Volume 4, Issue 1 , Pages 10-13, January 2009
Endoscopic treatment of vallecular cyst in newborn
Article Outline
Summary
Congenital vallecular cyst is a rare disease in neonates and infants. Even though benign in nature, it owns a high potential morbidity and mortality. Stridor, dyspnea, feeding difficulties, coughing, voice changes, failure to thrive are the most common symptoms. The authors report a case of a 4-month-old infant who presented with a failure to thrive and respiratory distress due to a congenital vallecular cyst. Marsupialization was performed with total symptoms resolution.
Keywords: Vallecular cyst, Failure to thrive, Congenital laryngeal stridor
1. Introduction
Congenital vallecular cyst represents a rare disease occurring in newborns and early childhood. Even though benign in nature, it owns a high potential morbidity and mortality [1]. Stridor, dyspnea, feeding difficulties, coughing, voice changes, failure to thrive are the most common symptoms [2], [3]. In adulthood, such lesions tend to show softer symptoms and mostly, they are diagnosed incidentally [4]. The cyst arises from submucosal glands obstructed and it grows for the pooling of secretions [5]. The authors report a case of a 4-month-old infant presenting failure to thrive and respiratory distress due to the presence of a vallecular cyst and the treatment adopted in this case.
2. Case report
A 4-month-old infant presented with a perinatal history of dyspnea worsening with crying. Cyanosis or stridor was present. Backward bending of the head was present. Failure to thrive was observed with only 1
kg weight gain in the first 3 months. In the meanwhile, recurrent episodes of vomiting occurred after feeding. At the third month, he was admitted in another pediatric department where nasogastric feeding was attempted with unsatisfactory results.
On admission in our department, the patient showed an important dyspnea, low oxygenation (84% during the sleep), opisthotonus. Neurological exam was normal. A fiberoptic endoscopy was performed. It revealed the presence of a cyst of the vallecular space, overwhelming the epiglottis during inspiration. Magnetic nuclear resonance (MNR) confirmed the presence of a rounded lesion with a clear boundary hyperintense in the T2-weighted sequences and hypointense in the T1-weighted sequences without enhancement after gadolinium (Fig. 1, Fig. 2). On surgical treatment, an incision was made on the lingual surface of the cyst under rigid endoscopy control and the cyst was punctured (Fig. 3, Fig. 4). A large amount of mucoid material was suctioned and the remaining mucosa of the cyst was removed. The patient received intravenous antibiotics and was extubated immediately without any respiratory compromise. On the following day, no symptom was present and patient was discharged. A week after, at the follow-up control, a residual mucosal layer of the cyst was observed. One month after, to avoid any recurrence of disease, the remaining layer of the mucosa cyst was removed with Nd-Yag laser under endoscopic vision. Patient was dismissed on the following day. Further fiberoptic endoscopies showed normal healing process and absence of recurrence. The pathologic specimens confirmed the 2cm×2cm×1.5cm epiglottic cyst.
Fig. 1.
T1-weighted MNR showing a hypointense rounded lesion overwhelming the epiglottis.
Fig. 2.
T2-weighted MNR showing a hyperintense lesion.
Fig. 3.
Surgical setting for the treatment of the vallecular cyst.
Fig. 4.
Puncturing of the vallecular cyst under endoscopic control.
3. Discussion
Laryngeal congenital cysts are rare lesions. Firstly described by Abicombie in 1881 [1], they are analyzed in three big articles in the scientific literature. Among 20 congenital laryngeal cysts, Mitchell and colleagues found 5 vallecular cysts, while Hsieh and Liu diagnosed respectively 11 and 14 congenital vallecular cysts [6], [7].
Laryngeal cysts are divided in saccular and ductal type. The first one results from the saccular plane following the enlargement of such structure. The latter arises from the block of the submucosal gland and it is detected in vallecula. Rarely, it has been reported vallecular cysts are due to infection of the upper airways [8]. Burton described a case of vallecular cyst in an old patient secondary to an infection of Nocardia asteroides [9]. In our patient, no micro-organism has been detected.
Symptoms of VC are linked with obstruction of the upper airways. The main symptom is the congenital stridor present in almost all patients described in the scientific literature. Other signs and symptoms include thoracic indrawing, apnoea, chronic cough and cyanosis. Furthermore, affected children could present dysphagia, conation, and difficulty in feeding. Such symptoms worsen in the supine position. Finally, low weight and failure to thrive could reveal the presence of a vallecular cyst [10]. Differential diagnosis include thyroglossal duct inner cyst, dermoid cyst, thyroid lingual tissue, lymphangioma and emangioma [11]. Even though it allows to identify a mass in the vallecula, flexible endoscopy cannot distinguish between these different diseases. Preoperatively, it is necessary to perform diagnostic imaging to evacuate the structure, the borders and the relationship with the adjacent structures. Computed tomography is less worth in differential diagnosis because thyroglossal duct cyst, dermoid cyst, lymphangioma and emangioma appear as low density mass. Only, lingual thyroid tissue shows high density signal [12]. Nowadays, magnetic resonance imaging in T1- and T2-weighted sequences and gadolinium are the best choice in the diagnosis of vallecular cyst.
Several classifications of laryngeal cyst have been proposed secondary to the localization and extension. The first one distinguishes saccular, ductal and thyroid cartilage foramina-arising cyst [8]. Arens introduced the hystomorphologic aspect, differentiating them in congenital, retention and inclusion cysts [13]. Finally, Forte proposed a classification only of the congenital cyst based only of anatomical and hystomorphologic peculiarity. So we can identify intralaryngeal cyst or type I, included in the laryngeal border with only endodermal elements and extralaryngeal cyst. Furthermore, the latter is divided in IIa, with only endodermal elements, in type IIb, where we can identify mesodermal elements (laryngotracheal duplication of diverticulum) [14]. Our patient can be classified as IIa for the extralaryngeal localization and the absence of mesodermal elements.
Treatment of the vallecular cyst is basically surgical. Recurrence of disease is very high and the simple incision and drainage of the serous fluid tend to fail. In this case, it is mandatory to perform a marsupialization of the cyst with laryngeal knife, laser or diathermy. CO2 laser has been proposed for the dissection or vaporization of epithelial lining [14]. In our case, after a first marsupialization with diathermy, we perform a revision of the remaining fragments of the lesion cystic walls, using Nd-Yag laser. This technique allowed a sharp surgical excision, no oedema and patient's dismission in the first post-operative day.
4. Conclusions
Vallecular cyst is a rare disease, even though it should be hypotized in the presence of stridor and failure to thrive, fiberoptic endoscopy should ever be performed in such patients. Endoscopic treatment allows a more radical excision and it avoids any recurrence.
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PII: S1871-4048(08)00031-2
doi:10.1016/j.pedex.2008.04.003
© 2008 Elsevier Ireland Ltd. All rights reserved.
Volume 4, Issue 1 , Pages 10-13, January 2009
