Literature DB >> 26023524

Bilateral maxillo-mandibular syngnathia in a newborn.

A El Madi1, K Khattala1, M Rami1, Y Bouabdallah1.   

Abstract

Entities:  

Year:  2014        PMID: 26023524      PMCID: PMC4420337     

Source DB:  PubMed          Journal:  J Neonatal Surg        ISSN: 2226-0439


× No keyword cloud information.
A 5-day-old newborn, product of non-consanguineous marriage, presented with inability to completely open oral cavity and feeding difficulty. On examination, there was a good sucking reflex and limited mouth opening (Fig. A) with retrognathia (Fig. B). Maxillary CT with 3D reconstruction revealed hypoplasia of the ascending branches of the mandible; the temporomandibular joints were normal (Fig. C, D) with bilateral Maxillomandibular Syngnathia (Fig. E, F). Figure 1 : Clinical and radiological demonstration. Congenital fusion of the maxilla and mandible is a rare anomaly which is usually diagnosed at birth owing to inability to open mouth and feed. Congenital synostosis of the mandible and maxilla is even less common than synechiae, with only 25 cases reported in the literature [1]. Congenital maxillomandibular fusion may be associated with other anomalies such as Van der Woude syndrome, or aglossia-adactylia syndrome [2]. Dawson et al [3] divided bony syngnathia into two broad categories: Type 1 - simple syngnathia without any associated congenital anomalies of head and neck and Type 2 - complex syngnathia with associated congenital anomalies of head and neck. This is further sub classified into Type 2a with associated aglossia and Type 2b with associated agenesis or hypoplasia of proximal mandible [3]. No consensus on management is there given the rarity of the disease and anesthesia is difficult in these patients.

Footnotes

Source of Support: Nil Conflict of Interest: Nil
  3 in total

Review 1.  Congenital bony syngnathia: a proposed classification.

Authors:  K H Dawson; J S Gruss; R W Myall
Journal:  Cleft Palate Craniofac J       Date:  1997-03

2.  Congenital unilateral maxillomandibular fusion.

Authors:  Xiaojun Shao; Ge Yang
Journal:  Br J Oral Maxillofac Surg       Date:  2014-01-18       Impact factor: 1.651

Review 3.  Congenital fusion of the maxilla and mandible.

Authors:  Kemal Uğurlu; Semra Karsidag; Ilkay Huthut; Kemalettin Yildiz; Lutfu Bas
Journal:  J Craniofac Surg       Date:  2005-03       Impact factor: 1.046

  3 in total
  2 in total

1.  Maxillomandibular Syngnathia: 3D Planning and Review of the Literature.

Authors:  Josep Rubio-Palau; Alejandra Prieto-Gundin; Leydi Mar de Abreu Graterol; Herman Vercruysse
Journal:  Craniomaxillofac Trauma Reconstr       Date:  2017-08-25

2.  Congenital Maxillomandibular Syngnathia: Review of Literature and Proposed New Classification System.

Authors:  Vijay Kumar; Vidya Rattan; Sachin Rai
Journal:  J Maxillofac Oral Surg       Date:  2019-11-20
  2 in total

北京卡尤迪生物科技股份有限公司 © 2022-2023.