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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| Format: | Article |
| Language: | English |
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Elsevier
2016-02-01
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| Series: | Pediatrics and Neonatology |
| Subjects: | |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S1875957213000818 |
| _version_ | 1818295660915458048 |
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| author | M. Broome Y. Vial S. Jacquemont C. Sergi D. Kamnasaran E. Giannoni |
| author_facet | M. Broome Y. Vial S. Jacquemont C. Sergi D. Kamnasaran E. Giannoni |
| author_sort | M. Broome |
| collection | DOAJ |
| 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. |
| first_indexed | 2024-12-13T03:51:11Z |
| format | Article |
| id | doaj.art-8b7db9cbb8604f86a4ea13f6bf1d37d8 |
| institution | Directory Open Access Journal |
| issn | 1875-9572 |
| language | English |
| last_indexed | 2024-12-13T03:51:11Z |
| publishDate | 2016-02-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Pediatrics and Neonatology |
| spelling | doaj.art-8b7db9cbb8604f86a4ea13f6bf1d37d82022-12-22T00:00:43ZengElsevierPediatrics 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 |
| 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 |
| title | 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_short | Complete Maxillo-Mandibular Syngnathia in a Newborn with Multiple Congenital Malformations |
| title_sort | complete maxillo mandibular syngnathia in a newborn with multiple congenital malformations |
| topic | choanal atresia maxillo-mandibular fusion newborn syngnathia synostosis |
| url | http://www.sciencedirect.com/science/article/pii/S1875957213000818 |
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