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DOI: 10.1055/a-2070-8590
Congenital Maxillomandibular Synechia with Multiple Malformations in a Very-Low-Birth-Weight Infant: A Case Report
Abstract
Congenital maxillomandibular synechia is a rare malformation that is characterized by a fusion of the maxilla and mandible. The fusion is fibrous or bony and prevents mouth opening, which causes difficulties in feeding and occasionally in breathing. Although extremely rare, neonatologists must understand the disease because it can be fatal and require emergency treatment after birth. We report the case of a very-low-birth-weight (VLBW) infant with congenital maxillomandibular synechia and other malformations, including cleft palate, syndactyly, and cryptorchidism. The patient presented with extremely limited mouth opening, and endotracheal intubation seemed impossible; fortunately, the patient did not have respiratory distress syndrome. The patient underwent surgical release of the fibrous bands on days 10 and 17, and good mouth opening was achieved. The patient was able to consume breast milk orally and was discharged home at a corrected gestational age of 1 month without recurrence of difficulty in mouth opening or any sequelae. This is the first reported case of a VLBW infant with congenital maxillomandibular synechia who required more complicated management of feeding, surgical intervention, and anesthesia.
Keywords
congenital maxillomandibular synechia - fibrous or bony fusion - difficulty in feeding and breathing - popliteal pterygium syndromePublication History
Received: 10 December 2022
Accepted: 23 March 2023
Accepted Manuscript online:
08 April 2023
Article published online:
19 May 2023
© 2023. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)
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References
- 1 Gartlan MG, Davies J, Smith RJ. Congenital oral synechiae. Ann Otol Rhinol Laryngol 1993; 102 (3 Pt 1): 186-197
- 2 Naikmasur VG, Sattur AP, Joshi SK, Rai A. Congenital syngnathia: case report and review of literature. Cleft Palate Craniofac J 2010; 47 (06) 654-660
- 3 Olusanya AA, Akadiri OA. Congenital syngnathia: review of clinical profile and proposal of a new classification-based management protocol. Int J Oral Maxillofac Implants 2020; 49 (04) 505-514
- 4 Kumar V, Rattan V, Rai S. Congenital maxillomandibular syngnathia: review of literature and proposed new classification system. J Maxillofac Oral Surg 2021; 20 (01) 19-36
- 5 Shams MG, Motamedi MH, Abad HL. Congenital fusion of the maxilla and mandible: brief case report. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2006; 102 (02) e1-e3
- 6 Salahuddin O, Yousaf K, Rashid M, Yousaf S, Qazi UA. , Ishtiaq-ur-Rehman, Congenital alveolar synechiae with cleft palate. J Pak Med Assoc 2014; 64 (06) 703-704
- 7 Mevada K, Gopalakrishna A. Intraoral synechiae with cleft palate in an older child: a case report and review of literature. J Cleft Lip Palate Craniofacial Anomalies 2016; 3: 46-49
- 8 Kondo S, Schutte BC, Richardson RJ. et al. Mutations in IRF6 cause Van der Woude and popliteal pterygium syndromes. Nat Genet 2002; 32 (02) 285-289
- 9 Leslie EJ, Mancuso JL, Schutte BC. et al. Search for genetic modifiers of IRF6 and genotype-phenotype correlations in Van der Woude and popliteal pterygium syndromes. Am J Med Genet A 2013; 161A (10) 2535-2544