|Year : 2020 | Volume
| Issue : 3 | Page : 218-221
Congenital laryngeal saccular cyst leading to stridor in a newborn: Think beyond laryngomalacia
Jyoti Patodia, Sunil Garg, Jaikrishan Mittal
Department of Neonatology, Neoclinic Children Hospital, Jaipur, Rajasthan, India
|Date of Submission||28-Mar-2020|
|Date of Decision||31-Mar-2020|
|Date of Acceptance||03-Apr-2020|
|Date of Web Publication||07-Aug-2020|
Dr. Jyoti Patodia
Department of Neonatology, Neoclinic Children Hospital, 3-4, T. N. Mishra Marg, Nirman Nagar, Opp. Metro Pillar No. 35, Jaipur - 302 019, Rajasthan
Source of Support: None, Conflict of Interest: None
Laryngomalacia is the most common congenital anomaly of the larynx leading to stridor in newborns. However, laryngeal cyst, although rare, should also be considered in the differential diagnosis of stridor. Hence, every newborn with stridor should undergo flexible/rigid endoscopic evaluation of the airway so that these rare entities are not missed, as its early recognition and proper treatment are essential because it can cause life-threatening airway obstruction. We describe a newborn presenting to us with stridor and severe respiratory distress since birth, who was successfully treated with no recurrence, with transoral microscopic complete excision of the laryngeal saccular cyst. We emphasize the importance of a good clinical history along with endoscopic evaluation of the airway in every neonate presenting with stridor with an unexpected evolution to determine the causal lesion.
Keywords: Microscopic excision, newborn, saccular cyst, stridor
|How to cite this article:|
Patodia J, Garg S, Mittal J. Congenital laryngeal saccular cyst leading to stridor in a newborn: Think beyond laryngomalacia. J Clin Neonatol 2020;9:218-21
|How to cite this URL:|
Patodia J, Garg S, Mittal J. Congenital laryngeal saccular cyst leading to stridor in a newborn: Think beyond laryngomalacia. J Clin Neonatol [serial online] 2020 [cited 2022 Jan 16];9:218-21. Available from: https://www.jcnonweb.com/text.asp?2020/9/3/218/291645
| Introduction|| |
Laryngeal cyst, although rare, is among the important causes of airway obstruction in neonates. Their incidence is 1.8 in 100,000 live births. Congenital laryngeal cysts are of two types: ductal/vallecular and saccular. Ductal cysts, comprise 75% of laryngeal cyst, arise from the obstruction of the laryngeal epithelial mucous glands, and saccular cysts develop at the supraglottic level by blockage of the opening of the saccule of the laryngeal ventricle. The most common symptoms arising from laryngeal cysts are stridor, which may mimic laryngomalacia. Other symptoms include feeding difficulties, hoarse, or low crying and cyanotic/apneic episodes.
The diagnosis is confirmed by direct laryngoscopy or endoscopic visualization of the lesion and computed tomography (CT) scan, for its exact location and extension. Treatment of choice is complete excision of the cyst by a transoral approach with microscope or endoscope to avoid recurrence.
| Case Report|| |
A term (38 weeks), 2.6 kg male baby born to G2 P2 L2 A0, born by normal vaginal delivery, with no antenatal risk factors, cried immediately after birth (Apgarscore – 7, 8). Baby developed respiratory distress soon after birth and was shifted to our hospital on day 2 of life. On examination, he was having tachypnea (respiratory rate: 64/min), suprasternal/subcostal retractions, and stridor, which was persistent even on prone position but slightly decrease on the right lateral position. The baby also had a weak muffled cry. Baby initially required heated humidified high-flow nasal canal (HHFNC) support to maintain saturation. Septic screen and chest X-ray was done, which was normal. Initially, a probable diagnosis of laryngomalacia was kept, and the baby was started on orogastric feeds. However, respiratory distress and stridor further worsened, requiring intubation on day 5 of life. Thereafter, ENT consultation was taken, and endoscopic evaluation of the airway under general anesthesia was planned. A smooth cystic mass was seen on the right arytenoid region compressing the airway [Figure 1]. Contrast-enhanced CT neck was done showing cystic lesion (1.6 cm × 1.4 cm) on the right side, involving supraglottic, glottic, and mild extension into the subglottic area and compressing the airway [Figure 2]a and [Figure 2]b.
|Figure 1: A smooth saccular cystic mass on the right arytenoid region compressing the airway|
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|Figure 2: (a) Contrast-enhanced computed tomography neck (sagittal view) showing hypodense mass (1.6 cm × 1.4 cm) in the right side, involving supraglottic, glottic, and mild extension into the subglottic area andcompressing the airway. (b) Contrast-enhanced computed tomography neck (axial view)|
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On the 9th day of life, transoral endoscopic marsupialization of the right laryngeal cyst (saccular) was done with coablation [Figure 3]. About 1.5 cc of mucinous fluid came out of cyst, and the cavity was left wide open. Postoperatively, there was a drastic improvement, the baby was extubated on the next day and was on room air after 24 h. On postoperative day 3, the baby had a recurrence of mild stridor, so laryngoscopy was done, which showed some edema and slough at the postoperative site, there was no recurrence of the cyst. The baby was given supportive management (HHFNC, low-dose steroids, and H2 blockers) after which baby was improving. On postoperative day 9, the baby again had a recurrence of stridor, so baby was re-evaluated, confirmed as recurrence, and hence, complete excision of the cyst was done by transoral microscopic technique with coablation [Figure 4]. Baby was continued on nasogastric feeds and discharged on day 18 of life (1 week after the last surgery). Endoscopy was repeated after 15 days of cyst excision, which was normal, and no recurrence was seen. The plan is to do follow-up till 6–8 weeks.
| Discussion|| |
Congenital stridor can be caused by several causes: supraglottic, glottic, or subglottic conditions. Laryngomalacia was found to be the most common congenital laryngeal anomalies followed by subglottic stenosis, vocal cord paralysis, laryngeal webs, hemangiomas, papillomas, and laryngeal clefts.
Saccular cyst of the larynx remains a very rare cause of respiratory obstruction, which often can be missed. Saccular cyst represents 1.5% of congenital laryngeal anomalies. It can be differentiated from a laryngocele, as the latter contains air and communicates with the larynx.
The clinical presentation of laryngeal cysts depends on its location and size. Larger cysts may present at birth with severe airway obstruction, whereas smaller cysts may be asymptomatic or have mild distress, muffled cry, hoarseness, cyanotic episodes, and feeding difficulty. The most common symptom is inspiratory stridor mimicking laryngomalacia. However, in laryngomalacia, the stridor typically improves with prone position, it hardly causes dysphonia or severe respiratory obstruction requiring intubation, usually present in the 1st week of life and does not worsen with time. All these findings were contrary to our case, and this is when we suspected some other laryngeal anomaly apart from laryngomalacia. In addition, in laryngeal cysts, stridor decreases when the patient lies on the affected side, so was as in our case.
Hence, laryngomalacia, although common, is not always the cause of stridor in newborns. It can be differentiated from other causes by subtle clinical markers, which can be confirmed by endoscopic airway evaluation. Hence, emphasizing the importance of performing endoscopic airway evaluation in every newborn with stridor. Our case which presented with stridor on day 1 of life, associated with weak muffled cry, endoscopic airway evaluation confirmed the diagnosis of congenital laryngeal cyst. Further, CT or magnetic resonance imaging should be done to know the exact location and extent of the cyst.
There are different modalities of treatment for saccular cysts: endoscopic needle aspiration, marsupialization, and transoral microscopic excision of the cyst. Needle aspiration is a temporary measure used to relieve acute respiratory obstruction, but the recurrence is very high. Endoscopic marsupialization of the congenital saccular cyst is also documented as an effective line of treatment with little recurrence. Microscopic or endoscopic excision with complete removal of the cyst wall is essential to prevent a recurrence. Thus, transoral microscopic or endoscopic excision of the cyst is the treatment modality of choice for laryngeal cyst. Both CO2 laser and radiofrequency ablation/coablation can be used for marsupialization and vaporization of the cyst lining. Kirse et al. successfully treated a series of newborns with endoscopic extended ventriculotomy. Endoscopic excision is adequate for small cysts, but an external approach might be required for larger cysts.
Our aim at first surgery was to do an intervention with minimal surgical and anesthesia duration, minimum tissue handling, and minimum postoperative surgical site edema. To achieve all above goals, we planned for endoscopic marsupialization in our case in the first attempt. Unfortunately, there was recurrence on, so a complete excision of the cyst was done, with no recurrence on follow-up.
| Conclusion|| |
Endoscopic airway evaluation is mandatory in all cases of newborns presenting with stridor. A good clinical history and examination always helps to establish the diagnosis. Initial presentation may be mistaken for laryngomalacia, leading to delay in the diagnosis of rarer entities such as laryngeal cyst. Further, transoral microscopic excision with complete removal of the cyst wall is the mainstay of treatment to prevent a recurrence.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]