Background: Neurofibromatosis type 1 (NF1) is a genetic disorder with skeletal involvement. Periapical cemental dysplasia is a rare finding in the normal population.
Method: A total of 55 patients with NF1, 29 female and 26 male patients, were evaluated with orthopantomograms, supplemented with periapical radiographs if necessary. The vitality of the teeth was measured by two different testing methods.
Results: A novel finding was the occurrence of cemental dysplasia affecting the periapical area of vital mandibular teeth in 8 adult women with NF1. Thus, cemental dysplasia was detected in 34.8% of the adult female NF1 patients, while cemental dysplasia was not present in men or children with NF1.
Conclusion: Periradicular cemental dysplasia is indeed a new NF1 related bone lesion type. Our finding suggests that this is the first reported sexual dimorphism in the manifestations of NF1. Cemental dysplasia of NF1 patients should not be confused with periapical findings caused by endodontic pathoses. The former do not require active therapy whereas in the latter root canal treatment is necessary.
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http://dx.doi.org/10.1016/j.ejmg.2007.04.001 | DOI Listing |
Clin Case Rep
November 2022
Department of Ophthamology, ST.-Johannes-Hospital Dortmund Germany.
Periodontal Ehlers-Danlos syndrome (pEDS) is a rare disorder caused by heterozygous mutations in complement 1 subunit genes C1R and C1S. To date, 148 cases have been described in the literature.We describe a case of a suspected de novo-mutation of pEDS with generalized Periapical cemental dysplasia (PCD) and cerebral leukoencephalopathy.
View Article and Find Full Text PDFJ Korean Assoc Oral Maxillofac Surg
April 2022
Department of Oral and Maxillofacial Surgery, School of Dentistry, Pusan National University, Yangsan, Korea.
Objectives: The purpose of this study was to evaluate risk factors and symptoms in cemento-osseous dysplasia (COD) patients.
Materials And Methods: In this study, 62 patients who were diagnosed histologically with COD were investigated from 2010 to 2020 at the author's institution. We compared clinical and radiological characteristics of symptomatic and asymptomatic patients.
Radiol Case Rep
December 2021
Department of Oral and Maxillofacial Surgery, University Hospital "Carl Gustav Carus", Technische Universität Dresden, Dresden, Germany.
Periapical cemental dysplasia (PCD) is considered a non-neoplastic proliferation of fibrous tissues and cementum-like hard tissues, usually occurring in the periapical regions of teeth. PCD is characterized by the presence of vital pulp and is often accidentally discovered during a general radiographic survey. PCD may arise from the tissue of odontogenic origin or occur as a reactive process in the periapical tissue.
View Article and Find Full Text PDFDentomaxillofac Radiol
January 2021
Department of Oral Pathology and Oral Biology, Faculty of Health Sciences, University of Pretoria, Pretoria, South Africa.
Familial cases of benign fibro-osseous lesions of the jaws are rare and have been described under numerous terms including familial gigantiform cementoma, multiple cemento-ossifying fibromas, sclerotic cemental masses and familial florid cemento-osseous dysplasia. The synonymous and interchangeable use of these terms to describe distinct entities with overlapping features has resulted in confusion and inaccurate categorisation of these lesions. This study highlights three family members with diffuse fibro-osseous jaw lesions with areas of significant expansion.
View Article and Find Full Text PDFOrphanet J Rare Dis
August 2018
Department of Cell Biology and Anatomy, Institute of Biomedicine, University of Turku, Kiinamyllynkatu 10, 20520, Turku, Finland.
Neurofibromatosis type 1 (NF1) is one of the most common inherited syndromes. The literature on craniofacial alterations associated with NF1 has been limited and partially contradictory. This review is based on literature search and the results of the clinical study "Craniofacial and Oral Alterations and Speech in patients with Neurofibromatosis 1", carried out at the University of Turku and Turku University Hospital, Finland in 2006-2012.
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