J Korean Assoc Maxillofac Plast Reconstr Surg.  2009 Sep;31(5):429-434.

Case Report of Treatment of Multiple Odontogenic Keratocysts with Basal Cell Nevus Syndrome Using Preoperative Marsupialization and Orthodontic Extrusion

• Gang TI
• Park YJ
• Nam JH
• Ahn JH
• Kang HJ
• Song JH
• Chung JA
• Shin JE

Abstract

Basal cell nevus syndrome is a rare inherited disorder characterized by mulitple cutaneous basal cell carcinoma, pits of the palms and soles, cysts of the jaws, skeletal abnormalities and ectopic calcifications. Currently there are new lines of investigation based on biomolecular studies, which aim at identifying the molecules responsible for these cysts and thus early allowing an early diagnosis of these patients. We report a case of a 9-year-old boy with the various manifestation of basal cell nevus syndrome, which are multiple odontogenic keratocysts, pits of the soles, bifid ribs, ectopic calcification, macrocephaly, and hypertelorism, etc. Total five odontogenic keratocysts were found. For the reduction of the size of the odontogenic keratocysts, following preoperative marsupialization, there were surgically enucleated. And the impacted upper right lateral incisor and canine are tracted orthodontically.

Keyword

Odontogenic keratocyst; Basal cell nevus syndrome; Marsupialization; Orthodontic extrusion

MeSH Terms

Basal Cell Nevus Syndrome
Carcinoma, Basal Cell
Child
Early Diagnosis
Humans
Hypertelorism
Incisor
Jaw
Macrocephaly
Odontogenic Cysts
Orthodontic Extrusion
Ribs