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

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, Y. Vial, S. Jacquemont, C. Sergi, D. Kamnasaran, E. Giannoni
Format: Article
Language:English
Published: Elsevier 2016-02-01
Series:Pediatrics and Neonatology
Subjects:
choanal atresia
maxillo-mandibular fusion
newborn
syngnathia
synostosis
Online Access:http://www.sciencedirect.com/science/article/pii/S1875957213000818
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spelling doaj-8b7db9cbb8604f86a4ea13f6bf1d37d82020-11-24T21:05:40ZengElsevierPediatrics and Neonatology1875-95722016-02-01571656810.1016/j.pedneo.2013.04.009Complete Maxillo-Mandibular Syngnathia in a Newborn with Multiple Congenital MalformationsM. Broome0Y. Vial1S. Jacquemont2C. Sergi3D. Kamnasaran4E. Giannoni5Service of Maxillo-Facial Surgery, Centre Hospitalier Universitaire Vaudois, Lausanne, SwitzerlandDepartment of Obstetrics and Gynecology, Centre Hospitalier Universitaire Vaudois, Lausanne, SwitzerlandService of Medical Genetics, Centre Hospitalier Universitaire Vaudois, Lausanne, SwitzerlandDepartment of Pathology and Laboratory Medicine, University of Alberta, Edmonton, Alberta, CanadaDepartment of Pediatrics, Laval University, Quebec City, Quebec, CanadaService of Neonatology, Centre Hospitalier Universitaire Vaudois, Lausanne, SwitzerlandSyngnathia 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.http://www.sciencedirect.com/science/article/pii/S1875957213000818choanal atresiamaxillo-mandibular fusionnewbornsyngnathiasynostosis
collection DOAJ
language English
format Article
sources DOAJ
author M. Broome
Y. Vial
S. Jacquemont
C. Sergi
D. Kamnasaran
E. Giannoni
spellingShingle M. Broome
Y. Vial
S. Jacquemont
C. Sergi
D. Kamnasaran
E. Giannoni
Complete Maxillo-Mandibular Syngnathia in a Newborn with Multiple Congenital Malformations
Pediatrics and Neonatology
choanal atresia
maxillo-mandibular fusion
newborn
syngnathia
synostosis
author_facet M. Broome
Y. Vial
S. Jacquemont
C. Sergi
D. Kamnasaran
E. Giannoni
author_sort M. Broome
title Complete Maxillo-Mandibular Syngnathia in a Newborn with Multiple Congenital Malformations
title_short Complete Maxillo-Mandibular Syngnathia in a Newborn with Multiple Congenital Malformations
title_full Complete Maxillo-Mandibular Syngnathia in a Newborn with Multiple Congenital Malformations
title_fullStr Complete Maxillo-Mandibular Syngnathia in a Newborn with Multiple Congenital Malformations
title_full_unstemmed Complete Maxillo-Mandibular Syngnathia in a Newborn with Multiple Congenital Malformations
title_sort complete maxillo-mandibular syngnathia in a newborn with multiple congenital malformations
publisher Elsevier
series Pediatrics and Neonatology
issn 1875-9572
publishDate 2016-02-01
description 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.
topic choanal atresia
maxillo-mandibular fusion
newborn
syngnathia
synostosis
url http://www.sciencedirect.com/science/article/pii/S1875957213000818
work_keys_str_mv AT mbroome completemaxillomandibularsyngnathiainanewbornwithmultiplecongenitalmalformations
AT yvial completemaxillomandibularsyngnathiainanewbornwithmultiplecongenitalmalformations
AT sjacquemont completemaxillomandibularsyngnathiainanewbornwithmultiplecongenitalmalformations
AT csergi completemaxillomandibularsyngnathiainanewbornwithmultiplecongenitalmalformations
AT dkamnasaran completemaxillomandibularsyngnathiainanewbornwithmultiplecongenitalmalformations
AT egiannoni completemaxillomandibularsyngnathiainanewbornwithmultiplecongenitalmalformations
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