A man with facial disfigurement
Phatharaporn Kiatpanabhikul, Thiti Snabboon
The Pan African Medical Journal. 2018;30:196. doi:10.11604/pamj.2018.30.196.16299

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A man with facial disfigurement

Cite this: The Pan African Medical Journal. 2018;30:196. doi:10.11604/pamj.2018.30.196.16299

Received: 09/06/2018 - Accepted: 16/06/2018 - Published: 05/07/2018

Key words: Fibrous dysplasia, craniofacial, mccune-albright syndrome

© Phatharaporn Kiatpanabhikul et al. The Pan African Medical Journal - ISSN 1937-8688. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Available online at: http://www.panafrican-med-journal.com/content/article/30/196/full

Corresponding author: Thiti Snabboon, Excellence Center in Diabetes, Hormones and Metabolism, King Chulalongkorn Memorial Hospital, Department of Medicine, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand (Thiti.S@chula.ac.th)


A man with facial disfigurement

Phatharaporn Kiatpanabhikul1, Thiti Snabboon2,3,&

 

1Department of Medicine, Charoenkrungpracharak Hospital, Medical Service, Bangkok Metropolitan Administration, Bangkok, Thailand, 2Excellence Center in Diabetes, Hormones and Metabolism, King Chulalongkorn Memorial Hospital, Bangkok, Thailand, 3Department of Medicine, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand

 

 

&Corresponding author
Thiti Snabboon, Excellence Center in Diabetes, Hormones and Metabolism, King Chulalongkorn Memorial Hospital, Department of Medicine, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand

 

 

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A 21-year-old man presented with progressive facial disfigurement along with intermittent pain over his prominent mandible and forehead. Examination revealed asymmetrical facial figures especially the forehead and his left side (A). CT skull demonstrated typical features of fibrous dysplasia (FD) including multiple expansile lesions with heterogeneous pattern including radiolucency interspersed with ground glass opacity involving all craniofacial bones (B). No periosteal reaction or soft tissue involvement is noted. Extensive bone survey and hormonal assessments were unremarkable. The patient preferred conservative treatment with pain relief and undergoing regular follow-up. FD is a benign and progressive disorder in which the bone is replaced and expanded by fibro-osseous tissue, leading to fractures and deformities. It may affect a single (monostotic) or multiple (polyostotic) bones. The latter may be associated with endocrine dysfunctions and cafe' au lait spots (McCune-Albright syndrome) or soft tissue myxomas (Mazabraud syndrome). Craniofacial bone involvement has been reported in about one-fourth of the FD case. Its manifestations are varied depending on the involving bones including painless swelling with facial asymmetry, malocclusion, headache, visual or hearing impairment, nasal obstruction or neurological deficit. Diagnosis is based on the clinical ground and typical radiographic findings. Biopsy is reserved for cases suspicious of malignant change or indeterminate diagnosis. Treatment is aimed to alleviate cosmetic or functional problems, ranging from observation, medical treatment with bisphosphonates, to extensive surgery. Regular follow-up is mandatory to detect disease progression or malignant change.

 

 

Figure 1: A) the patient picture. Note asymmetrical facial figure especially the forehead and left side; B) a CT scan (axial view). Multiple expansile lesions with a heterogeneous pattern, including radiolucency interspersed with ground-glass opacity of craniofacial bones

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 


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Keywords

Fibrous dysplasia
Craniofacial
Mccune-albright syndrome

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