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Examine your orofacial cleft patients for Gorlin-Goltz syndrome.
Journal article

Examine your orofacial cleft patients for Gorlin-Goltz syndrome.

  • Lambrecht JT Department of Oral Surgery, Radiology, and Medicine, School of Dentistry, University of Basel, Switzerland.
  • Kreusch T
  • 1997-07-01
Published in:
  • The Cleft palate-craniofacial journal : official publication of the American Cleft Palate-Craniofacial Association. - 1997
Adult
Alveolar Process
Basal Cell Nevus Syndrome
Brain Diseases
Calcinosis
Cell Transformation, Neoplastic
Cleft Lip
Cleft Palate
Diagnosis, Differential
Eyelid Diseases
Face
Facies
Female
Follow-Up Studies
Foot Deformities
Frontal Bone
Hand Deformities
Humans
Hypertelorism
Incidence
Jaw Cysts
Male
Mouth Abnormalities
Nose
Parietal Bone
Prognathism
Strabismus
English 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.
Language
  • English
Open access status
closed
Identifiers
Persistent URL
https://sonar.ch/global/documents/232008
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