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Severe Cherubism Treated with Curettage, Osteotomy, and Bony Repositioning: A Case Series of Three Patients

Cherubism is a rare, autosomal dominant condition characterized by the replacement of medullary bone by fibro-osseous lesions, predominantly in the bilateral maxillae and/or mandibles. The clinical presentation of cherubism can vary widely, from clinically undetectable to severe facial disfigurement...

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Autores principales: Moss, Whitney, Pires, Giovanna, Zahid, Rehan, Tyrell, Richard, Rhemtulla, Irfan, Gociman, Barbu
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Lippincott Williams & Wilkins 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8849314/
https://www.ncbi.nlm.nih.gov/pubmed/35186633
http://dx.doi.org/10.1097/GOX.0000000000004079
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author Moss, Whitney
Pires, Giovanna
Zahid, Rehan
Tyrell, Richard
Rhemtulla, Irfan
Gociman, Barbu
author_facet Moss, Whitney
Pires, Giovanna
Zahid, Rehan
Tyrell, Richard
Rhemtulla, Irfan
Gociman, Barbu
author_sort Moss, Whitney
collection PubMed
description Cherubism is a rare, autosomal dominant condition characterized by the replacement of medullary bone by fibro-osseous lesions, predominantly in the bilateral maxillae and/or mandibles. The clinical presentation of cherubism can vary widely, from clinically undetectable to severe facial disfigurement. Although there are no established management guidelines for this condition, conservative management with observation is typically favored in most cases due to the possibility of spontaneous regression following puberty. In this article, we present three cases of moderate to severe cherubism managed with early surgical intervention utilizing curettage and osteotomy followed by bony repositioning. We aimed to show the feasibility and safety of this minimally invasive surgical technique in the management of moderate to severe cases of cherubism to provide improvement in patient quality of life, aesthetics, and function while also possibly mitigating the need for later reconstructive surgery.
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spelling pubmed-88493142022-02-18 Severe Cherubism Treated with Curettage, Osteotomy, and Bony Repositioning: A Case Series of Three Patients Moss, Whitney Pires, Giovanna Zahid, Rehan Tyrell, Richard Rhemtulla, Irfan Gociman, Barbu Plast Reconstr Surg Glob Open Craniofacial/Pediatric Cherubism is a rare, autosomal dominant condition characterized by the replacement of medullary bone by fibro-osseous lesions, predominantly in the bilateral maxillae and/or mandibles. The clinical presentation of cherubism can vary widely, from clinically undetectable to severe facial disfigurement. Although there are no established management guidelines for this condition, conservative management with observation is typically favored in most cases due to the possibility of spontaneous regression following puberty. In this article, we present three cases of moderate to severe cherubism managed with early surgical intervention utilizing curettage and osteotomy followed by bony repositioning. We aimed to show the feasibility and safety of this minimally invasive surgical technique in the management of moderate to severe cases of cherubism to provide improvement in patient quality of life, aesthetics, and function while also possibly mitigating the need for later reconstructive surgery. Lippincott Williams & Wilkins 2022-01-27 /pmc/articles/PMC8849314/ /pubmed/35186633 http://dx.doi.org/10.1097/GOX.0000000000004079 Text en Copyright © 2022 The Authors. Published by Wolters Kluwer Health, Inc. on behalf of The American Society of Plastic Surgeons. https://creativecommons.org/licenses/by-nc-nd/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND) (https://creativecommons.org/licenses/by-nc-nd/4.0/) , where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal.
spellingShingle Craniofacial/Pediatric
Moss, Whitney
Pires, Giovanna
Zahid, Rehan
Tyrell, Richard
Rhemtulla, Irfan
Gociman, Barbu
Severe Cherubism Treated with Curettage, Osteotomy, and Bony Repositioning: A Case Series of Three Patients
title Severe Cherubism Treated with Curettage, Osteotomy, and Bony Repositioning: A Case Series of Three Patients
title_full Severe Cherubism Treated with Curettage, Osteotomy, and Bony Repositioning: A Case Series of Three Patients
title_fullStr Severe Cherubism Treated with Curettage, Osteotomy, and Bony Repositioning: A Case Series of Three Patients
title_full_unstemmed Severe Cherubism Treated with Curettage, Osteotomy, and Bony Repositioning: A Case Series of Three Patients
title_short Severe Cherubism Treated with Curettage, Osteotomy, and Bony Repositioning: A Case Series of Three Patients
title_sort severe cherubism treated with curettage, osteotomy, and bony repositioning: a case series of three patients
topic Craniofacial/Pediatric
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8849314/
https://www.ncbi.nlm.nih.gov/pubmed/35186633
http://dx.doi.org/10.1097/GOX.0000000000004079
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