Portuguese Version

Year:  2003  Vol. 69   Ed. 1 - (3º)

Artigo Original

Pages: 16 to 18

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Laryngomalacia: experience with surgical management in São José do Rio Preto University School of Medicine.

Author(s): Raquel C. de Oliveira,
Fernando D. Molina,
Claudiane M. Assis,
Alexandre R. Coraçari,
José V. Maniglia

Keywords: laryngomalacia, congenital anomaly, stridor, gastroesophageal reflux, and supraglottoplasty.

Abstract:
Laryngomalacia is the most common cause of stridor and the most common congenital laryngeal anomaly in infants. Usually, it occurs in association with other abnormalities or diseases, such as gastroesophageal reflux disease. The etiology of laryngomalacia remains unknown; it's a self-limited condition that resolves without therapy on 85% of affected infants. However, surgical management is necessary in 10-15% of patients who present with severe upper airway obstruction. This study intends to review the experience with supraglottoplasty in 15 patients with severe laryngomalacia in the Department of Otolaryngology - Head and Neck Surgery, Section of Pediatric Otolaryngology, São José do Rio Preto Medical School, submitted to unilateral section of aryepiglotic fold and the diagnosis of associated synchronic anomalies.

INTRODUCTION

Laryngomalacia is the most common laryngeal malformation and the main cause of respiratory stridor in childhood1, more frequently affecting male subjects. It consists of a collapse of supraglottic structures2, 3, epiglottis, aryepiglottic folds, and aryepiglottic mucosa during inspiration, being characterized by inspiration stridor that can be present right after birth, but that normally is noticed by the parents after some days or weeks of life. Stridor is exacerbated by agitation, food, crying or the supine position with head and neck flexion, attenuated by placing the child in prone position or by extending the head and neck4. The etiology is unknown, but the theories point to laxity or incoordination of supraglottic structures that narrow during inspiration, causing stridor and respiratory obstruction. This laryngeal hypotony is probably the physiological dysfunction that resolves with growth, generally about the 2nd year of life. Some authors believe that laryngomalacia is a manifestation of the delay in neuromuscular development5, 6. The diagnosis can be confirmed by endoscopy examination, detecting epiglottis in omega or tubular, short aryepiglottic folds and redundant supraglottic mucosa, which during inspiration gets stuck in the laryngeal site and is expelled by exhalation.

The disease is normally self-limited, with increase in intensity of stridor in the first 6 to 9 months, followed by gradual resolution at about 18 to 24 months of life3, 4, 7. These children develop normally and are followed up only clinically. However, 10 to 15% of the patients present severe stridor, intense respiratory distress, sleep obstructive apnea, cyanosis episodes, and incapacity to maintain the appropriate oxygen blood saturation, leading to chronic hypoxia and cor pulmonale 1, 4, 5 and delay in weight and height gain and repetitive pneumonia. This small percentage of patients requires surgical intervention4.

The surgical procedure more commonly performed is supraglottoplasty, which consists of the excision of flaccid supraglottic tissues and/or section of the short aryepiglottic folds responsible for respiratory obstruction, which can be made either uni or bilaterally4. Tracheotomy is reserved for cases in which there is failure in the previous treatment1 and in those in which there are synchronous respiratory diseases.

Laryngomalacia is normally associated with other diseases and/or malformations, among which the gastroesophageal reflux disease that is present in approximately 100% of the cases and its treatment is fundamentally important to control the respiratory condition of the children2, 4, 7, 8.
This study intended to assess the results of the Service of Pediatric Otorhinolaryngology of the Medical School of São Jose do Rio Preto in managing severe laryngomalacia by means of unilateral section of aryepiglottic fold, as well as to assess the presence of associated pathologies.

MATERIAL AND METHOD

The present study analyzed medical charts of patients treated at the Service of Otorhinolaryngology, Medical School of São Jose do Rio Preto, between January 1996 and January 2002, in which 15 patients had clinical and nasofibroscopic diagnosis of severe laryngomalacia and were submitted to surgical treatment.

Surgical indications were basically clinical evidence of severe respiratory obstruction, sleep obstructive apnea, repetitive pneumonia, cyanosis episodes, with decreased levels of oxygen blood saturation below 80%, and failure in development.

The initially performed surgery was the section of the unilateral aryepiglottic fold, as follows: patient under general anesthesia and orotracheal intubation, exposure of the larynx using suspension laryngoscope, we sectioned the unilateral aryepiglottic fold using a pair of micro-scissors or high frequency knife and if there was redundant tissue in the laryngeal lumen or the post-cricoid region, we cauterized it. Next, we performed tracheostomy and bronchoscopy to identify synchronous pathologies of the respiratory tree. The patients that did not evolve satisfactorily were submitted to section of the contralateral aryepiglottic fold, using the same surgical technique and those who maintained significant respiratory obstruction were submitted to tracheotomy.

RESULTS

Out of 15 patients that participated in the study, nine were male and six were female subjects, whose mean age at diagnosis was 10.8 months, with interval for symptom onset ranging from birth to 3 months of age.

The surgical treatment initially made was the section of unilateral aryepiglottic fold, which was conducted in all cases. Among the 15 studied patients, three did not progress satisfactorily, and maintained the respiratory difficulty, submitted then to section of the contralateral aryepiglottic fold. Of these 3 patients, one maintained the respiratory obstruction and was submitted to tracheotomy.

Evidence of gastroesophageal reflux, frequent regurgitation, edema, hyperemia of posterior third of the larynx and post-cricoid area were present in 100% of the sample, congenital cardiopathy in 13.3% and unilateral choanal atresia in 6.66%.

DISCUSSION

In addition to being the most common congenital pathology of the larynx, laryngomalacia is self-limited in most cases, with spontaneous resolution in about 85% of the cases. The improvement in the general picture is gradual, passing by stridor noticed only in periods of agitation. The association with other pathologies in non-severe cases is quite common but the spontaneous resolution period is not changed6.

The severe cases, about 10 to 15% of the patients, should be surgically treated and uni or bilateral supraglottoplasty is the most used treatment. The patients present an important respiratory obstruction, with apnea crisis, cyanosis, delay in growth, repetitive pneumonia, and can evolve to pulmonary hypertension and cor pulmonale. Some authors, such as Bent et al., believed that in addition to the manifestations in childhood, as a result of short aryepiglottic folds and the tubular and elongated shape of the epiglottis, in adult life these subjects may present more marked respiratory difficulties than in the normal population in case of physical stress, being diagnosed as asthma triggered by physical stress or constitutional origin9.

In our study, 80% of the patients progressed to good respiratory pattern after unilateral section of aryepiglottic fold and in the cases in which it was necessary to section both aryepiglottic folds, the rate was 93.5%, with no surgical complications. These results are similar to those reported by Kelly and Gray, 1995, who reported 94% of success in the treatment of patients; Reddy and Bruce, 2001, in a sample of 47 children reported 85% of surgical success with unilateral aryepiglottic fold section, and 95% with contralateral aryepiglottic fold section, but there were complications only in the patients submitted to bilateral fold section, with 2 cases of supraglottic stenosis. Thus, the unilateral procedure is advantageous because it is quick and presents low incidence of complications, even if it has to be repeated.

A curious fact observed in our sample was of a 2-year-old male patient who presented good respiratory pattern, according to the mother, and developed an episode of profound neck abscess, Ludwig angina-like. He required emergency care and various attempts of orotracheal intubation failed, leading to tracheotomy. About 20 days after the resolution of the infectious process and removal of the cannula, the child came back to the hospital complaining of respiratory difficulty, significant stridor and laryngeal abnormalities compatible with laryngomalacia. He was submitted to section of unilateral aryepiglottic fold with resolution of the respiratory condition.

Another interesting case in our sample was a 4-year-old child with history of respiratory obstruction and repetitive pneumonia, submitted to adenotonsillectomy in another center, with no clinical improvement. Laryngomalacia was diagnosed after our evaluation; the child underwent unilateral aryepiglottic fold section and surgical treatment for associated gastroesophageal reflux, progressing with good respiratory pattern.

As to associated pathologies, the evidence of gastroesophageal reflux in this study was similar to those reported by other authors; Belmont and Grundfast, 1984 found signals of the pathology in 80% of the sample. Congenital cardiopathy was present in 13.3% and choanal atresia in 6.66% of our cases, showing the importance of performing a very careful examination of these patients. No other airway synchronous lesions were detected in our sample, even though the literature has reported up to 19% of them in children with laryngomalacia. The main lesions are glottic cystic, tracheomalacia, subglottic stenosis and unilateral vocal folds paralysis6. Thus, few patients that present respiratory obstruction caused by impairment of various anatomical sites can benefit from conservative procedures such as supraglottoplasty, requiring tracheotomy.9

CONCLUSION

Unilateral supraglottoplasty proved to be an effective and safe procedure in treating severe laryngomalacia.

REFERENCES

1. Fauroux B, Pigeot J, Polkey MI, Roger G, Boulé M, Clément A, Lofaso F. Chronic Stridor Caused by Laryngomalacia in Children. Am J Respir Crit Care Med 2001;164: 1874-1878.
2. Bibi H, Khvolis E, Shoseyov D, Ohaly M, Ben Dor D, London D, Ater D. The Prevalence of Gastroesophageal Reflux in Children With Tracheomalacia and Laryngomalacia. CHEST 2001;119:409-413.
3. Shah UK, Wetmore RF. Laryngomalacia: A Proposed Classification Form. International Journal of Pediatric Otorhinolaryngology 1998;46:21-26.
4. Reddy DK, Matt BH. Unilateral vs. Bilateral Supraglottoplasty for Severe Laryngomalacia in Children. Arch Otolaryngol Head Neck Surg 2001;127:694-699.
5. Belmont JR, Grundfast K. Congenital laryngeal stridor (laryngomalacia): etiologic factors and associated disorders. Ann otol Rhinol Laryngol 1984;93:430-437.
6. Olney DR, Greinwald Jr JH, Smith RJH, Bauman NM. Laryngomalacia and Its Treatment. Laryngoscope 1999;109:1770-1775.
7. Zalzal GH, Anon JB, Cotton R. T. Epiglottoplasty for the treatment of laryngomalacia. Ann Oto Rhinol Laryngol 1987;96:72-76.
8. Yellon RF, Goldberg H. Update on Gastroesophageal Reflux disease in Pediatric Airway Disorders. The American Journal of Medicine 2001;111:78-74.
9. Cotton RT, Myer CM et al. Practical Pediatric Otolaryngology. 2nd ed. Philadelphia: Lippincott-Raven Publishers; 1999. 30:497-537.

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1 - Resident Physician, Department of Otorhinolaryngology and Head and Neck Surgery FAMERP.
2 - Physician responsible for the Service of Otorhinolaryngology and Head and Neck Surgery FAMERP.
3 - Resident Physician, Service of Otorhinolaryngology and Head and Neck Surgery, Clinica Maniglia.
4 - Resident Physician, Department of Otorhinolaryngology and Head and Neck Surgery FAMERP.
5 - General Director, FAMERP and Head of the Discipline of Otorhinolaryngology and Head and Neck Surgery FAMERP.

Study conducted at the Medical School of São Jose do Rio Preto.

Address correspondence to: Claudiane Mozer de Assis - Rua Ondina n° 45, Redentora 15015-200 São Jose do Rio Preto, São Paulo. - Tel/fax (55 17)235.3366 - E-mail: clauorl@yahoo.com.br.

Study presented at the 35º Congresso Brasileiro de Otorrinolaringologia, on October 16 - 20, in Natal, RN.

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