In:
The Cleft Palate-Craniofacial Journal, SAGE Publications, Vol. 34, No. 4 ( 1997-07), p. 342-350
Abstract:
The Gorlin-Goltz syndrome is characterized by tour primary symptoms: multiple nevold 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 facles 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.
Type of Medium:
Online Resource
ISSN:
1055-6656
,
1545-1569
DOI:
10.1597/1545-1569_1997_034_0341_eyocpf_2.3.co_2
Language:
English
Publisher:
SAGE Publications
Publication Date:
1997
detail.hit.zdb_id:
2030056-6
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