Atak Firat, Koc Irem, Kosemehmetoglu Kemal, Bulut Elif Gunay
Department of Radiology, Mamak State Hospital, Ankara, Turkey.
Department of Ophthalmology, Faculty of Medicine, Hacettepe University, Ankara, Turkey.
Childs Nerv Syst. 2025 Aug 2;41(1):252. doi: 10.1007/s00381-025-06909-y.
Cranial fasciitis (CF) is a rare benign myofibroblastic proliferation predominantly affecting the pediatric skull, often mimicking malignant processes radiologically. While CF is well described pathologically, its imaging characteristics remain underreported, especially in orbital involvement.
We retrospectively reviewed the literature through PubMed and Scopus using the search terms "cranial fasciitis" and "nodular fasciitis AND orbit." We included only cases localized to the craniofacial region. Demographic, imaging, anatomical origin, and follow-up data were extracted. We categorized CF into five distinct types based on tissue involvement. Additionally, we report a new case of CF with orbital extension in a 32-month-old male, including detailed imaging and histopathologic findings.
A total of 142 CF cases were identified across 73 studies, including our case. Median age at diagnosis was 2 years, with a male predominance (1.5:1). Most cases presented with subcutaneous scalp masses (88.7%), often associated with bone erosion or intracranial extension. Only four cases involved the orbit. The most common imaging type was subcutaneous mass with calvarial destruction (45.1%). MRI findings typically included T2 hyperintensity, T1 hypo- to isointensity, marked contrast enhancement, and facilitated diffusion. Recurrence was rare (7.3%).
This study represents the largest review of CF to date and is the first to systematically describe imaging subtypes. Orbital involvement, while rare, may mimic aggressive lesions and lead to misdiagnosis. Recognizing imaging patterns of CF is essential for accurate diagnosis, avoiding overtreatment, and guiding appropriate surgical management in pediatric patients.
颅骨筋膜炎(CF)是一种罕见的良性肌纤维母细胞增生性疾病,主要累及小儿颅骨,在影像学上常酷似恶性病变。虽然CF在病理学上已有充分描述,但其影像学特征报道较少,尤其是在眼眶受累方面。
我们通过PubMed和Scopus数据库,使用“颅骨筋膜炎”和“结节性筋膜炎与眼眶”等检索词对文献进行了回顾性研究。仅纳入局限于颅面部区域的病例。提取了人口统计学、影像学、解剖学起源及随访数据。我们根据组织受累情况将CF分为五种不同类型。此外,我们报告了一例32个月大男性患有眼眶扩展型CF的新病例,包括详细的影像学和组织病理学发现。
共纳入73项研究中的142例CF病例,包括我们的病例。诊断时的中位年龄为2岁,男性居多(1.5:1)。大多数病例表现为头皮皮下肿块(88.7%),常伴有骨质侵蚀或颅内扩展。仅4例累及眼眶。最常见的影像学类型是伴有颅骨破坏的皮下肿块(45.1%)。MRI表现通常包括T2高信号、T1低信号至等信号、明显强化及扩散受限。复发罕见(7.3%)。
本研究是迄今为止对CF最大规模的综述,也是首次系统描述影像学亚型。眼眶受累虽罕见,但可能酷似侵袭性病变并导致误诊。认识CF的影像学表现对于小儿患者的准确诊断、避免过度治疗及指导恰当的手术管理至关重要。