Differential diagnosis and management of adult-onset laryngomalacia

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Abstract

Objective

Adult-onset laryngomalacia is a rare clinical entity that has been infrequently reported. This study aims to evaluate the clinical presentation, diagnosis, and management of adult-onset laryngomalacia through literature review and report of a case.

Methods

PubMed and Google Scholar databases were queried for articles published from 1960 to 2019 including only patients aged 18 years and older. Included keywords were: ‘laryngomalacia’, ‘adult laryngomalacia’, ‘acquired laryngomalacia’, ‘idiopathic laryngomalacia’, ‘laryngeal obstruction’, ‘floppy epiglottis’, ‘floppy epiglottis’, and ‘epiglottis prolapse’. Data extracted from literature included clinical presentation, diagnostic workup, surgical management, and follow-up care.

Sources

PubMed and Google Scholar.

Results

A total of 21 articles reported 41 cases of adult-onset laryngomalacia. Within these cases, 5 etiologies were identified: neurologic (n = 14), exercise-induced (n = 9), post-operative (n = 7), idiopathic (n = 7), and age-related (n = 4) laryngomalacia. Anterior prolapse of arytenoids and aryepiglottic folds was the most common laryngoscopic finding (n = 21), followed by posterior epiglottic prolapse (n = 20). Management included supraglottoplasty (n = 14), epiglottidectomy (n = 8) or epiglottopexy (n = 2). Neurologic etiology required tracheotomy more often than the other etiologies (n = 5, 36% vs. 15%). Three patients were managed expectantly without surgical intervention and reported symptom resolution.

Conclusion

Adult laryngomalacia is a rare diagnosis comprising a spectrum of disease. This diagnosis may be overlooked, but association with neurologic injury or trauma should encourage consideration. In comparison to pediatric laryngomalacia, patients often require surgical intervention. Surgical decision is based on the direction of supraglottic collapse, where supraglottoplasty and partial epiglottidectomy are effective interventions.

Level of evidence

N/A.

Introduction

Adult-onset laryngomalacia is an incompletely understood and perhaps underdiagnosed clinical entity. The term laryngomalacia is most frequently used in reference to pediatric patients; however, this condition can affect adults in the absence of congenital anomaly.

During normal breathing, the epiglottis tilts anteriorly to enable airflow into the larynx; in contrast, the supraglottic larynx in a patient with laryngomalacia collapses on inspiration. This collapse decreases airway diameter and can cause stridor and dyspnea. The differential diagnosis for an adult presenting with stridor and dyspnea is broad, encompassing conditions such as paradoxical vocal fold motion, laryngeal stenosis, asthma, and the less commonly reported adult-onset laryngomalacia. This study aims to assemble and analyze the reported cases of adult laryngomalacia in an effort to improve understanding of the clinical presentation, contributing factors, and treatment outcomes. A case report of adult-onset laryngomalacia is also presented.

Section snippets

Methods

PubMed and Google Scholar searches were conducted with the terms ‘laryngomalacia’, ‘adult laryngomalacia’, ‘acquired laryngomalacia’, ‘idiopathic laryngomalacia’, ‘laryngeal obstruction’, ‘floppy epiglottis’, ‘floppy epiglottis’, and ‘epiglottis prolapse’. Case reports or articles describing presentation and management of laryngomalacia in persons aged 18 or above were included. No restrictions were applied to study design, date of publication, or language. Articles were screened for the

Case report

A thirty-six-year-old female presented to laryngology clinic with 2–3 weeks of variable dyspnea, inspiratory stridor and throat irritation worsened by inspiring or laying supine. Flexible laryngoscopy demonstrated anteromedial prolapse of arytenoid cartilages and aryepiglottic folds during inspiration (Fig. 1). Initial trial of respiratory retraining therapy did not improve symptoms. CO2 laser supraglottoplasty was then performed with symptom resolution. Six years after initial surgery, the

Patient demographics

Forty-one cases of adult laryngomalacia in 21 reports (26M:14F; median 42 years old) were identified for review [[1], [2], [3], [4], [5], [6], [7], [8], [9], [10], [11], [12], [13], [14], [15], [16], [17], [18], [19]]. Most common symptoms at presentation were inspiratory stridor (n = 26) and dyspnea (n = 11); other associated symptoms included dysphonia (n = 3), throat irritation or cough (n = 2), and dysphagia (n = 2). Symptom onset coincided with extubation in four patients and decannulation

Discussion

The diagnosis of adult-onset laryngomalacia requires first consideration in the differential diagnosis and differentiation from similar pathologic states. Awake fiberoptic laryngoscopy is the gold standard for diagnosis and identifies the direction of supraglottic collapse, which guides surgical management. The etiology of laryngomalacia does not always dictate the direction of supraglottic collapse, but the majority of patients with post-operative (71.4%) and neurologic (64.3%) laryngomalacia

Conclusion

Adult laryngomalacia is a rare diagnosis comprising a wide spectrum of disease. Although the diagnosis is frequently overlooked within the adult population, association with neurologic injury, surgery, exercise, comorbidity, or age should encourage consideration. Adult patients more commonly require surgical intervention than pediatric patients. Surgery is based on the direction of supraglottic collapse, where partial epiglottidectomy and supraglottoplasty are effective interventions.

Declaration of competing interest

None.

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