CONGENITAL DISEASE AND SYNDROMES - Radiological Guide

 GORLIN SYNDROME (Basal Nevus Cell Carcinoma Syndrome)

⭐IMPORTANT POINTS TO PONDER

Gorlin syndrome is an autosomal dominant neurocutaneous (phakomatosis) disease characterized by a triad of multiple basal cell nevi, jaw odontogenic keratocysts, and skeletal anomalies. 

🔹An odontogenic (primordial) keratocyst is a cyst that arises from a tooth before its calcification.

🔹It is most commonly located posterior to the premolar region of the mandible or maxilla.

🔹In the mandible, it mostly affects the third molar tooth.

🔹In men, the disease causes hypogonadism, while in women it causes calcified ovarian fibroids.