关键词: Cleft lip Ectodermal dysplasia Peripheral giant cell Granuloma

Mesh : Humans Cleft Lip / surgery complications pathology Granuloma, Giant Cell / pathology surgery diagnostic imaging Cleft Palate / complications surgery pathology Ectodermal Dysplasia / complications pathology Male Female Child, Preschool

来  源:   DOI:10.1186/s12903-024-04585-z   PDF(Pubmed)

Abstract:
BACKGROUND: Ectrodactyly-ectodermal dysplasia-cleft lip/palate (EEC) syndrome mainly affects ectodermal and mesodermal tissues. It is usually manifested as split hands and feet, ectodermal dysplasia, and orofacial clefting, along with other signs and symptoms. A multidisciplinary approach to treatment is required, in which dentists play an important role in identifying and treating various oral conditions that may be genetically linked to or may be the result of EEC syndrome.
METHODS: The present case describes the oral condition of a young child suffering from EEC syndrome and presenting with peripheral giant cell granuloma (PGCG) in the mandibular anterior region. After obtaining a thorough medical and family history and a clinical examination, the lesion was surgically excised under local anesthesia. The patient was followed up at periodic intervals for the next twenty four months, during which no recurrence of the lesion was observed.
CONCLUSIONS: This report highlights the role of a dentist in the management of the oral conditions of patients suffering from EEC syndrome.
摘要:
背景:外植体-外胚层发育不良-唇腭裂(EEC)综合征主要影响外胚层和中胚层组织。它通常表现为分开的手和脚,外胚层发育不良,和口面裂开,以及其他症状和体征。需要多学科的治疗方法,其中牙医在识别和治疗可能与EEC综合征遗传相关或可能是EEC综合征的各种口腔疾病方面发挥重要作用。
方法:本案例描述了一名患有EEC综合征并在下颌前区表现为周围巨细胞肉芽肿(PGCG)的幼儿的口腔状况。在获得全面的病史和家族史以及临床检查后,在局部麻醉下手术切除病灶.在接下来的二十四个月中,定期对患者进行随访,在此期间未观察到病变复发。
结论:本报告强调了牙医在治疗EEC综合征患者口腔疾病中的作用。
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