Rachel Rosen, MD MPHAssociate Professor of Pediatrics

Director, Aerodigestive ClinicBoston Children's HospitalHarvard Medical School

Matthew F. Abts, MDAssistant Professor of Pediatrics

Director of BronchoscopyCo-Director, Aerodigestive Medicine

Division of Pulmonology and Sleep MedicineSeattle Children's HospitalUniversity of Washington

The Aero Year in Review

Nancy M. Bauman, MD FACS FAAPProfessor of OtolaryngologyDirector, Aerodigestive Clinic

Co-Director, Vascular Anomalies ClinicChildren’s National Medical Center

George Washington University

Eliminated topics covered in other talksTracheomalacia, Ankyloglossia, Vocal Cord Dysfunction

Topics Addressed Today

• Primary ciliary dyskinesia• Endoscopic cricopharyngeal myotomy• Therapeutic benefit of honey• Type 1 laryngeal clefts• Unilateral vocal cord injection• Bacterial bronchitis• Plastic bronchitis• Long term risk of adenotonsillectomy

PCD Consensus Statement

• A must read!Clinical Phenotype: 3 or more features

– Nearly 100% daily cough, usually wet, year round– 80% full term babies had respiratory distress 12-24 hours after birth

• Upper or middle lobe atelectasis • Required supplemental O2 for days-weeks

– 2/3 have situs inversus totalis or situs ambiguus• More subtle anomalies too: intestinal malrotation, ANY cardiac defect

– 80% have recurrent acute or chronic otitis media• Absence does not exclude PCD

– 80% year round nasal congestion or chronic sinusitis– 80% recurrent pneumonia or bronchitis

• Bronchiectasis: 50% children and 100% adults– Reduced fertility: 100% males and many females (ectopic pregnancy)

Pediatr Pulm 2016;51:115-32

• Diagnosis: Clinical features and diagnostic testing, no single test captures all cases• One visit to PCD Center advised to confirm diagnosis • Diagnostic Studies

1. Respiratory epithelial biopsy with EM for cilia ultrastructure: detects 70% 20-50 cilia neededNormal does not exclude oligocilia and some genetic anomalies with normal structure Inner dynein arm absence may be from secondary causes

2. Nitrous Oxide measurements <77 nl/min in children >5 years 30% Cystic Fibrosis have low NO values98% for ciliary axonemal defects

3. Functional Ciliary Beat waveform Analysis (Europe) No viral illness or pathogen exposure

4. Immunofluorescence Testing: promising, limited sitesAntibodies against dynein arms and radial spoke head

5. Genetic testing: 33 known genes, All autosomal recessive inheritance except 2 X-linked syndromic Testing panel of 19 genes through next generation sequencing : $2000Biallelic DNAH11 mutations: classic phenotype, low NO levels but normal EM studies and nearly normal waveform analysis

• Diagnostic tests NOT recommended: saccharin testing, ciliary beat frequency calculation, visual assessment of ciliary motion without high speed recording devices.

• Rule out CF

Non-specific

PCD

• Endoscopic CO2 laser division of the cricopharyngeusmuscle in 2 patients with achalasia of upper esophageal sphincter

• 6 month old, discharged 2 days later• 10 month old, discharged 4 days later

• Advantages:• More often permanent improvement than botox

injection• Less invasive than open myotomy

• Bactericidal and wound healing properties– Hyperosmolarity, low pH, H2O2 production and antioxidant contents– Manuka honey: methyglycoxal disrupts biofilms of MRSA and Pseudomonas– Medicinal honey products irradiated to remove Clostridia botulinum

• Medihoney gel, SurgiHoneyRO gel, Therahoney gel, Nasumel ointment, Otomel otic drops, HoneDoc sinus rinse, Thyme honey

– 63 studies: 6 meta analyses, 44 RCTs, 5 case reports, 8 animal studies• Useful for acute cough with URI, 5 RCTs

– 2.5-10 ml honey qhs improves nocturnal cough and parental sleep in children compared to no treatment, placebo, dextromethorphan and diphenhydramine.

– Not more effective than albuterol– Side effects: GI upset, nervousness and sleep disturbance

• Useful adjunct for post tonsillectomy pain, 2 RCTs– 2-15 ml honey 5 x/day, in children > 1 year

• Allergic rhinitis: 3 RCTs , limited benefit • Useful for chemotherapy induced mucositis

• Interarytenoid mucosal height measured in 182 patients (59 months) did not vary between groups and does not necessarily correlate with aspiration

• Physiologic factors in addition to anatomy play a role in pharyngeal dysphagia. • This may explain augmentation success in some patients regardless of height.

VFSS: Thickened diet

n=82

interarytenoid mucosa at true vocal fold level.

VFSS:Normal diet

n=19

Control, No VFSS n=81

• Tested reliability of IAAP protocol to standardize assessment for interarytenoid area, what defines “deep interarytenoid notch” laryngeal cleft

– to correlate anatomy and video swallows and FEES– to correlate anatomy and outcome of treatment

• Suspend larynx, insert spreaders, assess height with right angle probe– Above false vocal folds– At false vocal folds– In ventricle– At true vocal folds– Below True vocal folds

• 4 pediatric otolaryngologists reviewed 30 videos, on 2 occasions mean 144 seconds

• Good Interrater and intrarater reliability ( kappa 0.71 and 0.70)

• 15 patients injected with prolaryng gel plus– All with pre-op aspiration by VFSS, – 80% documented GERD, 50% bronchiolitis or asthma and

60% hospitalized for aspiration sequelae– 95% on thickened feeds– Post op VFSS obtained in all, mean of 16 days later (9-29)

• 15.2 months, 13/15 full term• 9/15 (60%) improved swallowed consistency with 13%

resolved• 40% no change

• Transoral approach to cleft repair possible – One infant with penetration – One infant with aspiration.

• McIvor mouthgag placed – Intubated patient, loupes and headlight

• Advantages– Shorter and easier technique – Ideal if desaturates under suspension

laryngoscopy with spontaneous ventilation– Ideal if limited operating room resources

Monopolar cautery

Vicrylsutures

Repairedcleft

• Thick purulent exudates and pseudomembranes, high fever, acute onset respiratory distress, toxic

• 36 children, 6.7 years, 2011-16• Cough:85%, stridor:77%, hoarseness:67%, fever 67%• PICU admision 70% intubaiton 45%• 1 fatality: airway obstruction, “croup” unrespoinsve to racemi epinephrine• Viral prodrome common, and S. Aureus common• Poor response ot racemic epinephrine• High index of suspicion: immediate OR• Lower clinical suspicion: flexible alryngscopy and if purulent secretions noted

rigid bronchoscopy• Suspect if presumed “croup” fails to respond to racemic epinephrine tretment

• Plastic bronchitis arises after single ventricle surgical palliation• Thyroglossal duct flow is altered and chyle flows from TD into lungs

- elevated central venous pressures• Recurrent airway casts of mucin concretions that asphyxiate if not

expectorated or removed by bronchoscopy• Fatal in 33% of cases!

• Medical treatment of limited benefit (sildenafil and steroids), • Surgical treatment temporary benefit • Interventional radiology life-saving!

• Dynamic lymphangiogram with MR scanning, cannulation of TD and embolization of pulmonary lymphatic ducts

• Treated 18 patients, most with daily to weekly casts• 88% significantly improved

• Decreased cast rate (p<0.0001) and medication use (p<0.0001)

Children’s Hospital of Philadelphia

Submucosal lymphatics evidentafter Lymphazurin injection

Several minutes laterblue tinged new cast evident

• Temporary intervention while awaiting possible return of mobility• 108 injected between 2/06-2/18, usually prolaryng gel• 38 met inclusion criteria, pre and post video swallow studies

• 44 months mean age (2 weeks - 12 years) • Iatrogenic: > 90%

• Outcome: • 63% advanced diet at least one level• Higher success if:

• Normal diet prior to cord injury (p<0.05)• Injected within 6 months of injury (p<0.05)

l

• Large case-controlled study from Danish Health Registry assessed risk of diseases in population who had tonsillectomy and/or adenoidectomy < 10 years of age

• 60,000 surgical patients versus 1.2 million controls born between 1979-1999– 30-42 weeks GA – 1-21 years of follow up, – Included those not diagnosed with outcome disease prior to surgery (ie not included if

diagnosed with asthma before surgery)– Outcome diseases selected that were thought affected by changes in immunity

• Disease typically increased after surgery compared to control group– Tonsillectomy 2.72 increased risk of disease (RR) of the upper respiratory tract– Adenoidectomy 2.11 increased risk of COPD and 1.99 increased risk of disease of the upper

respiratory tract– 2-5 fold increased risk of acute otitis media post surgery

• Limitations: – Asthma cannot be diagnosed until after age of 5 – Adenotonsillectomy candidates have a predisposition to upper respiratory tract disease including

asthma , allergy and sinusitis– Did not control for smoking status of parents or patients, Better control group would have

been patients meeting criteria for surgery who did not have it.

• Inducible Laryngeal Obstruction• Exercise Inducible Laryngeal Obstruction (EILO)

• Vocal Cord dyskinesia, Paradoxical Vocal Cord Motion• Exercise Induced Laryngomalacia

• Noxious Stimuli Inducible Laryngeal Obstruction• Continuous view of larynx during inducible maneuvers• Improved biofeedback

Thank You!

Plastic Bronchus• CHOP has been pioneer• Happens in Fontan procedures:

– Lymphatic duct drains into subclavian but pressure high in subclavian vein so pressure gradient causes lymph to be diverted and forms peribronchial channels and leaks into bronchi. Similar to protein losing enteropathy

• Dynamic lymphangiogram with MR scans while injecting • Kildane needs a pacemaker too. Might this help> should it be

staged?• Plan to coil and glue lymphatic duct, do up to thyroglossal duct,

above and below hilum. CHO can be a whole day affair• No one knows long term, does chyle get rerouted and become

peritoneal chylous ascites• Eventual surgery like germany and japan to reanastamose

• External silk stent for severe tracheomalacia– Sufficient radial strength– Degrade over time– Easy to affix

• Silk fibroin from cocoons of Bombyx mori silkworms• Designed an image based assay to quantify changes in

tracheal area during spontaneous respiration.• Induced tracheomalacia in 3 NZ rabbits and applied

silk stents• Assessed airway area at baseline, at time of

tracheomalacia and 3-4 weeks after stent placement

NIH RO1AR068048

NO

Will not discuss24 patients followed for 6 or more months, mean age at injection was 15 monthsShowed that long term results continue to be excellent even though material may not last.Overall ¼ of patients (6/24) required an open procedure.