J Korean Soc Plast Reconstr Surg.  2010 Nov;37(6):819-822.

Removal of Odontogenic Keratinocyst using Versatile Maxillary Window in BCNS

• Moon MS
• Lee HK
• Jeong HS
• Song JS

Abstract

PURPOSE
Basal cell nevus syndrome (BCNS), also known as Gorlin syndrome, is a rare autosomal dominant disorder. It is characterized by complex neoplastic syndrome with multisystemic manifestations, involving six major features. This article presents a rare genetic disorder and usage of the author's methods for odontogenic keratocyst, developed in the maxillary sinus.
METHODS
A 67-year-old man was presented with large calcified maxillary mass and multisystemic manifestations and findings that matched with basal cell nevus syndrome. The calcified maxillary mass was removed via the versatile maxillary window and maxillary bone segment was repositioned.
RESULTS
Histopathologic findings revealed that maxillary and mandibular lesions were odontogenic keratocysts and the skin lesions were basal cell carcinoma.
CONCLUSION
Basal cell nevus syndrome is a rare genetic disease that requires surveillance and care for basal cell carcinoma and multisystemic problems. The author's method was satisfactory for maxillary odontogenic keratocyst in the aspect of the approach and reconstruction.

Keyword

Basal cell nevus syndrome; Calcifying odontogenic cyst

MeSH Terms

Aged
Basal Cell Nevus Syndrome
Carcinoma, Basal Cell
Humans
Maxilla
Maxillary Sinus
Odontogenic Cyst, Calcifying
Odontogenic Cysts
Skin