Ailumerab Haider A, Aguiar Cassyanne L
Department of Pediatrics, Children's Hospital of The King's Daughters, Eastern Virginia Medical School, Norfolk, VA, USA.
Department of Pediatric Rheumatology, Children's Hospital of The King's Daughters, Eastern Virginia Medical School, Norfolk, VA, USA.
Mod Rheumatol Case Rep. 2023 Jan 3;7(1):298-301. doi: 10.1093/mrcr/rxac058.
Chronic nonbacterial osteomyelitis (CNO) is a chronic, sterile, inflammatory disease. It primarily presents with nonspecific bone pain and swelling but ultimately can cause bone destruction and deformities, if left untreated. The involvement of the cranial bones (apart from the mandible) is rare in CNO. In this report, we present a rare case of CNO affecting facial and cranial bones presenting as facial palsy with a review of the literature about similar affection. A 10-year-old, previously healthy female was initially evaluated for swelling of the left side of her face with slight tenderness on palpation, but no fever. Her complete blood count was unremarkable, her inflammatory markers were elevated (C-reactive protein 7.5 mg/dl and erythrocyte sedimentation rate 104 mm/h), and CT of facial and skull bones and MRI of brain showed a destructive osseous process involving the left maxillary, zygomatic, sphenoid bones and the clivus. Bone biopsy of the left maxilla showed fibrous dysplasia with abscess formation, most consistent with an infectious aetiology (acute osteomyelitis). She was started on oral clindamycin for a 3-month course. The facial swelling improved after starting clindamycin, but on her sixth week of treatment, she developed right-sided Bell's palsy. An MRI of the brain showed hyperenhancement of the right seventh cranial nerve. A month later, she was evaluated for right wrist and knee swelling, pain, and limitation of movement. Skeletal survey and MRI showed multifocal lesions with mixed sclerosis and lucency. Her inflammatory markers continued to be elevated. Another bone biopsy of the right radius showed similar findings of destruction with no evidence of malignancy. She was ultimately diagnosed with CNO. She was started on nonsteroidal anti-inflammatory drugs with gastric protection and regular follow-up. Over more than a year of follow-up, the patient's inflammatory markers remain normal, and joint swelling/limitation has remained in remission. We found five additional cases in the literature that presented with a similar presentation. To our knowledge, our patient is the first reported case in the USA involving the cranial/facial bones apart from the mandible presenting with facial palsy. The affection of the facial bones (apart from the mandible) in CNO is very rare, but the awareness of such a presentation by the clinician is an important aspect of reaching the diagnosis.
慢性非细菌性骨髓炎（CNO）是一种慢性、无菌性炎症性疾病。其主要表现为非特异性骨痛和肿胀，但如果不治疗，最终可导致骨质破坏和畸形。CNO累及颅骨（下颌骨除外）的情况罕见。在本报告中，我们呈现了1例罕见的CNO累及面颅骨并表现为面瘫的病例，并对有关类似情况的文献进行了综述。一名10岁、既往健康的女性最初因左侧面部肿胀且触诊时有轻微压痛而接受评估，但无发热。她的全血细胞计数无异常，炎症指标升高（C反应蛋白7.5mg/dl，红细胞沉降率104mm/h），面部和颅骨CT以及脑部MRI显示左侧上颌骨、颧骨、蝶骨和斜坡有骨质破坏过程。左侧上颌骨活检显示纤维发育异常伴脓肿形成，最符合感染性病因（急性骨髓炎）。她开始口服克林霉素，疗程为3个月。开始使用克林霉素后面部肿胀有所改善，但在治疗的第6周，她出现了右侧贝尔面瘫。脑部MRI显示右侧第七颅神经强化明显。1个月后，她因右手腕和膝关节肿胀、疼痛及活动受限接受评估。骨骼检查和MRI显示有多灶性病变，伴有混合性硬化和透亮区。她的炎症指标持续升高。右侧桡骨的另一次骨活检显示有类似的破坏表现，无恶性证据。她最终被诊断为CNO。她开始使用有胃保护作用的非甾体抗炎药并定期随访。经过一年多的随访，患者的炎症指标保持正常，关节肿胀/活动受限一直处于缓解状态。我们在文献中又发现了5例有类似表现的病例。据我们所知，我们的患者是美国首例报道的除下颌骨外累及颅/面骨并表现为面瘫的病例。CNO累及面颅骨（下颌骨除外）的情况非常罕见，但临床医生对这种表现的认识是做出诊断的一个重要方面。
