Lame Cheikh Ahmedou, Loum Birame, Diallo Thierno Boubacar, Ndiaye Cheikhna Ba, Diouf Khady Marie Agnès, Fall Amat
Service d´ Otorhinolaryngologie (ORL) et de Chirurgie Cervico-Faciale, Hôpital Principal de Dakar, Dakar, Sénégal.
Département d´Imagerie Médicale, Hôpital Principal de Dakar, Dakar, Sénégal.
Pan Afr Med J. 2020 Nov 25;37:271. doi: 10.11604/pamj.2020.37.271.21350. eCollection 2020.
Fibrous dysplasia (FD) of bone is a benign, congenital and rare disease in which normal bone is replaced by fibrous bone tissue, resulting in bone deformities. It can affect any bone in the body, however craniofacial fibrous dysplasia is characterized by specific clinical manifestations, progression and therapeutic issues. The purpose of our study was to describe the diagnostic, therapeutic and evolutionary features of craniofacial FD. This study involved six patients with craniofacial FD followed up in the Department of Otolaryngology at the Principal Hospital of Dakar. The average age of patients was 26.16 years, ranging from 11 to 58 years. Sex ratio favoured women (83% of the cases). Bone deformity was the main feature of craniofacial FD leading to diagnosis. One patient presented with unilateral nasal obstruction with epistaxis. In all cases, scanner enabled diagnosis and topographic balance. Two female patients underwent surgery. One case of recurrence was reported. Craniofacial FD is a rare bone disease that can manifest as serious sensory and functional disorders. It poses real therapeutic issues; hence adequate interdisciplinar management is essential.
骨纤维异常增殖症(FD)是一种良性、先天性且罕见的疾病,在该疾病中正常骨被纤维骨组织所取代,从而导致骨骼畸形。它可累及身体的任何骨骼,然而颅面部纤维异常增殖症具有特定的临床表现、病情发展过程及治疗问题。我们研究的目的是描述颅面部FD的诊断、治疗及病情发展特征。本研究纳入了在达喀尔主要医院耳鼻喉科接受随访的6例颅面部FD患者。患者的平均年龄为26.16岁,年龄范围在11至58岁之间。性别比有利于女性(占病例的83%)。骨骼畸形是导致颅面部FD诊断的主要特征。1例患者出现单侧鼻塞伴鼻出血。在所有病例中,CT扫描有助于诊断及确定病变部位。2例女性患者接受了手术治疗。报告了1例复发病例。颅面部FD是一种罕见的骨病,可表现为严重的感觉和功能障碍。它带来了实际的治疗问题;因此,充分的多学科管理至关重要。