*Winner, Philippine Medical Association National Case Report Contest 2004, PMA Annual Convention, Manila Hotel, Philippines
Introduction
Snoring in the adult has always been thought of as a benign condition, more of a nuisance than a medical problem. But if it is heard from a child, it is thought of as a significant indicator of an underlying pathology that needs to be looked into. Literature commonly associates it with Obstructive Sleep Apnea (Ejercito, 1995; Krespi and Kacker, 2003). Specifically, it has been attributed to anatomic abnormalities such as a large lingual tonsil (Dundar, et al, 1996), laryngomalacia (Chetty, et al, 1994), and epiglottic prolapse (Woo, 1992). Other reported conditions include obesity, nasal obstruction (Chen and Kushida, 2003), unfavorable tongue to mandible relationship, pharyngeal hypotonia and pharyngeal space occupying tumors1. There has been no report attributing it to a laryngeal mass like the case presented.
This is a case report of a 3-year old boy who was brought for consultation because of snoring and related restless sleep. History seemed to point to an upper pharyngeal abnormality resulting to an obstructive sleep disordered breathing but thorough examination revealed a left lateral laryngeal saccular cyst which was causing an airway obstruction, producing the noisy breathing. There was disappearance of the snoring after marsupialization of the cyst.
The Case
A 3-year old male presented with a 2-year history of snoring, restless sleep, and intermittent difficulty of breathing during sleep. He was initially managed as a case of Tonsillopharyngitis by a general physician but when no change in the condition was noted after medical treatment, an impression of Obstructive Tonsillar Hyperplasia was given and observation was advised until an age ‘suitable’ for tonsillectomy was reached. He was treated with Anti-Koch’s medication for six months because of a Chest x-ray finding of Hilar Lymphadenopathies. The snoring and restless sleep persisted with the occasional difficulty of breathing noted even when awake. Hoarseness was also already noted. He was brought for consultation with a pediatrician who attributed the symptoms to Acute Bronchitis, Rule out cardiac pathology. He was confined in a local hospital for three days, given intravenous antibiotics, and discharged unimproved after 3 days. Referral to a pediatric cardiologist was made and he was apparently ‘cleared’ of any cardiac abnormality. The cardiologist advised evaluation from an otorhinolaryngologist.
On physical examination, the patient was well nourished, fully developed and active. There was no palpable lymph node in the neck. Inspiratory stridor was noted. There was no nasal turbinate congestion and the adenoids were not enlarged. The faucial tonsils, uvula and tongue were unremarkable. Fiberoptic flexible endoscopy of the larynx revealed a smooth bulging mass at the area of the left ventricle and pushing the left aryepiglottic fold medially. The glottis cannot be visualized properly although there was discernible movement of the right vocal fold. In order to secure the airway for a more thorough examination and to avoid any airway complication that could arise perioperatively, a pediatric tracheostomy was done with insertion of a size 4.5 Shiley tracheostomy tube. On direct laryngoscopy and bronchoscopy, the epiglottis and vallecula were unremarkable. The smooth compressible globular mass obscured the normal contour of the left ventricle and pushed the left aryepiglottic fold medially. The vocal folds were pushed to the right. The left pyriform sinus was obliterated. No mass was noted in the subglottis and trachea. 5 cc of brownish mucoid fluid admixed with blood was aspirated from the mass using a gauge 23 spinal needle.
Multiple plain and Gadolinium enhanced axial and coronal Magnetic Resonance Imaging of the neck revealed a well defined mass along the left paralaryngeal space, extending from the level of the true vocal cord up to the proximal aryepiglotic fold. This measures 1.8 x 1.4 x 2.2 cm with no significant contrast enhancement. The aryepiglottic fold is displaced medially causing narrowing of the supraglottic airway. The subglottic area and cervical trachea are intact. The left pyriform sinus is obliterated.
Endoscopy guided marsupialization was done. Incision was made along the lateral edge of the cyst from anterior to posterior. The mucosa was then grasped and dissected along with the roof of the cyst. The content of the mass, 7 cc of brownish mucoid material, was then suctioned out.
Postoperatively, the tracheostomy tube was kept in place for 10 days. At the time of successful decanulation in the operating room, repeat direct laryngocopy and bronchoscopy revealed granulation tissue overlying the previous area of the cyst. This was excised and bleeders cauterized. Flexible fiberoptic endoscopy of the larynx done after 7 days showed a healing mucosa over the left ventricle with return of its normal contour. Both vocal folds were noted to be freely mobile up to full abduction.
No more snoring, difficulty of breathing and hoarseness reported.
Discussion
Snoring is a sound of breathing described from the extreme of being gentle as a kitten’s purr, to earthshaking like a locomotive’s roar, produced by breathing through the nose and mouth at the same time, causing vibration of the soft palate between the 2 currents of air2.This results from airway obstruction in the nose, nasopharynx or oropharynx and supraglottis. This is different, although hard to distinguish, from the harsh, medium-pitched sound caused by rapid turbulent airflow through a narrowed segment of the large airways, termed stridor. This signifies obstruction in the supraglottis, glottis, subglottis or tracheal regions3.
Presented with a history of snoring with associated restless sleep and difficulty of breathing during sleep, our first consideration of the patient’s condition was an upper pharyngeal obstruction causing obstructive sleep disordered breathing. Anatomic abnormalities like a low hanging soft palate, long uvula, prominent vertical pharyngeal folds, large base of tongue, large adenoids and large faucial tonsils were our initial considerations. However, these were not seen in the patient prompting us to do examination of the lower pharynx and larynx. The associated hoarseness and inspiratory stridor also gave us the suspicion of a possible laryngeal problem. We initially did transnasal flexible laryngoscopy, followed by a more definitive direct laryngoscopy and bronchoscopy under general anesthesia, after securing the airway by tracheostomy tube insertion. It was then that we noted the left lateral saccular cyst of the larynx. Magnetic resonance imaging of the neck confirmed our finding.
A saccular cyst is an abnormal mucous-filled dilatation of the laryngeal saccule that does not communicate with the laryngeal lumen. This arises as a result of either atresia or from inflammation, trauma, or tumor which may occlude the ventricular orifice4. As no history of previous trauma or infection was elicited, the saccular cyst of our patient might have resulted from atresia of the ventricular orifice. Laryngeal saccular cysts can be anterior, extending medially and posteriorly, protruding into the laryngeal lumen between the true and false vocal folds, or lateral, extending postero-superiorly and laterally into the false vocal fold and aryepiglottic fold5, just like in our case.
The question whether the noisy breathing noted from the patient was really snoring or actually a stridor exists. Considering the mechanism of how both sounds are produced, and with the result of our physical examination, we think it could be both. Given the slowly growing left lateral saccular cyst, progressive narrowing of the laryngeal inlet increased the velocity of airflow producing turbulence that could be heard as inspiratory stridor, and the resulting increase in the negative pressures near the pharyngeal wall (Bernoulli principle), exceeding the tension provided by the dilatory muscles of respiration, produced airway collapse during sleep, manifested as snoring (Figure 6). The limited movement of the vocal folds, as both were pushed to the right, produced the associated hoarseness.
Management includes securing the airway immediately, especially in patients with severe respiratory obstruction, and removal of the cyst6. For our case, a tracheostomy tube was inserted to secure the airway for a more thorough examination and to avoid any airway complication that could arise perioperatively. A choice between endoscopic removal (where the cyst can be marsupialized, excised or de-roofed) and external approach (in the form of laryngofissure or lateral cervical approach) exists7 Endoscopic removal is regarded as the treatment of choice, with external approach reserved for recurrence8. For the patient, we did endoscopy guided marsupialization of the cyst which, post-operatively, resolved the ‘snoring’ and dyspnea of the patient.
Conclusion
Lateral laryngeal saccular cysts, a rare condition occurring in 1.82 per 100,000 live births, can cause narrowing of the upper airway and thus present with snoring and signs of obstruction. It should therefore be included as a consideration in cases of snoring child. Thorough naso-,oro-, and hypopharyngeal examination should always be done in patients presenting with such signs and symptoms. Transnasal flexible laryngoscopy is useful in establishing the diagnosis. Endoscopy guided marsupialization of the cyst is an adequate treatment.
Introduction
Snoring in the adult has always been thought of as a benign condition, more of a nuisance than a medical problem. But if it is heard from a child, it is thought of as a significant indicator of an underlying pathology that needs to be looked into. Literature commonly associates it with Obstructive Sleep Apnea (Ejercito, 1995; Krespi and Kacker, 2003). Specifically, it has been attributed to anatomic abnormalities such as a large lingual tonsil (Dundar, et al, 1996), laryngomalacia (Chetty, et al, 1994), and epiglottic prolapse (Woo, 1992). Other reported conditions include obesity, nasal obstruction (Chen and Kushida, 2003), unfavorable tongue to mandible relationship, pharyngeal hypotonia and pharyngeal space occupying tumors1. There has been no report attributing it to a laryngeal mass like the case presented.
This is a case report of a 3-year old boy who was brought for consultation because of snoring and related restless sleep. History seemed to point to an upper pharyngeal abnormality resulting to an obstructive sleep disordered breathing but thorough examination revealed a left lateral laryngeal saccular cyst which was causing an airway obstruction, producing the noisy breathing. There was disappearance of the snoring after marsupialization of the cyst.
The Case
A 3-year old male presented with a 2-year history of snoring, restless sleep, and intermittent difficulty of breathing during sleep. He was initially managed as a case of Tonsillopharyngitis by a general physician but when no change in the condition was noted after medical treatment, an impression of Obstructive Tonsillar Hyperplasia was given and observation was advised until an age ‘suitable’ for tonsillectomy was reached. He was treated with Anti-Koch’s medication for six months because of a Chest x-ray finding of Hilar Lymphadenopathies. The snoring and restless sleep persisted with the occasional difficulty of breathing noted even when awake. Hoarseness was also already noted. He was brought for consultation with a pediatrician who attributed the symptoms to Acute Bronchitis, Rule out cardiac pathology. He was confined in a local hospital for three days, given intravenous antibiotics, and discharged unimproved after 3 days. Referral to a pediatric cardiologist was made and he was apparently ‘cleared’ of any cardiac abnormality. The cardiologist advised evaluation from an otorhinolaryngologist.
On physical examination, the patient was well nourished, fully developed and active. There was no palpable lymph node in the neck. Inspiratory stridor was noted. There was no nasal turbinate congestion and the adenoids were not enlarged. The faucial tonsils, uvula and tongue were unremarkable. Fiberoptic flexible endoscopy of the larynx revealed a smooth bulging mass at the area of the left ventricle and pushing the left aryepiglottic fold medially. The glottis cannot be visualized properly although there was discernible movement of the right vocal fold. In order to secure the airway for a more thorough examination and to avoid any airway complication that could arise perioperatively, a pediatric tracheostomy was done with insertion of a size 4.5 Shiley tracheostomy tube. On direct laryngoscopy and bronchoscopy, the epiglottis and vallecula were unremarkable. The smooth compressible globular mass obscured the normal contour of the left ventricle and pushed the left aryepiglottic fold medially. The vocal folds were pushed to the right. The left pyriform sinus was obliterated. No mass was noted in the subglottis and trachea. 5 cc of brownish mucoid fluid admixed with blood was aspirated from the mass using a gauge 23 spinal needle.
Multiple plain and Gadolinium enhanced axial and coronal Magnetic Resonance Imaging of the neck revealed a well defined mass along the left paralaryngeal space, extending from the level of the true vocal cord up to the proximal aryepiglotic fold. This measures 1.8 x 1.4 x 2.2 cm with no significant contrast enhancement. The aryepiglottic fold is displaced medially causing narrowing of the supraglottic airway. The subglottic area and cervical trachea are intact. The left pyriform sinus is obliterated.
Endoscopy guided marsupialization was done. Incision was made along the lateral edge of the cyst from anterior to posterior. The mucosa was then grasped and dissected along with the roof of the cyst. The content of the mass, 7 cc of brownish mucoid material, was then suctioned out.
Postoperatively, the tracheostomy tube was kept in place for 10 days. At the time of successful decanulation in the operating room, repeat direct laryngocopy and bronchoscopy revealed granulation tissue overlying the previous area of the cyst. This was excised and bleeders cauterized. Flexible fiberoptic endoscopy of the larynx done after 7 days showed a healing mucosa over the left ventricle with return of its normal contour. Both vocal folds were noted to be freely mobile up to full abduction.
No more snoring, difficulty of breathing and hoarseness reported.
Discussion
Snoring is a sound of breathing described from the extreme of being gentle as a kitten’s purr, to earthshaking like a locomotive’s roar, produced by breathing through the nose and mouth at the same time, causing vibration of the soft palate between the 2 currents of air2.This results from airway obstruction in the nose, nasopharynx or oropharynx and supraglottis. This is different, although hard to distinguish, from the harsh, medium-pitched sound caused by rapid turbulent airflow through a narrowed segment of the large airways, termed stridor. This signifies obstruction in the supraglottis, glottis, subglottis or tracheal regions3.
Presented with a history of snoring with associated restless sleep and difficulty of breathing during sleep, our first consideration of the patient’s condition was an upper pharyngeal obstruction causing obstructive sleep disordered breathing. Anatomic abnormalities like a low hanging soft palate, long uvula, prominent vertical pharyngeal folds, large base of tongue, large adenoids and large faucial tonsils were our initial considerations. However, these were not seen in the patient prompting us to do examination of the lower pharynx and larynx. The associated hoarseness and inspiratory stridor also gave us the suspicion of a possible laryngeal problem. We initially did transnasal flexible laryngoscopy, followed by a more definitive direct laryngoscopy and bronchoscopy under general anesthesia, after securing the airway by tracheostomy tube insertion. It was then that we noted the left lateral saccular cyst of the larynx. Magnetic resonance imaging of the neck confirmed our finding.
A saccular cyst is an abnormal mucous-filled dilatation of the laryngeal saccule that does not communicate with the laryngeal lumen. This arises as a result of either atresia or from inflammation, trauma, or tumor which may occlude the ventricular orifice4. As no history of previous trauma or infection was elicited, the saccular cyst of our patient might have resulted from atresia of the ventricular orifice. Laryngeal saccular cysts can be anterior, extending medially and posteriorly, protruding into the laryngeal lumen between the true and false vocal folds, or lateral, extending postero-superiorly and laterally into the false vocal fold and aryepiglottic fold5, just like in our case.
The question whether the noisy breathing noted from the patient was really snoring or actually a stridor exists. Considering the mechanism of how both sounds are produced, and with the result of our physical examination, we think it could be both. Given the slowly growing left lateral saccular cyst, progressive narrowing of the laryngeal inlet increased the velocity of airflow producing turbulence that could be heard as inspiratory stridor, and the resulting increase in the negative pressures near the pharyngeal wall (Bernoulli principle), exceeding the tension provided by the dilatory muscles of respiration, produced airway collapse during sleep, manifested as snoring (Figure 6). The limited movement of the vocal folds, as both were pushed to the right, produced the associated hoarseness.
Management includes securing the airway immediately, especially in patients with severe respiratory obstruction, and removal of the cyst6. For our case, a tracheostomy tube was inserted to secure the airway for a more thorough examination and to avoid any airway complication that could arise perioperatively. A choice between endoscopic removal (where the cyst can be marsupialized, excised or de-roofed) and external approach (in the form of laryngofissure or lateral cervical approach) exists7 Endoscopic removal is regarded as the treatment of choice, with external approach reserved for recurrence8. For the patient, we did endoscopy guided marsupialization of the cyst which, post-operatively, resolved the ‘snoring’ and dyspnea of the patient.
Conclusion
Lateral laryngeal saccular cysts, a rare condition occurring in 1.82 per 100,000 live births, can cause narrowing of the upper airway and thus present with snoring and signs of obstruction. It should therefore be included as a consideration in cases of snoring child. Thorough naso-,oro-, and hypopharyngeal examination should always be done in patients presenting with such signs and symptoms. Transnasal flexible laryngoscopy is useful in establishing the diagnosis. Endoscopy guided marsupialization of the cyst is an adequate treatment.
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