Craniofacial Fibrous Dysplasia: Experience at San José Hospital, Bogotá, Colombia

Author:

Cantini J. Ernesto1,Vergel M. Fernanda2,Tapiero Xiomara3,Gómez-Ortega Viviana4

Affiliation:

1. University Foundation of Health Sciences—FUCS, Hospital de San José. Bogota, Colombia

2. Fundación Universitaria de Ciencias de la Salud—FUCS, Hospital de San José. Bogota, Colombia

3. Fundación Universitaria de Ciencias de la Salud—FUCS, Hospital de San José. Bogotá, Colombia

4. Burn Unit of the Fundación Santa Fe de Bogota, Colombia

Abstract

Introduction: Fibrous dysplasia is a disorder in which normal bone is gradually replaced by immature fibro-osseous tissue, with an incidence of less than 7% of all benign bone tumors. The management of this disease is a challenge for plastic surgeons and neurosurgeons. Goal: To describe the diagnostic, therapeutic, and outcome approach of patients with craniofacial fibrous dysplasia seen at the Plastic Surgery Service of the Hospital San José in Bogotá, Colombia. Methods: This is a descriptive and retrospective case series study of patients diagnosed with monostotic and polyostotic fibrous dysplasia treated at the Plastic Surgery Department of Hospital San José during the period from January 1, 2010, to July 31, 2023. Results: All (n=10) of the patients had monostotic craniofacial fibrous dysplasia. The most affected bones in patients with monostotic fibrous dysplasia were zone I bones (n=10, 100%), followed by zone II bones (n=2, 20%). Patients with zone I and II involvement manifested throbbing headaches associated with phosphenes and tinnitus (n=8, 80%) and pain during occlusion associated with edema in the affected cheek (n=5, 50%). Physical examination showed that patients with orbital wall involvement (zone I bone) had ocular dystopia (n=7, 70%). Regarding the treatment received by the patients, 90% (n=9) of the patients received surgical management as primary treatment, with orbitotomy, replacement, and/or remodeling of the roof and lateral wall of the orbit with bone graft, drilling, canthoplasty, ciliary suspension being the most frequently performed procedure (n=6, 60%). Of the patients, 20% (n=2) required reintervention. Conclusions: FD is a slowly progressive benign fibro-osseous disease that requires a timely, individualized, and multidisciplinary diagnosis and treatment to obtain favorable clinical and surgical results. The mainstay of treatment is surgery as a preventive measure since it is important to avoid future functional alterations that, depending on the location of the dysplasia, would cause a high risk of alteration of adjacent structures.

Publisher

Ovid Technologies (Wolters Kluwer Health)

Reference6 articles.

1. Fibrous dysplasia pathophysiology, evaluation, and treatment;DiCaprio;J Bone Jt Surg,2005

2. Displasia fibrosa craneofacial;Rojas;An Radiol México,2003

3. Craniofacial fibrous dysplasia;Ricalde;Oral Maxillofac Surg Clin North Am,2012

4. Medical therapy in adults with fibrous dysplasia of bone;Chapurlat;J Bone Miner Res,2006

5. Our experience in the surgical management of craniofacial fibrous dysplasia: what has changed in the last 10 years?;Valentini;Acta Otorhinolaryngol Ital,2017

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