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

Details

Serval ID
serval:BIB_5F7B52BFFD3F
Type
Article: article from journal or magazin.
Publication sub-type
Case report (case report): feedback on an observation with a short commentary.
Collection
Publications
Title
Complete Maxillo-Mandibular Syngnathia in a Newborn with Multiple Congenital Malformations.
Journal
Pediatrics and Neonatology
Author(s)
Broome M., Vial Y., Jacquemont S., Sergi C., Kamnasaran D., Giannoni E.
ISSN
2212-1692 (Electronic)
ISSN-L
1875-9572
Publication state
Published
Issued date
2016
Volume
57
Number
1
Pages
65-68
Language
english
Notes
Publication types: Case Reports ; Journal Article ; Research Support, Non-U.S. Gov't
Publication Status: ppublish
Abstract
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.
Keywords
Abnormalities, Multiple/surgery, Cleft Palate/surgery, Humans, Infant, Newborn, Male, Mandible/abnormalities, Maxilla/abnormalities, Synostosis/surgery, Zygoma/abnormalities
Pubmed
Web of science
Open Access
Yes
Create date
31/01/2014 15:42
Last modification date
20/08/2019 15:17
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