Palacios E, Serou M, Restrepo S, Gonzalez I, Rojas R

Language: English
References: 9
Page: 23-26
PDF size: 71.89 Kb.

ABSTRACT

Introduction: The nevoid basal cell syndrome (Gorlin’s Syndrome) is a dominant autosomic disorder, characterized by multiple basal cell carcinomas, odontegenic keratocysts, dural calcifications, bone and face malformations, tumors including meduloblastoma and ovaric fibromas, as well as different degrees of mental retardation.
Characteristic imagine findings of the Gorlin’s Syndrome are odontogenic keratocysts in the jaw and jawbone, prognathism, cleft lip and palate, macrocephalia, prominent paranasal cavities, inter-hemispheric bone calcifications, vertebrae malformations (cifoescoliosis and abnormal segmentation), ribs merging, short forth metacarpian and sclerotic bone lesions.
Material and methods:A case of a 13-year old male patient is presented, with family background of Gorlin’s Syndrome who presented mouth fetid serum-bloody drain, prognathism and hypertelorism. Imaging studies showed bi-lateral cystic lesions on the jaw’s angle and maxillary antra. MRI with T2 and T1 powered images with Gadolinium showed multiple cystic lesions with lobe-like contours, which had their peripheral contour highlighted after the administration of Gadolinium, some with a secondary liquid level at hemorrhagic component. Brain MRI showed minimum slimming of the callous body and a small prominence of the ventricle system for his age. The bone series did not show any alterations different from those already described.
Discusion: Even though the CT is useful in diagnosing face abnormalities related to Gorlin’s Syndrome, MRI is better in it capacity to show the internal composition and the structures of odontogenic keratocysts.

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