背景:腭发育涉及通过复杂的分子机制进行的遗传调控,该机制可能会受到环境因素的干扰,导致受损的融合和腭裂形成。由于背舌错构瘤而遇到的left裂病例促使我们进行了这项系统审查。
目的:回顾1例腭裂合并舌错构瘤的临床特点和治疗方法。
方法:在PubMed中使用与left裂和舌错构瘤相关的关键词进行了系统的文献检索,Scopus,MEDLINE,和Scielo数据库到2021年12月,没有时间或语言限制。
方法:研究报告了腭裂和舌错构瘤患者。
方法:与临床概况有关的信息,诊断测试,组织病理学,管理,并提取结果。
到目前为止,共发现了14篇相关出版物,报告了16例病例。其中,13例患者为女性(81.25%),男性3人(18.75%)。介绍的年龄从出生到19岁不等。口腔-面部-数字综合征(II型)是最常见的相关综合征。
先天性舌错构瘤伴腭裂是一种罕见的表现,可以表现为孤立的实体或综合症的一部分。基因评估是有必要的,特别是对于多发性错构瘤性病变。优选的治疗是立即切除错构瘤,同时遵循用于腭成形术的标准时间表。
Palate development involves a genetic regulation through a complex molecular mechanism that may be disrupted by environmental factors, resulting in impaired fusion and cleft palate formation. An encounter with a case of cleft palate due to dorsal tongue hamartoma prompted us to perform this systematic review.
To review the clinical profile and management approach for a case with cleft palate and tongue hamartoma.
A systematic literature search was conducted using keywords related to cleft palate and tongue hamartoma in PubMed, Scopus, MEDLINE, and Scielo databases through December 2021, with no time or language restrictions.
Studies reporting patients with cleft palate and tongue hamartoma were included.
Information related to clinical profile, diagnostic tests, histopathology, management, and outcomes were extracted.
Fourteen relevant publications were identified with 16 cases reported so far. Among them, thirteen patients were females (81.25%), and 3 were males (18.75%). The age of presentation varied from birth to 19 years. Oral-facial-digital syndrome (type II) was the most commonly associated syndrome.
Congenital tongue hamartoma with cleft palate is a rare presentation, which can present as an isolated entity or part of a syndrome. Genetic evaluation is warranted, particularly for multiple hamartomatous lesions. The preferred treatment is immediate excision of hamartoma while following a standard timeline for palatoplasty.