Congenital Bilateral Zygomatico-Maxillo-Mandibular Fusion Associated With Gum Fusion

Al-Mahdi, Akmam H.; Koppel, David; Al‐jumaily, Hassanien A.; Mohammed, Ali Abdul Hameed; Boyd, Deborah

doi:10.1097/scs.0000000000002293

Cited by 6 publications

(3 citation statements)

References 25 publications

(60 reference statements)

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“…Syngnathia is often accompanied by additional congenital defects, such as microglossia, micrognathia, and TMJ abnormalities. There are approximately 60 cases of congenital bony syngnathia reported in the literature[3], which reveal a high degree of variability in the location and extent of jaw fusion, and indicate that bony syngnathia may be isolated or syndromic. It is estimated that only 18% of cases are associated with known syndromes, such as aglossia-adactylia syndrome or hemifacial microsomia[4].…”

Section: Discussionmentioning

confidence: 99%

Application of computer-assisted navigation in treating congenital maxillomandibular syngnathia: A case report

Lin¹,

Bai²,

Wei³

2019

WJCC

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BACKGROUND Congenital maxillomandibular syngnathia is an extremely rare disorder characterized by craniofacial malformations and inability to open the mouth adequately, which leads to problems with feeding, swallowing, and breathing as well as temporomandibular joint ankylosis. The main goal of the surgery is to release the ankylosis, establish functioning mandible, and prevent re-fusion. However, surgical procedures for this disease are rarely reported. CASE SUMMARY Here, we report a 7-mo-old girl with bilateral maxillomandibular syngnathia. The patient presented with difficulty in feeding, breathing, sounding, and swallowing and had developmental dysplasia. For treatment, we performed bone isolation by computer-assisted navigation and used silicone to fix the wound surface to prevent refusion of bone. To our knowledge, this is the only syngnathia case in the literature treated using computer-assisted navigation. With the guidance of precise navigation, we were able to minimize operation time by at least one hour, the patient's blood vessels, nerves, and tooth germs were well protected, and excessive bleeding was avoided. After six weeks, the patient showed improvement in mouth opening and no major issues of feeding. CONCLUSION Application of computer-assisted navigation can significantly improve accuracy, effectiveness, and surgical safety in correcting congenital maxillomandibular syngnathia.

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Section: Discussionmentioning

confidence: 99%

Application of computer-assisted navigation in treating congenital maxillomandibular syngnathia: A case report

Lin¹,

Bai²,

Wei³

2019

WJCC

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“…It represents several types such as mandibulo-maxillary fusion, alveolar fusion and gum fusion [ 1 ]. Sixty-two cases of congenital bone syngnosis have been reported in the literature up to 2019 [ 2 ]. Sygnathia manifests itself from birth by a complete closure of the mouth leading to difficulties in feeding and swallowing.…”

Section: Introductionmentioning

confidence: 99%

Congenital bilateral zygomatico-maxillo-mandibular fusion associated with gumfusion: Case report

Raiteb

Elmrini

Hassani

et al. 2021

International Journal of Surgery Case Reports

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Introduction Maxillomandibular sygnathia is a rare and severe craniofacial deformity defined by gingival mucosal fusion (synechia) or bony fusion (synostosis). Case report We will present a case of complete closure of the mouth since birth from eastern Morocco treated in our department of maxillofacial surgery at the University Hospital of Casablanca. The patient was referred after two months by the pediatrician of the provincial hospital and following the placement of a nasogastric tube. On admission, she presented with facial dysmorphosis, signs of malnutrition with closure of the mouth and fusion of both gums with pro-alveoli and retromandibulia. CT scan with 3D reconstruction confirmed maxillomandibular synostosis. 5 days later, she underwent a fixed mucosal incision with osteotomy at the maxillomandibular joints with early and prolonged active physical therapy. The evolution was marked by the recurrence of mouth closure, the little girl was operated a second time and then she died by a mucous plug at the level of her tracheostomy cannula during resuscitation. Discussion Maxillomandibular sygnathia is a very rare pathology whose origin remains unknown. Very few cases published in the literature. Conclusion The therapeutic difficulties encountered outside the ideal age of the operation and the management of recurrences were also linked to socio-economic factors making it difficult to ensure adequate postoperative follow-up.

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“…It is commonly associated with cleft lip, cleft palate, cleft mandible, oblique facial cleft, mandibular hypoplasia, hemifacial microsomia, glossopalatine ankylosis, microglossia, low-set ears, and other ear-related deformities. 1 Although syngnathia may also be present in isolation, it can also manifest syndromes like oromandibular limb hypogenesis syndrome (OLHS), Nager syndrome, Horner syndrome, Popliteal pterygium syndrome, and Van der Woude syndrome 2 both unilateral and bilateral deformity can be observed in this disease. It varies in severity with single mucosal bands, or even complete bony fusion.…”

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confidence: 99%

Application of Computer-Assisted Preoperative Planning in Treating Congenital Bilateral Zygomatico-Maxillo-Mandibular Fusion: A Case Report and Literature Review

Chen

Jie

et al. 2022

The Cleft Palate Craniofacial Journal

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Congenital fusion of the jaws (syngnathia) is a rare facial malformation with an unknown etiology. This disease may vary in severity with adhesion of soft tissue and bony fusion. It can be anterior fusion, unilateral or bilateral fusion, and complete fusion. The main problem of these patients is the difficulty of airway maintenance and feeding, and the most common postoperative complication is the relapse of bony fusion. Here, we report a young male patient with bony syngnathia, involving bilateral fusion of the ascending ramus and body of the mandible with the maxillary complex. We performed bone isolation by computer-assisted preoperative planning and used an insertional temporalis flap to fix the wound surface to prevent refusion of bone.

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Congenital Bilateral Zygomatico-Maxillo-Mandibular Fusion Associated With Gum Fusion

Cited by 6 publications

References 25 publications

Application of computer-assisted navigation in treating congenital maxillomandibular syngnathia: A case report

Application of computer-assisted navigation in treating congenital maxillomandibular syngnathia: A case report

Congenital bilateral zygomatico-maxillo-mandibular fusion associated with gumfusion: Case report

Application of Computer-Assisted Preoperative Planning in Treating Congenital Bilateral Zygomatico-Maxillo-Mandibular Fusion: A Case Report and Literature Review

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