International Journal of Pediatric Otorhinolaryngology Extra
Volume 5, Issue 1 , Pages 28-31, January 2010

Stridor in a newborn caused by a congenital laryngocele and bifid epiglottis: A case report and review of the literature

Duzce University, Duzce Medical Faculty, Department of ENT and Head & Neck Surgery, Turkey

Received 29 December 2008; received in revised form 14 January 2009; accepted 14 January 2009. published online 13 February 2009.

Article Outline

Summary 

A laryngocele is a rare, an abnormal cystic dilatation of the laryngeal saccule. A bifid epiglottis is also an extremely rare congenital anomaly of larynx that is often associated with other congenital defects. In this article, we report a case of an internal laryngocele and bifid epiglottis in a one hour old newborn girl that presented as acute airway obstruction who also have imperforate hymen, hydrocolpos, bilateral hydronephrosis and polydactyly. The patient underwent endoscopic microsurgical excision of internal laryngocele using suspension laryngoscopy and cold blade. We also review the anatomy, etiology and clinic course of the different types of laryngoceles, bifid epiglottis and related congenital defects.

Keywords: Congenital laryngocele, Bifid epiglottis, Airway obstruction, Endoscopic resection

 

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Introduction 

A laryngocele is a rare, an abnormal cystic dilatation of the laryngeal saccule. The etiology of laryngoceles is not well understood, but congenital and acquired factors are considered to play a role in their development [1], [2], [3]. A bifid epiglottis is also an extremely rare congenital anomaly of larynx that is often associated with other congenital defects [4]. The most commonly reported associated defects are abnormalities of the polydactyl which occur in as many as 75% of patients, and oral anomalies including cleft palate, cleft uvula, micrognathia and microglossia which occur in as many as 84% of patients [4], [5]. Internal laryngoceles is confined to the false vocal cord and it does not pass through the thyrohyoid membrane. Many laryngoceles are usually asymptomatic [6]. The most common symptoms for internal laryngoceles are hoarseness and dysphagia. Symptomatic internal laryngoceles may also cause functionally airway obstruction as a result of an increase of the intralaryngeal pressure [7], [8], [9], [10].

In this article, we report a case of an internal laryngocele and bifid epiglottis in a one hour old newborn girl that presented as acute airway obstruction who also have imperforate hymen, hydrocolpos, bilateral hydronephrosis and polydactyly. We also review the anatomy, etiology and clinic course of the different types of laryngoceles.

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Case report 

Our patient was a one hour female newborn who has a respiratuary distress, hoarsenesss and no crying in the newborn intensive care. She was being followed up by the perinatologists because of bilateral hydronephrosis and polydactyly on the four extremities seemed on intrauterine USG. According to maternal medical history she has a 8-year-old healthy boy and two boys with polydactylies died on the first week after delivery, respectively six and 10 years ago. During the direct laryngoscopic examination, we saw a mass with cystic appearance under the uvula arised from the left ventriculer fold and the left aryepiglottic fold. Epiglottis was bifid. Neck inspection and palpation revealed nothing pathologic. We aspired 2ml serous fluid from cystic mass thus temporary airway relief was obtained. Culture of the serous mucus was negative. We continued to examine by direct laryngoscopy after aspiration and saw left laryngeal saccule bulging and dilatation by breathing and crying. We avoided tracheostomy and followed up carefully.

We repeated the physical examination on the fifth day in the ENT department. Newborn had no stridor, dysphagia and had no mass on the neck. But the indirect fiberoptic laryngoscopic examination revealed a mass bulging from the left ventriculer fold and the left aryepiglottic fold and dilatation was seen by breathing and crying and the shape of the epiglottis was bifid (Fig. 1). No external laryngeal component was found. The vocal cords and arytenoids showed nothing pathologic and mobility was normal.

The CT examination of the neck confirmed the diagnosis of the internal laryngocele by the presence of an air-fluid filled saccular cavity on the left ventriculer fold (Fig. 2). On the sixth day the patient underwent an operation by the pediatric surgery because of imperforate hymen, hydrocolpos and bilateral hydronephrosis. There were no postoperative problem corresponding these pathologies.

On the 25th day stridor were developed again and the patient underwent endoscopic microsurgical excision of internal laryngocele using suspension laryngoscopy and cold blade. The intralaryngeal portion of the laryngocele was removed. The histopathologic report confirmed the diagnosis of laryngocele with pseudostratified epitelium and underlying seromucous glands in fibrous stroma. There was no evidence of neoplasia or any coinciding laryngeal disease. Polydactyly of the extremities are seen on the plain radiography (Fig. 3). Cranial MRI of the patient did not show any abnormality like hypothalamic hamartoma usually associated with bifid epiglottis.

Follow up examination with videolaryngoscopy revealed no recurrence and airway problems 6 months after surgery (Fig. 4). The patient's multiple congenital abnormalities (laryngocele, bifid epiglottis, polydactyly and imperforate hymen) corresponded with no known syndrome.

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Discussion 

The laryngocele is a sac like structure with an internal lumen that is dilated and filled with air. The incidence is estimated to be 1 per 2.5 million population per year [1]. They are classified as internal if they remain within the laryngeal cartilage or external if they extend through the thyrohyoid membrane [11]. Combined laryngoceles may also occur. Fortunately these are rare in infants. Laryngoceles occur predominantly in males. Saccular cysts result from the obstruction of the laryngeal saccule orifice, or the collecting ducts of the submucosal glands located around the ventricle produce the saccular cysts. Laryngoceles produce intermittent upper airway obstruction and hoarseness because of episodic filling with air, whereas saccular cysts produce constant symptoms because of mucoid fluid within the cyst. Dilatation of the laryngocele could be seen by breathing and crying but saccular cysts do not. Diagnosis is made at the time of flexible or direct laryngoscopy.

A bifid epiglottis is also an extremely rare congenital anomaly of larynx. It has been reported in 40% of patients with Pallister–Hall Syndrome and rarely in other syndromes [2], [4], [12]. The primary feature of Pallister–Hall Syndrome is the hypothalamic hamartoma. In our case the patient was free of hypothalamic hamartoma. The most commonly reported associated defects with bifid epiglottis are abnormalities of the polydactyly which occur in as many as 75% of patients, and oral anomalies which occur in as many as 84% of patients [4], [5]. Twenty-two patients reported in the literature with bifid epiglottis and no patient had bifid epiglottis as an isolated anomaly. Other malformations include clefts, micropenis, renal anomalies, anal malformations, hypospadias, hypothalamic hamartomas, hypopituitarism, heart defects and Hirschprung disease [12]. In our case the patient has laryngocele, bifid epiglottis, polydactyly and imperforate hymen.

The epiglottis is thought to arise from the hypobranchial eminence and ventral ends of the third and fourth branchial arches beginning at five weeks of gestation. Multiple theories have been proposed to explain the embryologic origin of the epiglottis [2], [13]. Controversy exists with regard to whether the hypobranchial eminence is of midline or bilateral origin. The pattern of associated anomalies supports the hypothesis that bifid epiglottis is the result of a teratogenic event rather than a genetic mutation since the embryologic development of the epiglottis occurs at the same time as that of the hand, hypothalamus, larynx, and oral cavity [2]. As a result, the true etiology behind these anomalies is still unknown.

It is believed that formation of laryngoceles depend on two factors; the presence of a congenital long saccule and the prolonged periods of increased intralaryngeal pressure. Half of laryngoceles are mixed, 30% are external and 20% are internal. There is an equal frequency between the right and left sides and most of the laryngoceles are unilateral, as only 15% of the cases present bilaterally [14]. Laryngoceles are developed predominantly in males in the fifth to seventh decades of life [1], [2]. If the laryngocele becomes infected, a potentially fatal condition may quickly arise so the recommended treatment of a laryngopyocele is immediate endoscopic drainage [15]. The additional surgery can be performed either immediately after endoscopic drainage or at a later date but still there is currently no consensus on the timing of surgery. The timing of excision should be decided according to patients’ symptoms. If the patients have respiratuary distress, dyspnea the surgery should be done as soon as possible.

Various surgical techniques have been described including external approach by way of thyrotomy, endoscopic excision with cold blade or CO2 laser and endoscopic marsupialization for internal laryngoceles. Thome et al. [16] reported seven different surgical techniques of the external approach like thyrotomy or thyrohyoid membrane dissection. Myssiorek and Perksy [17] have described successful use of laser endoscopic excision of internal laryngoceles. They also found that marsupialization has a higher risk of recurrence. Güven et al. [18] reported a case of laryngocele that is treated with endoscopic excision via cold blade. In our case the patient underwent endoscopic microsurgical excision of internal laryngocele using suspension laryngoscopy and cold blade. The intralaryngeal portion of the laryngocele was removed.

A laryngocele in a one hour old female newborn patient with a bifid epiglottis, polydactyly and imperforate hymen is extremely rare and can be excised with endoscopic technique using cold blade without complication.

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References 

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PII: S1871-4048(09)00007-0

doi:10.1016/j.pedex.2009.01.003

International Journal of Pediatric Otorhinolaryngology Extra
Volume 5, Issue 1 , Pages 28-31, January 2010

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