Transconjunctival Orbital Decompression in Cherubism

Cherubism is a rare fibro-osseous disease characterized by the progressive expansion of the mandible and maxilla during childhood. Orbital involvement occurs in a subset of patients and is clinically manifested as upward displacement of the affected eye. The bony changes tend to spontaneously diminish or even regress after puberty. The authors report a case of a young adult female who underwent craniofacial correction of the maxilla and mandible changes but continued to have unilateral scleral show. CT scans of the orbits revealed a residual mass in the right orbit, associated with superior displacement of the inferior orbital rim. The abnormal relationship between the eye and eyelid margin was fully corrected through mass debulking combined with inferomedial orbital decompression.

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Publication Details

Journal
Ophthalmic Plastic and Reconstructive Surgery
Published
2024-12-27
DOI
https://doi.org/10.1097/iop.0000000000002878
Primary Topic
Bone Tumor Diagnosis and Treatments
Type
article
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article

Transconjunctival Orbital Decompression in Cherubism

Talissa Feltrini, Antônio Augusto Velasco e Cruz, Patrícia Akaishi
Ophthalmic Plastic and Reconstructive Surgery
Bone Tumor Diagnosis and Treatments
article

Transconjunctival Orbital Decompression in Cherubism

Talissa Feltrini, Antônio Augusto Velasco e Cruz, Patrícia Akaishi
article en

Abstract

Cherubism is a rare fibro-osseous disease characterized by the progressive expansion of the mandible and maxilla during childhood. Orbital involvement occurs in a subset of patients and is clinically manifested as upward displacement of the affected eye. The bony changes tend to spontaneously diminish or even regress after puberty. The authors report a case of a young adult female who underwent craniofacial correction of the maxilla and mandible changes but continued to have unilateral scleral show. CT scans of the orbits revealed a residual mass in the right orbit, associated with superior displacement of the inferior orbital rim. The abnormal relationship between the eye and eyelid margin was fully corrected through mass debulking combined with inferomedial orbital decompression.

Ophthalmic Plastic and Reconstructive SurgeryVol. 41(4)
Universidade de São Paulo (BR)
Openalex Percentile: Top 58%
Bone Tumor Diagnosis and Treatments
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