Epiglottopexy in Infants Younger Than 6 Months Old: A Case Series

Introduction

Laryngomalacia (LM) is the most common congenital disease of the larynx.^1,2 Children typically present with inspiratory stridor secondary to the dynamic collapse of the supraglottic structures during inspiration. Flexible fiberoptic laryngoscopy is the gold standard for diagnosis of LM; Findings may include omega-shaped epiglottis, retroflexed epiglottis, shortened aryepiglottic folds, and supra-arytenoids mucosal redundancy.^1,2 Swallowing difficulties are common and can present among children with LM due to poor coordination of the suck-swallow-breathing cycle, decreased laryngeal sensation, or laryngeal sensory-motor dysfunction.^{3 -7} In 10%-20% of patients, LM may present with severe, life-threatening symptoms that would warrant surgical intervention.

Supraglottoplasty (SGP) has been established as the treatment of choice for severe LM although the procedure may not necessarily address the posterior displacement of the epiglottis during inspiration.^{7 -9} Epiglottopexy, securing the epiglottis to the base of the tongue, is a well-described intervention to address epiglottic prolapse. ¹⁰ The technique has regained popularity in recent years due to the widespread use of drug-induced sleep endoscopy (DISE)^{11 -13} and the ability to identify epiglottic prolapse as a significant factor in airway obstruction. Post-surgical alterations in the position and closure of the larynx may impact the swallow mechanism.^13,14 Few studies have investigated the effect of epiglottopexy on swallowing in otherwise healthy children^11,13 and found the surgical technique to be safe and the free epiglottic rim to be sufficient to seal the glottic inlet and prevent aspiration during swallowing.

Infants younger than 6 months old and neonates are particularly prone to laryngeal penetration and aspiration due to the longer duration of the oropharyngeal phase of swallowing. Surgical intervention with SGP and epiglottopexy in neonates and younger infants may disrupt the balance between pharyngeal swallowing and airway protection and result in dysphagia; yet to date, no studies are dedicated to SGP and epiglottopexy outcomes in this age-group population.

The goal of our study was to review swallowing outcomes and the efficacy of SGP and epiglottopexy in our cohort of infants younger than 6 months old with severe LM and posterior displacement of the epiglottis. The knowledge gained from this analysis would provide a better understanding of the short-and long-term postoperative outcomes, as well as guide discussions with families and set expectations of potential complications.

Material and Methods

Surgical Technique

The patient was placed supine and underwent direct laryngoscopy and bronchoscopy. The infant Benjamin-Lindholm laryngoscope device was applied, and SGP was performed with anesthetic insufflation utilizing the traditional cold-steel technique. The patient was then intubated trans-nasally. The mucosa of the vallecular sulcus was denuded using a Coblator wand or a CO₂ laser. A similar denuding of the mucosa was performed on the epiglottic surface. A single PDS suture was then placed through the mid-third of the epiglottic perichondrium and the base of the tongue in a horizontal mattress fashion and secured to lift the epiglottis into the tongue base (Figure 1). Care was taken to avoid demucosalization of the epiglottis tip to prevent scarring of the epiglottis rim to the base of the tongue, allowing some motion of the epiglottis to remain during swallowing and providing airway protection. In infants with challenging laryngeal exposure, a suture was not placed. The patients were kept intubated for at least 24 hours with the tube placed in the midline of the epiglottis to allow for healing.

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Figure 1.

Epiglottopexy—surgical technique. (A) Denudation of the mucosa of the vallecula using a Coblator wand, (B) denuding of the mucosa on the epiglottic surface, and (C) a single PDS suture through the mid-third of the epiglottic perichondrium and the base of the tongue.

Results

Thirteen patients (4 females and 9 males) age 1.3 week-5.2 months met inclusion criteria. Table 1 summarizes the demographic data of the patients. The mean age at surgery was 10.5 weeks. The mean gestational age was 36.8 ± 1.9 weeks. Eight (62%) children were diagnosed with a neurodevelopmental disease or a syndrome, 3 (23%) children were found to have a vallecular cyst, and 5 (39%) children had congenital heart disease. Table 2 summarizes patients’ comorbidities and pre-and post-operative data for each patient. All patients presented with stridor and dysphagia. One patient was intubated shortly after birth due to a supraglottic mass, and 3 patients required positive pressure respiratory support. Four patients underwent SGP prior to this intervention.

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Table 1.

Patient Demographics.

Patient characteristics
Age at surgery (mean, SD), wk	10.5 ± 7.5
Gestational age (mean, SD), wk	36.8 ± 1.9
Sex, N (%)
Female	4 (31)
Male	9 (69)
Race, N (%)
White	7 (54)
Black	5 (39)
Other	2 (16)
Comorbidities, N (%)
Syndromes/chromosomal/Neurodevelopmental disease	8 (62)
Congenital heart disease	5 (39)
Vallecular cyst	3 (23)

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Table 2.

Patient Clinical Data Prior to and Following Surgical Intervention.

Patient number	Gender	Age (wk) at surgery	Gestational age (weeks)	Comorbidities	Stridor pre-op	Dysphagia pre-op	Previous surgical interventions	Operative findings	Procedure performed	Postoperative day for extubation	Postoperative complications	Follow-up (mo)	Need for further interventions
1	M	22.29	37	22q deletion syndrome, congenital cardiac disease, vocal cord paresis	Yes	Yes	NA	Shortened aryepiglottic folds, arytenoid redundancy, retroflexed epiglottis	SGP epiglottopexy with suture	3	NA (slow ventilation weaning)	38.10	Tracheostomy and vent dependent due to cardiopulmonary disease
2	M	21.43	35	Spina bifida and tethered cord, hypotonia, GERD	Yes	Yes	SGP	Shortened aryepiglottic folds, arytenoid redundancy, retroflexed epiglottis	Revision SGP epiglottopexy with suture	1	NA	35.13	Adenotonsillectomy
3	M	9.57	39	Retrognathia	Yes	Yes	NA	Shortened aryepiglottic folds, arytenoid redundancy, retroflexed epiglottis, Glossoptosis	SGP epiglottopexy with suture	3	NA (slow ventilation weaning)	7.43	Tongue base suspension
4	F	11.86	37	Genetic anomaly, hypotonia craniosynostosis, neurodevelopmental delay	Yes	Yes	NA	Shortened aryepiglottic folds, arytenoid redundancy, retroflexed epiglottis	SGP epiglottopexy with suture	1	NA	15.97	None
5	M	2.29	36	Vallecular cyst	Yes	Yes	NA	Shortened aryepiglottic folds, arytenoid redundancy, retroflexed epiglottis, vallecular cyst	SGP epiglottopexy without suture	1	NA	2.53	None
6	M	19.71	35	Congenital heart disease	Yes	Yes	SGP	Shortened aryepiglottic folds, arytenoid redundancy, retroflexed epiglottis, trapped first ring	Revision SGP epiglottopexy with suture	3	NA (kept intubated for planned imaging)	14.93	None
7	F	9.14	37	Trisomy 21, hypotonia hypothyroidism	Yes	Yes	NA	Shortened aryepiglottic folds, arytenoid redundancy, retroflexed epiglottis, grade 1 sub glottis stenosis	SGP epiglottopexy without suture	3	NA (slow ventilation weaning)	8.50	None
8	F	9.71	33	DiGeorge syndrome, neurodevelopmental delay, congenital heart disease	Yes	Yes	SGP	Shortened aryepiglottic folds, arytenoid redundancy, retroflexed epiglottis	Revision SGP epiglottopexy without suture	4	Sepsis secondary to gastrostomy tube	12.17	None
9	M	1.29	39	Coffin-siris syndrome, cleft palate, congenital heart disease	yes	yes	NA	Shortened aryepiglottic folds, arytenoid redundancy, retroflexed epiglottis	SGP epiglottopexy with suture	1	NA	0.73	Tracheostomy
10	M	17.29	38	Cleft palate, retrognathia, macrocephaly, cerebral palsy	Yes	Yes	NA	Shortened aryepiglottic folds, arytenoid redundancy, retroflexed epiglottis	SGP epiglottopexy with suture	1	NA	7.90	None
11	F	11.00	39	Vallecular cyst	Yes	Yes	NA	Shortened aryepiglottic folds, arytenoid redundancy, retroflexed epiglottis, vallecular cyst	SGP epiglottopexy with suture	1	NA	2.10	None
12	M	1.86	36	Vallecular cyst, IUGR	Yes	Yes	NA	Shortened aryepiglottic folds, arytenoid redundancy, retroflexed epiglottis, vallecular cyst	SGP epiglottopexy with suture	1	NA	1.01	None
13	M	15.57	37	Genetic anomaly, hypotonia, neurodevelopmental delay, severe GERD, Poor GI motility	Yes	Yes	SGP	Shortened aryepiglottic folds, arytenoid redundancy, retroflexed epiglottis	Revision SGP epiglottopexy with suture	5	Atelectasis of lung, slow wean from ventilator	20.0	Revision SGP and epiglottopexy

Abbreviations: GERD, gastroesophageal reflux disease; GI, gastrointestinal; IUGR, intrauterine growth restriction; Pre-op, preoperative; SGP, supraglottoplasty.

Pre-operative swallowing evaluation was performed on 9 patients. Four patients were fed via nasogastric (NG) tube due to respiratory distress (1 patient was intubated since birth, and 3 patients were placed on high flow nasal cannula (HFNC)). Seven patients were evaluated with a video-fluoroscopic swallowing study (VFSS), while 6 patients underwent fiberoptic evaluation of swallowing (FEES). There was aspiration or concern for aspiration in 6 (46%) children and penetration in an additional 3. Only 1 child was primarily orally fed prior to surgery. Overall, 10 children had an NG tube for feeding, and 2 patients had a gastrostomy-tube placed prior to epiglottopexy. All patients underwent SGP (or revision SGP) and epiglottopexy.

Twelve patients underwent both aryepiglottic fold division, and supra-arytenoid mucosal resection; 1 patient had only aryepiglottic folds division. Additionally, 3 patients had an excision of the vallecular cyst. Three patients had epiglottopexy performed without suturing the epiglottis to the base of the tongue due to suboptimal laryngeal exposure (mean age at surgery 7.0 weeks).

All the patients were kept intubated at the end of the procedure to re-enforce adhesion of the epiglottis to the base of the tongue and promote scarring. The patients were extubated in the pediatric ICU on day 1.9 ± 1 on average. None had a failed extubation. There were no upper airway post-operative complications during admission. All patients were extubated on postoperative day 1 to 3, with significant improvement in upper airway obstruction. The patients who required positive pressure support (n = 3) were able to wean to room air. Two patients with genetic anomaly and congenital cardiac disease subsequently required tracheostomy tube placement (time interval between epiglottopexy to tracheostomy was 1 and 2 months).

Instrumental swallowing evaluation with VFSS was performed in all patients following surgery. Aspiration was found in 10 patients. New-onset aspiration was found in 4 children. Penetration with a higher level of consistency was noted in 4 patients. Table 3 summarizes swallowing evaluation findings prior to and following epiglottopexy for each patient. On the most recent follow-up (mean 12.1 months, range 1-38.1 months), 1 child was taking a full oral diet, 1 patient was primarily NG-tube fed, and 10 patients required continued gastrostomy-tube feeds. One of the gastrostomy-tube-dependent patients was lost to follow-up, and 1 patient was cleared for oral intake yet has not transitioned to a regular diet due to severe oral aversion.

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Table 3.

Swallowing Evaluation with FEES and VFSS Prior to and Following Surgical Intervention.

	Preoperative evaluation					Postoperative evaluation					Feeding route at the most recent follow-up
Patient number	Oral dysphagia	Early spillage	Residue	Laryngeal penetration	Laryngeal aspiration	Oral dysphagia	Early spillage	Residue	Laryngeal penetration	Laryngeal aspiration
1	+	+	+	Syrup, nectar, & honey	Nectar & syrup	+	−	−	None seen with solids and puree	None seen with solids and puree	G-tube, cleared for oral feed but has severe oral aversion
2	+	+	+	Thins	None	+	−	−	Thins	None	G-tube, lost to follow up
3	+	+	NA	Thins	None	+	+	+	Thins & nectar	Thins & nectar	G-tube
4	+	+	+	Thins	Thins	+	+	+	Thins, nectar, & syrup	Thins, nectar, & syrup	G-tube
5	Bedside evaluation by SLP—No cough/congestion with feeds, noisy breathing at baseline, unclear if worsened with intake.					+	+	+	Thins & nectar	Thins & nectar	G-tube
6	+	+	+	Thins & nectar	-	+	+	+	Thins, nectar, & syrup	Thins, nectar, syrup, & honey	G-tube
7	Patient required positive pressure respiratory support since birth					+	+	−	None	None	G-tube
8	Patient required positive pressure respiratory support since birth					+	+		Thins	None	G-tube
9	Intubated at birth due to supraglottic mass and respiratory distress					+	+	+	Thins & nectar, syrup, & honey	Thins & nectar syrup & honey	G-tube
10	Bedside evaluation by SLP: facial reddening, direct cough, concern for aspiration					+	+	+	Thin, nectar, syrup & honey	Thin, nectar, syrup & honey	G-tube, cleared for solids and puree
11	+	NA	NA	Thins	−	+	+	+	Thins	Thins	Full oral diet
12	The patient required positive pressure respiratory support. Oral feeds associated with worsening stridor					+	+	+	Thins & nectar	Thins	NG
13	+	+	+	+	+/−	+	+	+	Secretions	Secretions	G-tube

Abbreviations: G-tube, gastrostomy tube; NG, nasogastric tube; SLP, speech-language pathologist.

Five patients underwent further airway evaluation and interventions due to persistent respiratory symptoms, upper airway obstruction, or persistent noisy breathing. One child required revision SGP and epiglottopexy within 5 weeks from her primary surgery due to a scar formation that pulled the arytenoids over the glottis and caused some separation between the epiglottis and the base of tongue. One child with retrognathia had tongue base suspension, and 2 required a tracheostomy secondary to cardiorespiratory failure. Adenotonsillectomy and pillar plication was performed on 1 child several years later.

Discussion

The goal of this study was to evaluate the outcomes of SGP and epiglottopexy in our cohort of infants younger than 6 months old. Our data demonstrate that medical comorbidities are common among infants with epiglottic prolapse and severe LM. Surgical intervention with SGP and epiglottopexy resulted in improvement in obstructive airway symptoms in all patients. Two patients who subsequently required tracheostomy tube placement were diagnosed with medical syndrome and congenital heart disease. There were no postoperative complications related to upper airway edema or obstruction following extubation.

Feeding difficulties and dysphagia were noted in all of the patients prior to intervention, and 12 out of 13 patients required enteral feeds via NG tube or gastrostomy-tube. Following surgery, 10 patients were diagnosed with aspiration, 4 of them had new onset of aspiration. On follow-up (mean 10.7 months), 10 patients were primarily fed via gastrostomy-tube, one of these patients was lost to follow-up, and 1 child was cleared for unrestricted oral diet but demonstrated severe oral aversion.

Our data regarding the efficacy of SGP and epiglottopexy in relieving upper airway obstruction correlates with previous studies.^2,8,9,11 Durvasula et al ⁹ investigated the success rate of SGP among infants and children with neurologic and syndromic comorbidities and found that these children require revision SGP, tracheostomy tube, and gastrostomy-tube placement more commonly than healthy children with LM. Similarly, Thompson ² and Hoff et al ⁸ described a direct correlation between medical comorbidities and the need for revision SGP and tracheostomy tube placement.

Whymark et al ¹⁵ investigated the effect of epiglottopexy without suturing as a treatment method for LM (regardless of epiglottis position) in 76 children age 5 days-32 months, and found significant improvement in their respiratory symptoms. Those that required additional interventions or repeat surgery were all diagnosed with neurological/syndromic comorbidities or other airway anomalies.

Dysphagia is common among children with LM, although the effect of disease severity and comorbidities is controversial.^4,5 SGP in children with neurological or syndromic disease is associated with long-term dysphagia^9,16; however, the effect of epiglottopexy on swallowing function has only scarcely been described. Kanotra et al ¹³ presented a study involving 5 healthy children age 6 to 14 years old who underwent epiglottopexy with lingual tonsillectomy for residual obstructive sleep apnea and were evaluated with a FEES 1 week following surgery. The authors found epiglottopexy to be safe and not associated with dysphagia in this cohort of patients. Our findings are different from the swallowing evaluations which have been previously described.^12,13,15 Several plausible explanations for these differences include a younger patient cohort, a higher proportion of patients with significant comorbidities, and instrumental postoperative swallowing evaluation, which has not been performed in other studies.

This is one of the first studies looking at the efficacy and safety of SGP and epiglottopexy in infants younger than 6 months old. However, there are a few limitations to our study that should be considered when interpreting these results; such as small cohort of patients and retrospective design. Other limitations include the heterogeneity of our cohort, the presence of significant comorbidities and dysphagia prior to surgery in almost all of the patients, and variable duration of follow-up, all of which may affect the generalizability of these results.

Conclusions

The addition of epiglottopexy to SGP in infants younger than 6 months old with LM and epiglottic prolapse may further improve upper airway obstruction in properly selected patients. Long and short-term dysphagia are common and should be explicitly evaluated in all patients prior to and following surgery.