家族多发性牙源性角化囊肿2例报告及文献复习  

Familial multiple odontogenic keratocysts:A report of two cases and literature review

作　　者：宋丽娜 吴春月 秦清岩 楚天舒 刘麒麟[1] SONG Lina;WU Chunyue;QIN Qingyan;CHU Tianshu;LIU Qilin(Third Department of Oral and Maxillofacial Surgery,Stomatology Hospital,Jilin University,Changchun 130021,China)

机构地区：[1]吉林大学口腔医院口腔颌面外三科,吉林长春130021

出　　处：《吉林大学学报（医学版）》2022年第4期1035-1039,共5页Journal of Jilin University：Medicine Edition

基　　金：国家自然科学基金项目(81600890)。

摘　　要：目的:分析家族多发性牙源性角化囊肿(OKC)患者的临床表现、诊断和治疗方法,以提高临床医生对该病的认识。方法:收集2例OKC患者的临床资料和随访结果,分析病情并进行相关文献复习,并对与痣样基底细胞癌综合征(NBCCS)相关的多发性OKC的临床表现和诊疗进展等进行探讨。结果:患者1,女性,32岁,临床表现为眶距宽伴多发性OKC;左下颌骨OKC分别行刮治术、开窗减压术和刮治术+囊肿外围磨除部分骨质手术治疗;7年前左上颌骨病变行囊肿刮治术并磨除囊肿外围部分骨质,随访无复发;最近一次随访发现右上颌和右下颌骨出现放射性低密度影,考虑为新发囊肿,观察随诊。患者2为患者1的女儿,9岁,临床表现为额部突出,眼距宽,左眼角区及左颈部大量黑色素痣伴多发OKC。患儿6岁时拍摄颌骨曲面断层片未发现颌骨异常,半个月前因治牙拍摄颌骨曲面断层片发现左、右下颌骨低密度影;行双侧下颌骨囊肿摘除术,并拔除囊内累及牙33和47,囊肿外围磨除部分骨质。术后半月复查见创口愈合良好,现随访中。结论:NBCCS作为一种有家族遗传倾向的系统性疾病,通常以多发性OKC为首要表现。临床上发现多发性OKC患者,建议筛查家族多发性OKC和NBCCS的可能性,做到早期诊断和早期治疗。Objective:To analyze the clinical manifestations,diagnosis and treatment method of the patients with familial multiple odontogenic keratocysts(OKC),and to improve the clinicians’awareness of the disease.Methods:The clinical data and follow-up results of 2 cases of OKC patients were collected,the disease was analyzed and the related literatures were reviewed;the diagnosis and treatment progress of multiple OKC related to nevus basal cell carcinoma syndrome(NBCCS)were analyzed Results:Patient 1 was a 32-year-old female,presented with wide orbital space with multiple OKC;the left mandibular bone cyst received curettage,marsupialization and curettage+grinding treatment of the bone wall,respectively;the left maxillary lesion was removed by curettage with grinding treatment of the bone wall before 7 years.and no recurrence was found during follow-up;a recent follow-up showed radiolucency in the right maxilla and mandible,considering the occurrence of new cysts.Patient 2 was the 9-year-old daughter of patient 1;the clinical manifestations were prominent forehead,severe hypertelorism,a large number of melanin nevus on the left canthus and left neck with multiple OKC;the imageological examination was taken when the child was 6 years old and no abnormalities were found in her jaw bone.Before half a month,the radiolucent areas were found in the left and right mandible by panoramic radiography due to routine examination for dental treatment;curettage of cyst of both mandiblar bones was performed,the teeth involved in the cyst 33 and 47 were simultaneously removed,and part of bone was ground outside the cyst;half a month after surgery,the child took follow-up and wound healing was well.Conclusion:As a systemic disease with familial genetic predisposition,multiple OKC is usually the primary manifestation of NBCCS.Therefore,if multiple OKC patients have been found clinically,it is recommended to screen the possibility of familial multiple OKC and NBCCS,and early diagnosis and treatment should be performed.

关 键 词：家族性 牙源性角化囊肿 痣样基底细胞癌综合征 复发 随访 

分 类 号：R782.05[医药卫生—口腔医学]