Gorlin-Goltz syndrome: A rare case report
A 13-year-old boy visited our hospital with a chief complaint of swelling on lower anterior region of the jaw since 10 days. Swelling was approximately 2 cm × 1 cm in size, soft to firm in consistency and tender on palpation. Intraoral examination revealed his deciduous teeth were still present and few permanent teeth were missing [].\nThe orthopantomogram was advised, which revealed three cystic lesions in the mandible and two involving the maxillary sinus on either side with the displaced permanent teeth []. Owing to the presence of multiple cysts like lesions in the jaw, GGS was suspected and further investigations were carried out.\nThe radiograph of the skull showed bilamellar calcification of the falx cerebri []. Physical examination revealed macrocephaly with a head circumference of 92 cm (normal for a 13-year-old boy is 54-57 cm), frontal bossing, depressed nasal bridge, hypertelorism and mandibular prognathism []. Palmar pits were brown coloured and measuring 1-3 mm in diameter []. No other anomalies of the skeletal, cardiovascular, or central nervous system were present. On the basis of clinical findings, diagnosis of GGS was made. Prior to the surgical procedure, an arch bar was placed on the lower jaw to prevent fracture of the mandible as well as to splint the unsupported permanent teeth. The cyst enucleation was done under general anesthesia via intraoral approach. After the cystic lesions enucleated, large areas of bone loss were seen and the displaced permanent teeth were visible on the floor of the cystic cavity []. The enucleated cystic lining was sent for histopathological examination.\nCarnoy's solution was then applied using cotton rolls for a period of 5 min. The excess solution was then irrigated using saline and the cavity was packed with bismuth iodine paraffin paste. The pack was removed after 7 days and regular irrigation of the cavity was done.\nThe histopathological examination of the enucleated tissue showed parakeratinized uniform squamous epithelial lining, five to eight cells thick with fairly flat base. The epithelium demonstrated a well-developed basal layer of palisaded columnar cells with polarized hyperchromatic nuclei. Multiple satellite and daughter cysts were seen in the connective tissue wall []. The histopathological examination confirmed the diagnosis of OKC.\nThe parents of the patient were examined and underwent radiological evaluation; neither of them had any features of the GGS.