Examine your orofacial cleft patients for Gorlin-Goltz syndrome.
The 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.
Lambrecht
J T
JT
Department of Oral Surgery, Radiology, and Medicine, School of Dentistry, University of Basel, Switzerland.
Kreusch
T
T
D000328:Adult; D000539:Alveolar Process / Q000002:abnormalities; D001478:Basal Cell Nevus Syndrome / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy; D001927:Brain Diseases / Q000473:pathology; D002114:Calcinosis / Q000473:pathology; D002471:Cell Transformation, Neoplastic / Q000473:pathology; D002971:Cleft Lip / Q000175:diagnosis*; D002972:Cleft Palate / Q000175:diagnosis*; D003937:Diagnosis, Differential; D005141:Eyelid Diseases / Q000473:pathology; D005145:Face / Q000002:abnormalities*; D019066:Facies; D005260:Female; D005500:Follow-Up Studies; D005530:Foot Deformities / Q000473:pathology; D005624:Frontal Bone / Q000002:abnormalities; D006226:Hand Deformities / Q000473:pathology; D006801:Humans; D006972:Hypertelorism / Q000473:pathology; D015994:Incidence; D007570:Jaw Cysts / Q000473:pathology; D008297:Male; D009056:Mouth Abnormalities / Q000175:diagnosis*; D009666:Nose / Q000002:abnormalities; D010294:Parietal Bone / Q000002:abnormalities; D011378:Prognathism / Q000473:pathology; D013285:Strabismus / Q000473:pathology