Chapter 48 - chronic pediatric airway diseases Flashcards
2 most common causes of stridor in infant
laryngomalacia
unilateral VC paralysis (usually iatrogenic)
By what age do kids outgrow laryngomalacia?
18-24 mo
Most common tumor of infancy. And where are most found?
infantile hemangioma, H/N
Which types of HPV must be targeted by vaccines to decrease risk of RRP?
6, 11
When is stridor worse with laryngomalacia
feeding, supine, or agitated
Percentage of laryngomalacia pts that experience significant UA obstruction. What does this result in?
10%
feeding diff/FTT, pectus excavitum, apneic episodes, cyanosis, hypoxia
These pts warrant consideration for surgery
Relationship between GERD and laryngomalacia
GERD may contribute to airway edema and further compromise the airway
Acid suppression can improve mild cases of laryngomalacia
Size of term infant subglottic lumen
4.5-5.5mm
What does a 4.0 ETT mean?
inner diameter 4mm
Formula to choose ETT size…and which age group does this work in
inner diameter = 4 + age/4
More accurate for older children
Causes of congenital subglottic stenosis
elliptical cricoid congenital narrowing (Downs, trapped 1st tracheal ring)
Causes of acquired subglottic stenosis
ETT (duration, size) neck trauma laryngeal procedures caustic ingestion radiotherapy tracheal infection
Grading system for SGS
Myer-Cotton I- 0-50% obstruction II- 51-70% III- 71-99% IV- no detectable lumen
Ways to treat SGS
scar lysis with balloon dilation
augmentation (cartilage graft to make airway bigger)
resection (remove affected segment, reanastamose)
If mature, thick lesion, more superior to inferior dimension, may need more than balloon
Embryology of laryngeal cleft
TE septum forms caudal to cranial, cleft forms if incomplete development/fusion
Classification system of laryngeal cleft
Benjamin-Inglis
1- isolated to supraglottic interarytenoid region, above or at VC
2- extends to upper cricoid, not through lower cricoid
3- through inferior border cricoid, possibly into cervical trachea
4- into thoracic trachea, possibly to carina
Symptoms of laryngeal cleft based on grade
I-II: hoarse, stridor, chronic cough, lingering URI, recurrent pneumonia
III-IV: respiratory distress, aspiration, presents at birth
Most frequent operation leading to VC paralysis
PDA ligation –> left TVC paralysis
Others: TEF repair, esophageal atresia repair, also birth trauma, Chiari (b/l), neoplasm of CNS/neck/mediastinum
Name 4 syndromes associated with laryngeal cleft
Opitz-Frias
Pallister Hall
VACTERL
CHARGE
Opitz-Frias syndrome
craniofacial (cleft), GU abnl, midline airway abnl
Pallister-Hall syndrome
Congenital hypothalamic hamartoblastoma, hypopituitarism, polydactyly, imperforate anus, cardiac abnl, renal malformations
VACTERL
Vertebral, Anal atresia, Cardiac, TEF, Ear, Renal, Limb
Presentation and workup and prognosis of unilateral VC paralysis
stridor, weak cry, feeding difficult, possible asp.
Imaging course of RLN from SB to mediastinum
Barium swallow if feeding diff
70% resolve spontaneously within 6 mo age unless iatrogenic (35% recover by 16mo)
Treatment for severe sx of unilat VC paralysis
VC medialization to improvve speech/swallow
narrows airway at glottis though
How many patients with beard hemangiomas have airway ones?
50%
XR findings suggestive of airway hemangioma
asymmetric narrowing subglottis
Tx of airway (esp subglottis) hemangioma
steroids (stop angiogenesis, DEC inflammation) effective in proliferative phase only
propranolol - mechanism not identified
laser, steroid injection
tracheostomy until spontaneous resolution
Diagnosis of tracheomalacia
> 50% loss of airway diameter during coughing, with bronchoscopy
Expiratory/biphasic stridor, barky cough, pneumonia prolonged or recurrent
Intrinsic vs Extrinsic tracheomalacia
Int: cartilage weak/absent, outgrow in first few yrs of life
Tx inhaled steroids for edema
Associated with TEF
Ex: structure outside trachea is compressing the cartilage. Cardiovascular abnl. Do barium esophagram, rigid bronch, CT/MRI
Most common benign neoplasm of pediatric larynx
RRP
Also occurs in adults, but less aggressive
Sx of RRP
dysphonia, stridor
How HPV 11 RRP differs from 6
more airway obstruction, tracheal disease, pulmonary disease, need for trach
How many babies born to mothers with active genital condylomata will get RRP?
1 in 231
Should you do C-section to decrease risk of RRP?
ACOG currently does not recommend it
Risk factors for RRP development
first born child
teenage mother
vaginal delivery (but several cases demonstrate in utero transmission)
Tx of RRP
cold steel, KTP/CO2 laser
Topical cidofovir
Which HPV types does gardasil prevent?
6, 11, 31, 33
Four phases of swallowing
Oral prep - bolus form
Oral propulsive - transport to pharynx
Pharyngeal - pharynx to esophagus, nasopharynx closes, breathing stops, VC adduct, larynx rises, BOT/pharyng mm propel bolus
Esophageal- peristalsis
What is aspiration vs penetration
Pen: material passing into larynx but not past TVC
Asp: material passing from upper airway to below VC
Anatomical abnormalities and neurological conditions associated with aspiration/swallowing difficulty
cleft palate, TEF, laryngeal cleft, macroglossia, micrognathia, hypotonia, Downs
History items suggestive of aspiration
choke with feeds, recurrent pneumonia, wheezing, respiratory distress
FEES
endoscopic eval of swallowing
nasopharyngeal scope used to watch patient swallow