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lüll Examine your orofacial cleft patients for Gorlin-Goltz syndrome Lambrecht JT; Kreusch TCleft Palate Craniofac J 1997[Jul]; 34 (4): 342-50The Gorlin-Goltz syndrome is characterized by four primary symptoms: multiple nevoid basal cell epitheliomas that usually undergo malignant transformation; jaw keratocysts that show constant growth; skeletal anomalies; and intracranial calcifications. A myriad of additional findings may also be noted. Among the most frequent are: palmar and plantar pits, a characteristic flattened facies and broad nasal root, frontal and parietal bossing, mandibular prognathia, hypertelorism, strabismus, dystrophia of the canthi, and clefts of the lip, alveolus, and/or palate. In this study, we review the literature and our 25 cases of Gorlin-Goltz syndrome patients, questioning their incidence of cleft formations (8.5%) as compared to the general population (0.1%). It is our contention that all patients who present with an orofacial cleft warrant deeper investigation as to the presence of additional signs indicative of Gorlin-Goltz syndrome. The nevi turn malignant with time, and thus, early diagnosis, follow-up, and treatment are imperative.|Adult[MESH]|Alveolar Process/abnormalities[MESH]|Basal Cell Nevus Syndrome/*diagnosis/pathology/therapy[MESH]|Brain Diseases/pathology[MESH]|Calcinosis/pathology[MESH]|Cell Transformation, Neoplastic/pathology[MESH]|Cleft Lip/*diagnosis[MESH]|Cleft Palate/*diagnosis[MESH]|Diagnosis, Differential[MESH]|Eyelid Diseases/pathology[MESH]|Face/*abnormalities[MESH]|Facies[MESH]|Female[MESH]|Follow-Up Studies[MESH]|Foot Deformities/pathology[MESH]|Frontal Bone/abnormalities[MESH]|Hand Deformities/pathology[MESH]|Humans[MESH]|Hypertelorism/pathology[MESH]|Incidence[MESH]|Jaw Cysts/pathology[MESH]|Male[MESH]|Mouth Abnormalities/*diagnosis[MESH]|Nose/abnormalities[MESH]|Parietal Bone/abnormalities[MESH]|Prognathism/pathology[MESH]|Strabismus/pathology[MESH] |