Inherited craniofacial fibrous dysplasia

doi:10.1016/0030-4220(85)90263-4

Oral Surgery, Oral Medicine, Oral Pathology

Volume 60, Issue 4, October 1985, Pages 403-409

Oral Surgery, Oral M...

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Abstract

Craniofacial fibrous dysplasia occurring in a mother and two of her three daughters is reported. In all three patients, the condition was characterized by extensive bilateral and bimaxillary involvement. All patients exhibited radiographic evidence of abnormal maxillary and mandibular bone and tooth displacement. Morphologic examination of deciduous teeth from the daughters showed replacement of normal pulp tissue by fibro-osseous lesion. Because of the familial occurrence of the cases, it is proposed that they comprise an example of inherited craniofacial fibrous dysplasia.

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Cited by (11)

Healing of a Fibrous Dysplastic Lesion in a Permanent Molar after Endodontic Therapy
2007, Journal of Endodontics
Fibrous dysplasia presents in two forms: monostotic and polyostotic. Both forms are more widespread among children and juveniles and may result in facial asymmetry. Neoplastic bone lesions, localized over the root apices and mimicking periapical pathosis, have been observed. Irregular pulp morphology not previously reported is described in the following case report.
A 14-year-old boy diagnosed with fibrous dysplasia was referred for endodontic treatment as the result of a necrotic pulp in an upper molar. A periapical lesion was diagnosed when the tooth was intact. After complex root canal treatment the periapical lesion healed.
Clinical and radiological evaluation of cherubism: A sporadic case report and review of the literature
2003, International Journal of Pediatric Otorhinolaryngology
Many cases have been published on cherubism since Jones described it first time in three children of the same family [Am. J. Cancer 17 (1933) 946]. Cherubism is an autosomal-dominant disorder in which the normal bone is replaced by cellular fibrous tissue and immature bone. Extracranial skeletal involvement is rarely seen in hereditary and non-hereditary forms of the disorder. The mandible is the most severely affected craniofacial component, in which uncontrolled growth of the malady deteriorates the aesthetic balance of the face. Bilateral swelling of the cheeks, mandibular enlargement and maxillary spongious hypertrophy cause orbital manifestations and tendency of eyes looking up to the sky. Thus, the pathognomic clinical feature resembles the appearance of “raised to heaven’’ Renaissance cherubs. The sporadic case concerns a child affected by cherubism. Radiographic and clinical data of the patient are presented and brief review of the literature is included.
Fifteen-year follow-up of a family with inherited craniofacial fibrous dysplasia
1996, Journal of Oral and Maxillofacial Surgery
Fibrous dysplasia and cherubism as an hereditary familial disease. Follow-up of four generations
1989, Journal of Cranio-Maxillofacial Surgery
Five cases of dysplasia of the jaws in one family which has been under our observation since 1970 are reported. The disease appeared as a mixed display of jaw lesions, in some members as fibrous dysplasia and in others as cherubism. We were able to trace the disorder through an unbroken line of four generations, and thus to demonstrate autosomal dominant inheritance. Cytogenetic analysis performed on three members of this family revealed a significantly increased rate of chromosomal breakage.
Successive generations with inherited craniofacial fibrous dysplasia
2012, Oral Radiology
Autosomal dominant mesomandibular fibro-osseous dysplasia: A self-resolving inherited fibro-osseous lesion of the jaws
2012, Frontiers in Physiology

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Inherited craniofacial fibrous dysplasia

Abstract

Orthop Clin North Am

Br J Oral Surg

Am J Surg

Oral Surg Oral Med Oral Pathol

Int J Oral Surg

Benign fibro-osseous enlargements of the jaws

Br Dent J

Fibro-osseous lesions of the jaws

J Oral Surg

Fibrous dysplasia of the jaws

Arch Otolaryngol

Histological typing of bone tumours, international histological classification of tumours

Fibrous dysplasia of bone

Arch Pathol

Fibrous dysplasia of single bones (monostotic fibrous dysplasia)

Milit Surg

Polyostotic fibrous dysplasia: review of the literature with two additional cases

Radiology

Fibrous dysplasia of maxilla and mandible: clinicopathologic study and comparison of facial bone lesions with lesions affecting general skeleton

J Oral Surg

Polyostotic fibrous dysplasia

Arch Surg

Syndrome characterized by osteitis fibrosa disseminata, areas of pigmentation and endocrine dysfunction, with precocious puberty in females; report of five cases

N Engl J Med