Original article | Volume 30, Article 196, 05 Jul 2018 | 10.11604/pamj.2018.30.196.16299

A man with facial disfigurement

Phatharaporn Kiatpanabhikul, Thiti Snabboon

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

Received: 09 Jun 2018 - Accepted: 16 Jun 2018 - Published: 05 Jul 2018

Domain: Endocrinology,Maxillofacial surgery

Keywords: Fibrous dysplasia, craniofacial, mccune-albright syndrome

©Phatharaporn Kiatpanabhikul et al. Pan African Medical Journal (ISSN: 1937-8688). This is an Open Access article distributed under the terms of the Creative Commons Attribution International 4.0 License (https://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Cite this article: Phatharaporn Kiatpanabhikul et al. A man with facial disfigurement. Pan African Medical Journal. 2018;30:196. [doi: 10.11604/pamj.2018.30.196.16299]

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

Home | Volume 30 | Article number 196

Original article

A man with facial disfigurement

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

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

Original article

A man with facial disfigurement

Original article

A man with facial disfigurement

Original article

A man with facial disfigurement