Document Detail

Atlanto-occipital ligament calcification: a novel sign in nevoid basal cell carcinoma syndrome.
MedLine Citation:
PMID:  21036751     Owner:  NLM     Status:  MEDLINE    
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.
Rosalia Leonardi; Andrea Santarelli; Ersilia Barbato; Domenico Ciavarella; Susanne Bolouri; Franz Härle; Giuseppe Palazzo; Lorenzo Lo Muzio
Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Anticancer research     Volume:  30     ISSN:  1791-7530     ISO Abbreviation:  Anticancer Res.     Publication Date:  2010 Oct 
Date Detail:
Created Date:  2010-11-01     Completed Date:  2010-12-10     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8102988     Medline TA:  Anticancer Res     Country:  Greece    
Other Details:
Languages:  eng     Pagination:  4265-7     Citation Subset:  IM    
Department of Medical and Surgical Sciences, II Dental Unity, University of Catania, Catania, Italy.
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MeSH Terms
Atlanto-Occipital Joint / metabolism,  pathology*,  radiography
Basal Cell Nevus Syndrome / diagnosis,  metabolism,  pathology*,  radiography
Calcinosis / pathology*,  radiography
Middle Aged
Young Adult

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