Objective

To describe typical clinical presentation of patients with microfistular, capillary-venule (CV) malformation as a variant form of arteriovenous malformations (AVM).

Methods

A retrospective clinical analysis of 15 patients with CV-AVM confirmed by a computational flow model enrolled in a prospective database of patients with congenital vascular malformation between January 2008 and May 2018.

Results

The mean age of the patients at first time of presentation was 30 years with balanced sex ratio. Presentation was dominated by soft tissue hypertrophy (n = 12 [80.0%]) and atypical varicose veins (n = 11 [73.3%]). The anatomic location of enlarged varicose veins gave no uniform pattern and did not correspond with the typical picture of primary varicose vein disease. Most often, symptomatic CV-AVM was found at the lower extremities in this series of unselected patients. The most frequent compartment affected was the subcutis (n = 14 [93.3%]), involvement of muscle was recorded in one-third and cutis in one-fourth of patients.

Conclusions

A high grade of clinical suspicion is needed to recognize CV-AVM and to prevent inadequate therapy owing to missed diagnosis.

中文翻译：

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毛细血管畸形是动静脉畸形的微瘘变体。

目的

描述微瘘，毛细血管（CV）畸形作为动静脉畸形（AVM）的变体形式的患者的典型临床表现。

方法

在2008年1月至2018年5月之间对先天性血管畸形患者的前瞻性数据库中登记的计算流模型证实了对15例CV-AVM患者的回顾性临床分析。

结果

首次就诊时患者的平均年龄为30岁，性别比例均衡。表现以软组织肥大（n = 12 [80.0％]）和非典型静脉曲张（n = 11 [73.3％]）为主。扩大的曲张静脉的解剖位置没有给出均匀的图案，并且与原发性曲张静脉疾病的典型图片不符。在这一系列未选择的患者中，最常见的是在下肢发现有症状的CV-AVM。受影响最频繁的隔室是皮下组织（n = 14 [93.3％]），记录到三分之一的患者有肌肉受累，四分之一的患者有皮肤受累。

结论

需要高度的临床怀疑以识别CV-AVM并防止由于漏诊而导致治疗不足。