Approach

Treatment of laryngomalacia (LM) should be individualised according to disease severity.[21] Disease severity does not correlate with the intensity or frequency of stridor, but with the presence of associated symptoms.[7]

Most cases are mild, and it is appropriate to adopt a conservative approach with regular review and monitoring of growth using centile charts. If an expectant approach is not appropriate due to the severity, then surgery to address the LM should be considered. Endoscopic surgical treatment is undertaken if the patient becomes compromised by airway obstruction or if feeding is disrupted sufficiently to prevent normal growth. In some patients (e.g., in cases where endoscopic surgery has failed or when there are medical comorbidities), tracheostomy may be necessary. This facilitates bypass of the supraglottic obstruction until spontaneous resolution occurs with growth.

Pressure-assisted ventilation (e.g., bilevel positive airway pressure [BiPAP]) may be used in patients with obstructive sleep apnoea (OSA) who are not surgical candidates or where surgery has failed to improve airway obstruction. It may also be useful as an interim measure: for example, to enable further surgical procedures to be delayed in the early postoperative period.

Co-existing GORD should be assessed and treated appropriately in all patients, with either changes to feeding techniques, medication, or, in severe cases, surgical intervention. Eosinophilic oesophagitis should be excluded by oesophagoscopy and biopsy in cases with persistent laryngeal oedema contributing to LM.[21]

Research continues to address the relative lack of high-level evidence examining treatments, outcomes, and effects of comorbidities of laryngomalacia.[33]

Mild LM

These patients have an audible stridor and endoscopic features of LM, but no respiratory distress and no evidence of failure to thrive (i.e., steady growth on weight centile charts).

Most patients with LM are managed conservatively with observation without surgical intervention. They should be kept under regular review until the condition resolves to ensure the LM is not progressing in severity.

Reflux and minor feeding difficulties should be assessed and treated appropriately. Parents can be reassured that this condition typically resolves spontaneously.

Moderate LM

Moderate disease is associated with stridor, increased work of breathing, progressive feeding difficulties, and either weight loss or inadequate weight gain.

A conservative approach may be taken where the child is kept under observation and regular review to ensure that he or she is developing adequately. Reflux and minor feeding difficulties should be assessed and treated appropriately.

If the child has significant airway obstruction or feeding difficulties affecting growth, then endoscopic supraglottoplasty to modify the supraglottis and relieve the obstruction is appropriate.

Severe LM

Severe disease occurs in 10% to 15% of patients.[34][35][36] These infants may have significant shortness of breath and airway obstruction, failure to thrive, marked dysphagia, associated apnoeas, hypoxia or hypercapnia, pulmonary hypertension, cor pulmonale, delayed neuropsychomotor development, obstructive sleep apnoea, or severe chest deformity (pectus excavatum).

Endoscopic supraglottoplasty is the treatment of choice for all patients with severe disease. Treatment of severe LM underwent a significant change in the 1980s when endoscopic surgery became available as an alternative to tracheostomy or prolonged nasogastric feeding.[35][37][38][39]

Role of surgery

Endoscopic supraglottoplasty (aryepiglottoplasty)

  • This procedure to modify the supraglottis to relieve obstruction has an excellent success rate, with reports of 79% to 98% of cases having a good outcome.[7][35]

  • Several forms of supraglottoplasty have been described. Surgery is selected according to the observed anatomical features in order to address the main source of supraglottic obstruction. Options include division or excision of the aryepiglottic folds, with removal of redundant supra-arytenoid mucosa, with or without cuneiform or corniculate cartilage if necessary, taking care to preserve the interarytenoid mucosa.[1][40] Alternatively, epiglottopexy may be undertaken, to adhere the epiglottis to the tongue base using a laser, with or without sutures.[3] Partial amputation of the epiglottis has also been described. Unilateral supraglottoplasty with division of a single aryepiglottic fold is recommended by some authors to minimise the risk of subsequent supraglottic stenosis or aspiration.[41][42]

  • No difference in outcome has been found using CO₂ laser, laryngeal microscissors, or microdebrider.[7][36][37][38][39]

  • Repeat procedures may be performed if necessary to address other components of the obstruction. Patients with additional congenital anomalies have poorer outcomes but no higher rate of complications than patients with isolated LM.[35] Poor outcomes also occur with widespread pharyngolaryngomalacia, and these patients may require eventual tracheostomy.[7][43]

  • Complications occur in less than 8% of cases, and are related to the extent of surgery, amount of tissue excised, and mode of excision.[35] Complications include granulomas, synechiae, aspiration, and supraglottic stenosis.[35][42] Supraglottic stenosis occurs in 2% to 4% of cases and is difficult to treat.[35][41] Unilateral supraglottoplasty with division of a single aryepiglottic fold has the lowest morbidity, but has the disadvantage of having a higher chance of multiple procedures.[1][35][41]

  • Comorbidities of neurological disorders, syndromes, and congenital heart disease need to be recognised and factored into surgical decision-making. Patients with additional disorders were shown to have an increased risk of aspiration following surgery than those without comorbidities, and delayed post-operative diagnosis of a co-existing neurological disorder has been shown to be significantly associated with surgical failure.[44][45] The potential benefits of favourable outcomes versus the risks of complications require careful consideration in these complex cases.

    [Figure caption and citation for the preceding image starts]: Laryngeal appearance after supraglottoplasty using cold steelFrom the personal teaching collection of Simone J. Boardman, MBBS, FRACS (OHNS) and C. Martin Bailey, BSc, FRCS, FRCSEd [Citation ends].com.bmj.content.model.Caption@584d1340

Tracheostomy

  • May be necessary in children with severe LM. It serves to bypass the supraglottic obstruction until spontaneous resolution occurs with growth.

  • It is used in cases where endoscopic surgery has failed or when there are other indications for tracheostomy due to medical comorbidities.

  • There is substantial potential for short- and long-term morbidity with tracheostomy, including a tracheostomy-related mortality rate of about 2%.[46]

Management of GORD

All patients require assessment and treatment of GORD if necessary. Reflux ought to be treated regardless of whether or not surgery is undertaken for the LM. Both conditions are closely related and one may exacerbate the other. Control of reflux may improve the degree of airway obstruction by reducing laryngeal inflammation and oedema.[40] Reflux also often improves significantly with supraglottoplasty due to a decrease in negative intrathoracic and intra-oesophageal pressures.[17] Untreated reflux may delay healing after surgery.[16]

Simple treatment options include nursing upright, using bottles to minimise aerophagia, or using thickening feeds. If these conservative measures fail, then options for medical treatment include an H2 antagonist (e.g., famotidine) or a proton-pump inhibitor (e.g., omeprazole). Persistent GORD can be treated surgically with Nissen's fundoplication.

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