Complete Maxillo-Mandibular Syngnathia in a Newborn with Multiple Congenital Malformations

Syngnathia is an extremely rare condition involving congenital fusion of the maxilla with the mandible. Clinical presentations vary from simple mucosal bands (synechiae) to complete bony fusion (synostosis). Most cases are unilateral incomplete fusions. We report the case of a severely growth-retard...

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Main Authors: M. Broome (Author), Y. Vial (Author), S. Jacquemont (Author), C. Sergi (Author), D. Kamnasaran (Author), E. Giannoni (Author)
Format: Book
Published: Elsevier, 2016-02-01T00:00:00Z.
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100 1 0 |a M. Broome  |e author 
700 1 0 |a Y. Vial  |e author 
700 1 0 |a S. Jacquemont  |e author 
700 1 0 |a C. Sergi  |e author 
700 1 0 |a D. Kamnasaran  |e author 
700 1 0 |a E. Giannoni  |e author 
245 0 0 |a Complete Maxillo-Mandibular Syngnathia in a Newborn with Multiple Congenital Malformations 
260 |b Elsevier,   |c 2016-02-01T00:00:00Z. 
500 |a 1875-9572 
500 |a 10.1016/j.pedneo.2013.04.009 
520 |a Syngnathia is an extremely rare condition involving congenital fusion of the maxilla with the mandible. Clinical presentations vary from simple mucosal bands (synechiae) to complete bony fusion (synostosis). Most cases are unilateral incomplete fusions. We report the case of a severely growth-retarded newborn infant with complete synostosis of the mandible with the maxilla and the zygoma associated with cleft palate, choanal atresia, deafness, delayed cerebral white matter development, and genital and limb malformations. Extensive genetic analysis did not reveal any mutations. This association of multiple congenital malformations may represent an entity distinct from previously described syndromes associated with syngnathia. 
546 |a EN 
690 |a choanal atresia 
690 |a maxillo-mandibular fusion 
690 |a newborn 
690 |a syngnathia 
690 |a synostosis 
690 |a Pediatrics 
690 |a RJ1-570 
655 7 |a article  |2 local 
786 0 |n Pediatrics and Neonatology, Vol 57, Iss 1, Pp 65-68 (2016) 
787 0 |n http://www.sciencedirect.com/science/article/pii/S1875957213000818 
787 0 |n https://doaj.org/toc/1875-9572 
856 4 1 |u https://doaj.org/article/8b7db9cbb8604f86a4ea13f6bf1d37d8  |z Connect to this object online.