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Gorlin-Goltz syndrome

Gorlin-Goltz syndrome is an inherited autosomal dominant disorder with complete penetrance and extreme variable expressivity. The authors present a case of an 11-year-old girl with typical features of Gorlin-Goltz syndrome with special respect to medical and dental problems which include multiple bony cage deformities like spina bifida with scoliosis having convexity to the left side, presence of an infantile uterus and multiple odonogenic keratocysts in the maxillofacial region.

in the eruption of permanent teeth. A brief medical history revealed that during late infancy (11 months) she was diagnosed to have rickets and treated for the same. Apart from this, her past history was uneventful. Her psychomotor development was normal and her school performance was above average.
At the Department of Oral and Maxillofacial surgery, Saraswati Dental College and Hospital, the patient was completely examined. On inspection, the patient appeared to have sprengel deformity [ Figure 1]. AP view of the chest revealed multiple bony cage abnormalities. Significant findings elicited were spina bifida of C5 and C6 vertebrae with scoliosis having convexity to the left side and left and right 4 th and 5 th ribs partially fused with narrow intercostal space [ Figure 2]. Complete blood count was done and appeared to be within normal limits. Panoramic radiograph revealed multiple radiolucent areas in the region suggestive of a cyst [ Figure 3]. Aspirates of right ramus region of mandible and the biochemical analysis revealed a total protein content of 0.8 g/dl, suggestive of a keratocyst. Enucleation with open dressing (packing the cavity with iodoform gauze) of the right ramus region cyst was done. Enucleation of the left body mandible cyst was done. The cystic lining was sent for histopathologic examination and was confirmed as odontogenic keratocyst.
Hitherto fore, the patient was advised to get an ultrasonography of the abdomen which revealed a small nodular structure in uterine fossa giving an impression of an infantile uterus. Left ovary could not be visualized.
The patient was also referred to a tertiary care centre -Sanjay Gandhi Post Graduate Institute, Lucknow, for genetic evaluation where karyotyping from blood was done and no detectable abnormality was elicited.
Based on the clinical, radiographic, and histologic findings, and referring to the diagnostic criteria for

dIscussIon
Gorlin-Goltz syndrome is an autosomal dominant disorder with high penetrance and variable expressivity. It is caused by the mutations in the patched tumor suppressor gene (PTCH), a human homologue of the Drosophila gene mapped to the long arm of chromosome 9q22.3-q31. Gorlin-Goltz syndrome's typical malformative patterns suggest that the main function is to control the growth and development of normal tissues [3] . It is associated with multiple keratocysts in patients in the second decade of their life. In the case presented here, one of the first signs was multiple cystic lesions involving the maxilla and mandible, which have been histopathologically diagnosed as odontogenic keratocysts. The association with odontogenic keratocysts, however, is not clearly understood and appears in more than 90% of the cases. All the other disorders are less frequent. Acral pits that are often overlooked during physical examination have a characteristic dermoscopy with red globules that are mainly distributed in parallel lines inside flesh-colored, irregular-shaped, and slightly depressed lesions [4] . Dermoscopy improves the visualization of these pits.
Despite the name of the syndrome, multiple BCCs occur only in 50% of the cases. They may vary in number from a few to 1000 and range in size from 1 to 30 mm in diameter. BCC most often involves face and nonexposed areas such as the back and chest. Management of superficial BCC without hair follicle involvement can be accomplished by the topical application of 0.1% Retinoin cream, 5% 5-Florouracil cream, 5% Imiquimod cream, cryosurgery, and surgical excision.
The diagnostic criteria for nevoid BCC were established by Evans et al, and modified by Kimonis et al. in 1973 [5] . According to them, diagnosis of Gorlin-Goltz syndrome can be established when two major or one major and two minor criteria are present which are described below.
Major criteria 1 More than two BCCs or one BCC under the age of 20 years. 2. Histologically proven odontogenic keratocyst of the jaw. 3. Three or more cutaneous palmar or plantar pits. 4. Bifid, fused or markedly splayed ribs. 5. First degree relative with nevoid basal cell carcinomas.