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 Table of Contents  
CASE REPORT
Year : 2019  |  Volume : 14  |  Issue : 4  |  Page : 401-404

Surgical technique in the management of congenital unilateral syngnathia: A case report of a 4-year-old female


Department of Oral and Maxillofacial Surgery, Sharad Pawar Dental College and Hospital, Datta Meghe Institute of Medical Sciences, Wardha, Maharashtra, India

Date of Submission10-Oct-2019
Date of Decision30-Oct-2019
Date of Acceptance30-Nov-2019
Date of Web Publication16-Jul-2020

Correspondence Address:
Dr. Rooshay Trivedi
Department of Oral and Maxillofacial Surgery, SPDC, Wardha, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jdmimsu.jdmimsu_152_19

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  Abstract 


Congenital disorders involving orofacial region represent approximately 20% of all birth defects. Syngnathia is an extremely rare condition involving congenital fusion of the maxilla with the mandible. Usually, syngnathia is associated with other anomalies and syndromes. This case report presents a novel surgical technique in the management of unilateral fusion of maxilla and mandible.

Keywords: Congenital unilateral maxillomandibular fusion, craniofacial abnormalities, syngnathia, synostosis


How to cite this article:
Trivedi R, Bhola N, Jadhav A, Gaikwad P, Patil C, Sharma A. Surgical technique in the management of congenital unilateral syngnathia: A case report of a 4-year-old female. J Datta Meghe Inst Med Sci Univ 2019;14:401-4

How to cite this URL:
Trivedi R, Bhola N, Jadhav A, Gaikwad P, Patil C, Sharma A. Surgical technique in the management of congenital unilateral syngnathia: A case report of a 4-year-old female. J Datta Meghe Inst Med Sci Univ [serial online] 2019 [cited 2020 Aug 11];14:401-4. Available from: http://www.journaldmims.com/text.asp?2019/14/4/401/289836




  Introduction Top


Maxillomandibular fusion (syngnathia) is an uncommon anomaly, which was first reported by Burket.[1] The latest case was reported by Hegab et al.[2] Since then, only about 34 cases have been reported in literature. Congenital disorders of the maxillofacial region represent approximately 20% of all natal disorders. They may clinically manifest based on severity from adhesion-only mucosal (synechiae) to severe bony fusion (synostosis). The condition restricts sufficient opening of mouth, leading to difficulty in not only feeding and swallowing but also breathing. Syngnathia may or may not be in combination with other facial abnormalities such as cleft lip and palate, hypoplasia of the proximal mandible, temporomandibular fusion, and autosomal recessive hypomandibular craniofacial dysostosis. In addition, it has a strong syndromic association like popliteal pterygium syndrome, Van der Woude syndrome, and aglossia-adactylia syndrome. Early diagnosis with prompt treatment is very important for normal growth and development in such patients. However, no standard operative protocol or sufficient methodology has been advocated in literature.


  Case Report Top


A 4-year-old female was referred to our hospital, complaining of nil mouth opening since birth. The child was the second child of parents with two generations of consanguineous marriage at maternal as well as paternal sides. Her elder sister had the same complaint since birth. On clinical examination, there was unilateral fusion between the mandible and the maxilla over the right side with a deficient growth over the right side of the face [Figure 1] and [Figure 2]. Computed tomography scans showed complete unilateral bone fusion between the mandibular horizontal and ascending rami with the maxilla [Figure 3]. Temporomandibular joints (TMJs) showed normal morphological features bilaterally [Figure 4]. The parents reported no previous surgical intervention. The patient was submitted to general anesthesia using fiberoptic intubation. Full-thickness incisions were placed along the occlusal plane over the right side [Figure 5]. The maxilla and mandible were thus separated [Figure 6], yielding mobility along both the TMJs bilaterally. An active mouth opening of 45 mm was achieved intraoperatively [Figure 7]. A rubber-based material was used for impression of the maxillary arch intraoperatively for the fabrication of postoperative occlusal splint to avoid further fusion of the separated segments [Figure 8].
Figure 1: Preoperative frontal view

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Figure 2: Preoperative worm's view

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Figure 3: Preoperative three-dimensional reconstruction of computed tomography scan

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Figure 4: Preoperative computed tomography scan showing normal anatomy of both the condyles

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Figure 5: Exposure of bony fusion site

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Figure 6: Osteotomy cut placed to separate the maxilla and mandible

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Figure 7: Intraoperative mouth opening achieved

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Figure 8: Intraoperative impression for occlusal splint

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Postoperative physiotherapy was indicated to maintain mouth opening, to be performed three times daily for 3 min each. The patient was discharged after 2 weeks. The patient was thus kept on sequential follow-ups, and passive mouth opening after 6-month postoperative period was 25 mm [Figure 9].
Figure 9: Mouth opening on 6-month follow-up

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  Discussion Top


Syngnathia is an extremely rare condition and usually refers to bony fusion of the jaws. Moreover, it can also imply soft-tissue fusion between the gums, referred to as synechiae.[2] Most of the 26 cases reported in literature were incomplete.[3],[4] Early treatment is very important to avoid respiratory and feeding difficulties and prevent future problems such as ankylosis of the TMJ. Different etiological factors have been suggested, such as abnormality of the stapedial artery in the fetus, early loss of neural crestal cells, persistence of the buccopharyngeal membrane, amniotic constriction bands in the region of the developing first branchial arch, teratogenic agents, or trauma.[2],[3],[5] Consanguinity, as in this patient, has also been described by other authors.[2] Two cases of acquired syngnathia have been reported as a result of a fibrotic tubed pedicle and from myositis ossificans.[6] Syngnathia can be associated with Van der Woude's syndrome,[7] popliteal pterygium syndrome, and aglossia-adactylia syndrome.[4]

According to Subramanian et al.,[8] anomalies associated with syngnathia can be cleft palate, mandibular hypoplasia, absent or abnormal tongue morphology, hemifacial microsomia, congenital amputation of arms and legs, glossopalatine ankylosis, Horner's syndrome, cleft mandible, coloboma, scoliosis, and oblique facial clefts. Inman et al.[9],[10],[11],[12],[13],[14],[15],[16],[17] reported that Foxc1 null embryos display bony syngnathia together with defects in the maxillary and mandibular structures and agenesis of the TMJ.

Laster et al.[5] proposed a modified classification for bony syngnathia: Type 1a: simple anterior syngnathia, characterized by bony fusion of the alveolar ridge only and without other congenital deformity in the head and neck; Type 1b: complex anterior syngnathia, characterized by bony fusion of the alveolar ridges only and associated with other congenital deformity in the head and neck; Type 2a: simple zygomaticomandibular syngnathia, characterized by bony fusion of the mandible to the zygomatic complex causing only mandibular micrognathia; and Type 2b: complex zygomaticomandibular syngnathia, characterized by bony fusion of the mandible to the zygomatic complex and associated with clefts or TMJ ankylosis. The patient presented here had maxillomandibular syngnathia, belonging to the Type 1b category.

Surgical treatment is a challenge as anesthesiologic management is difficult.[2],[3] We followed Hegab's treatment protocol:[2] Three-dimensional surgical planning, nasotracheal intubation using fiberoptic endoscope with a tracheostomy set on standby, and long-term postoperative physiotherapy. When treatment for this fusion is not undertaken early or in long-standing cases, growth anomalies from TMJ ankylosis may occur. Excessive force may easily lead to jaw fracture and should be avoided because TMJs are stiffened due to disuse, the alveolar ridges are soft, and infantile bone is fragile.

The management of congenital fusion when associated with multiple malformations needs a team approach. In late cases, the baby may need a tracheostomy to manage respiratory obstruction. Early surgical division of the occlusion is recommended for normal feeding and avoidance of upper airway obstruction and to allow for normal mandibular function and growth. Early intervention also reduces the possibilities of TMJ ankylosis.


  Conclusion Top


We present the case of a congenital maxillomandibular syngnathia, with mild hemifacial microsomia, and a disordered dental eruption. Separation of the fused fragment by horizontal osteotomy cuts and aggressive long-term physiotherapy for 3 months led to a stable increase in mouth opening from 0 to 25 mm interincisor distance. The patient reported an improvement in speech, was able to eat without restriction regarding food consistency, and could maintain a good oral hygiene.

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.
Burket LW. Congenital bony temporomandibular ankylosis and facial hemiatrophy: Review of the literature and report of a case. J Am Med Assoc 1936;106:1719-22.  Back to cited text no. 1
    
2.
Hegab A, ElMadawy A, Shawkat WM. Congenital maxillomandibular fusion: A report of three cases. Int J Oral Maxillofac Surg 2012;41:1248-52.  Back to cited text no. 2
    
3.
Fallahi HR, Naeini M, Mahmoudi M, Javaherforoosh F. Congenital zygomatico-maxillo-mandibular fusion: A brief case report and review of literature. Int J Oral Maxillofac Surg 2010;39:930-3.  Back to cited text no. 3
    
4.
Parkins GE, Boamah MO. Congenital maxillomandibular syngnathia: Case report. J Craniomaxillofac Surg 2009;37:276-8.  Back to cited text no. 4
    
5.
Laster Z, Temkin D, Zarfin Y, Kushnir A. Complete bony fusion of the mandible to the zygomatic complex and maxillary tuberosity: Case report and review. Int J Oral Maxillofac Surg 2001;30:75-9.  Back to cited text no. 5
    
6.
Choi JY, Min CG, Myoung H, Hwang SJ, Kim MJ, Lee JH. Acquired syngnathia. Br J Oral Maxillofac Surg 2004;42:448-50.  Back to cited text no. 6
    
7.
El Madi A, Khattala K, Rami M, Bouabdallah Y. Bilateral maxillomandibular syngnathia in a newborn. J Neonatal Surg 2014;3:53.  Back to cited text no. 7
    
8.
Subramanian B, Agrawal K, Panda K. Congenital fusion of the jaws: A management protocol. Int J Oral Maxillofac Surg 2010;39:925-9.  Back to cited text no. 8
    
9.
Inman KE, Purcell P, Kume T, Trainor PA. Interaction between foxc1 and Fgf8 during mammalian jaw patterning and in the pathogenesis of syngnathia. PLoS Genet 2013;9:e1003949.  Back to cited text no. 9
    
10.
Kashikar SV. Congenital Unilateral Infiltrating Facial Lipomatosis. West Indian Med J 2017;66:189-90. Available from: https://doi.org/10.7727/wimj.2014.250. [Last accessed on 2019 Sep 29].  Back to cited text no. 10
    
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Kasatwar A, Borle R, Bhola N, Rajanikanth K, Prasad GSV, Jadhav A. Prevalence of Congenital Cardiac Anomalies in Patients with Cleft Lip and Palate – Its Implications in Surgical Management. J Oral Biol Craniofac Res 2018:8:241-44. Available from: https://doi.org/10.1016/j.jobcr.2017.09.009. [Last accessed on 2019 Sep 29].  Back to cited text no. 11
    
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Borle RM, Jadhav A, Bhola N, Hingnikar P, Gaikwad P. Borle's Triangle: A Reliable Anatomical Landmark for Ease of Identification of Facial Nerve Trunk during Parotidectomy. J Oral Biol Craniofac Res 2019;9:33-6. Available from: https://doi.org/10.1016/j.jobcr.2018.08.004. [Last accessed on 2019 Sep 29].  Back to cited text no. 12
    
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Patil C, Jadhav A, RK, Bhola N, Borle RM, Mishra A. Piezosurgery vs Bur in Impacted Mandibular Third Molar Surgery: Evaluation of Postoperative Sequelae. J Oral Biol Craniofac Res 2019;9:259-62. Available from: https://doi.org/10.1016/j.jobcr.2019.06.007. [Last accessed on 2019 Sep 29].  Back to cited text no. 13
    
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Sharma P, Gawande M, Chaudhary M, Ranka R. T-Cell Lymphoma of Oral Cavity: A Rare Entity. J Oral Maxillofac Pathol 2018;22:104–7. Available from: https://doi.org/10.4103/jomfp.JOMFP_153_16. [Last accessed on 2019 Sep 29].  Back to cited text no. 14
    
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Kapse S, Surana S, Shastri AK. Transpalatal Wiring for the Management of Sagittal Fracture of the Maxilla/Palate. Int J Oral Maxillofac Surg 2017;46:1059-60. Available from: https://doi.org/10.1016/j.ijom.2017.02.1271. [Last accessed on 2019 Sep 29].  Back to cited text no. 15
    
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Oswal S, Borle R, Bhola N, Jadhav A, Surana S, Oswal R. Surgical Staples: A Superior Alternative to Sutures for Skin Closure After Neck Dissection—A Single-Blinded Prospective Randomized Clinical Study. J Oral Maxillofac Surg 2017;75:2707.e1-2707.e6. Available from: https://doi.org/10.1016/j.joms.2017.08.004. [Last accessed on 2019 Sep 29].  Back to cited text no. 16
    
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Jain A. Accidental Displacement of MandibularFirst Molar Root into Buccal Space: A Unique Case. J Stomatol Oral Maxillofac Surg 2018;119:429-31. Available from: https://doi.org/10.1016/j.jormas.2018.04.004. [Last accessed on 2019 Sep 29].  Back to cited text no. 17
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9]



 

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