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儿童内生软骨瘤病的罕见放射学表现:柱状模式:回顾性队列研究。

Rare radiological manifestation of enchondromatosis in children: Columnar pattern: A retrospective cohort study.

机构信息

Department of Orthopaedics and Traumatology, Istanbul University, Istanbul Faculty of Medicine, Istanbul, Turkey.

Department of Radiology, Istanbul University, Istanbul Faculty of Medicine, Istanbul, Turkey.

出版信息

Medicine (Baltimore). 2024 Jul 26;103(30):e39106. doi: 10.1097/MD.0000000000039106.

Abstract

The columnar cartilage pattern is characterized by parallel aligned cartilage tissue columns related to the physis without matrix calcification separated by the surrounding osseous tissue. Usually, it is seen in patients with multiple enchondromas. The objective of this study was to elucidate the clinical and radiological features of this rare radiological pattern in the physis, which remains unfamiliar to most physician. We retrospectively evaluated the clinical features and imaging findings of 15 patients (9 men and 6 women) who have a columnar pattern with varied spectrum of enchondromatosis. On X-ray and computed tomography (CT) examination, all these lesions were seen as vertical or oblique oriented tubular zones, which have relatively low radiologic density compared with normal bone. The lesions have similar signal characteristics relative to epiphyseal cartilage plates, on T1W and T2W magnetic resonance images. Columnar pattern was observed in different appearances from one single column in one physis to multiple columns in multiple physis. The mean follow-up was 62 months (range: 36-96 months). The mean age was 9.7 (range: 4-14) years at the initial admission. Eight patients had 3 or less affected physis. Five patients had only one affected physis. We defined these patients' group who had up to 3 affected physis as "limited enchondromatosis with columnar pattern (LE-CP)." We observed that most of the columnar cartilage was turning into the normal bone via endochondral ossification. Based on our observations, the columnar pattern is a rare manifestation of the enchondromas. Columnar pattern, along with the related physis, acts as a normal endochondral ossification process, and surgery is not necessary unless there is a risk of fracture or severe deformity. Further awareness of this unique subset of patients may improve our understanding of the disease and lead to better patient outcomes. We have modified non-hereditarily enchondromatosis into 2 categories: limited enchondromatosis with the columnar pattern and multiple enchondromatosis. We believe that LE-CM reflects a developmental anomaly of the physis rather than a true neoplasia, and it acts as a normal endochondral ossification process. Level IV (case series).

摘要

柱状软骨模式的特征是平行排列的软骨组织柱与骺板相关,没有基质钙化,被周围的骨组织分隔。通常见于多发性内生软骨瘤患者。本研究的目的是阐明这种在骺板中罕见的放射学模式的临床和放射学特征,这对大多数医生来说仍然很陌生。我们回顾性评估了 15 名患者(9 名男性和 6 名女性)的临床特征和影像学发现,这些患者具有不同频谱的内生软骨瘤病的柱状模式。在 X 线和计算机断层扫描(CT)检查中,所有这些病变均表现为垂直或斜向管状区域,与正常骨相比,其放射密度相对较低。病变在 T1W 和 T2W 磁共振图像上与骺软骨板具有相似的信号特征。柱状模式在单一骺板中的单个柱状到多个骺板中的多个柱状表现出不同的外观。平均随访时间为 62 个月(范围:36-96 个月)。初次就诊时的平均年龄为 9.7 岁(范围:4-14 岁)。8 名患者有 3 个或更少受累骺板。5 名患者只有一个受累骺板。我们将这些患者中受累骺板数量在 3 个以内的患者定义为“局限性内生软骨瘤伴柱状模式(LE-CP)”。我们观察到,大多数柱状软骨通过软骨内骨化转变为正常骨。基于我们的观察,柱状模式是内生软骨瘤的一种罕见表现。柱状模式与相关骺板一起作为正常的软骨内骨化过程发挥作用,除非存在骨折或严重畸形的风险,否则不需要手术。进一步了解这一独特的患者群体可能会提高我们对疾病的认识,并导致更好的患者结局。我们已经将非遗传性内生软骨瘤病分为两类:局限性内生软骨瘤伴柱状模式和多发性内生软骨瘤病。我们认为 LE-CM 反映了骺板的发育异常,而不是真正的肿瘤,它作为一个正常的软骨内骨化过程发挥作用。IV 级(病例系列)。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e238/11272238/3125376e7a7d/medi-103-e39106-g001.jpg

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