Head and Neck Imaging

Basal cell naevus syndrome

(also called GorlinGotz syndrome), autosomal dominant disorder with a variable clinical picture. The syndrome consists of multiple basal cell carcinoma head and neck, odontogenic keratocysts, palmar and plantar pits, skeletal abnormalities (bifid and anteriorly broadened ribs, short fingers, hypertelorism, prognathia), ectopic calcification (cerebral falx), hamartomas, epidermal cysts (milia) and eye muscle palsies. The keratocysts often develop before the basal cell carcinomas appear.

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