Chen Chen, Yan Hanlei, Yao Wei, Wang Zuopeng, Li Kai
Department of Pediatric Surgery, Children's Hospital of Fudan University, National Children's Medical Center, Shanghai, China.
Department of Pediatric Surgery, The Second Affiliated Hospital, Xi'an Jiaotong University, Xi'an, China.
J Pediatr Surg. 2025 Feb;60(2):161932. doi: 10.1016/j.jpedsurg.2024.161932. Epub 2024 Sep 28.
This study generalized and analyzed the clinical attributes observed in patients afflicted with Kaposiform hemangioendothelioma (KHE) with the aim of elucidating the risk factors contributing to the manifestation of Kasabach-Merritt phenomenon (KMP).
We retrospectively analyzed 96 pediatric cases diagnosed with KHE at the Children's Hospital of Fudan University from January 2013 to December 2021. Among them, 62 patients (65%) showed KMP (KHE + KMP group), while 34 patients (35%) did not (KHE-KMP group). The risk factors for KMP associated with KHE were analyzed using univariate analysis and binary logistic regression analysis, comparing the differences between KHE + KMP group and KHE-KMP group.
Univariate analysis indicated no statistically significant differences between the two groups in gender, prematurity, family history, or color of involved skin. However, statistically significant differences were observed in age of onset, lesion site, and lesion depth. Multivariate analysis revealed significant associations: children with onset age ≤1 month had a 51-fold increased risk of KMP compared to those with onset age >1 month (95% CI 5.238-501.663); non-extremity lesion sites exhibited a 21-fold higher risk of KMP compared to extremity sites (95% CI 3.970-105.958); deeper lesions conferred a 5-fold higher risk of KMP compared to superficial lesions (95% CI 1.073-21.005); lesions >60 mm carried a 17-fold higher risk of KMP compared to lesions ≤60 mm (95% CI 2.999-96.157). A comprehensive predictive model was developed using the fitting formula: Logit (P) = 3.937∗(age at onset) + 1.558∗(lesion depth) + 3.021∗(lesion site) + 2.832∗(lesion size), demonstrating an accuracy of 82.9%. Furthermore, a scoring system was established to assess the likelihood of KMP occurrence. Children diagnosed with KHE were likely to have KMP if their score exceeded 72.5, as determined by Receiver Operating Characteristic (ROC) curve analysis.
Age of onset ≤1 month, deeper lesions, non-extremity sites, and lesions ＞60 mm are independent risk factors for KMP in children with KHE. The cumulative presence of these factors escalates the likelihood of KMP development. Additionally, the identification of these factors allows for the early recognition of potential KMP cases among children with KHE, facilitating prompt therapeutic intervention.
Clinical Research article.
LEVEL Ⅲ.
本研究归纳并分析了卡波西样血管内皮瘤（KHE）患者的临床特征，旨在阐明导致卡萨巴赫-梅里特现象（KMP）出现的危险因素。
我们回顾性分析了2013年1月至2021年12月在复旦大学附属儿科医院诊断为KHE的96例儿科病例。其中，62例患者（65%）出现KMP（KHE+KMP组），而34例患者（35%）未出现（KHE-KMP组）。采用单因素分析和二元逻辑回归分析KHE相关的KMP危险因素，比较KHE+KMP组和KHE-KMP组之间的差异。
单因素分析表明，两组在性别、早产、家族史或受累皮肤颜色方面无统计学显著差异。然而，在发病年龄、病变部位和病变深度方面观察到统计学显著差异。多因素分析显示存在显著关联：发病年龄≤1个月的儿童发生KMP的风险是发病年龄>1个月儿童的51倍（95%CI 5.238-501.663）；非四肢病变部位发生KMP的风险比四肢部位高21倍（95%CI 3.970-105.958）；深部病变发生KMP的风险比浅表病变高5倍（95%CI 1.073-21.005）；病变>60mm发生KMP的风险比病变≤60mm高17倍（95%CI 2.999-96.157）。使用拟合公式Logit（P）=3.937×（发病年龄）+1.558×（病变深度）+3.021×（病变部位）+2.832×（病变大小）建立了综合预测模型，准确率为82.9%。此外，还建立了一个评分系统来评估KMP发生的可能性。根据受试者工作特征（ROC）曲线分析，诊断为KHE的儿童如果评分超过72.5，则可能发生KMP。
发病年龄≤1个月、深部病变、非四肢部位和病变>60mm是KHE患儿发生KMP的独立危险因素。这些因素的累积存在会增加KMP发生的可能性。此外，识别这些因素有助于早期识别KHE患儿中潜在的KMP病例，便于及时进行治疗干预。
临床研究文章。
Ⅲ级。
