Taylor Tristen Noble, Bridges Callie Shawhan, Ezeokoli Ekene Uchenna, Smith Tyler Scott, Montgomery Nicole Irene
Baylor College of Medicine, Houston, TX.
Texas Children's Hospital, Houston, TX.
J Pediatr Soc North Am. 2024 Feb 12;5(1):575. doi: 10.55275/JPOSNA-2023-575. eCollection 2023 Feb.
Chronic Recurrent Multifocal Osteomyelitis (CRMO) is a rare aseptic autoinflammatory disease with a wide and vague clinical presentation that often mimics infection, malignancy, or benign conditions, leading to a delayed diagnosis. We aimed to evaluate the clinical characteristics, differential diagnoses from evaluating pediatric orthopaedic surgeons, and compared the number of patients that could have avoided a biopsy in 80 patients with CRMO. Children diagnosed with CRMO at a single tertiary pediatric hospital in the United States between 2012 and 2022 who were evaluated by a pediatric orthopaedic surgeon were retrospectively reviewed. The differential diagnoses from the surgeons were recorded from their initial presentation. The Jansson criteria and Bristol Criteria were retrospectively applied to evaluate patients who could have been spared a biopsy. 80 children (65% female) with CRMO were identified. The mean age at diagnosis was 10.28 ± 3.52 years, follow-up of 37.13 ± 27.67 months, and delay in diagnosis of 6.21 ± 9.75 months. Common presenting symptoms were antalgic gait (45%), local inflammation (30%), and fever/fatigue (26.25%). 58% presented clinically with unifocal symptoms, but 81% had multifocal disease on imaging. Radiographs were unremarkable in 35%, had periosteal reaction/sclerosis (23%), or a lytic lesion (22%). On MRI, 72% of patients had marrow edema, periosteal reaction (23%), and/or osteitis (19%). 69% of patients received a whole-body MRI, and 75% received a bone biopsy. The femur, tibia, pelvis, and spine were involved in >30% of patients. The most common initial differential diagnoses were related to infection (34%) and neoplasm (21%). The Jansson criteria was found to be more sensitive than the Bristol criteria for diagnosing CRMO (OR 3.94, P < 0.001) and identified 80% that could have been spared biopsy. This cohort of 80 patients with CRMO in the U.S. displayed an ambiguous presentation. Whole-body MRI was useful for identifying multifocal lesions. In conjunction with clinical reasoning, the Jansson criteria may be useful in the diagnosis of CRMO and perhaps avoid an unnecessary bone biopsy. Level IV •Chronic Recurrent Multifocal Osteomyelitis is a rare condition in children with a delay in definitive diagnosis of approximately 6 months after initial presentation.•Chronic Recurrent Multifocal Osteomyelitis often has unremarkable radiographs and MRI findings similar to a neoplasm or infectious osteomyelitis.•The most common lesions were found in the femur, tibia, pelvis, and spine.•Pediatric orthopaedic surgeons evaluating these patients placed neoplasm/malignancy and infection on their differential most frequently.•The Jansson criteria may be used in some patients to possibly prevent unnecessary bone biopsies.
慢性复发性多灶性骨髓炎(CRMO)是一种罕见的无菌性自身炎症性疾病,临床表现广泛且不明确,常类似感染、恶性肿瘤或良性疾病,导致诊断延迟。我们旨在评估其临床特征、小儿骨科医生的鉴别诊断情况,并比较80例CRMO患者中可避免活检的患者数量。对2012年至2022年间在美国一家三级小儿专科医院被诊断为CRMO且由小儿骨科医生进行评估的儿童进行回顾性研究。记录医生从患者初诊时做出的鉴别诊断。回顾性应用扬松标准和布里斯托尔标准来评估可避免活检的患者。共识别出80例CRMO患儿(65%为女性)。诊断时的平均年龄为10.28±3.52岁,随访时间为37.13±27.67个月,诊断延迟时间为6.21±9.75个月。常见的首发症状为疼痛性步态(45%)、局部炎症(30%)以及发热/疲劳(26.25%)。58%的患者临床表现为单灶症状,但81%的患者影像学检查显示为多灶性病变。35%的患者X线片无明显异常,23%有骨膜反应/骨质硬化,22%有溶骨性病变。在磁共振成像(MRI)上,72%的患者有骨髓水肿、骨膜反应(23%)和/或骨炎(19%)。69%的患者接受了全身MRI检查,75%的患者接受了骨活检。超过30%的患者股骨、胫骨、骨盆和脊柱受累。最常见的初始鉴别诊断与感染(34%)和肿瘤(21%)有关。发现扬松标准在诊断CRMO方面比布里斯托尔标准更敏感(比值比3.94,P<0.001),并识别出80%可避免活检的患者。这组80例美国CRMO患者表现不明确。全身MRI有助于识别多灶性病变。结合临床推理,扬松标准可能有助于CRMO的诊断,或许可避免不必要的骨活检。IV级•慢性复发性多灶性骨髓炎在儿童中较为罕见,确诊延迟约为初诊后6个月。•慢性复发性多灶性骨髓炎的X线片和MRI表现通常不明显,类似于肿瘤或感染性骨髓炎。•最常见的病变部位是股骨、胫骨、骨盆和脊柱。•评估这些患者的小儿骨科医生最常将肿瘤/恶性肿瘤和感染列入鉴别诊断。•扬松标准可用于部分患者,可能避免不必要的骨活检。