Basal cell nevus syndrome: a case report. 1994

T J Ocholla, and S W Guthua, and S S Kimaro
Division of Oral and Maxillofacial Surgery, University of Nairobi, College of Health Sciences, Kenya.

A case is reported of a 13 year old Kenyan girl who presented at the Kenyatta National Hospital Dental Clinic with multiple mandibular and maxillary cysts, cutaneous lesions and mandibular prognathism. This combination of clinical and radiographic features led to a diagnosis of basal cell nevus syndrome. This paper is the first reported case of the syndrome in Kenya. The significance of thorough clinical inspection and radiographic screening of suspected cases is discussed.

UI MeSH Term Description Entries
D008339 Mandibular Neoplasms Tumors or cancer of the MANDIBLE. Mandibular Neoplasm,Neoplasm, Mandibular,Neoplasms, Mandibular
D008441 Maxillary Neoplasms Cancer or tumors of the MAXILLA or upper jaw. Maxillary Neoplasm,Neoplasm, Maxillary,Neoplasms, Maxillary
D011378 Prognathism A condition marked by abnormal protrusion of the mandible. (Dorland, 27th ed) Prognathisms
D011859 Radiography Examination of any part of the body for diagnostic purposes by means of X-RAYS or GAMMA RAYS, recording the image on a sensitized surface (such as photographic film). Radiology, Diagnostic X-Ray,Roentgenography,X-Ray, Diagnostic,Diagnostic X-Ray,Diagnostic X-Ray Radiology,X-Ray Radiology, Diagnostic,Diagnostic X Ray,Diagnostic X Ray Radiology,Diagnostic X-Rays,Radiology, Diagnostic X Ray,X Ray Radiology, Diagnostic,X Ray, Diagnostic,X-Rays, Diagnostic
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000293 Adolescent A person 13 to 18 years of age. Adolescence,Youth,Adolescents,Adolescents, Female,Adolescents, Male,Teenagers,Teens,Adolescent, Female,Adolescent, Male,Female Adolescent,Female Adolescents,Male Adolescent,Male Adolescents,Teen,Teenager,Youths
D001478 Basal Cell Nevus Syndrome Hereditary disorder consisting of multiple basal cell carcinomas, odontogenic keratocysts, and multiple skeletal defects, e.g., frontal and temporoparietal bossing, bifurcated and splayed ribs, kyphoscoliosis, fusion of vertebrae, and cervicothoracic spina bifida. Genetic transmission is autosomal dominant. Gorlin Syndrome,Nevoid Basal Cell Carcinoma Syndrome,Nevus Syndrome, Basal Cell,Fifth Phacomatosis,Gorlin-Goltz Syndrome,Multiple Basal Cell Nevi, Odontogenic Keratocysts, and Skeletal Anomalies,NBCCS,Fifth Phacomatoses,Gorlin Goltz Syndrome,Syndrome, Gorlin,Syndrome, Gorlin-Goltz

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