"Oh, Baby, Baby": Respiratory Problems in 'les tout petits’
Laryngomalacia and Tracheomalacia: An Update
Sam J Daniel Emily Kay-Rivest
Professor of Otolaryngology and Pediatric Surgery Director Pediatric Otolaryngology Montreal Children’s Hospital, McGill University
Faculty Disclosure
No conflicts of interest to declare Objectives • Review of presentation, classification, diagnosis and managament of laryngomalacia and tracheomalacia
• Specific focus on surgical management options for both of these conditions Laryngomalacia
• Laryngomalacia is defined as the collapse of supraglottic structures during inspiration • It is the most common cause of stridor in neonates and children • Historically believed to be an anatomic abnormality of laryngeal cartilage • Now, a neuromuscular etiology is suspected
Thorne, Marc C., and Susan L. Garetz. "Laryngomalacia: review and summary of current clinical practice in 2015." Paediatric respiratory reviews 17 (2016): 3-8. Fraga, Jose Carlos, Russell W. Jennings, and Peter CW Kim. "Pediatric tracheomalacia." Seminars in Pediatric Surgery. Vol. 25. No. 3. WB Saunders, 2016. Reinhard A. et al, January 2017 Classification: Laryngomalacia Classification: Laryngomalacia
Laryngomalacia Severity Scale
Severity Level Respiratory Symptoms Feeding symptoms
Inspiratory stridor Mild Occasional cough or regurgitation Average resting SpO2 98-100%
Inspiratory stridor Frequent regurgitation or other feeding Moderate Average resting SpO2 ∼96% issues
Inspiratory stridor with cyanosis or apnea Severe Failure to thrive or aspiration Average resting SpO2 ∼86%
D.M. Thompson. Laryngomalacia: factors that influence disease severity and outcomes of management. Curr Opin Otolaryngol Head Neck Surg., 18 (6) (2010), pp. 564–570 Tracheomalacia
• Tracheomalacia refers to a weakness of the trachea, due to reduction and/or atrophy of the longitudinal elastic fibers of the pars membranacea, or impaired cartilage integrity
Fraga, Jose Carlos, Russell W. Jennings, and Peter CW Kim. "Pediatric tracheomalacia." Seminars in Pediatric Surgery. Vol. 25. No. 3. WB Saunders, 2016.
Classification: Tracheomalacia
Primary/Congenital TM Secondary/Acquired TM
Idiopathic TM – normal infant Prolonged intubation Prematurity Tracheostomy Cartilage congenital abnormalities Severe tracheobronchitis Congenital syndromes Resulting from compression Vascular Cardiac Skeletal Tumors and cysts Infection Post-traumatic
Fraga, Jose Carlos, Russell W. Jennings, and Peter CW Kim. "Pediatric tracheomalacia." Seminars in Pediatric Surgery. Vol. 25. No. 3. WB Saunders, 2016. Clinical presentation and diagnosis
Laryngomalacia Tracheomalacia
Inspiratory stridor – intermittent, variable, harsh inspiratory and/or expiratory stridor, NORMAL CRY wheezing, barking, croupy cough, (worsened by feeding, agitation, supine position) recurrent respiratory infections difficulty clearing endobronchial secretions,
Feeding concerns (retarded growth, choking, reflux Sometimes dramatic attacks of “reflex apnea” or “dying symptoms, pneumonia) spells.”
Laryngomalacia Tracheomalacia Flexible nasolaryngoscopy Rigid bronchoscopy with spontaneous ventilation flexible bronchoscopy Initial presentation algorithm. John Carter, Reza Rahbar, Matthew Brigger, Kenny Chan, Alan Cheng, Sam J. Daniel, Alessandro De Alarcon, Noel Garabedian, Catherine Hart, Christopher Hartnick, Ian Jacobs, Bryan Liming, Richard Nicollas, Seth Pransky... International Pediatric ORL Group (IPOG) laryngomalacia consensus recommendations International Journal of Pediatric Otorhinolaryngology, Volume 86, 2016, 256–261 Management: Laryngomalacia
• Conservative/Medical – Reserved for mild to moderate symptoms with no associated feeding difficulties – Careful weight monitoring, feeding interventions, positional therapy • Surgical – For those who fail to respond to conservative management Management: Laryngomalacia
John Carter, Reza Rahbar, Matthew Brigger, Kenny Chan, Alan Cheng, Sam J. Daniel, Alessandro De Alarcon, Noel Garabedian, Catherine Hart, Christopher Hartnick, Ian Jacobs, Bryan Liming, Richard Nicollas, Seth Pransky...
International Pediatric ORL Group (IPOG) laryngomalacia consensus recommendations
International Journal of Pediatric Otorhinolaryngology, Volume 86, 2016, 256–261 Reflux?
Luebke, Pepsin as a Biomarker for Laryngopharyngeal Reflux in Children With Laryngomalacia, 2017 Surgical indications for laryngomalacia
• Stridor with respiratory distress • Dyspnea with retractions • Pectus excavatum • Pulmonary hypertension • Cor pulmonale • Severe OSA • Episodic cyanosis with feeding • Recurrent aspiration pneumonia • Failure to thrive Surgical options for laryngomalacia • Supraglottoplasty – Division of AE folds – Resection of redundant supra-arytenoid mucosa – Epiglottopexy may be necessary in patients with obstruction from posterior collapse of epiglottic • Tracheostomy always remains part of the surgical options Supraglottoplasty
Thorne, Marc C., and Susan L. Garetz. "Laryngomalacia: review and summary of current clinical practice in 2015." Paediatric respiratory reviews 17 (2016): 3-8. Surgical Treatment of Laryngomalacia Supraglottoplasty continued
• Has had great success in treatment of severe laryngomalacia, reported rates over 90%
• Complications can occur, but are rare, they include: – Granulomas, airway edema, small webs – Supraglottic stenosis – Recurrence
Preciado, Diego, and George Zalzal. "A systematic review of supraglottoplasty outcomes." Archives of Otolaryngology–Head & Neck Surgery 138.8 (2012): 718-721. Management: Tracheomalacia
• Medical – Most children can be treated non-surgically, and symptoms are expected to improve by age 1 • Surgical – Reserved for children with severe symptoms – Primary indications include • Dying spells • Recurrent pneumonia • Intermittent respiratory obstruction
Fraga, Jose Carlos, Russell W. Jennings, and Peter CW Kim. "Pediatric tracheomalacia." Seminars in Pediatric Surgery. Vol. 25. No. 3. WB Saunders, 2016. Surgical Management: Tracheomalacia • Tracheal resection – Ideally resect no more than 30% of total length • Aortopexy – Mainstay for intrathoracic TM – Partial sternotomy – Thoracoscopic aortopexy • Anterior and or posterior tracheopexy • Splinting
Fraga, Jose Carlos, Russell W. Jennings, and Peter CW Kim. "Pediatric tracheomalacia." Seminars in Pediatric Surgery. Vol. 25. No. 3. WB Saunders, 2016. Aortopexy
-airway is loosely attached anteriorly to major vessels -by suturing the aorta (+/- the innominate artery +/- pericardium to the sternum, you stent open the tracheal lumen -various surgical approaches exist: anterior thoracotomy, lateral thoracotomy and newly thoracoscopic approaches are reported
Fraga, Jose Carlos, Russell W. Jennings, and Peter CW Kim. "Pediatric tracheomalacia." Seminars in Pediatric Surgery. Vol. 25. No. 3. WB Saunders, 2016. Splinting
• Considered in patient with diffuse TM • External splinting with both autologous and prosthetic materials – Autologous materials include rib cartilage – Prosthetic materials include dacron reinforced silastic mesh, ceramic rings • Internal tracheal stent
Fraga, Jose Carlos, Russell W. Jennings, and Peter CW Kim. "Pediatric tracheomalacia." Seminars in Pediatric Surgery. Vol. 25. No. 3. WB Saunders, 2016. Huang et al, J Thorac Dis. 2016 Nov; 8(11): 3323– 3328 Internal tracheal stenting
• Placed by endoscopy • Silicone and metallic expandable stents • Absorbable biopolymer stents are currently in development • Issue remains: – Patient is growing (quickly, and so is their trachea!) – Risk of granulation tissue formation – Risk of stent migration
• Future directions: stem cell-based tissue-engineered tracheal replacement
Filler RM,Forte V,Fraga JC,et al.The use of expandable metallic airway stents for tracheobronchial obstruction in children. J PediatrSurg. 1995;30: 1050–1055. 89. Valerie EP,Durrant AC,Forte V,et al.A decade of using intraluminal tracheal/bronchial stents in the management of tracheomalacia and/or bronchomalacia: is it better than aortopexy? J PediatrSurg. 2005;40:904–907. 90. Fig. 2. Congenital tracheomalacia (1-week-old boy). A: Severe anteroposterior collapse of the lower-middle tracheal segment. B: Widely patent trachea on bronchoscopy after application of SX-Ella Biodegradable stent (6 mm diameter × 15 mm length).
B. Sztanó, G. Kiss, K. Márai, G. Rácz, I. Szegesdi, K. Rácz, G. Katona, L. Rovó
Biodegradable airway stents in infants – Potential life-threatening pitfalls
International Journal of Pediatric Otorhinolaryngology, Volume 91, 2016, 86–89 http://dx.doi.org/10.1016/j.ijporl.2016.10.013 Fig. 3. Autopsy findings from 13-month-old girl. The stent inserted 2 days prior is shown at the bifurcation (black arrow). Total closure of the left main bronchus caused by degradation products and granulation inserted 11 weeks prior (yellow arrow). The left ...
B. Sztanó, G. Kiss, K. Márai, G. Rácz, I. Szegesdi, K. Rácz, G. Katona, L. Rovó
Biodegradable airway stents in infants – Potential life-threatening pitfalls
International Journal of Pediatric Otorhinolaryngology, Volume 91, 2016, 86–89 http://dx.doi.org/10.1016/j.ijporl.2016.10.013 Conclusions
• Laryngomalacia and tracheomalacia are conditions encountered by the both the pediatric otolaryngologist and the pediatric respirologist
• Surgical intervention is usually reserved for severe cases
• A multidisciplinary team approach is crucial for appropriate management of these patients
Looking Forward to Your Comments and Questions
References
• Snijders, Deborah, and Angelo Barbato. "An update on diagnosis of tracheomalacia in children." European Journal of Pediatric Surgery 25.04 (2015): 333-335. • Carter, John, et al. "International Pediatric ORL Group (IPOG) laryngomalacia consensus recommendations." International journal of pediatric otorhinolaryngology 86 (2016): 256-261. • Thorne, Marc C., and Susan L. Garetz. "Laryngomalacia: review and summary of current clinical practice in 2015." Paediatric respiratory reviews 17 (2016): 3-8. • Fraga, Jose Carlos, Russell W. Jennings, and Peter CW Kim. "Pediatric tracheomalacia." Seminars in Pediatric Surgery. Vol. 25. No. 3. WB Saunders, 2016.