Basal cell nevus syndrome: 4 case reports and a literature review
10.12016/j.issn.2096-1456.2019.01.008
- Author:
WANG Shipeng
1
;
ZHANG Andong
2
;
CHENG Li
3
;
CHEN Baoyong
3
;
WEN Li
2
;
LIU Huawei
2
Author Information
1. 1.Department of Stomatology, General Hospital of China stomatology 2.Department of Stomatology, Laiwu Iron and Steel Group Co., LTD Hospital
2. Department of Stomatology, General Hospital of China stomatology
3. Department of Stomatology, Laiwu Iron and Steel Group Co., LTD Hospital
- Publication Type:Case Reports
- Keywords:
Basal cell nevus syndrome;
Odontogenic keratocyst;
Autosomal dominant inheritance;
Photodynamic therapy;
Intracranial calcification;
Cysts in the jaw
- From:
Journal of Prevention and Treatment for Stomatological Diseases
2019;27(1):41-45
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To summarize the incidence, clinical manifestations, diagnosis and treatment of basal cell nevus syndrome and to provide reference for clinical diagnosis and treatment.
Methods : Retrospective analysis of 4 cases of basal cell nevus syndrome admitted to the General Hospital of PLA during January 2017 to January 2018 and recent cases reported in the literature.
Results:In this study, 1 males and 3 females were included. The patients included 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 manifestations 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.
- Full text:4例基底细胞痣综合征临床报道及文献复习.pdf
