 Difficult problem, difficult diagnosis  Stridor at birth-worse with feeding, crying  May have history of difficult or forceps delivery, but not always  Post cardiac surgery  Assessment includes CXR, CNS  Often need repetitive flexible bedside laryngoscopy to assess vocal cord motion  Often needs operative endoscopy Vocal Cord Paralysis

 Bilateral: Airway issues, Aspiration issues  Expectant (can take years to resolve)  Close monitoring of O 2 sat, apnea, weight gain  Tracheotomy  Posterior laryngeal expansion  Botox (for spastic paralysis)  Cordotomy  Arytenoidectomy  Re-innervation procedures  Unilateral: Voice Issues, Aspiration Issues  Observation  Vocal Cord Injection  Multiple approaches  Thyroplasty Vocal Cord Paralysis

 Glottic webs  Tend to be thick and extend to subglottis  Laryngeal atresia variant  22 q deletion association  Rare familial pattern  Surgical Options  Endoscopic vs. Open  Keel placement Congenital Airway Abnormalities: Anterior Glottic Web

 Laryngeal Clefts  High index of suspicion needed  Stridor & aspiration problems  Assess with microlaryngoscopy  2 handed distraction technique  Palpation of interarytenoid region Congenital Airway Abnormalities: Laryngeal Cleft

 Laryngeal cleft: types 1-4  1-observation  2-endoscopic repair  3-Laryngofissure with buttress and multilayer closure  4-Thoracotomy Congenital Airway Abnormalities: Laryngeal Cleft

 Innominate artery compression  “Pulsatile” tracheomalacia  “Dying spells”  ? vascular ring – esophagram, MRI, CT  Bronchoscopy – fishmouth appearance  Repair - Aortopexy Congenital Airway Abnormalities: Vascular Rings and Slings

 History  Hoarseness  May have history “asthma” or recurrent croup  Correct diagnosis generally made 1 years after onset of symptoms  Usually identified around age 3  Has been found in neonates  Must ask about maternal vaginal condylomata  If preadolescent, think about sexual abuse  Office NPL Recurrent Respiratory Papillomatosis

 Frequent recurrence  Spread of disease  6-13% to distal airways  2-7% to lungs  Devastating impact  Surgical extirpation rarely curative  No predictive therapeutic intervention available  Malignant transformation in 1- 4% Recurrent Respiratory Papillomatosis: Particular Challenges

 Communicate with anesthesia  Interval debulking for airway and voice  LASER, Forceps, Microdebrider,  Conservative approach (“First do no harm”)  Counsel parents  Protect anterior commissure  Avoid tracheostomy  Aims:  reduce tumor burden  decrease spread of disease  create a safe and patent airway  preserve voice quality  increase interval between surgical procedures Recurrent Respiratory Papillomatosis: General Treatment Principles

Severe RRP- Cidofovir  View at initial endoscopy (s/p 4 procedures elsewhere)  View at 1st cidofovir injection (s/p 11 monthly procedures)

Cidofovir Impact

RRP and Cidofovir  Often see dramatic initial response  Disease that persists/recurs after cessation of cidofovir often is less extensive and easier to control with some individuals progressing to lasting remission  Can achieve prolonged remission in many, but not a universal “cure all”  Patient selection- Major benefit is control of “severe disease” (those patients requiring repetitive surgery or with airway risk)  The current histological and blood chemistry information is reassuring  No liver or kidney toxicity noted  Malignant transformation may occur-especially in adults; use with caution and reason

Foreign Bodies- Airway  History is key  There may not be witnessed event  If witnessed, have parents bring similar object  Signs:  Transient/ Unilateral wheeze  Barky cough, Hoarseness, Intermittent dyspnea  Clinical Stages  Acute choking/ gagging episode  Asymptomatic interval- FB becomes lodged/ reflexes become fatigued  Complications- Erosion/ Obstruction/ Infection

Foreign Bodies- Airway

 Esophageal  Can cause respiratory distress  Carefully review Xray  Repeat Xray immediately before OR  Warn parents – foreign body may pass with anesthesia  Look for more than one foreign body  Esophageal edema can be considerable Foreign Bodies- Esophageal

Gastroesophageal Reflux Disease  Airway issues  High index of suspicion  “Silent” GERD vs. Classic symptoms Pediatric Airway Manifestations of Gastroesophageal Reflux Deborah M. Burton, M.D. Seth M. Pransky, M.D. Richard Katz, M.D. Donald B. Kearns, M.D. Allan B. Seid, M.D. Annals Otol Rhinol Laryngol Sept 1992

 Classic symptoms  Emesis  Dysphagia  Burping  Choking  Gagging  Rumination  Irritability  “colic”  Arching  Torticollis  Sleep disturbance Magit/Pransky: AAO Stridor 2007 Gastroesophageal Reflux Disease

 Rhinitis of infancy  Recurrent Otitis  Chronic Sinusitis  Stertor / Stridor  Hoarseness  Chronic cough (especially nocturnal)  Halitosis  Laryngospasm / Apnea  Severe Laryngomalacia  Recurrent Croup  Subglottic stenosis exacerbation  Recurrent Bronchitis  Asthma exacerbation Multiple ENT Minfestations of Extra-esophageal Reflux

Gastroesophageal Reflux Disease  Non-operative Assessment:  Often not very conclusive or accurate  Radiologic  pH probe: double lumen  Newer pharyngeal probes  Empiric treatment if clinical picture suggestive of GERD

 Operative Assessment  DL&B  Mucosal changes  Biopsy  Tracheal aspirate for LLM  EGD with biopsy Gastroesophageal Reflux Disease

Eosinophilic Esophagitis  Symptoms similar to GERD  No response to GERD Rx  EGD with biopsy distal esophagus  Eosinophilic infiltrate  Allergy evaluation-esp foods  Dietary manipulation  Inhaled steroids (swallowed)

Gastroesophageal Reflux Extra-Esophageal Reflux  Connect refractory or unexplained airway symptoms to acid reflux  Prophylactic treatment before and after airway surgery

 Not a bad word!  Avoid a trach, create a crisis! Tracheostomy

 Assess urgency of intervention  Thorough evaluation of the situation: age, history, clinical presentation  Anticipate risks and benefit of examination  Relationship with anesthesia critical-spontaneous ventilation  Pediatric endoscopic equipment  Consider all possibilities, co-morbidities and synchronous lesions Pediatric Stridor: Conclusions