International Journal of Pediatric Otorhinolaryngology xxx (2014) xxx–xxx

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Case Report

A congenital mucocele of the anterior dorsal tongue

J.E.R.E. Wong Chung a, R.J.H. Ensink b,*, H.F.H. Thijs c, F.J.A. van den Hoogen a

a Radboudumc Nijmegen, Department of Otorhinolaryngology, The Netherlandsb Gelre ziekenhuizen Zutphen, Department of Otorhinolaryngology, The Netherlandsc Gelre ziekenhuizen Zutphen, Department of Pediatrics, The Netherlands

A R T I C L E I N F O

Article history:

Received 7 March 2014

Received in revised form 31 March 2014

Accepted 6 April 2014

Available online xxx

Keywords:

Mucocele

Congenital

Tongue

Diagnosis

Treatment

A B S T R A C T

We report on a new-born with a congenital mucocele on the anterior dorsal side of the tongue. The

presentation as well as the differential diagnosis of congenital oral swellings is discussed.

Because of breastfeeding problems the mucinous swelling was incised and drained two days after

birth. Immediately after drainage the swelling disappeared.

Congenital oral swellings are rare. Most of them are mucoceles. Post-partum treatment is surgically,

but spontaneous remission has been described. High incidence of recurrence should be taken into

account when (micro-)marsupialization or incision as sole treatment is performed.

� 2014 Elsevier Ireland Ltd. All rights reserved.

Contents lists available at ScienceDirect

International Journal of Pediatric Otorhinolaryngology

jo ur n al ho m ep ag e: ww w.els evier . c om / lo cat e/ i jp o r l

1. Introduction

A congenital swelling of the tongue forms an interestingdiagnostic challenge for pediatricians, ENT-surgeons and maxil-lofacial surgeons as the differential diagnosis is very broad anddifferent lesions are not always easy to distinguish [1]. Amucocele is one of the most common (congenital) lesions andcan be found at different sites in the oral cavity. Mucoceles mainlyarise from salivary glands of the lower lip, buccal mucosa, anteriorventral tongue and floor of the mouth(ranula). Mucoceles of theretromolar region, palate, lingual frenum, and dorsal tongue arerare [2].

We report on a neonate, with feeding problems, caused by alarge cystic lesion of the anterior dorsal tongue. To our knowledge,a mucocele at this location has once been described until now [3].

2. Case presentation

A new-born was referred because of feeding problems. Ongeneral pediatric examination an aural tag on the right side wasfound as well as a hypospadias. Prolonged neonatal icterus wastreated by phototherapy. On general ENT examination no furtheranomalies, except a short tongue frenulum, were found. A soft

* Corresponding author. Tel.: +31 575592457.

E-mail addresses: Justin.ere@gmail.com (J.E.R.E. Wong Chung),

r.ensink@gelre.nl (R.J.H. Ensink).

Please cite this article in press as: J.E.R.E. Wong Chung, et al., A coOtorhinolaryngol. (2014), http://dx.doi.org/10.1016/j.ijporl.2014.04.0

http://dx.doi.org/10.1016/j.ijporl.2014.04.012

0165-5876/� 2014 Elsevier Ireland Ltd. All rights reserved.

mobile bluish mass, sized approximately 2 cm was found onthe anterior side of the dorsal tongue, localized in the midline(Fig. 1A and B). Because of breastfeeding problems, the lesion, withthe macroscopic appearance of a mucocele, was incised and thecontent was analyzed. The content was amylase rich and thereforemucin related. This confirmed the diagnosis of a mucocele,therefore further histological analysis seemed unnecessary. Fourweeks after drainage no re-occurrence of the lesion was found. Onthe anterior side of the tongue however a small fistula was seen(Fig. 2).

3. Discussion

Congenital cyst-like lesions in the oral cavity are rare. A PubMedsearch (1980–2013) using the MESH terms ‘‘congenital’’, ‘‘oral’’ and‘‘cysts’’ disclosed 66 articles. Mucoceles are diagnosed mostfrequently. On macroscopic appearance a mucocele presents asa painless solitary circular swelling filled with mucus, thatfluctuates on palpation, but can also become firm after keratiniza-tion. Their color is in general pink or bluish translucent. Mucocelescan cause different symptoms based on their location, size andthe age of the patient. A mucocele of the posterior dorsal tonguecan cause trouble breathing and swallowing. Mucoceles moreanteriorly can give occlusion problems of the mouth [4]. In ourcase the patients main problem was related to breastfeeding.

Most mucoceles, about 78%, are located at the lower lip, an areaeasily subject to trauma (2). The incidence in the tongue is 10%. In

ngenital mucocele of the anterior dorsal tongue, Int. J. Pediatr.12

Fig. 1. (A) Macroscopic appearance of the lesion of the anterior dorsal tongue,

diagnonal view. (B) Macroscopic appearance of the lesion of the anterior dorsal

tongue, frontal view.

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all reports this mucocele was located on the posterior dorsal side ofthe tongue only twice, but never on the anterior dorsal side.

There are three pathogenic ways a mucocele can develop.Mostly a rupture of the excretory duct caused by trauma, orinitiated by mucosal inflammation leads to blockage, dilatationand eventually rupture of the duct causing mucus to flow intosurrounding tissues (extravasation mucocele). Mucoceles canalso arise following duct obstruction without rupturing of thegland (retention mucocele) [5]. Congenital mucoceles can alsooccur as a failure of duct-canalization [1,6]. Trauma to a salivarygland or duct in a neonate can be caused by thumb-sucking inutero, during birth due to passage through the birth canal or whenforceps is used or after birth [7].

Fig. 2. Small fistula with shortened tongue frenulum present four weeks after

incision.

Please cite this article in press as: J.E.R.E. Wong Chung, et al., A coOtorhinolaryngol. (2014), http://dx.doi.org/10.1016/j.ijporl.2014.04.0

The tongue contains three main sets of salivary glands.The serous Von-Ebner glands, located around circumvallate andfoliate papillae in the tongue. The mucus glands of Weber, locatedat the superior pole of the peritonsillar space and the mixed mucusand serous glands of Blandin and Nuhn, or ‘‘anterior lingualglands’’, located at the ventral part of the anterior tongue. In theoryeach of these salivary glands can give rise to a mucocele (4).Mucoceles of the anterior lingual glands are very uncommon inchildren under 8 years of age [8] and are exclusively found inthe ventral surface of the tongue. Mucoceles of the glands ofVon Ebner or Weber have not been described till date. To ourknowledge no glands are found on the dorsal anterior tongue.

We think the mucocele in this neonate has originated from anunusual located anterior lingual gland. The short frenulum foundin this neonate indicates abnormalities during the fusion process ofthe three tongue buds which eventually form the anterior twothirds of the tongue. When, during development, the two distaltongue buds stay separated from each other for a longer period oftime, the acini in the anterior lingual glands can expand distal andbe found deep inside the tongue. These fusion problems alsocreates possibilities for the ducts to drain into the medial sulcus ofthe tongue, enabling a mucocele to form at the anterior dorsal sideof the tongue [9].

When encountering a congenital swelling of the tongue, thereis, apart from a mucocele, a wide differential diagnosis to consider.Swellings of the posterior dorsal tongue may be caused byhypertrofic lingual tonsils, lingual ectopic thyroid and thyroglossalduct cysts. In the anterior tongue ‘‘foregut duplication cysts’’ can befound [10]. (Epi)dermoid cysts, lymfangiomas [11], hemangiomas,venous lakes, lipomas, papillomas, adenomas and fibromas can beseen in every part of the tongue as well as the floor of mouth [8,12].Ranulas are typically located in the floor of the mouth, are largerand related to the sublingual glands.

Additional imaging (ultra-sound, CT or MRI scan) is onlynecessary when diagnosis is not clear. A distinction is essential fora cystic or vascular lesion because of its consequences fortreatment [13]. Prenatal diagnosis of a mucocele is rare, onlyseven cases, mostly ranulae, have been described [14]. Onultrasound mucoceles present as a sharply bordered, anechoicor homogeneously hypoechoic lesions [14] with distal acousticenhancement. MRI or CT can be helpful in an effort to determinethe salivary gland of origin of the lesion.

If diagnosed prenatally the risk of airway obstruction caused bythe growing congenital mucocele should be considered with a sizeover 40 mm [15]. If respiratory distress is expected at birth anextra-uterine intrapartum procedure should be considered [16].

Histopathology can confirm a clinical diagnosis. Mucus reten-tion can also be confirmed by high amylase and protein levels onchemical analysis. Clinicians should note that endothelial elementsof the ductal lining of a thyroglossal duct cysts produce mucus aswell [12].

Postnatal treatment is preferably surgical resection with coldsteal, electrosurgery, cryosurgery or CO2 laser vaporization. Othertherapeutic options are (micro-) marsupialization and simpleincising. Recurrence is frequent because of damage of surroundingminor salivary glands. Watchful waiting should be consideredwhen a mucocele does not cause any problems for the patient sinceit may resolve spontaneously if rupture takes place, for exampleduring feeding [6].

In our case the mucocele was first seen after birth. Prenatalultrasounds, routinely performed at 10 and 20 weeks of gestationalage were normal. The diagnosis of a mucocele was confirmed byproving that the lesion was rich of amylase. The anterior dorsaltongue as location for this mucocele is very rare. This mucocelemost likely originated from an anterior lingual gland whichdrained through a small, closed ended, duct into the dorsal tongue.

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Although only incising a mucocele results in high recurrencerates, we believe this approach should be considered for neonatesbecause of its limited invasive nature. Follow-up discloseddisappearance of the lesion. The non-recurrence can probably beexplained by the persistent fistula in the tongue, enabling thegland to drain freely into the oral cavity. If complications, namelyrecurrence, infection or dysplasia occurs, surgical enucleationshould be performed. Follow up until now at the age of 7 month’sdid not show any recurrence of the mucocele.

4. Conclusion

Congenital masses in the oral cavity are rare. Mucoceles are themost frequent findings. Although all locations in the oral cavityhave been described, this is the first report of a (congenital)mucocele of the anterior dorsal tongue, most likely originatingfrom an ectopic anterior lingual gland draining into the anteriordorsal tongue. Treatment of a mucocele is surgically, butspontaneous remission has been described. High incidence ofrecurrence should be taken into account when (micro-)marsupia-lization or incision as sole treatment is performed.

Conflicts of interests

None to declare.

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