|Year : 2008 | Volume
| Issue : 3 | Page : 113-115
Syngnathia-congenital unilateral bony fusion of the maxilla and mandible
GV Ramachandra Reddy1, KS Ganapathy2, V Sanjay1, N Srinath3, Sunil Dutt Christopher3
1 Department of Oral Medicine and Radiology, M. R. Ambedkar Dental College, 1/36 Cline Road, Cooke Town, Bangalore-560 005, India
2 Department of Oral Medicine and Radiology, Oxford Dental College, Bangalore, India
3 Department of Oral and Maxillofacial Surgery, M. R. Ambedkar Dental College, 1/36 Cline Road, Cooke Town, Bangalore-560 005, India
|Date of Web Publication||16-Jun-2009|
G V Ramachandra Reddy
Department of Oral Medicine and Radiology, Maruti Dental College, Bangalore
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Congenital disorders involving orofacial region represents approximately 20% of all birth defects. Out of these disorders, congenital bony fusion of the maxilla and mandible (syngnathia) is rare. Usually syngnathia is associated with other anomalies and syndromes. This case report presents a unilateral fusion of maxilla and mandible with no other anomalies.
Keywords: Bony fusion, congenital syngnathia, maxilla and mandible and synnechiae
|How to cite this article:|
Ramachandra Reddy G V, Ganapathy K S, Sanjay V, Srinath N, Christopher SD. Syngnathia-congenital unilateral bony fusion of the maxilla and mandible. J Indian Acad Oral Med Radiol 2008;20:113-5
|How to cite this URL:|
Ramachandra Reddy G V, Ganapathy K S, Sanjay V, Srinath N, Christopher SD. Syngnathia-congenital unilateral bony fusion of the maxilla and mandible. J Indian Acad Oral Med Radiol [serial online] 2008 [cited 2022 Dec 1];20:113-5. Available from: http://www.jiaomr.in/text.asp?2008/20/3/113/52779
| Introduction|| |
Congenital bony fusion of the maxilla and mandible without any other anatomic oral anomalies is extremely uncommon. Syngnathia is a rare disorder and only 24 cases have been reported since 1936.  This condition usually appears in combination with cleft lip, cleft palate, aglossia, poplitial pterygium syndrome, Vander Woude syndrome, aglossia adactylia syndrome, hypoplasia of the proximal mandible, bifid tongue, tempromandibular fusion and other regional, systemic abnormalites and is also seen in autosomal recessive hypomandibular craniofacial dysostosis.  This report describes an unusual case of unilateral intermaxillary syngnathia with no other oral and maxillofacial anomalies.
| Case Report|| |
A two year old female patient was brought to the department of oral medicine and radiology at M. R. Ambedkar dental college and hospital, Bangalore with complaint of inability to open her mouth since birth. As a result, the child could not be fed with semisolid or solid food [Figure 1]. Examination revealed severe trismus due to fusion of the left maxillary and mandibular alveolar ridges, extending from 61, 71 region to molar region posteriorly and separation on the right side. All the deciduous teeth were present in the upper and lower right quadrants and 61, 62, 71, 72 on the left side [Figure 1] and [Figure 2].
The child was the product of normal vaginal delivery and the first child of a healthy and normal 26 year old mother and 31 year old father. No similar affliction could be elicited in the past generation of either of the parents. Systemic examination revealed no anomalies in the rest of the body, but her general condition and appearance was poor because of difficulty in oral feeding. Because of technical difficulties and non-cooperation, the radiographs were of poor quality. However, standard computed tomography scan was helpful in depicting the site and extent of the bony fusion which was extending from left incisor to the maxillary tuborosity and ramus of the mandible [Figure 3].
| Discussion|| |
Congenital fusion of the jaws is extremely rare and unusual malformation that may be unilateral or bilateral and may be either soft tissue fusion (synnechiae) or bony fusion (syngnathia). , Patients with congenital fusion of jaws may present with other malformations. It is commonly associated with underdevelopment of facial structures on the affected side, micrognathia and ramus hypoplasia. Various anomalies of the facial soft tissues and palate which include colobomas of the eyelids, low set ears and other ear deformities, hypertelorism and cleft palate have also been described. Syngnathia could also occur with popliteal pterygium syndrome, Vander Woude syndrome, aglossia adactylia syndrome. ,,,
Bony syngnathia is less common than fibrous syngnathia, Burkett described a case of congenital unilateral bony alveolar fusion with left facial hemiatrophy.  Peterson and Hogging reported cases of bony fusion of the jaws with congenital aglossia and cleft palate. These congenital syngnathia rarely occur as isolated event and are often associated with various other anomalies.  The significant points about this case is that it was an isolated case of unilateral bony fusion without any other anomalies and only few such cases have been reported in the literature.
The cause of synechia or syngnathia is still unknown. However, many explanations for these rare malformations have been proposed by several authors. Snijman and Prinshoo believe that the condition is caused by an abnormal process occurring in late stages of intrauterine development.  Various other experimental studies performed on the embryological basis include, amniotic constriction bands in the region of the developing branchial arches, environmental insults, drugs such as meclozine and large doses of vitamin A.  Humprey has suggested that depressed fetal swallowing reflexes may delay palatal shelf elevation and this prolongs palatopharyngeal and palatoglossal epithelial contact leading to adhesion and cleft.  But the present case had no cleft and the fusion was unilateral.
Laster et al . have proposed the following classification for bony syngnathia of the maxillofacial region. 
Type 1a: Simple anterior syngnathia characterized by bony fusion of the alveolar ridges 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 zygomatico - mandibular syngnathia characterized by bony fusion of the mandible to the zygomatic complex causing only mandibular micrognathia.
Type 2b: Complex zygomatico - mandibular syngnathia. Characterized by bony fusion of the mandible to the zygomatic complex and associated with clefts or tempromandibular joint ankylosis.
Surgical separation of the bony fusion, under general anesthesia (blind intubation or via tracheostomy) is the treatment for the simple syngnathia in isolated occurrence or cases with the presence of other anatomic abnormalities. If the fusion is soft tissue only and not associated with other malformations, the synechiae should be divided early and the infant commenced on normal feeding. Jaw exercises should be commenced and normal function expected in these patients. ,
| Conclusion|| |
Unilateral bony syngnathia of maxilla and mandible is a rare occurrence. Such a rare case has been presented here in a two year old female patient with no other anomalies. The cause for this defect is still obscure. Some defect which has occurred during developmental stages in which separation of maxilla and mandible failed to occur. Surgical separation of the fusion is necessary to allow normal feeding and for normal mandibular movements and the growth.
| References|| |
|1.||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. [PUBMED] [FULLTEXT]|
|2.||Yazdi I, Fakhraee AH. Congenital fusion maxilla and mandible (bony syngnathia): A case report. Br J Plast Surg 2000;3:3. |
|3.||Kenneth H, Myall D, Myall RW. Congenital bony syngnathia: A proposed classification. Cleft Palate Craniofac J 1997;34:241-6. |
|4.||Gorlin RJ, Pindborg JJ. Text book of Syndromes of head and neck. New York, Toronto, London: McGraw-Hill Book Company; p. 96-102. |
|5.||Denion E, Capon N, Martinot V, Pellerin P. Neonatal permanent jaw constriction because of oral synechiae and Pierre Robbin sequence in a child with van der woude syndrome. Cleft Palate Craniofac J 2002;39:115-9. [PUBMED] [FULLTEXT]|
|6.||Kamata S, Satoh K, Uemura T, Onizuka T. Congenital bilateral zygomatico-mandibular fusion with mandibular hypoplasia. Br J Plast Surg 1996;49:251-3. [PUBMED] |
|7.||Behnia H, Shamse MG. Congenital unilateral fusion of the mandibular and maxillary alveolar ridges, tempromandibular joint and coronoid process: A case report. J Oral Maxillofac Surg 1996;54:773-6. [PUBMED] [FULLTEXT]|
|8.||Chadha R, Bagga D, Mohta A, Malhotra CJ, Dhar A. Congenital fusion of the gums. Oral Surg Oral Med Oral Pathol 1992;74:30-2. [PUBMED] |
[Figure 1], [Figure 2], [Figure 3]