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Familial gigantiform cementoma with Ehlers - Danlos syndrome: A report of 2 cases.

Şakar O, Aren G, Mumcu Z, Ünalan F, Aksakallı N, Tolgay CG - J Adv Prosthodont (2015)

Bottom Line: Ehlers-Danlos syndrome is an autosomal dominant hereditary disorder of connective tissue, while familial gigantiform cementoma is a condition that usually manifests as multiple radiopaque cementum-like masses throughout the jaws.This case report discusses the oral management and prosthetic rehabilitation of two patients presenting familial gigantiform cementoma with Ehlers-Danlos Syndrome.

View Article: PubMed Central - PubMed

Affiliation: Department of Prosthodontics, School of Dentistry, İstanbul University, İstanbul, Turkey.

ABSTRACT
Ehlers-Danlos syndrome is an autosomal dominant hereditary disorder of connective tissue, while familial gigantiform cementoma is a condition that usually manifests as multiple radiopaque cementum-like masses throughout the jaws. This case report discusses the oral management and prosthetic rehabilitation of two patients presenting familial gigantiform cementoma with Ehlers-Danlos Syndrome.

No MeSH data available.


Related in: MedlinePlus

Extraoral view of the patient before prosthodontic treatment.
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Figure 5: Extraoral view of the patient before prosthodontic treatment.

Mentions: The 38-year-old sister of the first patient also selfreferred to the Istanbul University Faculty of Dentistry. Unlike her brother, she did not have skin hyperelasticity on clinical examination. Head and neck examination showed no abnormalities or facial asymmetry, but the temporomandiular joint did demonstrate hypermobility. A difference between the upper and lower facial height and Class III malocclusion were diagnosed (Fig. 5). Early-onset periodontitis was also noted. Teeth 14, 17, 18, 26, 38, 41 and 45 were missing, besides teeth 36 and 37 were impacted (Fig. 6). The incisors and canines had mottled enamel, and there was only occlusal contact between teeth 15 and 46. Panoramic radiography showed radio-opaque masses scattered throughout the mandible and maxilla. Like her brother, she was diagnosed with familial gigantiform cementoma with EDS type VIII.


Familial gigantiform cementoma with Ehlers - Danlos syndrome: A report of 2 cases.

Şakar O, Aren G, Mumcu Z, Ünalan F, Aksakallı N, Tolgay CG - J Adv Prosthodont (2015)

Extraoral view of the patient before prosthodontic treatment.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4414950&req=5

Figure 5: Extraoral view of the patient before prosthodontic treatment.
Mentions: The 38-year-old sister of the first patient also selfreferred to the Istanbul University Faculty of Dentistry. Unlike her brother, she did not have skin hyperelasticity on clinical examination. Head and neck examination showed no abnormalities or facial asymmetry, but the temporomandiular joint did demonstrate hypermobility. A difference between the upper and lower facial height and Class III malocclusion were diagnosed (Fig. 5). Early-onset periodontitis was also noted. Teeth 14, 17, 18, 26, 38, 41 and 45 were missing, besides teeth 36 and 37 were impacted (Fig. 6). The incisors and canines had mottled enamel, and there was only occlusal contact between teeth 15 and 46. Panoramic radiography showed radio-opaque masses scattered throughout the mandible and maxilla. Like her brother, she was diagnosed with familial gigantiform cementoma with EDS type VIII.

Bottom Line: Ehlers-Danlos syndrome is an autosomal dominant hereditary disorder of connective tissue, while familial gigantiform cementoma is a condition that usually manifests as multiple radiopaque cementum-like masses throughout the jaws.This case report discusses the oral management and prosthetic rehabilitation of two patients presenting familial gigantiform cementoma with Ehlers-Danlos Syndrome.

View Article: PubMed Central - PubMed

Affiliation: Department of Prosthodontics, School of Dentistry, İstanbul University, İstanbul, Turkey.

ABSTRACT
Ehlers-Danlos syndrome is an autosomal dominant hereditary disorder of connective tissue, while familial gigantiform cementoma is a condition that usually manifests as multiple radiopaque cementum-like masses throughout the jaws. This case report discusses the oral management and prosthetic rehabilitation of two patients presenting familial gigantiform cementoma with Ehlers-Danlos Syndrome.

No MeSH data available.


Related in: MedlinePlus