J Adv Prosthodont.  2015 Apr;7(2):178-182. 10.4047/jap.2015.7.2.178.

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

• Sakar O
• Aren G
• Mumcu Z
• Unalan F
• Aksakalli
• Tolgay CG

• KMID: 2118250
• DOI: http://doi.org/10.4047/jap.2015.7.2.178

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.

Keyword

Ehlers-Danlos syndrome; Familial gigantiform cementoma; Overlay removable partial dentures; Tooth supported complete dentures

MeSH Terms

Cementoma*
Connective Tissue
Ehlers-Danlos Syndrome
Humans
Jaw
Rehabilitation

Figure

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  Fig. 1 (A) Extraoral view of the patient, (B) Intraoral view of the patient, (C) Panoramic radiography of the patient showing periapical lesions of teeth 36 and 46. Absence of teeth 12, 15, 17, 25, 27, 28 and transposition of maxillary right premolar can be seen.

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  Fig. 2 (A) Extracted mandibular left first molar. (B) Microscopic view of tooth (loop magnification H&E). (C) Mineralized material (M) is observed surrounding the root and at the root apex (Ra) (×40, H&E).

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  Fig. 3 (A) Mandibular framework was prepared using Cr-Co alloy. (B) Maxillary overdentures were fabricated using a heatprocessed silicone liner (Molloplast-B).

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  Fig. 4 (A) Intraoral view after prosthodontic treatment, (B) Extraoral view after prosthodontic treatment.

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  Fig. 5 Extraoral view of the patient before prosthodontic treatment.

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  Fig. 6 Panoramic radiography showing periapical lesions and missing teeth.

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  Fig. 7 (A) Extraoral view of the patient after prosthodontic treatment, (B) Intraoral view of the dentures.

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