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先天性上颌下颌融合:文献综述及新分类系统建议

Congenital Maxillomandibular Syngnathia: Review of Literature and Proposed New Classification System.

作者信息

Kumar Vijay, Rattan Vidya, Rai Sachin

机构信息

Unit of Oral and Maxillofacial Surgery, Oral Health Sciences Centre, Postgraduate Institute of Medical Education and Research, Chandigarh, 160012 India.

出版信息

J Maxillofac Oral Surg. 2021 Mar;20(1):19-36. doi: 10.1007/s12663-019-01308-8. Epub 2019 Nov 20.

Abstract

PURPOSE

Maxillomandibular fusion (syngnathia) is a rare craniofacial anomaly. It occurs as either fibrous (synechiae) or bony (synostosis) fusion of jaw or fusion of mandible to zygoma, tuberosity, hard palate and temporal bone. There are several documentations delineating this condition but owing to its varying presentation, association with both intraoral and extraoral anomalies and syndromes, reviewing it has been as uphill task. The non-unanimous description of cases in the literature and the use of different nomenclatures make it difficult to classify this condition.

METHODS

Extensive search of the literature was done from the year 1936 to 2018 which included a total of 118 cases with 62 cases of bony fusion, 48 cases of fibrous fusion and 8 cases of combined fusion, i.e., fibrous on one site and bony on another side.

RESULTS

This paper proposes a novel, simple and explicit classification system on the basis of nature of fusing tissues, location and extent, association with syndrome after reviewing the existing literature. Furthermore, this review reports 4 new cases of syngnathia along with their clinical, radiographic features and their management protocol.

CONCLUSIONS

This classification can be generalized to all the cases of syngnathia and can further facilitate in its appropriate diagnosis and treatment plan.

摘要

目的

上颌下颌融合(并颌畸形)是一种罕见的颅面畸形。它表现为颌骨的纤维性(粘连)或骨性(骨性融合)融合,或者下颌骨与颧骨、结节、硬腭及颞骨的融合。虽然有几篇文献描述了这种情况,但由于其表现多样,与口腔内和口腔外异常及综合征相关,对其进行综述一直是一项艰巨的任务。文献中对病例的描述不一致以及使用不同的命名法使得对这种情况进行分类变得困难。

方法

对1936年至2018年的文献进行了广泛检索,共纳入118例病例,其中62例为骨性融合,48例为纤维性融合,8例为联合融合,即一侧为纤维性融合而另一侧为骨性融合。

结果

在回顾现有文献的基础上,本文根据融合组织的性质、位置和范围、与综合征的关联,提出了一种新颖、简单且明确的分类系统。此外,本综述报告了4例并颌畸形的新病例及其临床、影像学特征和治疗方案。

结论

这种分类可推广至所有并颌畸形病例,并可进一步促进其准确诊断和治疗方案的制定。

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