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Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_4518_Библиотеки_им_академика_М_И_Перельмана

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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, decient 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 andEsophagology
AnatoliF.Karas andJanetWaiminLee
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 >20mmHg 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 andAdult 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 dening angle.
• Infant vocal cords shorter, 4–4.5mm long at birth, adults 14–23mm.
– 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):
4.5–7mm 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 difculties – Cyanosis – Sleep disordered breathing – Radiography
• Pediatric airway abnormalities that may improve in prone position
– Laryngomalacia – Pierre Robin sequence – Vascular compression – Mediastinal mass
• Common gastroesophageal reux 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 6months 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
– Reux 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 reux
A. F. Karas and J. W. Lee
Laryngeal Cleft
• Can contribute to chronic cough, aspiration, chronic lung disease
• May be suspected on modied barium swallow
• Diagnosis requires microdirect laryngoscopy and palpation of the interarytenoid space—cannot be ruled out on exible laryngoscopy
• Classication
– Type 1: supraglottic interarytenoid cleft above the level of the vocal cords
Treatment: observation, diet modication, injection laryngoplasty, endo­scopic repair
– Type 2: partial cricoid cleft, extends below the level of the vocal cords
Treatment: observation, diet modication, injection laryngoplasty, endo­scopic/open repair
– Type 3: total cricoid cleft, without extension into the thoracic inlet
Treatment: diet modication, 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 – Modied 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, 5–7days 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 (<12years 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 inammatory 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 classication
– 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/inammatory – 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 1–3years
• 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/inltrate
• 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 inammation).
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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 (supercial)
• Alkaline ingestion leads to liquefactive necrosis (deep)