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 Table of Contents  
CASE REPORT
Year : 2018  |  Volume : 7  |  Issue : 2  |  Page : 99-101

Congenital sublingual cyst masking the airway: Ex utero intrapartum treatment to life!


Department of Neonatology, Manipal Hospital, Bengaluru, Karnataka, India

Date of Web Publication10-Apr-2018

Correspondence Address:
Dr. Iyer Harohalli Venkatesh
Department of Neonatology, Manipal Hospital, Bengaluru, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jcn.JCN_117_17

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  Abstract 


A term male baby weighing 3000 g was delivered by elective cesarean section with antenatal diagnosis of mass in the oral cavity. The ex utero intrapartum treatment was performed. The magnetic resonance imaging of head and neck delineated the mass. The mass was excised and histopathology of the specimen suggested sublingual cyst. Postoperatively, he did well and got discharged home safe on full feeds.

Keywords: Ex Utero Intrapartum Treatment, mass in oral cavity, prenatal diagnosis, sublingual cyst


How to cite this article:
Venkatesh IH. Congenital sublingual cyst masking the airway: Ex utero intrapartum treatment to life!. J Clin Neonatol 2018;7:99-101

How to cite this URL:
Venkatesh IH. Congenital sublingual cyst masking the airway: Ex utero intrapartum treatment to life!. J Clin Neonatol [serial online] 2018 [cited 2021 Aug 2];7:99-101. Available from: https://www.jcnonweb.com/text.asp?2018/7/2/99/229663




  Introduction Top


Obstructed airway is a life-threatening event. Oral cysts are rare at birth or at the early neonatal period. Most infants present with respiratory problems due to airway obstruction. The diagnosis should be suspected when the tongue appears to protrude or appear elevated. A fetus with the antenatal diagnosis of oral mass with apparent airway obstruction can be effectively managed by ex UteroIintrapartum Treatment (EXIT) procedure with a well-coordinated multidisciplinary approach.


  Case Report Top


A term male baby with a large, antenatally detected oral cyst was born to a primi mother by elective cesarean section. The mother had antenatal history of polyhydramnios with a fetal oral cavity mass [Figure 1] measuring 3.5 cm × 3 cm in size on ultrasound [Figure 2]. The mother was taken for a planned lower segment cesarean section in the presence of multidisciplinary team comprising the obstetrician, the anesthesiologist, the ENT surgeon, the pediatric surgeon, the fetal medicine specialist, and the neonatologist. After the spinal anesthesia to the mother, the fetus was injected with anesthetic medications (succinylcholine and rocuronium) by fetal medicine specialist under direct ultrasound guidance, by passing a needle through the mother's abdomen into the fetal thigh.
Figure 1: Fetal US demonstrating fetus with a mass in mouth

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Figure 2: Fetal US demonstrating mass measuring 3.5 cm × 3cm

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Once the baby's head was delivered, the airway was secured with 3.5 size endotracheal tube using video-assisted laryngoscopy [Figure 3]. The remaining portion of the baby was then delivered and then the umbilical cord was severed. He was then shifted under radiant warmer and stabilized. Later, he was transported to the neonatal intensive care unit.
Figure 3: Ex-utero intrapartum intubation

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On examination, the cyst was seen to be arising from the dorsal surface of the tongue, approximately 4 cm in diameter [Figure 4]. Magnetic resonance imaging (MRI) of the head and neck was performed the next day to determine the extent and characteristics of the soft-tissue mass and demonstrated a well-defined homogeneous lesion arising from the anterior two-thirds of the tongue measuring 3.5 cm × 2.9 cm [Figure 5]. The lesion was reaching up to sublingual space posteriorly; however, it is not invading it. It is confined to intramuscular location and mainly the upper two-thirds of muscle. The lesion was hypointense on T1-weighted and very hyperintense on T2-weighted images. On the 2nd day of life, the cyst was excised. Histopathology revealed sublingual cyst lined by pseudostratified columnar epithelium with underlying loose connective tissue [Figure 6].
Figure 4: A mass arising from tongue

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Figure 5: Magnetic resonance imaging of the head and the neck – Sagittal section demonstrating a mass arising from tongue

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Figure 6: Histopathology of the excised specimen demonstrating pseudostratified columnar epithelium

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  Discussion Top


Congenital sublingual/lingual cysts in neonates are rare, and most of them are small in size (<1 cm) and asymptomatic, but giant cysts causing feeding and respiratory difficulty have been reported.[1],[2],[3],[4],[5] The differential diagnosis of cystic lesion of the oral cavity includes mucocele, epignathus, gingival cyst, palatal cyst, thyroglossal cyst, ranula, congenital epulis, vascular hamartoma, lymphangioma, and oropharyngeal teratoma.[6],[7],[8],[9] Aspiration cytology and enzyme studies, radiological investigations (computed tomography and MRI), and histology after will confirm diagnosis in majority of the cases. Prenatal diagnosis using two- and three-dimensional sonography, with guided aspiration, has also been reported.[10]

The EXIT procedure was stressed by Langer describing the importance of delivering the upper half of the fetal body and maintaining the fetomaternal circulation permitting uninterrupted fetal oxygenation and gas exchange.[11]

The tracheal occlusion, the neck mass, unilateral pulmonary agenesis, thoracic abnormality, conjoint twins, and congenital high airway obstruction are some of the indications for this novel procedure. In our index case, the mass was found in the oral cavity on antenatal scan and planned for the procedure at birth.

The multidisciplinary team comprising of the fetal ultrasonologist, the anaesthetist, the pediatric surgeon, the ENT surgeon and the neonatologist is very essential in smooth transition of the high risk neonate. The induction of anesthesia and its maintenance, uterine relaxation and maintaining the perfusion, surgical procedure, and delivering the baby are the main stages in EXIT procedure.

Different techniques are described to treat sublingual cysts. Spontaneous resolution has been observed, but usually, a surgical approach is recommended. It can involve marsupialization or unroofing of the cyst, simple excision of the cyst, or excision combined with excision of the ipsilateral sublingual gland. The recurrence rate in epithelium-free cyst is lowest after excision including the ipsilateral sublingual gland.

In our case, the cyst was lined by an epithelium. They develop from a mucus leak following disruption of the sublingual gland elements and are considered as pseudocysts. However, 7.5% of cysts are mucosal retention cysts with epithelium. The glands involved may then frequently display metaplasia of the epithelium lining. Even though the baby presented with cyst on the dorsum of the tongue, we got the cyst suggestive of sublingual cyst on histopathology.

Learning points

  1. A large oral cyst, occupying most of the oral cavity with threatened airway, is a definite challenge to the treating physician
  2. A qualified multidisciplinary team performing EXIT procedure can safely deliver the baby
  3. Once the cyst is surgically excised, the baby can lead a normal life.


Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Karam O, Pfister RE, Extermann P, La Scala GC. Congenital lingual cysts. J Pediatr Surg 2007;42:E25-7.  Back to cited text no. 1
    
2.
Kumar KV, Joshi M, Vishwanath N, Akhtar T, Oak SN. Neonatal lingual gastric duplication cyst: A rare case report. J Indian Assoc Pediatr Surg 2006;11:97-8.  Back to cited text no. 2
    
3.
Singh GB, Rai AK, Arora R, Garg S, Abbey P, Shukla S. Case Rep Otolaryngol 2013;2013:841930. doi: 10.1155/2013/841930.  Back to cited text no. 3
    
4.
Soni A, Suyal P, Suyal A. Congenital ranula in a newborn: A rare presentation. Indian J Otolaryngol Head Neck Surg 2012;64:295-7.  Back to cited text no. 4
    
5.
Ameh EA, Jimoh AO, Rafindadi AH, Shehu SM. Sublingual gastric duplication cyst causing respiratory obstruction: Case report. East Afr Med J 2007;77:394-5.  Back to cited text no. 5
    
6.
Onderoglu L, Saygan-Karamürsel B, Deren O, Bozdag G, Tekşam O, Tekinalp G, et al. Prenatal diagnosis of ranula at 21 weeks of gestation. Ultrasound Obstet Gynecol 2003;22:399-401.  Back to cited text no. 6
    
7.
Bonet Coloma C, Ata-Ali Mahmud J, Minguez Martinez I, Peñarrocha MA. Congenital oral mucoceles: Presentation of four new clinical cases. An Pediatr (Barc) 2011;75:424-5.  Back to cited text no. 7
    
8.
Joshi SR, Pendyala GS, Choudhari S, Kalburge J. Mucocele of the glands of blandin-nuhn in children: A clinical, histopathologic, and retrospective study. N Am J Med Sci 2012;4:379-83.  Back to cited text no. 8
    
9.
Kaneko T, Horie N, Shimoyama T. Congenital mucocele in the tongue: Report of a case. J Oral Maxillofac Surg 2012;70:2596-9.  Back to cited text no. 9
    
10.
Pires P, Pereira M, Machado L, Bonilla-Musoles F. Prenatal diagnosis of a ranula with 2- and 3-dimensional sonography and sonographically guided aspiration. J Ultrasound Med 2006;25:1499-502.  Back to cited text no. 10
    
11.
Langer JC, Fitzgerald PG, Desa D, Filly RA, Golbus MS, Adzick NS, et al. Cervical cystic hygroma in the fetus: Clinical spectrum and outcome. J Pediatr Surg 1990;25:58-61.  Back to cited text no. 11
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]


This article has been cited by
1 Diagnosis and Management of Congenital Floor of Mouth Masses: A Systematic Review
Jordyn P. Lucas,Meredith Allen,Bianca Siegel,Nathan Gonik
International Journal of Pediatric Otorhinolaryngology. 2020; : 110541
[Pubmed] | [DOI]



 

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