Background: The nevoid basal cell carcinoma syndrome (NBCCS), first described by Gorlin and Goltz in 1960, is a hereditary autosomal dominant disease with high penetrance and variable expressivity. Almost 70% of patients with NBCCS have some degree of craniofacial anomaly. Among these, the presence of ectopic calcification have been reported but Atlanto-occipital ligament calcification has never been described. Therefore this investigation was carried out to determine the prevalence of atlanto-occipital ligament calcification on lateral x-ray of NBCCS patients aiming to assess the effectiveness of this sign in NBCCS diagnosis. Patients and Methods: Lateral and frontal cephalometric radiographs of 18 patients (11 males and 7 females), aged 8-61 years, with the diagnosis of NBCCS were evaluated for the presence of intracranial calcifications (diaphragma sellae and falx cerebri) and or calcification of the atlanto-occipital ligament. Results: A total of 11 patients presented calcification of atlanto-occipital ligament to various degrees and in three cases this represented the only sign of ectopic calcification. When compared to the other two sites of ossification, atlanto-occipital ligament calcification had a similar prevalence. Conclusion: The calcification of the atlanto-occipital ligament should be considered in addition to the other major criteria for NBCCS diagnosis.

Atlanto-occipital Ligament Calcification: A Novel Sign in Nevoid Basal Cell Carcinoma Syndrome / R., Leonardi; A., Santarelli; Barbato, Ersilia; D., Ciavarella; S., Bolouri; F., Harle; G., Palazzo; L., Lo Muzio. - In: ANTICANCER RESEARCH. - ISSN 0250-7005. - 30:10(2010), pp. 4265-4267.

Atlanto-occipital Ligament Calcification: A Novel Sign in Nevoid Basal Cell Carcinoma Syndrome

BARBATO, Ersilia;
2010

Abstract

Background: The nevoid basal cell carcinoma syndrome (NBCCS), first described by Gorlin and Goltz in 1960, is a hereditary autosomal dominant disease with high penetrance and variable expressivity. Almost 70% of patients with NBCCS have some degree of craniofacial anomaly. Among these, the presence of ectopic calcification have been reported but Atlanto-occipital ligament calcification has never been described. Therefore this investigation was carried out to determine the prevalence of atlanto-occipital ligament calcification on lateral x-ray of NBCCS patients aiming to assess the effectiveness of this sign in NBCCS diagnosis. Patients and Methods: Lateral and frontal cephalometric radiographs of 18 patients (11 males and 7 females), aged 8-61 years, with the diagnosis of NBCCS were evaluated for the presence of intracranial calcifications (diaphragma sellae and falx cerebri) and or calcification of the atlanto-occipital ligament. Results: A total of 11 patients presented calcification of atlanto-occipital ligament to various degrees and in three cases this represented the only sign of ectopic calcification. When compared to the other two sites of ossification, atlanto-occipital ligament calcification had a similar prevalence. Conclusion: The calcification of the atlanto-occipital ligament should be considered in addition to the other major criteria for NBCCS diagnosis.
2010
atlanto-occipital ligament; calcification; gorlin; nbccs; ponticulus posterior
01 Pubblicazione su rivista::01a Articolo in rivista
Atlanto-occipital Ligament Calcification: A Novel Sign in Nevoid Basal Cell Carcinoma Syndrome / R., Leonardi; A., Santarelli; Barbato, Ersilia; D., Ciavarella; S., Bolouri; F., Harle; G., Palazzo; L., Lo Muzio. - In: ANTICANCER RESEARCH. - ISSN 0250-7005. - 30:10(2010), pp. 4265-4267.
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11573/357220
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