Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

ONGOING

mild disease

1st line – observation

Back
ONGOING
|
mild disease
1st line – 

observation

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).

They may be safely observed without surgical intervention. The parents can be reassured of the high likelihood of spontaneous improvement and resolution.

Regular review and monitoring of growth using centile charts is essential until resolution, to assess any increase in disease severity.

Consider – GERD therapy

Back
ONGOING
|
mild disease
Consider – 

GERD therapy

Treatment recommended for SOME patients in selected patient group

All patients require assessment and treatment of GERD if necessary. 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 edema.[41]

Simple treatment options include nursing upright, using bottles to minimize 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 GERD can be treated surgically with Nissen fundoplication.

Primary options

famotidine: neonates and infants <3 months: 0.5 to 1 mg/kg orally once daily; children ≥3 months of age: 0.5 to 1 mg/kg orally twice daily, maximum 80 mg/day

OR

omeprazole: 0.5 to 1 mg/kg orally once daily, maximum 20 mg/day

moderate disease

1st line – observation

Back
ONGOING
|
moderate disease
1st line – 

observation

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

A conservative approach may be taken. Reflux and minor feeding difficulties should be assessed and treated appropriately.

Patients should be monitored closely to detect any progression in the severity of airway obstruction or feeding difficulties. Weights can be regularly plotted on a centile chart and provide an excellent guide for monitoring appropriate growth.

Consider – GERD therapy

Back
ONGOING
|
moderate disease
Consider – 

GERD therapy

Treatment recommended for SOME patients in selected patient group

All patients require assessment and treatment of GERD if necessary. 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 edema.[41]

Simple treatment options include nursing upright, using bottles to minimize 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 GERD can be treated surgically with Nissen fundoplication.

Primary options

famotidine: neonates and infants <3 months: 0.5 to 1 mg/kg orally once daily; children ≥3 months of age: 0.5 to 1 mg/kg orally twice daily, maximum 80 mg/day

OR

omeprazole: 0.5 to 1 mg/kg orally once daily, maximum 20 mg/day

2nd line – surgical therapy

Back
ONGOING
|
moderate disease
2nd line – 

surgical therapy

Surgical treatment is undertaken if the patient becomes compromised by airway obstruction or if feeding is disrupted sufficiently to prevent normal growth.

Endoscopic supraglottoplasty (aryepiglottoplasty) to modify the supraglottis to relieve obstruction is the preferred treatment. This may include: division or excision of the aryepiglottic folds, epiglottopexy, or partial amputation of the epiglottis.[1][3][41] Unilateral supraglottoplasty may minimize the risk of subsequent supraglottic stenosis or aspiration.[42][43]

Comorbidities of neurologic disorders, syndromes, and congenital heart disease need to be recognized 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 postoperative diagnosis of a coexisting neurologic disorder has been shown to be significantly associated with surgical failure.[45][46] The potential benefits of favorable outcomes versus the risks of complications requires careful consideration in these complex cases.

Tracheostomy may be preferred to supraglottoplasty in children with 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%.[47]

Consider – GERD therapy

Back
ONGOING
|
moderate disease
Consider – 

GERD therapy

Treatment recommended for SOME patients in selected patient group

All patients require assessment and treatment of GERD if necessary. 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 edema.[41]

Reflux also often improves significantly with supraglottoplasty due to a decrease in negative intrathoracic and intraesophageal pressures.[17] Untreated reflux may delay healing after surgery.[16]

Simple treatment options include nursing upright, using bottles to minimize 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 GERD can be treated surgically with Nissen fundoplication.

Primary options

famotidine: neonates and infants <3 months: 0.5 to 1 mg/kg orally once daily; children ≥3 months of age: 0.5 to 1 mg/kg orally twice daily, maximum 80 mg/day

OR

omeprazole: 0.5 to 1 mg/kg orally once daily, maximum 20 mg/day

3rd line – positive airway pressure

Back
ONGOING
|
moderate disease
3rd line – 

positive airway pressure

Positive airway pressure may be used in patients with obstructive sleep apnea (OSA) where surgery has failed to improve airway obstruction or in those who are not surgical candidates.

Positive airway pressure interventions may also be useful as an interim measure: for example, to enable further surgical procedures to be delayed in the early postoperative period.

Consider – GERD therapy

Back
ONGOING
|
moderate disease
Consider – 

GERD therapy

Treatment recommended for SOME patients in selected patient group

All patients require assessment and treatment of GERD if necessary. 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 edema.[41]

Reflux also often improves significantly with supraglottoplasty due to a decrease in negative intrathoracic and intraesophageal pressures.[17] Untreated reflux may delay healing after surgery.[16]

Simple treatment options include nursing upright, using bottles to minimize 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 GERD can be treated surgically with Nissen fundoplication.

Primary options

famotidine: neonates and infants <3 months: 0.5 to 1 mg/kg orally once daily; children ≥3 months of age: 0.5 to 1 mg/kg orally twice daily, maximum 80 mg/day

OR

omeprazole: 0.5 to 1 mg/kg orally once daily, maximum 20 mg/day

severe disease

1st line – surgical therapy

Back
ONGOING
|
severe disease
1st line – 

surgical therapy

Severe disease occurs in 10% to 15% of patients.[35][36][37]

Endoscopic supraglottoplasty (aryepiglottoplasty) to modify the supraglottis to relieve obstruction is the preferred treatment. This may include: division or excision of the aryepiglottic folds, epiglottopexy, or partial amputation of the epiglottis.[1][3][41] Unilateral supraglottoplasty may minimize the risk of subsequent supraglottic stenosis or aspiration.[42][43]

Comorbidities of neurologic disorders, syndromes, and congenital heart disease need to be recognized 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 postoperative diagnosis of a coexisting neurologic disorder has been shown to be significantly associated with surgical failure.[45][46] The potential benefits of favorable outcomes versus the risks of complications requires careful consideration in these complex cases.

Tracheostomy may be preferred to supraglottoplasty in children with 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%.[47]

Consider – GERD therapy

Back
ONGOING
|
severe disease
Consider – 

GERD therapy

Treatment recommended for SOME patients in selected patient group

All patients require assessment and treatment of GERD if necessary. 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 edema.[41]

Reflux also often improves significantly with supraglottoplasty due to a decrease in negative intrathoracic and intraesophageal pressures.[17] Untreated reflux may delay healing after surgery.[16]

Simple treatment options include nursing upright, using bottles to minimize 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 GERD can be treated surgically with Nissen fundoplication.

Primary options

famotidine: neonates and infants <3 months: 0.5 to 1 mg/kg orally once daily; children ≥3 months of age: 0.5 to 1 mg/kg orally twice daily, maximum 80 mg/day

OR

omeprazole: 0.5 to 1 mg/kg orally once daily, maximum 20 mg/day

2nd line – positive airway pressure

Back
ONGOING
|
severe disease
2nd line – 

positive airway pressure

Positive airway pressure may be used in patients with obstructive sleep apnea (OSA) where surgery has failed to improve airway obstruction or in those who are not surgical candidates.

Positive airway pressure may also be useful as an interim measure: for example, to enable further surgical procedures to be delayed in the early postoperative period.

Consider – GERD therapy

Back
ONGOING
|
severe disease
Consider – 

GERD therapy

Treatment recommended for SOME patients in selected patient group

All patients require assessment and treatment of GERD if necessary. 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 edema.[41]

Reflux also often improves significantly with supraglottoplasty due to a decrease in negative intrathoracic and intraesophageal pressures.[17] Untreated reflux may delay healing after surgery.[16]

Simple treatment options include nursing upright, using bottles to minimize 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 GERD can be treated surgically with Nissen fundoplication.

Primary options

famotidine: neonates and infants <3 months: 0.5 to 1 mg/kg orally once daily; children ≥3 months of age: 0.5 to 1 mg/kg orally twice daily, maximum 80 mg/day

OR

omeprazole: 0.5 to 1 mg/kg orally once daily, maximum 20 mg/day

back arrow

Choose a patient group to see our recommendations

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer

Use of this content is subject to our disclaimer