CASE REPORT

Diagnosis and management of a neonatalintralingual cyst of foregut origin

Sunil Patel a,b,*, Shamir Chandarana b, Murad Husein b, Nancy Chan b,c

a London Health Sciences Centre, 250 North Centre Road Unit 30, London, Ontario, Canada N6G 5A4bChildren’s Hospital, London, Health Sciences Centre, 800 Commissioners Road East,London, Ontario, Canada N6A 5W9c London Health Sciences Centre, 800 Commissioners Road East, London, Ontario, Canada N6A 5W9

Received 1 October 2008; accepted 9 December 2008Available online 18 January 2009

International Journal of Pediatric Otorhinolaryngology Extra (2010) 5, 1—4

www.elsevier.com/locate/ijporl

KEYWORDSCongenital oral cavitycyst;Intralingual cyst;Congenital foregut cyst

Summary Congenital intralingual cysts of foregut origin are extremely rare. Thesecysts have the potential to compromise the fetal airway at the time of delivery. Wepresent a case of a 37-week fetus with an intralingual cyst identified by antenatalultrasound and subsequently further delineated by MRI. Information acquired fromthe imaging allowed for controlled delivery with necessary precautions to manage animpending airway obstruction. Excision of the cyst occurred at day 8 of life. We havereviewed the literature as it pertains to our case as well as the role of imaging inmanaging fetal oral cavity cysts.# 2009 Elsevier Ireland Ltd. All rights reserved.

Introduction

Congenital oral cavity cysts can present a challengeto the airway management of the neonate.Advances in antenatal imaging have allowed forearly identification and treatment of these cystsand can improve a neonate’s outcome. Typically,congenital cysts and pseudocysts in the oral cavityoriginate from the minor salivary glands. It is rarefor cysts of foregut origin to arise in the oral cavity.The case presented here is one of five reportedcases of an in-utero identification of an intralingualcyst of foregut origin. Subsequent management isdiscussed.

* Corresponding author. Tel.: +1 5197012535.E-mail address: spatel2009@meds.uwo.ca (S. Patel).

1871-4048/$ — see front matter # 2009 Elsevier Ireland Ltd. All rigdoi:10.1016/j.pedex.2008.12.006

Case report

A 28-year-oldmother at 37weeks gestation,whowasnoted antenatally to have mild polyhyrdamnios,underwent a fetal ultrasound. A diagnosis of a lingualcyst of the oral tongue was made. Ultrasound of thefetal mouth, showed a 1.4 cm � 1.0 cm � 1.5 cmlesion on the tongue of the fetus (Figs. 1 and 2).The cyst was noted to move with tongue protrusionand retraction with no evidence of vascularity withinthe cyst. As a result, the otolaryngology service wasconsulted to manage potential airway obstruction atthe time of delivery. A fetal MRI was performed, tofurther characterize thecyst (Fig. 3).TheMRIdemon-strated amniotic fluid present beyond the cyst, indi-cating an incomplete obstruction. The delivery wasinduced with otolaryngology present. The child wasdelivered in a controlled setting with preparations to

hts reserved.

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Figure 3 Antenatal MRI demonstrating lingual cyst.

Figure 1 Antenatal ultrasound demonstrating lingualcyst.

intervene in case of airway obstruction. Upon deliv-ery, the infant demonstrated moderate obstructivebreathing necessitating the use of an oral airway. Theoral airway was removed once the infant exhibitedincreased tone. The patient was then admitted instable condition to the pediatric critical care unit forobservation.

Figure 2 3D reconstruction of the fetal head with lin-gual cyst.

Elective surgery to remove the cyst was sched-uled for 8 days after birth. The baby was intubatedby otolarynogology under direct visualization of theglottis with a size 0 Miller laryngoscope.

The cyst was located on the ventral or lingualsurface of the tongue, to the left of the midline(Fig. 4). Sharp dissection along the midline of theventral surface of the tongue with elevation of amucosal flap overlying the cyst was carried out(Fig. 5). The cyst was then freed from the soft tissuesurrounding it. At no point was the wall of the cystpenetrated. The cyst was enucleated and measuredapproximately 2 cm in greatest dimension (Fig. 6).The wound bed was closed in a straight line with 4—0vicryl sutures and the patient was transferred to thepediatric critical care unit. The patient was keptintubated for 24 h in anticipation of swelling of thetongue.

Figure 4 Preoperative image of the ventral tongue cyst.

Diagnosis and management of a neonatal intralingual cyst 3

Figure 6 Cyst fully excised.

Figure 5 Cyst partial excised.

Pathologic examination of the cyst revealed apale tan fluid filled sac like structure. The fluidwas viscous and opaque. The cyst was lined withciliated columnar and cuboidal cells, resemblingthat of gastric epithelium, with focal mucin-produ-

Figure 7 Cyst lining with focal area of gastric epithe-lium.

cing cells (Fig. 7). The diagnosis was consistent witha cyst of foregut origin.

The patient was discharged to home 2 days post-surgery. The patient was stable, and had beenbreathing and feeding well. On follow up 1 monthlater, the patient was doing well with good tonguemobility and good feeding.

Discussion

Cysts of foregut origin can present along the diges-tive tract, most commonly in the small intestine,with only 0.3% occurring in the oral cavity [1].Specifically, these cysts have been reported to occurfrom the esophagus to the colon, and in the gall-bladder, pancreas, lungs, larynx and urinary blad-der. When these cysts occur in the oral cavity,approximately 60% have been located on the ventralaspect of the tongue [2].

Although not well understood, it has been pro-posed that the pathogenesis of these cysts is asso-ciated with a defect in the migration of the islandsof endoderm of the primordial stomach in the fourthweek of fetal development [3]. The differentialdiagnosis of a congenital oral cavity cyst includesa mucocele, ranula, thyroglossal duct cyst, subman-dibular lymphatic malformation, hemangioma, lin-gual thyroid and dermoid cyst [3].

The most common location in the oral cavity for acyst of foregut origin is the ventral surface of theanterior tongue [4]. They are a rare occurrence withapproximately 30 such cases reported in the Englishliterature in the last 100 years [5]. The cysts aremore common in males (approximately 75% of thecases) and have been diagnosed in patients up to 35years old with an average age of presentation of 10years old [5]. Patients can present with a variety ofsymptoms based on the size and location of the cyst.Symptoms can range from no symptoms to airwaycompromise, dysphagia, feeding difficulties orrecurrent bleeding [1,2].

Only five cases have previously been diagnosed inutero [3,6—9]. Most commonly, the cyst has beenidentified using routine ultrasound at later gesta-tional dates, nevertheless, the cyst has been iden-tified as early as 20 weeks gestation [8]. MRI can beuseful in localizing the cyst as well as its extent, andto differentiate it from a plunging ranula.

Management of these patients centers aroundprotecting the neonatal airway. Adequate pre-paration is the key to avoid deleterious outcomes.Management can range from oral airway insertionto an ex-utero intrapartum treatment (EXIT) pro-cedure. EXIT success is dependent upon the main-tenance of the uteroplacental perfusion while the

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airway is being secured. A caesarean section isperformed under general anesthetic allowing20—30 min of placental perfusion. During thistime, the fetal airway can be secured using anorotracheal tube, bronchoscopy or by performinga tracheostomy. Decompression of cysts can alsobe achieved [10]. Identification and characteriza-tion of the lesion is essential to ensure appropriateplanning and to manage a difficult airway inthe neonate. Ultrasound is a good modality foridentifying a lesion, determining calcifications andevaluating swallowing (via colour doppler). FetalMRI is an excellent modality to discern morespecific details of the lesion’s location, thenature of the lesion and the degree of airwaydistortion and compression [11]. In our case, thefetal MRI indicated fluid posterior to the cyst,implying a patent airway. It was elected that anEXIT procedure would not be required, althoughpreparation for a difficult airway was deemedmost appropriate.

Conclusion

We report on the unique finding of an enteric cystin the oral cavity, in hopes to contribute tothe small but growing body of literature surround-ing this pathologic finding. Emphasis shouldbe on early detection of the pathology and pre-paration for a complicated delivery. Therebyavoiding unexpected and potentially deleteriousoutcomes.

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