Multiple Keratocyst Glimpses Falx Cerebri and Bifid Rib- A Case of Gorlin-Goltz Syndrome

Abstract

Multiple Odontogenic keratocysts with multi-organ abnormalities are a feature of Gorlin-Goltz Syndrome (GGS) which is an autosomal dominant inherited disorder. The most common oral manifestation of GGS is odontogenic keratocyst. Early diagnosis of GGS without cutaneous manifestations is important as this syndrome has basal cell carcinoma as one of its major criteria. This paper includes a case report of a 24-year-old female who reported with swelling and pus discharge in lower right posterior region. The swelling was clinico-radiologically diagnosed as odontogenic keratocyst on a panoramic radiograph which revealed the presence of multiple cystic lesions in symphysis, parasymphysis, and bilateral maxillary region. Postero-Anterior (PA) chest showed bifid ribs and PA skull showed calcification of falxcerebri. Histopathological diagnosis confirmed the Odontogenic keratocyst. Ultrasonography (USG) abdomen revealed a simple ovarian cyst on the right side. Clinical examination findings and radiological imaging evaluation with histopathological examination of cystic lesion confirms Gorlin-Goltz. Surgical enucleation of the keratocyst, peripheral ostectomy followed by bone graft substitute, and primary closure under General Anaesthesia (GA) were done in the Department of Oral and Maxillofacial Surgery. Follow-up healing was uneventful after one month.