Deveza E, Puzenat E, Manzoni P, Humbert P, Aubin F
Service de dermatologie, CHU Besançon, 3, boulevard Fleming, 25030 Besançon cedex, France.
Service de dermatologie, CHU Besançon, 3, boulevard Fleming, 25030 Besançon cedex, France.
Arch Pediatr. 2015 Jul;22(7):685-92. doi: 10.1016/j.arcped.2015.04.003. Epub 2015 Jun 3.
Congenital hemangiomas (CHs) are rare congenital vascular tumors seldom mentioned in the literature.
We carried out a retrospective study of all the cases of CH diagnosed and treated at Besançon Hospital from 2008 to 2014. The clinical, radiological, and histological data of each case were collected. All the children were seen again in 2014.
Ten CHs (seven rapidly involuting CHs, RICH and three non-involuting CH, NICH), predominantly full-term eutrophic male infants, were enrolled. RICHs were located on the head (n=2), trunk (n=2), and lower limbs (n=3), and NICHs were found on the hands. Diagnosis was clinical for all ten infants. All CHs resembled "tumor" congenital lesions: single, oval-shaped, nonpulsatile, and well delimited, and their size did not increase after birth. Two RICHs were warm, one had phlebolites, and two had draining veins at the first visit. The mean age of the RICH involution onset was 1.7 months and the mean time to complete involution was 10.4 months. One CH was classified as a PICH (partially involuting CH) due to partial regression, two RICHs were still in the involution process at the age of 10 and 15 months, and one regressed very quickly within 7 days. No complications were observed in the NICH. Two RICHs presented benign complications (ulcerations and bleeding). Two RICHs regressed entirely, and five regressed with sequelae: lipoatrophy (n=3), cutaneous excess (n=2), dysplastic veins (n=3), a pigmented area (n=1), and an anemic halo (n=2).
The small number of patients in our cohort, in spite of the length of the study, confirms the rarity of CH. The sex-ratio in favor of male infants and the location of NICH on the hands have not been reported. The most discriminating element remained the follow-up over 1 year. The initial clinical aspect of the NICH and the progression of one RICH into a NICH suggested possible overlapping forms between RICH and NICH. Some CHs, including one PICH, presented clinical and radiological criteria similar to those of vascular malformations (warm lesion, dysplastic veins, and echo-Doppler results in favor of vascular malformation). RICH regressed with sequelae in most cases.
This study reveals a polymorphous clinical presentation of CH and provides a thorough description of their progression. It underlines the existence of overlapping phenomena between RICH and NICH, and between CH and vascular malformations, thus suggesting a possible link between proliferation and malformation phenomena at the origin of these lesions.
先天性血管瘤(CHs)是一种罕见的先天性血管肿瘤,在文献中很少被提及。
我们对2008年至2014年在贝桑松医院诊断和治疗的所有CH病例进行了回顾性研究。收集了每个病例的临床、放射学和组织学数据。所有儿童在2014年再次接受检查。
共纳入10例CH(7例快速消退型先天性血管瘤,RICH,和3例非消退型先天性血管瘤,NICH),主要为足月健康男婴。RICH位于头部(n = 2)、躯干(n = 2)和下肢(n = 3),NICH位于手部。所有10例婴儿均通过临床诊断。所有CH均类似“肿瘤”先天性病变:单个、椭圆形、无搏动且边界清晰,出生后其大小未增加。初次就诊时,2例RICH温热,1例有静脉石,2例有引流静脉。RICH开始消退的平均年龄为1.7个月,完全消退的平均时间为10.4个月。1例CH因部分消退被归类为部分消退型先天性血管瘤(PICH),2例RICH在10个月和15个月时仍处于消退过程中,1例在7天内迅速消退。NICH未观察到并发症。2例RICH出现良性并发症(溃疡和出血)。2例RICH完全消退,5例消退后有后遗症:脂肪萎缩(n = 3)、皮肤多余(n = 2)、发育异常的静脉(n = 3)、色素沉着区(n = 1)和贫血晕(n = 2)。
尽管研究时间较长,但我们队列中的患者数量较少,证实了CH的罕见性。男婴性别比偏高以及NICH位于手部的情况此前未见报道。最具鉴别性的因素仍然是超过1年的随访。NICH的初始临床表现以及1例RICH演变为NICH提示RICH和NICH之间可能存在重叠形式。一些CH,包括1例PICH,呈现出与血管畸形相似的临床和放射学标准(温热病变、发育异常的静脉以及支持血管畸形的超声多普勒结果)。大多数情况下,RICH消退后有后遗症。
本研究揭示了CH的多形性临床表现,并对其进展进行了全面描述。强调了RICH和NICH之间以及CH和血管畸形之间存在重叠现象,从而提示这些病变起源处增殖和畸形现象之间可能存在联系。