Journal of Jilin University(Medicine Edition) ›› 2022, Vol. 48 ›› Issue (4): 1035-1039.doi: 10.13481/j.1671-587X.20220425

• Clinical medicine • Previous Articles    

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

Lina SONG,Chunyue WU,Qingyan QIN,Tianshu CHU,Qilin LIU()   

  1. Third Department of Oral and Maxillofacial Surgery,Stomatology Hospital,Jilin University,Changchun 130021,China
  • Received:2021-11-11 Online:2022-07-28 Published:2022-07-26
  • Contact: Qilin LIU E-mail:qlliu@jlu.edu.cn

Abstract: 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.

Key words: Family hereditary, Odontogenic keratotic cyst, Nevus basal cell carcinoma syndrome, Recurrence, Follow-up

CLC Number: 

  • R782.05