Congenital syngnathia is an extremely rare condition with no standardized treatment. We hereby report a case highlighting the difficulties faced in its management and the postoperative complications.
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http://dx.doi.org/10.21699/jns.v6i1.402 | DOI Listing |
J Neonatal Perinatal Med
August 2024
Department of Neonatology, Sukena Susnerwala, MD, DM, Latifa Women's and Children's Hospital, Dubai, United Arab Emirates.
Background: Congenital maxillomandibular fusion is a rare disorder. Maxillomandibular fusion is usually discovered immediately after birth because the newborn is unable to open mouth or feed normally. The fusion defects can present with a wide range of severity, ranging from mucosal band (synechiae) to complete bony fusion (syngnathia).
View Article and Find Full Text PDFJ Med Genet
November 2023
Laboratory of embryology and genetics of human malformations, Institut National de la Santé et de la Recherche Médicale (INSERM) UMR 1163, Institut Imagine and Université Paris Cité, Paris, France
Background: Syngnathia is an ultrarare craniofacial malformation characterised by an inability to open the mouth due to congenital fusion of the upper and lower jaws. The genetic causes of isolated bony syngnathia are unknown.
Methods: We used whole exome and Sanger sequencing and microsatellite analysis in six patients (from four families) presenting with syngnathia.
J Craniofac Surg
May 2023
Department of Oral and Maxillofacial Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
Congenital syngnathia is a rarely reported malformation when there is a fusion between the maxilla and the mandible. It is necessary to modify it in childhood because congenital syngnathia causes incongruity in pronunciation, diet, and esthetics during the growth process. In this case report, 1 case of syngnathia, a rare craniofacial anomaly, is presented with a review of reports.
View Article and Find Full Text PDFItal J Pediatr
July 2022
Neonatal Intensive Care Unit, Department of Health Promotion, Mother-Child Care, Internal Medicine and Medical Specialties "G. D'Alessandro", University Hospital "P. Giaccone", Via Alfonso Giordano n. 3, 90127, Palermo, Italy.
Background: Congenital maxillomandibular syngnathia is a rare craniofacial anomaly leading to difficulties in feeding, breathing and ability to thrive. The fusion may consist of soft tissue union (synechiae) to hard tissue union. Isolated cases of maxillomandibular fusion are extremely rare, it is most often syndromic in etiology.
View Article and Find Full Text PDFJ Stomatol Oral Maxillofac Surg
November 2022
Department of Oral and Maxillofacial Surgery, University Medical Center Hamburg-Eppendorf, Hamburg 20246, Germany.
This case describes the treatment of syngnathia with a popliteal-pterygium syndrome. Although short-term successful surgical treatment has been described in literature, long-term follow up is essential, as the mouth opening limitations can be repetitively reoccurring. This requires new, individual treatment strategies as demonstrated in this case.
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