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

BACKGROUND

Nevoid 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 REPORT: We 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.

CONCLUSION

Clinicians 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.