A Rare Case of Gorlin-Goltz Syndrome in Children
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The Gorlin-Goltz syndrome, nevoid basal cell carcinoma syndrome, or basal cell nevus syndrome is an autosomal dominant condition disorder with high variability expression. It presents a series of relevant clinical manifestations that suggest its diagnosis in cutaneous, bone, dental, soft tissue, nervous, and ocular system disorders. This condition requires a great interaction of several specialists to improve the patient's life.

A 9-year-old male patient was referred to the Department of Oral and Maxillofacial Surgery due to failure of dental eruption in many teeth. The patient presented multiple cysts in the mandible and maxilla with a diagnostic hypothesis of odontogenic keratocysts.

The intraoral examination revealed several dental absences at the anterior and posterior regions of the maxilla and mandible, with teeth in the ectopic position, beside the bulging of the vestibular cortical bone in the region of the right inferior canine and premolars. On the panoramic radiograph, multiple radiolucent areas, causing dental dislocation, were observed . With the objective of obtaining the most precise limits of the multiple lesions, computed tomography was done .

Odontogenic keratocysts were histologically confirmed and treated by marsupialization, enucleation, and follow-up. Due to the possibility of multiple keratocysts being associated with Gorlin-Goltz syndrome, other manifestations of the Gorlin-Goltz syndrome were searched. After this clinical evaluation, three major criteria were found: odontogenic keratocysts, bifid ribs , and palmoplantar pits , along with 10 minor criteria: hypertelorism , falx cerebri calcification , macrocephaly , bifid uvula, frontal bossing , malocclusion, shortened fourth metacarpal, cardiac fibroma , ectopic dental position, and precocious development of genitals.

This case report stresses the importance of early diagnoses to prevent morbidity and mortality associated with this syndrome and reports a rare case of so many manifestations of the Gorlin-Goltz syndrome in an infant. In addition, the case report shows the importance of a multidisciplinary team including dentists, dermatologists, geneticists, and neurologists needed to improve the diagnosis and quality of life.

Source: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6942748/
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