Central Annals of Otolaryngology and Rhinology

Cite this article: Walner DL, Neumann DB (2017) The Classification of Laryngomalacia. Ann Otolaryngol Rhinol 4(2): 1165.

*Corresponding author

David L. Walner, Department of Surgery, Pediatric Otolaryngology, Pediatric Airway Center, Advocate Children’s Hospital, Park Ridge, IL, USA, 8780 W. Golf Rd, Niles, IL 60714, Tel: (847) 674-5585; Fax: (847) 674-5598, Email:

Submitted: 18 February 2017

Accepted: 20 March 2017

Published: 22 March 2017

ISSN: 2379-948X

Copyright© 2017 Walner et al.

OPEN ACCESS

Keywords•Airway obstruction•Classification•Laryngomalacia

Short Communication

The Classification of LaryngomalaciaDavid L. Walner1,2*, and Daniel B. Neumann3

1Department of Surgery, Pediatric Otolaryngology, Pediatric Airway Center, Advocate Children’s Hospital, USA2Department of Otolaryngology, Rosalind Franklin University of Medicine and Science, USA3Department of Pediatrics, Advocate Children’s Hospital, USA

Abstract

Objective: To evaluate existing classification systems for laryngomalacia and describe a more complete and comprehensive system

Methods: We searched the literature for previously described classification systems for laryngomalacia. We attempted to use each of these systems to classify a cohort of patients, but were unsuccessful due to deficiencies in each system. We subsequently developed a novel system which allows comprehensive and accurate classification of patients with laryngomalacia.

Results: We identified 6 existing systems to classify laryngomalacia. After trying to use these existing systems to classify a cohort of patients, we identified deficiencies with each system. We then created our own system which defines four major categories based on anatomical and pathophysiological variations. LM1 refers to anterior prolapse of the posterior (arytenoid) structures. It is subdivided into LM1a which describes prolapse of mucosa and cuneiform cartilage, and LM1b which also includes prolapse of the larger corniculate and arytenoid cartilages. LM2 describes short aryepiglottic folds. LM3 is comprised of anatomical abnormalities of the epiglottis. It is subdivided to describe a curled, tubular, or long epiglottis (LM3a), a posterior petiole (LM3b), or posterior epiglottic collapse (LM3c). LM4 refers to generalized collapse of the supraglottic structures.

Conclusion: Current classification systems to describe laryngomalacia have many deficiencies. Our newly described classification system more accurately portrays the anatomical and physiologic variations found in laryngomalacia.

INTRODUCTIONLaryngomalacia (LM) is the leading cause of stridor in infants

and the most common congenital laryngeal anomaly [1]. In a majority of cases, congenital LM presents within weeks of birth and is a self-limiting process that requires frequent monitoring and watchful waiting [2]. Cases involving severe respiratory compromise or failure to thrive often require surgical treatment to resolve symptoms. While the etiology of LM remains unclear, the variation of presentation among patients suggests a multifactorial origin [3].

Since the original description of LM in the late 1800s (then termed congenital laryngeal stridor), several different systems have been used to classify LM mainly based on anatomical differences [4-9]. To date, there is no universal or validated system to classify LM. A universal system would be ideal for several reasons. It would allow physicians to be less subjective

in their findings. It would permit more accurate and effective medical and surgical planning. It would also enable more accurate communication among researchers and physicians. In this paper we summarize several previously described classification systems for LM. In an effort to develop a universal system, we describe a novel classification method for LM based on anatomical and pathophysiological variations.

MATERIALS AND METHODSWe performed a review of the literature and identified 6

unique classification systems for LM (Table 1). After receiving Institutional Review Board approval from our institution, charts were reviewed to identify patients with severe LM who required surgical intervention from June 2007 to June 2012. We identified 23 patients and attempted to use each of the classification systems to categorize each patient. This categorization was based on results from preoperative awake flexible laryngoscopy

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as well as operative microlaryngoscopy under general anesthesia with spontaneous respirations. Because deficiencies in each classification system prevented us from accurately classifying the majority of patients, we developed our own system (Table 2). We were able to successfully classify each of the patients using the new system. Finally, we identified 5 patients from our cohort with varying types of LMto demonstrate the deficiencies in the older classification systems.

RESULTSOverall, we identified 6 classification systems for categorizing

patients with LM (each system is listed in Table (1). The earliest classification system was reported in 1977 by McSwiney et al [4] in which they reviewed characteristics of 21 infants with LM to describe the natural progression of patients with this disease. Overall, they identified 3 main anatomical variations which occur in isolation or in combination with each other. For more than a decade this system was used until Holinger and Konior [5] developed a novel system. They described 13 infants who were treated with supraglottoplasty for severe LM and suggested 6 classification types.

A third classification system was reported in 1995 by Roger et al. [6] in which they published the surgical outcomes of 115 children treated for severe LM. They identified three anatomical variations in their patients. A few years later, Olney et al. [7] performed a retrospective review of 58 infants with LM and described their outcomes. While they reported a new classification system, the categories they used (Types 1, 2,

and 3) appear to be nearly identical to categories C, B, and A, respectively, as described previously by McSwiney et al [4].

Kay and Goldsmith [8] appear to be the first to propose a classification system based not only on anatomical variations among patients (short AE folds, redundant supraglottic tissue), but also on the underlying pathophysiologic mechanism (neuromuscular dysfunction).Most recently, Lee et al. [9] described the postoperative outcomes of 138 infants with severe LM and classified patients using a 3-category system. Their 3 categories were based on the direction of supraglottic collapse.

After identifying these classification systems, we took a cohort of 23 patients with severe LM and attempted to categorize them using each previously described classification system. We were unable to accurately and comprehensively classify 17 of the 23 patients. We then created a classification system that allowed each patient to be accurately categorized and described (Table 2). To show the deficiencies in the older classification systems, we describe 5 patients from our cohort with varying types of LM. The age at time of surgery, type of surgical management, and comorbidities for each patient are presented in Table (3).

Patient 1 was found to have predominately right-sided prolapse of arytenoid mucosa and cuneiform cartilage (Figure 1a). Of the 6 previously described systems, none of them differentiate between prolapse of arytenoid mucosa and cartilaginous structures. Another deficiency of all 6 systems is that they don’t allow description of one side being more affected than the other side. For example, Patient 1 had the right side predominately

Table 1: Previously described classification systems for laryngomalacia.Year/Authors Categories Description

1977 McSwiney, Cavanagh, Languth [4]

A Long, curled (omega-shaped) epiglottis which prolapses posteriorly during inspiration

B Short AE folds

C Bulky arytenoids with collapse anteriorly during inspiration

1989 Holinger, Konior [5]

Type 1 Inward collapse of AE folds and cuneiform cartilage

Type 2 Long, tubular (omega-shaped) epiglottis

Type 3 Anterior/medial collapse of arytenoid cartilage

Type 4 Posterior collapse of epiglottis

Type 5 Short AE folds

Type 6 Overly acute angle of the epiglottis at the laryngeal inlet

1995 Roger, Denoyelle, Triglia, Garabedian [6]

Complete form Omega-shaped epiglottis, short AE folds, and redundant supraglottic mucosaPredominately posterior form Anterior displacement of supra-arytenoid mucosa

Isolated anterior form Posterior-medial displacement of the epiglottis

1999 Olney, Greinwald, Smith, Bauman [7]

Type 1 Anterior/medial collapse of supra-arytenoid mucosa

Type 2 Short AE folds

Type 3 Posterior collapse of epiglottis

2006 Kay, Goldsmith [8]

Type 1 Short AE folds

Type 2 Redundant tissue in the supraglottis

Refers to cases with a neuromuscular dysfunction etiology

2007 Lee, Chen, Yang, Chen [9]

Type A Short AE folds or collapse of redundant cuneiform/corniculate cartilage and soft tissue

Type B Long, tubular, curled epiglottis

Type C Posterior collapse of epiglottis

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Table 2: Laryngomalacia Classification System (2016: Walner, Neumann).Type Description Notes

LM1: Anterior prolapse of posterior (arytenoid) structures

One side may be more prominent than the other; R>L or L>RLM1a Mucosa and/or cuneiform cartilage

LM1b Mucosa/cuneiform cartilage and corniculate/arytenoid cartilage

LM2: Short aryepiglottic folds

LM3: Epiglottic abnormalities

LM3a Curled, tubular, or long

LM3b Posterior position of petiole

LM3c Posterior collapse (toward or abutting posterior pharyngeal wall)

LM4: Generalized collapse Often related to neurological issues or poor muscle tone

Table 3: Surgeries and comorbidities of select patients.

Patient/ Sex LM Type Age at Surgery (months) Surgery Performed Notes

1/F LM1a(R>L)

13.514.2

Right arytenoid mucosa/cuneiform trim, AE fold releaseLeft arytenoid mucosa/cuneiform trim, left AE fold release

Had complete resolution of respiratory/feeding problems

2/F LM1b, LM3b 31.1 Right corniculate/arytenoid trimHad complete resolution of respiratory/feeding problems

3/F LM1b, LM2, LM3a* 5.0 Right epiglottic trim, AE fold release, corniculate/

arytenoid trim

Had complete resolution of respiratory/feeding problems

4/MLM1b, LM2, LM3b, LM3c LM4

35.7 Tracheostomy recommended but refused; using CPAP CHARGE syndrome

5/MLM1b, LM2, LM3a, LM3b, LM4

4.3

5.38.610.5

AE fold release, arytenoid trim, bilateral lateral epiglottic cartilage trimLateral epiglottic trimAnterior epiglottic suspensionTracheostomy

Subsequently diagnosed with Fragile X Syndrome, neurodevelopmental delay, dysphagia

Worksheet 1 - Laryngomalacia type worksheetPatient Name: _____________________ MRN: ____________ Date: ____________Surgeon: ______________________ Institution: ______________________________Overall LM Type: ____________________________LM1 Arytenoidsa. Prolapse of mucosa and/or cuneiform cartilage Right___ Left___ b. Prolapse of mucosa/cuneiform and corniculate/arytenoid cartilage Right___ Left___ LM2 Shortened AE Folds Right___ Left___ LM3 Epiglottisa. Curled/tubular/long _____b. Posterior petiole _____c. Posterior collapse _____LM4 Generalized Collapse _____

affected. Using our new system, this patient is classified as LM1a (R>L).

Patient 2 was found to have prolapse of the arytenoid mucosa and corniculate/arytenoid cartilage as well as a posterior petiole (Figure 1b). In addition to the deficiencies as pointed out with Patient 1, none of the systems attempt to describe the position of the petiole of the epiglottis. One possible exception is the system by Holinger & Konior [5], in which they describe an overly acute angle of the epiglottis, though it is uncertain as to whether or not this refers to the petiole. Another deficit in at least two of the systems [6,7] is that they only allow for a patient to be classified in one group. As such, it is impossible to accurately categorize a

patient who has more than one supraglottic anomaly contributing to the LM. Using our newly proposed system, the patient would accurately be described as LM1b, LM3b.

Patient 3 was found to have arytenoid mucosa and corniculate/arytenoid cartilage prolapse, short AE folds, and an extreme epiglottic curl (Figure 1c). As mentioned previously, the other systems are unable to differentiate between arytenoid mucosa versus cartilage prolapse. We found the system by McSwiney et al. [4] to be overly inclusive. With their system, classifying the patient in category A describes the curled epiglottis, but also includes prolapse of the epiglottis, which Patient 3 did not have. Two of the systems [7,8] do not have categories describing

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Figure 1 Endoscopic operative images of select patients with varying types of laryngomalacia1a – Patient 1 (LM1a (R>L))1b – Patient 2 (LM1b, LM3b)1c – Patient 3 (LM1b, LM2, LM3a*)1d – Patient 4 (LM1b, LM2, LM3b, LM3c, LM4)1e – Patient 5 (LM1b, LM2, LM3a, LM3b, LM4)

abnormal epiglottic anatomy. Using our new classification system, the patient would be described as LM1b, LM2, LM3a* (the asterisk denotes the most significant type).

Patient 4 was noted to have prolapse of arytenoid mucosa and corniculate/arytenoid cartilage, short AE folds, a posterior petiole, collapse of the epiglottis, and generalized collapse (Figure 1d). In addition to the deficiencies already mentioned, only the system by Kay & Goldsmith [8] has a category describing generalized collapse of supraglottic structures. We find that this usually occurs in patients with neuromuscular disorders. Our system allows for this description and classifies this patient as LM1b, LM2, LM3b, LM3c, LM4.

Patient 5 had arytenoid mucosa and corniculate/arytenoid cartilage prolapse, short AE folds, a curled epiglottis with medial obstruction, a posterior petiole, and generalized supraglottic collapse (Figure 1e). The deficiencies mentioned previously also apply to this patient, in being unable to differentiate between the type of arytenoid prolapse, being unable to describe the petiole position, and being unable to describe generalized collapse of the tissue. Using our classification system, this patient is described as LM1b, LM2, LM3a, LM3b, and LM4.

It should be noted that Patient 5 had a tracheostomy placed after the surgical procedures performed did not resolve his upper airway obstruction and dysphagia. At the age of 4 years, his neuromuscular condition had improved following aggressive speech, occupational, and physical therapies, such that the tracheotomy was successfully removed. Follow up laryngoscopy showed the surgically treated portions of the LM to have been successful and the LM4 generalized collapse to have almost completely resolved. The child subsequently did well, having a patent airway and resolution of swallowing issues.

DISCUSSIONSince the late 1970s several classification systems have been

proposed to classify patients with LM, but as demonstrated above,

all the systems have deficiencies which render them less than useful in accurately categorizing patients. Our new classification system attempts to overcome each of the deficiencies and provide a much clearer and comprehensive way of classifying patients with LM.

One aspect of our classification system that is missing in the other previously described systems is differentiating between prolapse of the arytenoid mucosa and posterior cartilages. The larger cartilages of the posterior larynx include the corniculate and arytenoid cartilages. The smaller cuneiform cartilages are located anterior to the corniculate cartilages in the AE folds. We grouped together prolapse of the arytenoid mucosa and cuneiform cartilages because surgical treatment of LM involving these structures requires a superficial trim. On the other hand, treatment for prolapse of the larger cartilages (corniculate and arytenoid) often requires a deeper resection. This differentiation is not reflected in any other system.

Another component not found in other classification systems is a category to describe a posteriorly positioned epiglottic petiole. The petiole refers to the narrow stalk-like structure of the inferior epiglottis. It is attached to the inner surface of the thyroid cartilage by the thyroepiglottic ligament. Though not included in other classification systems, we find the petiole position to be important because it can contribute to the LM/upper airway obstruction to a significant degree in select cases [9-11].

One of the benefits of having a comprehensive classification system for LM is that it can assist in surgical planning. We found that patients with different LM types went on to require different surgical interventions (Table 3). For example, patients with LM1 required trimming of the arytenoid mucosa and cuneiform cartilage only. When one side was affected more than the other, unilateral or staged bilateral supraglottoplasty was utilized [12]. Patients with LM2 required release of the AE folds. In our cohort, both patients who had severe epiglottic curl (LM3a) subsequently underwent a lateral epiglottic trim as part of their management. Patients with posterior epiglottic collapse (LM3c) may be candidates for an epiglottic suspension (scarification of the petiole region with COBLATION or the laser and suturing the petiole to the base of tongue region). When there was generalized severe supraglottic collapse (LM4) as usually related to a neuromuscular disorder, we found that patients were more likely to eventually require tracheotomy [7]. Though, as seen with Patient 5, a combination of watchful waiting along with aggressive speech, occupational, and physical therapies may improve some portions of LM related to neuromuscular problems, and patients may be able to have their tracheotomy reversed at a later time.

While a more comprehensive classification system has many benefits, there are a few limitations. One limitation is that the proposed classification system may be difficult to remember in detail. To overcome this, a worksheet is enclosed which can be taken into the operating room to assist in documentation of the patient’s LM type (Worksheet 1). While the authors have found success using this classification system, further studies are needed to validate this system.

CONCLUSION

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Current classification systems to describe LM are less than ideal. Our newly proposed classification system very specifically describes the anatomical variations present in patients with LM.

REFERENCES1. Richter GT, Thompson DM. The surgical management of

laryngomalacia. Otolaryngol Clin North Am. 2008; 41: 837-864.

2. Thompson DM. Laryngomalacia: factors that influence disease severity and outcomes of management. Curr Opin Otolaryngol Head Neck Surg. 2010; 18: 564-570.

3. Thompson DM. Abnormal sensorimotor integrative function of the larynx in congenital laryngomalacia: A new theory of etiology. Laryngoscope. 2007; 117: 1-33.

4. McSwiney PF, Cavanagh NP, Languth P. Outcome in congenital stridor (laryngomalacia). Arch Dis Child. 1977; 52: 215-218.

5. Holinger LD, Konior RJ. Surgical management of severe laryngomalacia. Laryngoscope. 1989; 99: 136-142.

6. Roger G, Denoyelle F, Triglia JM, Garabedian EN. Severe laryngomalacia: Surgical indications and results in 115 patients. Laryngoscope. 1995; 105: 1111-1117.

7. Olney DR, Greinwald JH Jr, Smith RJ, Bauman NM. Laryngomalacia and its treatment. Laryngoscope. 1999; 109: 1770-1775.

8. Kay DJ, Goldsmith AJ. Laryngomalacia: a classification system and surgical treatment strategy. Ear Nose Throat J. 2006; 85: 328-331, 336.

9. Lee KS, Chen BN, Yang CC, Chen YC. CO2 laser supraglottoplasty for severe laryngomalacia: a study of symptomatic improvement. Int J Pediatr Otorhinolaryngol. 2007; 71: 889-895.

10. Erickson B, Cooper T, El-Hakim H. Factors associated with the morphological type of laryngomalacia and prognostic value for surgical outcomes. JAMA Otolaryngol Head Neck Surg. 2014; 140: 927-933.

11. Denoyelle F, Mondain M, Gresillon N, Roger G, Chaudre F, Garabedian EN, et al. Failures and complications of supraglottoplasty in children. Arch Otolaryngol Head Neck Surg. 2003; 129: 1077-1080.

12. Walner DL, Neumann DB, Hamming KK, Miller RP. Supraglottoplasty in Infants: A Staged Approach. Ann Otol Rhinol Laryngol. 2015; 124: 803-807.

Walner DL, Neumann DB (2017) The Classification of Laryngomalacia. Ann Otolaryngol Rhinol 4(2): 1165.

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