A full-term male baby weighing 2.8 kg was delivered by spontaneous vaginal delivery to an unbooked primigravida mother at a peripheral center. The child presented to us with severe respiratory distress with a heart rate of 180/min, a respiratory rate of 60/min along with inspiratory stridor and subcostal retraction. The air entry was diminished but equal on both the sides. The oxygen saturation on high flow oxygen, via high flow nasal cannula, at the rate of 10L/min, was 90-95%. On oral examination, there was a membrane extending from the floor of the mouth to the junction of the soft and hard palates with its lateral extension up to the molar trigone bilaterally. Nasal suction was performed to clear the secretions and to rule out associated choanal atresia.

The child deteriorated rapidly and progressed to respiratory failure. The respiratory rate fell to 20-30/min and saturation dropped to 75-80% on high flow oxygen. A diagnosis of the sub-glossopalatal membrane with severe respiratory distress and respiratory failure due to upper airway obstruction was made. The bag and mask ventilation was started. The distended stomach was decompressed with a infant feeding tube, though there was difficulty in negotiating it through the nasopharynx.

As the bag and mask ventilation was ineffective, an emergency excision of the membrane was performed. On excision of the membrane, a mass was seen to be arising from the dorsum of the tongue. The lesion was nearly filling the oropharynx and the nasopharynx (Fig. 1). The tongue was gently pulled out of the mouth, and the child was intubated to secure the airway. The child was shifted on the ventilator. After 24 hours, the baby improved and was extubated after which he was nursed in prone position.

Fig. 1 The mass arising from the dorsum of tongue with palatal cleft.

A computed tomography scan showed a cystic mass arising from the dorsum of the tongue extending into oropharynx and nasopharynx, and associated with cleft palate. Echocardiography and abdominal ultrasound were normal. An elective excision of the mass was planned. Per-operative, the mass was tense, cystic and lobulated with extension into the substance of tongue. The upper portion of the mass was filling the space of palatal cleft while the lower and posterior portions were occupying the nasopharynx and oropharynx. Primary closure of dorsum of the tongue was achieved after the complete excision of the mass. The postoperative course was uneventful. The child is awaiting cleft palate repair. Histopathology of the mass showed features suggestive of a dermoid cyst.

Congenital anomalies of the oral cavity are rare. As these result from abnormal development at a very early stage, they can be associated with multiple complications. Further, it is rare to find multiple congenital lesions affecting the oral cavity in one patient. A congenital lesion in the oral cavity may cause upper airway obstruction and lead to respiratory distress at the time of birth [2]. Similarly, the sub-glossopalatal membrane can compromise the upper airway patency and present with respiratory distress [3]. Both lesions presenting simultaneously in one patient is extremely unusual.

In this case, the child had respiratory distress due to upper airway obstruction caused by congenital anomalies. Upper airway in neonates may be blocked due to anomalies like choanal atresia, tumors such as glioma, encephalocele, teratoma or dermoid, vocal cord paralysis, and subglottic stenosis [1,5]. Many of these cases may require emergency surgical intervention for restoration of a secure airway [1]. Subglossopalatal membrane is a remnant of the buccopharyngeal membrane [3]. As neonates are obligate nasal breathers, the subglosso-palatal membrane may not cause respiratory distress in all cases [5]. This created a diagnostic dilemma in our case, and we looked for associated choanal atresia, as this association has previously been reported [6].

Congenital tongue mass can cause upper airway obstruction and child may have respiratory distress at birth [2]. The development of tongue is one of the earliest events in fetal life (4th–5th week) and can affect the development of palate and other maxillofacial structures [7]. Thus, a mass on the dorsum of the tongue can protrude into the defect between the palatal shelves (palate develops between 6th and 7th week) and prevent their fusion, and therefore, can cause palatal cleft [8]. Further, as the musculature of the tongue is formed there can be fusion, entrapment, and proliferation of epithelial debris, which can lead to the development of lingual dermoid cyst [9]. This can explain the infiltration of dermoid into the substance of tongue in our case [10]. The development of tongue and glossopharyngeal membrane and other maxillofacial structure overlap closely with each other. Thus, one anomaly may lead to the development of others.

Through this case, we documenting rare co-existence of subglosso-palatal membrane, cleft palate and dorsal tongue dermoid. Subglosso-palatal membrane excision should be performed in an emergency if the child is not improving on high flow oxygen because some other associated anomaly can be missed due to blocked view.

Contributors: All authors contributed to case management and manuscript.

Funding: None; Competing interest: None stated.

1. Grossfeld JL, Ballantine TVN. Surgical respiratory distress in infancy and childhood. Curr Probl Pediatr. 1976;6:1-64.

2. Celik M, Akkaya H, Arda IS, Hiçsönmez A. Congenital teratoma of the tongue: a case report and review of the literature. J Pediatr Surg. 2006;41:e25-8.

3. Nakajima T, Takahashi MTS. Subglosso-palatal membrane. Plast Reconstr Surg. 1979;63:574-6.

4. Kumar A, Bhatnagar V. Respiratory distress in neonates. Indian J Pediatr. 2005;72:425-8.

5. Coates H. Nasal obstruction in the neonate and infant. Clin Pediatr. 1992;31:25-9.

6. Pandey V, Tiwari P, Tapadar J, Gangopadhyay A. Subglosso-palatal membrane a rare cause of neonatal respiratory distress: A case report. J Indian Assoc Pediatr Surg. 2014;19:109-11.

7. Hong SJ, Cha BG, Kim YS, Lee SK, Chi JG. Tongue growth during prenatal development in Korean fetuses and embryos. J Pathol Transl Med. 2015;49:497-10.

8. Poretti A, Vitiello G, Hennekam RCM, Arrigoni F, Bertini E, Borgatti R, et al. Delineation and diagnostic criteria of oral-facial-digital syndrome type VI. Orphanet J Rare Dis. 2012;7:4.

9. Raewyn C, Paul W. Management of congenital lingual dermoid cysts. Int J Pediatr Otorhinolaryngol. 2010;74: 567-71.

10. Turki IM, Mouaffak-Zidi Y, Rajhi H, Triki H, Naija S, Ben JS, et al. A case of large dermoid cyst of the tongue. Egypt J Ear Nose Throat Allied Sci. 2011;12:171-4.