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Congenital fibrous fusion of the jaws (synechiae) with cleft palate: A case report.
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 PDFSubmucosal dissection to close wide cleft palate with folded mucoperiosteum for bilateral cleft lip and palate with popliteal pterygium syndrome.
J Surg Case Rep
October 2023
Department of Genetics, Kanagawa Children's Medical Center, Mutsukawa 2-138-4, Minami-ku, Yokohama 232-8555, Kanagawa, Japan.
Characteristic features of popliteal pterygium syndrome (PPS) associated with the craniofacial region include cleft palate, syngnathia, and difficulty with reconstruction. We developed a new procedure of submucosal dissection with periosteotomy to close the folded mucosa in bilateral cleft lip and palate patients with PPS. This technique could be applicable for patients with wide cleft palate.
View Article and Find Full Text PDFEarly Surgical Intervention of Congenital 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 PDFNovel de novo missense mutation in the interferon regulatory factor 6 gene in an Italian infant with IRF6-related disorder.
Ital 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 PDFA new surgical approach to treatment of bilateral syngnathia in a patient with popliteal pterygium-syndrome.
J 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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