The recurrence of odontogenic keratocysts in pediatric patients is associated with clinical findings of Gorlin-Goltz Syndrome.

LP. Silva,LS. Rolim,LP. Pinto,LB. Souza,LA. Silva

doi:10.4317/medoral.23185

Abstract

Background Odontogenic keratocyst (OKC) is an odontogenic developmental cyst that presents distinct clinical behavior. This lesion has been described as dental cysts with keratinization since the 1930s, however the term "OKC" was established in 1956. This study aims to determine the frequency and features of OKC in children aged 0 to 14 years in an oral pathology service in Brazil. Material and Methods A retrospective study was performed to review cases of OKC in children diagnosed between 1986 and 2017. Clinical data were evaluated from medical records (gender, race, age, anatomical location, treatment, radiographic findings and follow-up). Results Ninety-seven cases of OKC were diagnosed in a 31-year-period in all age groups and 10 were found in children (10.3%). Age ranged from 2 to 14 years (mean age=10.5±3.5), with 8 males and 2 females. The most frequent location was the anterior region of the mandible (n=4). Patients were predominantly asymptomatic. Moreover, in two children, clinical findings of Gorlin-Goltz Syndrome were observed. The most commonly used treatment was enucleation followed by curettage. In all cases of Gorlin-Goltz Syndrome were observed recurrences and occurrence of new keratocysts. Conclusions Although uncommon in pediatric patients, OKC should be considered a differential diagnosis in cases of osteolytic lesions in gnathic bones. Thus, the periodic assessment of children by dentists and pediatricians is essential to get a correct diagnosis and early treatment to avoid greater mutilation of these patients. Key words:Odontogenic cysts, children, odontogenic keratocyst, Gorlin-Goltz Syndrome.

Highlights

Since the 1930s, researchers have noted keratinization in dental cysts
This cyst may be associated with Nevoid Basal Cell Carcinoma Syndrome (NBCCS), or Gorlin-Goltz Syndrome, which is a rare autosomal dominant syndrome characterized primarily by multiple/recurrent Odontogenic keratocyst (OKC), basal cell carcinomas and other clinical findings which usually appear during puberty or even during the first decade of life [5,9,10]
The most frequent location was the anterior region of the mandible with mandible:maxilla ratio of 2.3:1 (Table 1)

Summary

Introduction

Since the 1930s, researchers have noted keratinization in dental cysts. the use of the term “odontogenic keratocyst” was first suggested by Philipsen for all odontogenic cysts that presented keratinization in the epithelium [1]. Most OKC appears as an isolated solitary lesion This cyst may be associated with Nevoid Basal Cell Carcinoma Syndrome (NBCCS), or Gorlin-Goltz Syndrome, which is a rare autosomal dominant syndrome characterized primarily by multiple/recurrent OKC (present in at least 75% of patients), basal cell carcinomas and other clinical findings which usually appear during puberty or even during the first decade of life [5,9,10]. The incidence of OKC is uncommon in patients from zero to 14 years, and few studies in literature have reported cases in non-syndromic children [11,12,13,14,15,16]. Odontogenic keratocyst (OKC) is an odontogenic developmental cyst that presents distinct clinical behavior This lesion has been described as dental cysts with keratinization since the 1930s, the term “OKC” was established in 1956. The periodic assessment of children by dentists and pediatricians is essential to get a correct diagnosis and early treatment to avoid greater mutilation of these patients

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