J Korean Assoc Oral Maxillofac Surg.  2012 Jun;38(3):171-176. 10.5125/jkaoms.2012.38.3.171.

Congenital syngnathia: a case report

• Kim CH
• Kim MY

• KMID: 2043847
• DOI: http://doi.org/10.5125/jkaoms.2012.38.3.171

Abstract

Congenital syngnathia refers to the fusion of bony tissues, a rare disorder with only 41 cases reported in the international literature from 1936 to 2009. The occurrence of syngnathia without any other associated systemic disease or congenital anomaly is extremely rare. This report presents a case of congenital syngnathia with unilateral maxillomandibular bony adhesion without any other oral or maxillofacial anomaly. No recommended protocol for surgery exists due to the rarity of the disorder. There is a very low survival rate for the few patients who have forgone surgical management. This case describes a 74-year-old female patient who was suffering from limitation of mouth opening and was subsequently diagnosed with congenital syngnathia. The surgical staff performed separation surgery and reconstructed the malformed oral vestibule and cheek using the radial forearm free flap operation.

Keyword

Jaw abnormalities; Synostosis; Congenital

MeSH Terms

Aged
Cheek
Female
Forearm
Free Tissue Flaps
Humans
Jaw Abnormalities
Mouth
Stress, Psychological
Survival Rate
Synostosis

Figure

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  Fig. 1 74-year-old woman with facial asymmetry due to left mandibular hypoplasia resulting in the fusion of the left maxillo-mandibular alveolar ridge.

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  Fig. 2 Adhesion of the left commissural and retrocommissural oral cheek to the fused arches on the left side, which continued posteriorly as complete adhesion of the buccal mucosa to the slopes of the upper and lower arches, resulting in the absence of the buccal vestibule.

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  Fig. 3 Panoramic view showing left mandibular ramus hypoplasia resulting in the unilateral bony fusion of the left maxillary-mandibular alveolar ridges.

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  Fig. 4 A computerized 3 dimension reconstruction of the skull showed the bony fusion of the alveolar crest of the maxilla and mandible.

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  Fig. 5 Computerized 3 dimension reconstruction of the skull. A. There was neither temporomandibular joint (TMJ) ankylosis nor coronoid process hyperplasia in the right skull. B. There was no TMJ ankylosis, but coronoid process hyperplasia was noted on the left side.

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  Fig. 6 The bone scan (99mTc-MDP) revealed a hot spot in the left maxillo-mandibular bony fusion area.

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  Fig. 7 Surgical incision. The incision line was lip-split and extended to apron flap incision.

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  Fig. 8 The maxillo-mandibular fusion area is separated by a saw, and left coronoidectomy was done to favor mouth opening training.

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  Fig. 9 Radial forearm free flap was inserted for the reconstruction of the buccal cheek mucosa.

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  Fig. 10 Debulking procedure for the reduction of volume of excessive radial forearm free flap during the second operation. Mouth opening of 45 mm was achieved.

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