endoscopic repair
– Type 2: partial cricoid cleft, extends below the level of the vocal cords
Treatment: observation, diet modication, injection laryngoplasty, endoscopic/open repair
– Type 3: total cricoid cleft, without extension into the thoracic inlet
Treatment: diet modication, endoscopic/open repair
– Type 4: laryngotracheoesophageal cleft, almost universally fatal
Vocal Cord Paresis/Immobility
• Presentation
– Unilateral: weak cry, aspiration.
– Bilateral: presentation depends on cord position.

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If immobile and aDducted → signs of airway obstruction, stridor
If immobile and aBducted → aspiration, weak/no cry, but no stridor or
airway obstruction
• Differential diagnosis of vocal cord palsy, unilateral or bilateral
– Idiopathic
– History of cardiac surgery (esp. PDA ligation with left cord paresis)
– Birth trauma, other trauma
– Neurologic disease
Arnold-Chiari malformation
Hydrocephalus
Cerebral palsy
Hypoxic encephalopathy
– Malignant disease: familial, brainstem lesions
– Drug related: vinca alkaloids (neurotoxic)
• Diagnostic workup
– MRI brain
– Modied barium swallow
– SLP evaluation
– Operative airway evaluation to rule out anatomic restriction of movement
(arytenoid xation, posterior glottic stenosis, etc.)
– Consider laryngeal EMG
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• Treatment
– Unilateral—rarely any airway/respiratory issues, primarily voice and
swallowing
Observation
Speech therapy
Injection laryngoplasty
Recurrent laryngeal nerve-ansa cervicalis anastomosis
– Bilateral—almost all present with some component of respiratory problems
Observation—spontaneous recovery possible, 5–7days of age
Lateral cordotomy, partial arytenoidectomy
Lateralization suture
Cricoid split—endoscopic, open, possible late failures secondary to
synkinesis
Tracheostomy

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A. F. Karas and J. W. Lee
Recurrent Respiratory Papillomatosis
• Etiology: HPV 6, 11; develops at the junction of squamous and respiratory epithelium
– Vertical maternal transmission, presents with dysphonia, stridor, respiratory
distress, ball-valving glottic lesion
– Pulmonary dissemination nearly uniformly fatal
• Types
– Juvenile onset (<12years old)—more common, more aggressive
– Adult onset
• Derkay staging/severity system
– Score of 1–3 (0 = absent, 1 = surface lesion, 2 = raised lesion, and
3=bulky lesion)
– Assign score to each site in aerodigestive tract
Larynx
• Epiglottis (lingual and laryngeal surface), aryepiglottic folds (Right/
Left), false vocal folds (R/L), true vocal cords (R/L), arytenoids (R/L),
anterior commissure, posterior commissure, subglottis
Trachea
• Upper 1/3, middle 1/3, lower 1/3, bronchi (R/L), tracheostomy stoma
Other
• Nose, palate, pharynx, esophagus, lungs, other
– Six questions regarding patient’s clinical course: interval of surgery, total
number of surgeries, urgency of this surgery, quality of voice, degree of stridor at the time of this surgery, and degree of respiratory distress
• Treatment
– Surgical debulking: laser, microdebrider, cold knife; may need staged
approach to avoid glottic webbing
– Tracheostomy, avoid unless absolutely necessary
– Adjuvant therapies: interferon, cidofovir, Avastin
Indications >4 surgeries/year, distal pulmonary spread, rapid regrowth of
disease with airway compromise
Laryngeal Webs
• Congenital—isolated or associated with 22q11 deletion syndrome
• Acquired—iatrogenic (postsurgical, prolonged intubation), caustic injection, trauma

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• Cohen grading system
– Type 1: thin, membranous, <35% of glottis
– Type 2: 35–50% of glottis
– Type 3: 50–75% of glottis, often extends to anterior cricoid cartilage
– Type 4: up to 99% of glottis
• CHAOS (congenital high airway obstruction syndrome), complete laryngeal stenosis
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Subglottic Stenosis
• Congenital
• Acquired—trauma/intubation, infection, chronic inammatory disease, neoplastic disease
• Cotton-Myer Grading system
– I: <50%
– II: 51–70%
– III: 71–99%
– IV: no detectable lumen, complete obliteration
• Surgical treatment
– Grade I/II: endoscopic laser, dilation, cold knife
– Grade III/IV: endoscopic management may be trialed for Grade III, but suc-
cess is less likely than for less severe cases. May require tracheostomy, laryngotracheal reconstruction (cricoid split with anterior ± posterior cricoid
augmentation), cricotracheal resection
– Single stage (no postoperative tracheostomy present) versus double stage
(persistent postoperative tracheostomy with staged decannulation)
Posterior Glottic Stenosis
• Can present as airway obstruction, dysphonia
• On exible laryngoscopy, may look like bilateral vocal cord paresis
• Bogdasarian classication
– I: interarytenoid adhesion
Treatment: observation, endoscopic lysis
– II: posterior commissure stenosis
Treatment: observation, endoscopic/open repair
– III: posterior commissure stenosis with unilateral cricoarytenoid xation
Treatment: endoscopic/open repair, tracheostomy

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– IV: posterior commissure stenosis with bilateral cricoarytenoid xation
Treatment: tracheostomy, open repair
A. F. Karas and J. W. Lee
Tracheal Obstruction
• Tracheal stenosis
– Acquired/inammatory
– Congenital—complete tracheal rings
– Surgical management: observation, endoscopic excision, tracheoplasty, seg-
mental resection, slide tracheoplasty
• Tracheobronchomalacia
– Immature tracheal cartilage with dynamic collapse
– May require surgical decompression if due to extrinsic vascular compression
Innominate artery (anterior)
Double aortic arch
• Predominantly airway symptoms (stridor, respiratory distress, cough)
• Surgical repair necessary
Right aortic arch
• Airway symptoms as well as dysphagia
Anomalous right subclavian artery (dysphagia lusoria)
• Vascular compression POSTERIOR to the trachea and esophagus
• Associated with non-recurrent right laryngeal nerve
Pulmonary artery sling/dilation
• Left pulmonary artery arises from right pulmonary artery
• Vascular compression BETWEEN trachea and esophagus on barium swallow
• Association with complete tracheal rings
– May require tracheostomy to bypass malacic segment or to allow use of posi-
tive pressure if severe
Airway Foreign Bodies
• Third most common cause of accidental death in children younger than age 1,
typically seen in children ages 1–3years
• May be food or non-food objects

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• Diagnosis
– History of choking episode, witnessed ingestion/aspiration, coughing
– Physical exam: abnormal chest auscultation (diminished breath sounds,
wheezing, rhonchi), respiratory distress
– Radiography: most objects are radiolucent on X-ray. Lateral decubitus or
inspiratory/expiratory lms may reveal air trapping. If FB present for some
time, may have atelectasis/inltrate
• Treatment
– Flexible laryngoscopy can be considered. However, a negative exam does not
rule out FB.Care should be taken as procedure can exacerbate symptoms.
– Microdirect laryngoscopy and bronchoscopy, urgency dictated by clinical
picture.
– May need ECMO (extracorporeal membrane oxygenation) available in cases
of severe obstruction or known large foreign body.
– If endoscopic management with rigid instrumentation fails, may need exible
bronchoscopy for more peripheral or upper lobe foreign bodies or open management in cases of severe impaction.
– Consider postoperative antibiotics and/or steroids depending on intraopera-
tive ndings, especially in cases of organic material (e.g., oils from nuts may
cause continued inammation).
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Tracheoesophageal Fistula
• Types
– Esophageal atresia with distal TEF (85%)
– Isolated esophageal atresia (10%)
– H-Type TEF (4%)
– Esophageal atresia with proximal TEF (0.5–1.0%)
– Esophageal atresia with proximal and distal TEF (0.5%)
• Generally present with immediate feeding problems and aspiration with inability
to pass feeding tube
– H-type may present later with recurrent pneumonia
– Diagnosed with uoroscopy, endoscopy
– Surgical correction required, risk of postoperative stricture
Caustic Ingestion
• Acidic ingestion leads to coagulative necrosis (supercial)
• Alkaline ingestion leads to liquefactive necrosis (deep)
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