Surgical treatment of laryngomalacia

Surgical treatment of laryngomalacia

Operative Techniques in Otolaryngology (2005) 16, 198-202 Surgical treatment of laryngomalacia Peggy E. Kelley, MD From the Department of Otolaryngol...

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Operative Techniques in Otolaryngology (2005) 16, 198-202

Surgical treatment of laryngomalacia Peggy E. Kelley, MD From the Department of Otolaryngology, University of Colorado Health Sciences Center, Denver, Colorado. KEYWORDS Laryngomalacia; Supraglottoplasty; Stridor

When evaluating an infant for stridor, laryngomalacia is the most commonly encountered diagnosis. In most cases, laryngomalacia is a benign, self-limited diagnosis, and treatment is expectant. In more severe cases, the child may have respiratory distress or feeding difficulties. Significant laryngomalacia may require medical and/or surgical therapy. Supraglottoplasty is indicated if airway or feeding symptoms persist after a careful preoperative evaluation and trial of medical therapy. A conservative surgical approach with division of aryepiglottic folds or unilateral mucosal ablation will avoid the serious complication of supraglottic stenosis. The success rate of supraglottoplasty for relieving symptoms of airway distress and feeding difficulties is excellent, and tracheotomy is rarely required for airway control. © 2005 Elsevier Inc. All rights reserved.

Laryngomalacia is the term most widely used to describe the “inward collapse of supraglottic structures during inspiration,” as originated by Jackson and Jackson in 1942.1 The inward collapse of the epiglottis or arytenoids results in stridor that is typically inspiratory and high-pitched. Laryngomalacia is often cited as the most common congenital laryngeal anomaly and most frequent cause of stridor in infants.1 The stridor is often present at birth and is usually noticed by 2 weeks of age. Laryngomalacia is usually positionally dependent and is worse at times of increased work of breathing. Older children and adults may be diagnosed with laryngomalacia occurring particularly during exercise. This result brings into question the idea that laryngomalacia is only a congenital problem. The stridor of laryngomalacia is well described, and the diagnosis can be easily made with fiberoptic laryngoscopy, but the pathogenesis is still unknown. The first proposed mechanism of pathogenesis was floppiness of the airway secondary to infantile cartilage abnormalities, but histologic study did not support this.2,3 Other investigators3 suggest that there is poor neuromuscular control with relative hypotonia of the supraglottic dilator muscles. There is a subset of children with laryngomalacia who have additional diagnoses,4,5 but laryngomalacia is usually isolated, and children with isolated laryngomalaAddress reprint requests and correspondence: Peggy E. Kelley, MD, Department of Otolaryngology, University of Colorado Health Sciences Center, B-455, 1056 East 19th Avenue, Denver, CO 80218. E-mail address: [email protected] 1043-1810/$ -see front matter © 2005 Elsevier Inc. All rights reserved. doi:10.1016/j.otot.2005.09.004

cia are developmentally normal. Many studies6-9 cite reflux disease as being at least a comorbid factor for explaining the symptoms of laryngomalacia. It is not clear whether the reflux is the primary problem or if reflux occurs secondary to the changes in intrathoracic pressure, with the increase work of breathing that occurs with more significant laryngomalacia. A third option is to consider reflux as either primary or secondary. To that end, it may be helpful to consider the diagnosis of laryngomalacia as having 2 subsets: (1) primary laryngomalacia (Figure 1), in which there is actual anatomic variation such as the shortening of the aryepiglottic folds or a more pronounced omega-shaped epiglottis; and (2) secondary laryngomalacia (Figure 2), in which reflux disease is the inciting factor. In primary laryngomalacia, the anatomic anomaly narrows the laryngeal inlet. Venturi principles of airflow then describe the resulting “floppy airway,”5 such as described in obstructive sleep apnea when the soft palate and tongue base are sucked against the posterior pharyngeal wall as a result of tonsils and/or adenoids narrowing the airway. Primary laryngomalacia may result in enough airway distress to induce secondary reflux, which then exacerbates the edema and swelling of the tissues that are being sucked into the airway with each breath, as seen on fiberoptic evaluation. Secondary laryngomalacia then is when the airway is anatomically normal to begin with, but in the child with reflux disease, the airway becomes edematous, which begins the process of narrowing and the resulting airway collapse with inspiration.

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Figure 1 (A) Primary laryngomalacia. Anatomic anomaly, such as shortened aryepiglottic folds, causes narrowing of the laryngeal inlet. Venturi principles describe forces that result in infolding of the arytenoids or epiglottis with inspiration. (B) Primary laryngomalacia after supraglottoplasty, with division of aryepiglottic folds.

Preoperative evaluation The preoperative evaluation of the patient with stridor begins with a history that includes how well the patient is eating, growing, and developing. This evaluation gives a sense as to how significant the stridor is and what treatment options may be necessary. The diagnosis of laryngomalacia is made when viewing the larynx during inspiration and expiration, and correlating the noise heard with infolding of the supraglottic structures. It is sometimes helpful to perform laryngoscopy with the patient in both sitting and laying positions. The evaluation of la-

ryngeal dynamics is most directly made in the awake patient because general anesthesia may affect the dynamics of the airway.10 The size of the flexible laryngoscope may also influence the airway dynamics by obstructing the nasal cavity, thus increasing the force for inspiration, therefore, the smallest scope that affords good visualization should be chosen for each patient. When performing laryngoscopy, the surgeon is evaluating the airway for edema, erythema, anatomic structure, and movement. Rotation of the epiglottis posteriorly, the arytenoids anteriorly, and areas of inward collapse of the supraglottic

Figure 2 (A) Secondary laryngomalacia. Narrowing of the laryngeal inlet caused by edema of the supraglottic secondary to reflux disease. (B) Secondary laryngomalacia with failure of medical therapy for reflux after supraglottoplasty with mucosal removal.

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structure are identified. In addition to noting the static end points of inspiration and expiration, evaluation should include where the larynx is positioned in the airway and if the larynx rotates as a whole up toward the tongue base into a protective position away from the esophagus. Shaker et al11 described the esophagoglottal closure reflex that occurs when the airway is exposed to reflux. This reflex may account for the neuromuscular abnormalities and uncoordinated swallowing seen in the infant with laryngomalacia. If the airway is sufficiently swollen, it may be impossible to determine if there is an underlying anatomic anomaly or if the obstruction is secondary only to edema. A medical trial with a proton pump inhibitor for mildto-moderate airway erythema and edema in the child who is not growing well or having feeding difficulties may result in improvement or resolution of the stridor.7,12 If the child is then able to feed and gain weight consistently, surgery may be avoided, particularly for secondary laryngomalacia. For severe airway obstruction, a burst of steroid for 2-5 days is often helpful for relieving acute symptoms and allowing a more precise evaluation of the underlying anatomy on repeating the laryngoscopy 1-2 weeks later. If the child presents in airway distress before 1 month of age, medical therapy is unlikely to succeed, and supraglottoplasty is offered. “Supraglottoplasty” is the term used to describe the division or removal of any supraglottic tissue for the relief of airway obstruction in the patient with laryngomalacia.10 Approximately 10% of infants with laryngomalacia require surgical intervention.13 Infants requiring surgery are those with significant primary laryngomalacia and those with secondary laryngomalacia in whom reflux therapy has been inadequate to relieve airway obstruction or feeding difficulties. Debate exists concerning the need for the evaluation of synchronous airway lesions with a full microlaryngoscopy and bronchoscopy in every child with laryngomalacia. It has been shown that the majority of secondary airway problems are found in patients who have progressive symptoms unresponsive to medical therapy or observation. Microlaryngoscopy and bronchoscopy should be performed on any child being taken to the operating room for possible supraglottoplasty but may not be necessary for every child with laryngomalacia.13

nosis.16 However, stenosis of the supraglottic is a very difficult complication to treat. Often a tracheostomy is required at least temporarily. Unilateral supraglottoplasty is highly successful and avoids the risk of supraglottic stenosis. The opposite side may require supraglottoplasty at a future date 15%15 to 17%14 of the time. Bilateral supraglottoplasty also has a reoperation rate of 5.1%.15 Therefore, unilateral supraglottoplasty is recommended. Once the surgical plan has been made, the larynx is then exposed. Options for laryngeal exposure include suspension laryngoscopy with a microscope for visualization or suspension laryngoscopy and the endoscope for visualization. Suitable laryngoscopes include the Cherry Jako, Jako-Cherry, Benjamin, and Lindholm operating laryngoscopes. The latter laryngoscopes provide superior binocular vision and a broad inlet to facilitate instrument manipulation.17 The laryngoscope tip may be placed in the vallecula or against the laryngeal surface of the epiglottis, depending on the portion of the larynx needing exposure and manipulation. Tissue can be divided or resected using microlaryngeal instruments or the carbon dioxide laser. Typical settings for the carbon dioxide laser are super-pulse mode at 6-8 W for a spot size of 0.5 mm. Microlaryngeal instruments required include straight and side-biting scissors, as well as cup forceps to grasp mucosal tissue. In the very young infant, suspension laryngoscopy is easily accomplished with the anesthesia intubating laryngoscope (Figure 3). The laryngoscope is lightweight and can be easily maneuvered but does require an assistant to hold the instrument. The advantage of the laryngoscope being handheld exists only when microlaryngeal instrumentation and the endoscope are the chosen techniques. Options for ventilation when using carbon dioxide laser or suspension microlaryngoscopy instrumentation include spontaneous ventilation, apneic anesthesia, or supraglottic jet ventilation. Intubation is recommended only for the specific technique described later for aryepiglottic fold division. As with any airway surgery, communication with the anesthesiologist is critical for a safe and successful outcome. The use of proximal jet ventilation is previously described.17

Surgical procedure Topical lidocaine without epinephrine is applied to the supraglottic and laryngeal mucosa as a mucosal anesthetic and to reduce reflex laryngeal adduction induced by laryngeal stimulation. Microlaryngoscopy/bronchoscopy is performed to elucidate the anatomic areas of concern in each patient with laryngomalacia and to exclude any additional airway anomalies. With adequate control of active reflux disease, the patient who remains symptomatic usually has a specific anatomic anomaly, such as shortened aryepiglottic folds. Based on the areas of anatomic narrowing, the surgical plan is made for division of aryepiglottic folds only or also for removal of unilateral inward collapsing mucosa.14,15 Bilateral mucosal excision may rarely result in supraglottic ste-

Figure 3 Use of intubating laryngoscope and endoscope instead of suspension laryngoscopy and microscope.

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Figure 4 View of larynx with Wis-blade laryngoscope and 4-mm Hopkins rod laryngoscope, before (A) and after (B) division of aryepiglottic folds.

Either the carbon dioxide laser or microlaryngeal instruments may be used to perform supraglottoplasty. The use of the carbon dioxide laser requires patient protection from divergent laser beams, with moist eye pads and towels to cover any exposed skin. The carbon dioxide laser is used to divide the aryepiglottic folds and remove edematous mucosa overlying the arytenoids. Microlaryngeal instruments divide sharply the aryepiglottic fold with scissors. Mucosal resection is accomplished using cup forceps and scissors (Figure 4). If division of the aryepiglottic folds is needed without mucosal resection, the patient may be intubated for microlaryngeal instrumentation. The endotracheal tube can be used to leverage open the narrowed laryngeal inlet by the endoscope and to expose the aryepiglottic fold for division. The patient is then extubated at the end of the procedure if possible. Using the endoscope and handheld laryngoscope instead of the microscope with full suspension laryngoscopy reduces surgical time because the microscope and suspension apparatus do not need to be positioned. The shorter duration also minimizes the length of time the larynx is traumatized with suspension instruments. Using the endoscope and anesthesia laryngoscope, the patient is routinely extubated at the end of the procedure. The patient who is extubated is monitored in an airway unit or pediatric intensive care unit until breathing well by mouth without supplemental oxygen and is discharged in 1-3 days. The patient who is intubated

Table 1

Complications Complications of supraglottoplasty are rare (10 of 136 [7.4%]).16 Although few in number, the complications of supraglottoplasty have significant morbidity. Therefore, it is incumbent on the surgeon to prevent and avoid as many of the complications as possible. Complications cited include granulomas,16 sepsis,10 need for further procedures,14-16 and supraglottic stenosis.15,16 Supraglottic stenosis is very difficult to correct once it develops. Multiple investigators14-16 now agree that the risk of a second surgery while performing a unilateral supraglottoplasty with mucosal ablation is less than the benefit of avoiding supraglottic stenosis altogether. Therefore, it is recommended to begin conservatively and remove only that mucosa that is necessary, and to avoid bilateral mucosal removal at the initial operations.

Supraglottoplasty

Indications ● ● ● ● ● ●

is usually extubated the day after the procedure, and feeding is begun. Hospital discharge is common in 2-5 days.14 The success rate for the procedure is reported between 80%16 and 100%14,15 of the time. It is noteworthy that the lower success rate was recorded in patients with additional airway abnormalities5,16 and before proton pump inhibitors were widely used. Highlights of the supraglottoplasty procedure are listed in Table 1.

Failure to thrive Airway obstruction Cyanosis with feeds Cor pulmonal Apneic spells Bradycardic spells

Contraindications

Special instrumentation

Tips and pearls

● Other uncorrectable airway anomalies ● Multiple levels of anomalies ● Uncontrollable edema of the airway

● Microlaryngeal scissors, straight and side opening ● Carbon dioxide laser

● Control reflux first ● Use antibiotics, steroids, and proton pump inhibitors postoperatively

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Postoperative care Postoperative care is chosen to minimize the chance of surgical complication. Empiric treatment with a proton pump inhibitor for the possible negative effects of reflux on airway healing should be considered for at least the first 6 weeks after supraglottoplasty.15,18 Steroids are helpful for controlling airway edema and modulation of healing. Steroids are given for 5 days postoperatively. If stridor returns at the conclusion of the steroids, a longer tapering dose over 2 weeks may be helpful early during the postoperative course. Antibiotics are given for 1–2 weeks, depending on the length of time a steroid is used. Follow-up laryngoscopy is performed at the 2-week postoperative visit to monitor healing and assess the potential for supraglottic stenosis.

Long-term follow-up Once outside the initial healing period, it is uncommon for symptoms to recur. Laryngomalacia generally resolves during the first year of life, even in children with additional diagnoses.13 Therefore, long-term follow-up is expectant as it is with the children who have milder laryngomalacia not requiring surgical therapy.

References 1. Holinger LD: Congenital laryngeal anomalies, in Holinger LD, Lusk RP, Green CG (eds): Pediatric Laryngology and Bronchoesophagology. Philadelphia, PA, Lippincott-Raven, 1997, pp 139-142 2. Kelemen G: Congenital laryngeal stridor. AMA Arch Otolaryngol 58:245-268, 1953

3. Chandra RK, Gerber ME, Holinger LD: Histological insight into the pathogenesis of severe laryngomalacia. Int J Pediatr Otorhinolaryngol 61:31-38, 2001 4. Portier F, Marianowski R, Morrisseau-Durand MP, et al: Respiratory obstruction as a sign of brainstem dysfunction in infants with Chiari malformations. Int J Pediatr Otorhinolaryngol 57:195-202, 2001 5. Sichel JY, Dangoor E, Eliashar R, et al: Management of congenital laryngeal malformations. Am J Otolaryngol 21:22-30, 2000 6. McClurg FL, Evans DA: Laser laryngoplasty for laryngomalacia. Laryngoscope 104:247-252, 1994 7. Roger G, Denoyelle F, Triglia JM, et al: Severe laryngomalacia: Surgical indications and results in 115 patients. Laryngoscope 105: 1111-1117, 1995 8. Belmont JR, Grundfast K: Congenital laryngeal stridor (laryngomalacia) etiologic factors and associated disorders. Ann Otol Rhinol Laryngol 93:430-437, 1984 9. Phelan PD, Gillam GL, Stocks JG, et al: The clinical and physiological manifestations of the “infantile larynx”: Natural history and relationship to mental retardation. Aust Paediatr J 7:135-140, 1971 10. Holinger LD, Konior RJ: Surgical management of severe laryngomalacia. Laryngoscope 99:136-142, 1999 11. Shaker R, Dodds WJ, Ren J, et al: Esophagoglottal closure reflex: A mechanism of airway protection. Gastroenterology 102:857-861, 1992 12. Polonovski JM, Contencin P, Francois M: Aryepiglottic fold excision for the treatment of severe laryngomalacia. Ann Otol Rhinol Laryngol 99:625-627, 1990 13. Olney DR, Greinwald JH Jr, Smith RJ, et al: Laryngomalacia and its treatment. Laryngoscope 109:1770-1775, 1999 14. Kelly SM, Gray SD: Unilateral endoscopic supraglottoplasty for severe laryngomalacia. Arch Otolaryngol Head Neck Surg 121:13511354, 1995 15. Reddy DK, Matt BH: Unilateral vs bilateral supraglottoplasty for severe laryngomalacia in children. Arch Otolaryngol Head Neck Surg 127:694-699, 2001 16. Denoyelle F, Mondain M, Gresillon N, et al: Failures and complications of supraglottoplasty in children. Arch Otolaryngol Head Neck Surg 129:1077-1080, 2003 17. Bauman NM, Smith RJH: Surgical management of laryngomalacia. Oper Tech Otolaryngol Head Neck Surg 10:253-258, 1999 18. Rudolph CD: Gastroesophageal reflux and airway disorders, in Myer CM, Cotton RT, Shott SR (eds): The Pediatric Airway: An Interdisciplinary Approach. Philadelphia, PA, Lippincott, 1995, pp 327-357