Basal cell nevus syndrome: 4 case reports and a literature review

Abstract

Objective To summarize the incidence, clinical manifestations, diagnosis and treatment of basal cell ne⁃ vus syndrome and to provide reference for clinical diagnosis and treatment. Methods Retrospective analysis of 4 cas⁃ es of basal cell nevus syndrome admitted to the General Hospital of PLA during January 2017 to January 2018 and re⁃ cent cases reported in the literature. Results In this study, 1 males and 3 females were included. The patients includ⁃ ed a mother and her child. All 4 cases were surgically resected. Pathological reports included all keratocysts of the jaws. There has been no recurrence since follow⁃up. Through literature summarization and analysis, the clinical manifesta⁃ tions of this syndrome were found to be diverse. Typical clinical manifestations include multiple keratocysts of the jaws, multiple blepharospasms or cancers, deformities of the spine or ribs, increased brachial distance, eye diseases or special face intracranial calcification. Conclusion Basal cell nevus syndrome is an autosomal dominant genetic disorder. The clinical manifestations are diverse and the diagnosis is often overlooked. The incidence of cysts in the jaws is one of the important clinical manifestations of this syndrome. Early diagnosis and proper treatment improve patient survival and quality of life.

Keywords

• Basal cell nevus syndrome
• Odontogenic keratocyst
• Autosomal dominant inheritance
• Photody⁃ namic therapy
• Intracranial calcification
• Cysts in the jaw