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上颌和下颌牙槽突融合并伴有下颌正中裂:一种罕见的先天性异常。

Fusion of maxillary and mandibular alveolar process together with a median mandibular cleft: a rare congenital anomaly.

作者信息

Uğurlu K, Turan T, Urganci N, Gözü A, Günay Y, Baş L

机构信息

Department of Plastic and Reconstructive Surgery, Istanbul Sişli Etfal Hospital, Turkey.

出版信息

J Craniomaxillofac Surg. 1999 Apr;27(2):105-8. doi: 10.1016/s1010-5182(99)80022-5.

DOI:10.1016/s1010-5182(99)80022-5
PMID:10342147
Abstract

Congenital fusion of the maxillary alveolar process and mandible is a very rare entity. A one day old male newborn was referred to our clinic with a diagnosis of a 'absence of oral cavity'. Physical examination revealed that there was no mouth opening bilaterally in the posterior region due to maxillary and mandibular bone fusion; and in the front there was only 1 mm of mouth opening. Examination of the intraoral space could not be achieved. No other congenital anomaly was detected on general examination. The infant, who was fed via a nasogastric tube for 1 month, was operated on. An intra-oral device which separated maxillary and mandibular alveolar arches from each other was designed and used for 8 weeks to avoid recurrence of the fusion. An adequate mouth opening was obtained and the infant could be fed orally.

摘要

先天性上颌牙槽突与下颌骨融合是一种非常罕见的病症。一名1日龄男性新生儿因“口腔缺失”的诊断被转诊至我们的诊所。体格检查发现,由于上颌骨和下颌骨融合,双侧后部区域无法开口;前部仅有1毫米的开口。无法对口腔内部空间进行检查。全身检查未发现其他先天性异常。该婴儿通过鼻胃管喂养了1个月后接受了手术。设计了一种将上颌和下颌牙槽弓彼此分开的口腔内装置,并使用8周以避免融合复发。获得了足够的开口度,婴儿能够经口喂养。

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J Maxillofac Oral Surg. 2021 Mar;20(1):19-36. doi: 10.1007/s12663-019-01308-8. Epub 2019 Nov 20.
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Mouse Models of Rare Craniofacial Disorders.罕见颅面疾病的小鼠模型
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