Conservative management of multiple odontogenic keratocysts in a child with nevoid basal cell carcinoma syndrome: A case report

Abstract

AbstractBackgroundNevoid basal cell carcinoma syndrome (NBCCS) is an autosomal dominant multisystemic disorder characterized by the presence of multiple odontogenic keratocysts (OKC), which are a hallmark feature of the syndrome. The treatment of these OKC poses challenges due to their high recurrence rates and the myriad of management options available.Case ReportWe describe here a case of NBCCS diagnosed in an 11‐year‐old girl who presented with multiple OKC in the jaws. Chest and cranial radiographs showed no abnormalities in the ribs and the cerebral falx, respectively. Cephalometric analysis indicated mandibular retrusion, a skeletal class II relationship, and a convex profile. The treatment approach involved a personalized strategy tailored for each cyst, comprising marsupialization followed by enucleation. This approach aimed to minimize surgical trauma and to reduce the risk of recurrence. The patient underwent regular follow‐up appointments, demonstrating successful outcomes with no signs of recurrence or de novo OKC observed over a 32‐month period.ConclusionClinicians should consider lesion characteristics and patient cooperation when determining treatment strategies for the optimization of outcomes for children and adolescents with NBCCS and multiple OKC.