Cherubism: A rare case report

There is no author summary for this article yet. Authors can add summaries to their articles on ScienceOpen to make them more accessible to a non-specialist audience.

Abstract

Cherubism is a rare congenital disease resulting in malformation of the jaw. It occurs before the age of 5 years and regress spontaneously after puberty. It can result into enlargement of the jaw bone, tooth displacement, facial disfigurement and psychological trauma to patient. Hence, the understanding about the condition, its progression and management is necessary.

Related collections

Most cited references 14

Record: found
Abstract: found
Article: found

Is Open Access

Cherubism: best clinical practice

Maria E Papadaki, Steven A Lietman, Michael Levine … (2012)

Cherubism is a skeletal dysplasia characterized by bilateral and symmetric fibro-osseous lesions limited to the mandible and maxilla. In most patients, cherubism is due to dominant mutations in the SH3BP2 gene on chromosome 4p16.3. Affected children appear normal at birth. Swelling of the jaws usually appears between 2 and 7 years of age, after which, lesions proliferate and increase in size until puberty. The lesions subsequently begin to regress, fill with bone and remodel until age 30, when they are frequently not detectable. Fibro-osseous lesions, including those in cherubism have been classified as quiescent, non-aggressive and aggressive on the basis of clinical behavior and radiographic findings. Quiescent cherubic lesions are usually seen in older patients and do not demonstrate progressive growth. Non-aggressive lesions are most frequently present in teenagers. Lesions in the aggressive form of cherubism occur in young children and are large, rapidly growing and may cause tooth displacement, root resorption, thinning and perforation of cortical bone. Because cherubism is usually self-limiting, operative treatment may not be necessary. Longitudinal observation and follow-up is the initial management in most cases. Surgical intervention with curettage, contouring or resection may be indicated for functional or aesthetic reasons. Surgical procedures are usually performed when the disease becomes quiescent. Aggressive lesions that cause severe functional problems such as airway obstruction justify early surgical intervention.

0 comments Cited 34 times – based on 0 reviews      Review now

Bookmark

Record: found
Abstract: found
Article: found

Is Open Access

Cherubism: Clinicoradiographic Features and Treatment

Gabriela Lima, Janete Almeida, Luiz Antonio Guimarães Cabral (2010)

ABSTRACT Objectives Cherubism is a congenital childhood disease of autosomal dominant inheritance. This disease is characterized by painless bilateral enlargement of the jaws, in which bone is replaced with fibrous tissue. The condition has sui generis clinical, radiographic and histological features, of which the clinician should be aware for a better differential diagnosis in the presence of a fibro-osseous lesion affecting the bones of the maxillomandibular complex. The purpose of present paper was to review the literature and to report the most important aspects of cherubism in order to facilitate the study of this disease Material and Methods Literature was reviewed about cherubism, emphasizing the relevant clinicoradiographic features and treatment. Literature was selected through a search of PubMed and Scielo electronic databases. The keywords used for search were adolescent, cherubism, cherubism/physiopathology, cherubism/treatment, cherubism/radiography. A manual search of the reference lists of the identified articles and the authors' article files and recent reviews was conducted to identify additional publications. Those studies that described new features about cherubism were included in this review. Results In total 44 literature sources were obtained and reviewed. Studies that described new features about cherubism physiopathology, diagnostics and treatment were reviewed. Conclusions Despite the exceptions, cherubism is a clinically well-characterized disease. In cases of a suspicion of cherubism, radiographic examination is essential since the clinical presentation, the location and distribution of the lesions may define the diagnosis. Histopathological examination is complementary. Nowadays, genetic tests should be used for final diagnosis of cherubism.

0 comments Cited 7 times – based on 0 reviews      Review now

Bookmark

Record: found
Abstract: found
Article: not found

An extreme case of cherubism.

Maria Silva, Andrew Barreto, Armando Palma … (2002)

We describe an 8-year-old boy who presented with severe facial swelling. This progressed rapidly and 17 months later he died of gastrointestinal and pulmonary infections. The diagnosis was initially brown tumour associated with hyperparathyroidism, but this was revised in the light of laboratory investigations that were within the reference ranges, and normal appearance of the parathyroids on exploration to that of an extreme case of cherubism that behaved in a locally aggressive manner. Copyright 2002 The British Association of Oral and Maxillofacial Surgeons.

0 comments Cited 6 times – based on 0 reviews      Review now

Bookmark

All references

Author and article information

Journal

Journal ID (nlm-ta): J Nat Sci Biol Med

Journal ID (iso-abbrev): J Nat Sci Biol Med

Journal ID (publisher-id): JNSBM

Title: Journal of Natural Science, Biology, and Medicine

Publisher: Medknow Publications & Media Pvt Ltd (India )

ISSN (Print): 0976-9668

ISSN (Electronic): 2229-7707

Publication date (Print): Jul-Dec 2014

Volume: 5

Issue: 2

Pages: 488-491

Affiliations

[1 ] Department of Oral Pathology, Raj Rajeswari Dental College, Udaipur, Rajasthan, India

[2 ] Department of Oral Pathology, Raj Rajeswari Dental College, Udaipur, Rajasthan, India

[3 ] Department of Pedodontics, Vyas Dental College, Jodhpur, Rajasthan, India

[4 ] Department of Orthodontics, Maharana Pratap Dental College, Kanpur, Utter Pradesh, India

[5 ] Department of Oral Pathology, Guardian Dental College, Ambernath, Maharastra, India

[6 ] Department of Prosthodontics, MIDSR Dental College, Latur, Maharastra, India

Author notes

Address for correspondence: Dr. Prashant Babaji, Department of Pedodontics, Vyas Dental College, Jodhpur - 342 001, Rajasthan, India. E-mail: babajipedo@ 123456rediffmail.com

Article

Publisher ID: JNSBM-5-488

DOI: 10.4103/0976-9668.136283

PMC ID: 4121945

PubMed ID: 25097445

SO-VID: eb13ef8a-91da-4b8f-b413-bfbe61a46930

License:

This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3.0 Unported, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Cherubism: A rare case report

Read this article at

Abstract

Related collections

Ticks and tick-borne pathogens

Most cited references 14

Cherubism: best clinical practice

Cherubism: Clinicoradiographic Features and Treatment

An extreme case of cherubism.

Author and article information

Journal

Affiliations

Author notes

Article

History

Categories

Comments

Comment on this article

Similar content 30

Cited by 5

Most referenced authors 42