Laryngology seminar Laryngomalacia and treatment R3 林世倉 Introduction ‧ Stridor Inspiratory low pitched sound at level of the nasopharynx or oropharynx: stretor or snoring Supraglottic and glottic obstructions: inspiratory stridor Intrathoracic airway lesion: expiratory stridor Fixed subglottic laryngeal and cervical tracheal lesion: biphasic stridor ‧ In 85% of children under age 2.5 years presenting with stridor, the etiology is congenital (others: inflammatory, trauma, or foreign) ‧ Congenital stridor is often not present at birth-> usually presents before the age of 4 months ‧ Laryngomalacia is the most common cause of stridor in infancy (65%-75%) ‧ French textbook of pediatrics by Rilliet and Barthez in 1853 first description of a child having stridor at birth, present on both inspiration and expiration Resolution at age 10 months Presumption of laryngeal compression from either the thymus or thyroid gland ‧ Sutherland an Lack in 1897: a series of 18 patients with laryngomalacia-> they called “ congenital laryngeal stridor” ‧ Jackson and Jackson in 1942: “Layngomalacia”, softness, flabbiness, or loss of consistency of laryngeal tissues. ‧ Ferguson in 1970: “Exaggerated infantile larynx”, a chronic softening or degenerating conditions such as in osteomalacia or chondromalacia Classification of laryngomalacia ‧ Type I: inward collapse of the aryepiglottic folds ‧ Type II: a long, tubular epiglottis which curls on itself, often occurs in association with type I laryngomalacia ‧ Type III: anterior, medial collapse of the cuneiform and corniculate cartilages to occlude the laryngeal inlet during inspiration ‧ Type IV: posterior inspiratory displacement of the epiglottis against the posterior pharyngeal wall or inferior collapse to the vocal folds ‧ Type V: short aryepiglottic folds ‧ (Type VI: overly acute angle of the epiglottis at laryngeal inlet) Type I Type II Type IV Type V Type III Alternative classification of laryngomalacia ( Olney et. al, 1999) ‧ Type I: prolapse of mucosa overlying the arytenoid cartilages ‧ Type II: foreshortened aryepiglottic folds ‧ Type III: posterior displacement of the epiglottis Symptoms ‧ Low pitch stridor with fluttering quality ‧ Most prominent with the child in the supine position or with agitation ‧ The symptoms often do not present for days to weeks after birth Contributing factor of laryngomalacia ‧ The etiology of laryngomalacia remains unknown ‧ Anatomic, neurologic, and inflammatory factors ‧ The most common underlying anatomic pathologies: shortening of the aryepiglottic folds and anterior collapse of the cuneiform cartilage Scott C. Manning et al. in Apr. 2005: The mean AE fold length-glottic length ratio for patients with severe laryngomalacia (0.380) was significantly lower than the mean ratio for controls (0.535) (p=0.004) The aryepiglottoplasy procedure resulted in an average AE length increase-glottic length ratio of 0.330 ‧ Immature neuromuscular control ‧ Gastroesophageal reflux is frequently associated with laryngomalacia (35-68%) negative intrathoracic pressure + partially collapsed supraglottic larynx: retrograde flow of gastric content into the esophagus pharyngoesophageal reflux: induce posterior supraglottic edema and secondarily laryngomalacia Diagnosis ‧ generally suspected on auscultation of stridor ‧ confirmed by flexible nasopharyngoscopy ‧ Synchronus airway lesions are reported in up to 19% of affected infants High-kilovolt radiographic films Barium swallow esophogram Airway fluoroscopy Rigid endoscopy ‧ Robert F. Mancuso in 1996 (233 cases): The necessary of rigid endoscopy in the diagnosis and management layngomalacia and its associated synchronous airway lesions (SAL) SAL were discovered in 44 patients (18.9%), 11 patients (4.7%) had SALs that considered clinically significant, 9 patients (3.9%) required surgical intervention Rigid endoscopy in evaluation of an infant with laryngomalacia is rarely necessary Appropriate indications for rigid endoscopy: a. uncharacteristic of laryngomalacia b. inconsisitent with findings observed with flexible fiberoptic laryngoscope c. abnormal high-kilovolt anteroposterior and lateral airway radiographic films Prognosis ‧ Prognosis is excellent ‧ Usually resolves without therapy by 12 to 18 months of age Treatment ‧ Rassurance: generally a self-limiting condition. ‧ Surgical indication in severe cases (10%): a. inability to feed orally b. Cor pulmonale c. Failure to thrive d. Life threatening episodes of airway obstruction ‧ In 1995 Roger, et al.: (115 patients) established criteria for the definition of severe laryngomalacia (3 of these criteria is a formal indication for endoscopic surgery) 1. Dyspnea at rest and/or severe dyspnea during effort 2. Feeding difficulties 3. Height and weight growth rate stagnation 4. Sleep apnea or obstructive hypoventilation 5. Uncontrollable gastroesophageal reflux 6. History of intubation for obstructive dyspnea 7. Effort hypoxia (10% higher than the normal values for the same age group) 8. Effort hypercapnia ( 10% higher than the normal values for the same age group) 9. Abnormal polysomnography with an increased apnea/obstructive hypoventilation index ‧ Before the 1980s, tracheotomy was often used: carries notable morbidity and mortality, including speech delay, lumen occlusion, and death ‧ Supraglottoplasty In 1922, Iglauer performed the first successful surgical alteration of the supraglottic larynx for laryngomalacia : amputated the redundant epiglottis with the wire snare Schwartz (1944): noted immediate improvement in a patient following removal of a V-shaped wedge of tissue from epiglottis: no long-term results In 1971, Fearon and Ellis: successfully decannulated a patient with severe laryngomalacia, shortly after suturing the epiglottis to the base of the tongue In 1971, hyomandibulopexy was advocated in France In 1981, Templer et al.: lateral phayngotomy approach to excise the epiglottis, ventricular bands, and aryepiglottic folds in an 18-year-old man In 1984, Lane et al.: using microcupped forceps and Belllucci scissors, the tips of the arytenoids, and a lateral band from each side of the epiglottis were removed In 1985 Seid et al.: division of abnormally short aryepiglottic folds was accompanied with the CO2 laser ( 3 patients with severe laryngomalacia) In 1987, Zalzal et al.: “epiglottoplasty”, trimming mucosa from the lateral edges of the epiglottis, the aryepiglottic folds, and the arytenoids and corniculate cartilages with microlaryngeal scissors In 1987, Solomons and Prescott: “ supraglottic trimming” and “ anterior epiglottopexy” In 1995 Kelly, et al.: a. An episode of asymptomatic, posterior, supraglottic stenosis following bilateral carbondioxide laser supraglottoplasty in 1991 b. Unilateral endoscopic supraglottoplasty for treatment of severe laryngomalacia (18 patients) c. Three required a contralateral supraglottoplasty d. All but one had good relief of airway obstructive symptoms In 2001 Reddy, et al.: unilateral vs bilateral supraglottoplasty for severe laryngomalacia in children In 2002, Werner et al.: isolated posterior displacement of the epiglottis, performing epiglotopexy for the treatment of severe layngomalacia In 2005, Zalzal et al.: a new approach to supraglottoplasty by making use of the microdebrider ‧ The occasional severely affected infant may still require a tracheostomy Failures and compilcations of supraglottoplasty in children ‧ F. Denoyelle et al. in 2003: Compare children with isolated layrngomalacia (IL) with those who have additional congenital anomalies (ACAs) Presence of ACAs is a risk factor for failure or partial failure, but the compication rate is similar to that observed in children with IL. The use of laser vs microinstruments was not statistically a risk factor Avoid large mucosal resections and propose patients to the parents to first attempt nasogastic feeding, oxygen therapy, or noninvasive ventilatory assistance Reference: • Bailey BJ. Head and Neck Surgery–Otolaryngology 3rd Edition. 2001: 902-3. • Denoyelle F, Mondain M, Gresillon N, Roger G, Chaudre F, Garabedian EN. Failures and complications of supraglottoplasty in children. Archives of Otolaryngology Head and Neck Surgery. 2003 Oct;129 (10): 1077-80. • Iglauer S. Epiglottidectomy for the relief of congenital laryngeal stridor, with report of a case. Laryngoscope. 1922; 32: 56-59. • Lane RW, Weider DJ, Steinem C, Marin-Padella M. Laryngomalacia: a review and case report of surgical treatment with resolution of pectus excavatum. Archives of Otolaryngology Head and Neck Surgery. 1984; 110: 546-51. • Mancuso RF, Choi SS, Zalzal GH, Grundfast KM. Laryngomalacia: The search for the second lesion. Archives of Otolaryngology Head and Neck Surgery. 1996 Mar; 122 (3): 302-6. • Manning SC, Inglis AF, Mouzakes J, Carron J, Perkins JA. Laryngeal anatomic differences in pediatric patients with severe laryngomalacia. Archives of Otolaryngology Head and Neck Surgery. 2005 Apr; 131 (4): 340-3. • Olney DR, Greinwald JH Jr, Smith RJ, Bauman NM. Laryngomalacia and its treatment. Laryngoscope. 1999 Nov; 109 (11): 1770-5. • Reddy DK, Matt BH. Unilateral vs. bilateral supraglottoplasty for severe laryngomalacia in children. Archives of Otolaryngology Head and Neck Surgery. 2001 Jun; 127 (6): 694-9. • Roger G, Denoyelle F, Triglia JM, Garabedian EN. Severe laryngomalacia: surgical indications and results in 115 patients. Laryngoscope. 1995 Oct;105: 1111-7. • Seid AB, Park SM, Kearns MJ, Gugeheim S. Laser division of the aryepiglottic folds for severe laryngomalacia. International Journal of Pediatric Otorhinolaryngology. 1985; 10: 153-8. • Werner JA, Lippert BM, Dunne AA, Ankermann T, Folz BJ, Seyberth H. Epiglottopexy for the treatment of severe laryngomalacia. European Archive of Otorhinolaryngology. 2002 Oct; 259: 459-64. • Zalzal GH, Anon JB, Cotton RT. Epiglottoplasty for the treatment of laryngomalacia. Annals of Otology, Rhinology, and Laryngology. 1987; 96: 72-6. • Zalzal GH, Collins WO. Microdebrider-assisted supraglottoplasty. International Journal of Pediatric Otorhinolaryngology. 2005 Mar; 69 (3): 305-9. • Holinger LD, Konior RJ. Surgical management of severe layrngomalacia. Laryngoscope 1989;99: 136-42. • Kelly SM, Gray SD. Unilateral endoscopic supraglottoplasty for severe laryngomalacia. Arch Otolaryngol Head and Neck Surg 1995; 121:1351-4.