International Journal of Pediatric Otorhinolaryngology Extra
Volume 2, Issue 4 , Pages 254-256, December 2007

Airway obstruction in a newborn due to a congenital laryngeal cyst

Department of Otolaryngology, Alder Hey Childrens’ Hospital, Eaton Road, West Derby, Liverpool L12 2AP, UK

Received 18 June 2006; received in revised form 5 August 2007; accepted 6 August 2007. published online 24 September 2007.

Article Outline

Summary 

Congenital laryngeal cysts are a rare cause of airway obstruction that may require urgent diagnosis and treatment. We report on a case of a neonate with a supraglottic laryngeal cyst and the successful outcome of endoscopic treatment.

Keywords: Stridor, Laryngeal cyst

 

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1. Introduction 

While stridor in infants is not a rare entity, congenital laryngeal cysts are a rare but potentially fatal cause of airway obstruction within minutes after birth [1], [2]. Therefore, they require prompt diagnosis and treatment, with involvement of a senior anaesthetist, ENT surgeon and radiologist. The origin of laryngeal cysts remains unknown. Theories of pathogenesis include possible obstruction of submucosal glands or atresia of the laryngeal saccule [3]. During acute airway obstruction due to a laryngeal cyst, intubation can be difficult in view of the mechanical deformity, and would require the presence of a senior anaesthetist. From a surgical point of view, tracheostomy in a neonate can also be fraught with problems. While endoscopic diagnosis and treatment by marupialisation can obviate the need for tracheostomy in the infant, the procedure may have to be repeated, with a risk of subglottic stenosis in some patients. An external approach, through a lateral incision towards the thyroid membrane may be necessary in older children, for recurrent cases, and for larger cysts [4], [5]. In contrast to the rapid development of stridor after birth, the patient may however, present months later with intermittent and chronic symptoms of airway obstruction such as cough, aspiration, failure to thrive, and hoarseness. Unfortunately, delays in diagnosis still lead to a fatal outcome. In one reported series, 50% of cases were diagnosed at autopsy [6], [7].

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2. Case report 

A newborn girl developed marked inspiratory stridor and respiratory distress immediately after birth. During intubation by a senior anaesthetist, a large laryngeal mass was noted in the supraglottic region. The newborn was subsequently referred to our hospital for evaluation and further management. She underwent an MRI scan, which showed a large laryngeal cyst filled with fluid (Fig. 1b). Direct laryngoscopy was performed 48h after birth (Fig. 2). A large supraglottic cyst was noted. The lesion was removed by microlaryngoscopic excision assisted by rigid endoscopes under general anaesthesia (Fig. 3). A postoperative MRI scan (Fig. 4) and a direct laryngoscopy (Fig. 5) showed essentially normal laryngeal structure at 3 months and there has been no further episodes of respiratory distress after 4 years.

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3. Discussion 

While laryngeal cysts are not unknown, they remain a rare entity. The examining surgeon must be aware that these cysts can present in a myriad of ways ranging from sever, life-threatening airway obstruction soon after birth, to chronic and recurrent airway symptoms in infants and small children.

Laryngeal cysts are classified into saccular, ductal and thyroid-cartilage foraminal cysts [2]. Less common forms of laryngeal cysts include hamartomas, choristomas, and teratomas. The saccular cysts (24%) are supraglottic lesions, beneath the normal laryngeal mucosal, can arise from the vallecula, the aryepiglottic fold, the ventricle or the false cord and can be differentiated into two subcategories: anterior and lateral or congenital. Ductal cysts (75%) are lesions coming from the mucosal glands and can form at any site except for the free edge of the true cords where there are no glands. The surgical procedures that are proposed for the treatment of congenital laryngeal cysts vary: e.g. needle aspiration [2], laryngoscopic deroofing and excision [9], laryngofissure [10], lateral pharyngotomy and tracheostomy [11] endoscopic carbon dioxide laser excision [12]. While smaller lesions can be excised, marupialiased during endoscopy, larger or recurrent cysts may require a lateral cervical approach. The choice of surgical technique depends on the particular surgeon's experience an available instruments. As some laryngeal cysts are small, they present later in infancy with subtle or intermittent airway symptoms. These patients require radiological and endoscopic assessment for full appraisal of their airway status.

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4. Conclusion 

Microlaryngoscopic excision assisted by rigid endoscopes following preoperative imaging evaluation is a safe and effective way of managing a congenital laryngeal cyst with minimal morbidity. Recurrent or larger cysts may require an open surgical approach. Smaller cysts present later in infancy and require surgical treatment similar to their larger counterparts.

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References 

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PII: S1871-4048(07)00065-2

doi:10.1016/j.pedex.2007.08.001

International Journal of Pediatric Otorhinolaryngology Extra
Volume 2, Issue 4 , Pages 254-256, December 2007

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