Open AccessGorlin-Goltz syndrome
Author(s) -
Monica Kohli,
Monica Kohli,
Naresh Kumar Sharma,
Saif Rauf Siddiqui,
Singel Tulsi
Publication year - 2010
Publication title -
national journal of maxillofacial surgery
Language(s) - English
Resource type - Journals
eISSN - 2229-3418
pISSN - 0975-5950
DOI - 10.4103/0975-5950.69171
Subject(s) - medicine , penetrance , expressivity , scoliosis , spina bifida , surgery , genetics , gene , biology , phenotype
Gorlin-Goltz syndrome is an inherited autosomal dominant disorder with complete penetrance and extreme variable expressivity. The authors present a case of an 11-year-old girl with typical features of Gorlin-Goltz syndrome with special respect to medical and dental problems which include multiple bony cage deformities like spina bifida with scoliosis having convexity to the left side, presence of an infantile uterus and multiple odonogenic keratocysts in the maxillofacial region.