Abstract
Gorlin-Goltz syndrome, is an uncommon, autosomal dominant inherited disorder, which is characterized by numerous basal cell carcinoma (known as nevoid basal cell carcinoma syndrome (NBCCS)). The common manifestations include multiple odontogenic keratocysts in the jaws are usually one of the first manifestations of the syndrome, macrocephaly, frontal boosing, multiple basal cell nevi on the skin with an early age onset, palmar pits, bifid or splayed ribs, high arched palate, calcified diaphragm sellae.
Highlights
Gorlin-Goltz syndrome (GGS) is an uncommon, autosomal dominant inherited disorder known as nevoid basal cell carcinoma syndrome (NBCCS) [1]
This syndrome characterized mainly by the presence of multiple basal cell carcinomas, odontogenic keratocysts of the jaw and palmar and plantar epidermal pits associated with a wide spectrum of developmental anomalies and neoplasms [2], hypertelorism, calcification of falx cerebri, palmer and planter pits, central nervous system, ocular lesions, rib anomalies, mandibular prognathism, cleft lip and palate Other findings of this syndrome in rare cases ovarian fibromas [3]
Diagnosis In the case of NBCCS it is of great importance to reduce the severity of complications, such as malignant skin and brain tumors and maxillofacial deformities related to the jaw cysts can be avoided [4]
Summary
Gorlin-Goltz syndrome (GGS) is an uncommon, autosomal dominant inherited disorder known as nevoid basal cell carcinoma syndrome (NBCCS) [1] This syndrome characterized mainly by the presence of multiple basal cell carcinomas, odontogenic keratocysts of the jaw and palmar and plantar epidermal pits associated with a wide spectrum of developmental anomalies and neoplasms [2], hypertelorism, calcification of falx cerebri, palmer and planter pits, central nervous system, ocular lesions, rib anomalies, mandibular prognathism (class III jaw relationship), cleft lip and palate Other findings of this syndrome in rare cases ovarian fibromas [3]. Temporal bossing, hypertelorism [Increased intercanthal distance 38mm normal is 32mm], wide nasal bridge and thick eye brows (Figure 1). Based on clinical history and radiographic findings the case was provisionally diagnosed as Gorlin Goltz syndrome. B) Congenital anomalies (cleft lip or palate, frontal bossing, coarse facies, and moderate or severe hypertelorism) c) Other skeletal anomalies (Sprengel deformity, marked pectus deformity, and marked syndactyly of the digits) d) Radiologic anomalies (such as bridging of the sellaturcica, vertebral anomalies, modeling defects of the hands and feet, or flame-shaped lucencies of the hands and the feet) e) Medulloblastoma, seizures, mental retardation, meningioma
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