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CC BY-NC-ND 4.0 · AJP Rep 2023; 13(02): e35-e39
DOI: 10.1055/a-2070-8590
Case Report

Congenital Maxillomandibular Synechia with Multiple Malformations in a Very-Low-Birth-Weight Infant: A Case Report

Ryosuke Uchi
1   Department of Neonatology, Japanese Red Cross Medical Center, Tokyo, Japan
2   Department of Neonatology, Yokohama City University Medical Center, Yokohama, Japan
,
Mayu Koto
1   Department of Neonatology, Japanese Red Cross Medical Center, Tokyo, Japan
,
Atsushi Nakao
1   Department of Neonatology, Japanese Red Cross Medical Center, Tokyo, Japan
,
Miku Hosokawa
1   Department of Neonatology, Japanese Red Cross Medical Center, Tokyo, Japan
,
Toshiko Ukawa
1   Department of Neonatology, Japanese Red Cross Medical Center, Tokyo, Japan
,
Chisa Tsurisawa
1   Department of Neonatology, Japanese Red Cross Medical Center, Tokyo, Japan
,
Yoshiya Hisaeda
1   Department of Neonatology, Japanese Red Cross Medical Center, Tokyo, Japan
,
Shusuke Amagata
1   Department of Neonatology, Japanese Red Cross Medical Center, Tokyo, Japan
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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 syndrome


Publication 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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