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 Table of Contents  
CASE REPORT
Year : 2017  |  Volume : 6  |  Issue : 1  |  Page : 8-10

Congenital lateral synechia with sacrococcygeal teratoma


Department of Pediatric Surgery, SMS Medical College, Jaipur, Rajasthan, India

Date of Web Publication18-Aug-2017

Correspondence Address:
Aditya Pratap Singh
Near The Mali Hostel, Main Bali Road, Falna, Pali - 306 116, Rajasthan
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijhi.ijhi_4_16

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  Abstract 


The presence of oral synechia along with sacrococcygeal teratoma (SCT) is a first case in the literature reported to date. We encountered a case of SCT accompanied by congenital lateral oral synechia without cleft palate. It was asymptomatic and accidental finding. It was resolved spontaneously. It may be considered Type 6 as isolated lateral synechia with extending from hard palate to the lateral part of the tongue.

Keywords: Isolated, oral synechia, sacrococcygeal teratoma


How to cite this article:
Singh AP, Mathur V, Tanger R, Gupta AK. Congenital lateral synechia with sacrococcygeal teratoma. Int J Histopathol Interpret 2017;6:8-10

How to cite this URL:
Singh AP, Mathur V, Tanger R, Gupta AK. Congenital lateral synechia with sacrococcygeal teratoma. Int J Histopathol Interpret [serial online] 2017 [cited 2017 Nov 23];6:8-10. Available from: http://www.ijhi.org/text.asp?2017/6/1/8/213270




  Introduction Top


The presence of congenital intra-oral epithelial bands is a very rare condition. Congenital intraoral bands may consist of epithelium supported by varying amounts of connective tissue. If only soft tissues are involved, the condition is termed as synechiae and the term syngnathia is used when bone is involved.[1] Congenital interalveolar synechia frequently shows syndromic associations despite the occurrence of isolated instances. In a report by Tanrikulu et al., the patient was a 10-month-old female infant with isolated congenital alveolar synechia without any other anomalies.[2] We present here a case of sacrococcygeal teratoma (SCT) with lateral oral synechia and review of literature.


  Case Report Top


The case involved a 2-day-old female neonate at the time of presentation. He was the first child of healthy parents. The infant was delivered normally at full-term with a birth weight of 2.7 kg. There were no particularly notable points in the family history, medical history or reproductive history. At the time of presentation, the parent came with the complaint of a swelling measuring approximately 2 cm × 2 cm in the right gluteal region. All routine blood investigations were within normal limits including complete blood counts, renal function test and serum electrolytes. Serum alpha-fetoprotein level was >2000 ng/ml. X-ray abdomen showed soft tissue mass in the pelvis [Figure 1]. Ultrasonography (USG) showed hydroureteronephrosis with Type 3 sacrococcygeal teratoma (SCT) (3328 mm) and vagina fluid filled, rectum pushed anteriorly. Contrast-enhanced computerised tomography showed the same finding as USG findings with heterogeneous enhancing solid cystic mass with few foci of internal calcification [Figure 2]. There were no other abnormalities found in the patient. No obvious auricular or nasal deformities were appreciated. Digits and extremities appeared normal as did genitalia. Examination of the heart revealed no abnormalities.
Figure 1: X-ray showed soft tissue mass in the pelvis with deviated sacrum

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Figure 2: Contrast-enhanced computerised tomography showed hydroureteronephrosis with mass in pelvis

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Baby planned for exploration, complete excision of the mass done with coccygectomy [Figure 3]. On routine oral examination, there was a band extending from the hard palate to the lateral part of dorsum of the tongue. The membranous adhesion was pale pink in colour and appeared to be avascular [Figure 4]. The baby did not have any feeding problem. On the 5th live day, it was resolved spontaneously [Figure 5]. Biopsy showed mature teratoma.
Figure 3: Post-operative surgical image

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Figure 4: Oral synechia

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Figure 5: Spontaneously resolved synechia

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  Discussion Top


The first report of oral synechia was by Illera in 1887,[3] Over the years, almost sixty cases of oral synechia have been reported, of which 52 are lateral synechia and eight were of the median variety.[4] In our case, it was lateral variety.

Oral synechia can be of various degrees adhering at various locations. These can be classified into five types:[4] synechia by cord-like adhesion of the alveolar mucosa on one or both sides of the upper and lower jaw (alveolar synechia); synechia by a membranous adhesion on the hard palate and floor of the mouth, excluding the rear of the tongue (lateral synechia); synechia in which the hard palate and tongue are partially involved; synechia in which the soft palate and tongue are widely involved, such that continuity is interrupted between the oral cavity and the pharynx; synechia by a membranous adhesion between the hard palate and lower lip. The membranous band was present between hard palate and lateral part of the tongue which was a rare presentation of oral synechia in our case and may be considered as Type 6. We named it as adi synechia. Fuhrmann et al.[5] reported that five family members had cleft palates and synechia, one having a cleft palate without synechia and one transmitted the gene but did not express it. It was isolated case of oral synechia without cleft palate in our case which is very rare. It was associated with the SCT Type 3. It is the first reported case of oral synechia associated with SCT.

The aetiology of intra-oral bands or synechia of epithelial tissue has been debated, but many theories have been proposed. During the 7th–8th week of embryological development, the alveolar ridges, tongue and palatal shelves are in contact with each other. The ensuing palatal closure depends on downward contraction of the tongue. When the tongue protrudes from the mouth as a result of medial movements of the oral cavity walls, it prevents the alveolar ridges from fusing. Genetic, teratogenic or mechanical insults during this critical stage may lead to periods of close, quiescent contact between oral structures, and this predisposes to abnormal fusion.[6]

Longacre asserts that oral synechia is due to the persistence of the buccopharyngeal membrane and is for that reason associated with micrognathia and cleft palate.[7] In our case, no such associations were seen. Kruger speculates that the mechanical effect of the tongue may contribute to cases, in which the periphery of the cleft palate adheres to mucous membranes on the floor of the mouth, and that adhesion in cases of cleft palate may occur as a result of obstruction by the tongue.[8] According to Mathis, when adhesion of the palatal shelf occurs during developmental stages, adhering epithelial rudiments, for some reasons, leads to synechia.[9]

The general consensus on the treatment of cleft palate lateral synechia syndrome is excision of the synechia and palatal closure. Dalal and Davison [10] have reported a case of intra-alveolar synechia in two siblings, one of whom had a spontaneous resolution of the adhesion. We believe that such a use of the synechia has been possible because the membranous adhesion, in this case, had fibromuscular bands in it unlike our situation where the synechia consisted of thin membranous tissue of unequal width. In our case, it was resolved spontaneously.

Obtaining a safe airway for the release of the bands may be difficult. The conventional orotracheal intubation or the use of a laryngeal mask may not be possible due to the presence of the synechia. Fibre-optic nasotracheal intubation may not be feasible due to technical difficulties of finding a bronchoscope small enough for a 1-week-old infant. Performing a procedure on the highly vascular floor of the mouth under local anaesthesia in an infant would involve the risk of bleeding complications and aspiration. In cases where immediate excision of the synechia is deemed necessary either due to breathing or feeding problems, sedation can be used under constant anaesthetic monitoring for the surgical procedure. In our case, it was asymptomatic and accidental finding on oral examination.

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.

Acknowledgement

We would like to acknowledge Dr. Annu Bhandari, MD Radiology, Professor, SMS Medical College, Jaipur, Rajasthan, India.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Gartlan MG, Davies J, Smith RJ. Congenital oral synechiae. Ann Otol Rhinol Laryngol 1993;102(3 Pt 1):186-97.  Back to cited text no. 1
    
2.
Tanrikulu R, Erol B, Görgün B, Ilhan O. Congenital alveolar synechiae – A case report. Br Dent J 2005;198:81-2.  Back to cited text no. 2
    
3.
Illera MD. Congenital occlusion of the pharynx. Lancet 1887;1:742.  Back to cited text no. 3
    
4.
Ogino A, Onish K, Maruyama Y. Congenital oral synechia associated with cleft palate: Cleft palate medial synechia syndrome? Eur J Plast Surg 2005;27:338-40.  Back to cited text no. 4
    
5.
Fuhrmann W, Koch F, Schweckendiek W. Autosomal dominant inheritance of cleft palate and synechias between the palate and floor of the mouth or tongue. Humangenetik 1972;14:196-203.  Back to cited text no. 5
    
6.
Haydar SG, Tercan A, Uckan S, Gurakan B. Congenital gum synechiae as an isolated anomaly: A case report. J Clin Pediatr Dent 2003;28:81-3.  Back to cited text no. 6
    
7.
Longacre JJ. Congenital atresia of the oropharynx. Plast Reconstr Surg 1951;8:341-8.  Back to cited text no. 7
    
8.
Kruger GO. Textbook of Oral Surgery. 6th ed. Jaypee Brothers, Mosby; 1990. p. 462.  Back to cited text no. 8
    
9.
Mathis H. Case report of patient with difficulty in eating due to congenital syngnathia. Ger Dent J 1962;16:1167-71.  Back to cited text no. 9
    
10.
Dalal M, Davison PM. Cleft palate congenital alveolar synechiae syndrome: Case reports and review. Br J Plast Surg 2002;55:256-7.  Back to cited text no. 10
    


    Figures

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



 

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