Volume 5, Issue 1 , Pages 1-4, January 2010
Diagnosis and management of a neonatal intralingual cyst of foregut origin
Article Outline
Summary
Congenital intralingual cysts of foregut origin are extremely rare. These cysts have the potential to compromise the fetal airway at the time of delivery. We present a case of a 37-week fetus with an intralingual cyst identified by antenatal ultrasound and subsequently further delineated by MRI. Information acquired from the imaging allowed for controlled delivery with necessary precautions to manage an impending airway obstruction. Excision of the cyst occurred at day 8 of life. We have reviewed the literature as it pertains to our case as well as the role of imaging in managing fetal oral cavity cysts.
Keywords: Congenital oral cavity cyst, Intralingual cyst, Congenital foregut cyst
Introduction
Congenital oral cavity cysts can present a challenge to the airway management of the neonate. Advances in antenatal imaging have allowed for early identification and treatment of these cysts and can improve a neonate's outcome. Typically, congenital cysts and pseudocysts in the oral cavity originate from the minor salivary glands. It is rare for cysts of foregut origin to arise in the oral cavity. The case presented here is one of five reported cases of an in-utero identification of an intralingual cyst of foregut origin. Subsequent management is discussed.
Case report
A 28-year-old mother at 37 weeks gestation, who was noted antenatally to have mild polyhyrdamnios, underwent a fetal ultrasound. A diagnosis of a lingual cyst of the oral tongue was made. Ultrasound of the fetal mouth, showed a 1.4
cm×1.0cm×1.5cm lesion on the tongue of the fetus (Figure 1, Figure 2). The cyst was noted to move with tongue protrusion and retraction with no evidence of vascularity within the cyst. As a result, the otolaryngology service was consulted to manage potential airway obstruction at the time of delivery. A fetal MRI was performed, to further characterize the cyst (Fig. 3). The MRI demonstrated amniotic fluid present beyond the cyst, indicating an incomplete obstruction. The delivery was induced with otolaryngology present. The child was delivered in a controlled setting with preparations to intervene in case of airway obstruction. Upon delivery, the infant demonstrated moderate obstructive breathing necessitating the use of an oral airway. The oral airway was removed once the infant exhibited increased tone. The patient was then admitted in stable condition to the pediatric critical care unit for observation.
Figure 1.
Antenatal ultrasound demonstrating lingual cyst.
Figure 2.
3D reconstruction of the fetal head with lingual cyst.
Figure 3.
Antenatal MRI demonstrating lingual cyst.
Elective surgery to remove the cyst was scheduled for 8 days after birth. The baby was intubated by otolarynogology under direct visualization of the glottis with a size 0 Miller laryngoscope.
The cyst was located on the ventral or lingual surface of the tongue, to the left of the midline (Fig. 4). Sharp dissection along the midline of the ventral surface of the tongue with elevation of a mucosal flap overlying the cyst was carried out (Fig. 5). The cyst was then freed from the soft tissue surrounding it. At no point was the wall of the cyst penetrated. The cyst was enucleated and measured approximately 2cm in greatest dimension (Fig. 6). The wound bed was closed in a straight line with 4–0 vicryl sutures and the patient was transferred to the pediatric critical care unit. The patient was kept intubated for 24h in anticipation of swelling of the tongue.
Figure 4.
Preoperative image of the ventral tongue cyst.
Figure 5.
Cyst partial excised.
Figure 6.
Cyst fully excised.
Pathologic examination of the cyst revealed a pale tan fluid filled sac like structure. The fluid was viscous and opaque. The cyst was lined with ciliated columnar and cuboidal cells, resembling that of gastric epithelium, with focal mucin-producing cells (Fig. 7). The diagnosis was consistent with a cyst of foregut origin.
Figure 7.
Cyst lining with focal area of gastric epithelium.
The patient was discharged to home 2 days post-surgery. The patient was stable, and had been breathing and feeding well. On follow up 1 month later, the patient was doing well with good tongue mobility and good feeding.
Discussion
Cysts of foregut origin can present along the digestive tract, most commonly in the small intestine, with only 0.3% occurring in the oral cavity [1]. Specifically, these cysts have been reported to occur from the esophagus to the colon, and in the gallbladder, pancreas, lungs, larynx and urinary bladder. When these cysts occur in the oral cavity, approximately 60% have been located on the ventral aspect of the tongue [2].
Although not well understood, it has been proposed that the pathogenesis of these cysts is associated with a defect in the migration of the islands of endoderm of the primordial stomach in the fourth week of fetal development [3]. The differential diagnosis of a congenital oral cavity cyst includes a mucocele, ranula, thyroglossal duct cyst, submandibular lymphatic malformation, hemangioma, lingual thyroid and dermoid cyst [3].
The most common location in the oral cavity for a cyst of foregut origin is the ventral surface of the anterior tongue [4]. They are a rare occurrence with approximately 30 such cases reported in the English literature in the last 100 years [5]. The cysts are more common in males (approximately 75% of the cases) and have been diagnosed in patients up to 35 years old with an average age of presentation of 10 years old [5]. Patients can present with a variety of symptoms based on the size and location of the cyst. Symptoms can range from no symptoms to airway compromise, dysphagia, feeding difficulties or recurrent bleeding [1], [2].
Only five cases have previously been diagnosed in utero [3], [6], [7], [8], [9]. Most commonly, the cyst has been identified using routine ultrasound at later gestational dates, nevertheless, the cyst has been identified as early as 20 weeks gestation [8]. MRI can be useful in localizing the cyst as well as its extent, and to differentiate it from a plunging ranula.
Management of these patients centers around protecting the neonatal airway. Adequate preparation is the key to avoid deleterious outcomes. Management can range from oral airway insertion to an ex-utero intrapartum treatment (EXIT) procedure. EXIT success is dependent upon the maintenance of the uteroplacental perfusion while the airway is being secured. A caesarean section is performed under general anesthetic allowing 20–30min of placental perfusion. During this time, the fetal airway can be secured using an orotracheal tube, bronchoscopy or by performing a tracheostomy. Decompression of cysts can also be achieved [10]. Identification and characterization of the lesion is essential to ensure appropriate planning and to manage a difficult airway in the neonate. Ultrasound is a good modality for identifying a lesion, determining calcifications and evaluating swallowing (via colour doppler). Fetal MRI is an excellent modality to discern more specific details of the lesion's location, the nature of the lesion and the degree of airway distortion and compression [11]. In our case, the fetal MRI indicated fluid posterior to the cyst, implying a patent airway. It was elected that an EXIT procedure would not be required, although preparation for a difficult airway was deemed most appropriate.
Conclusion
We report on the unique finding of an enteric cyst in the oral cavity, in hopes to contribute to the small but growing body of literature surrounding this pathologic finding. Emphasis should be on early detection of the pathology and preparation for a complicated delivery. Thereby avoiding unexpected and potentially deleterious outcomes.
References
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PII: S1871-4048(08)00081-6
doi:10.1016/j.pedex.2008.12.006
© 2009 Elsevier Ireland Ltd. All rights reserved.
Volume 5, Issue 1 , Pages 1-4, January 2010
