Hervella M, Iglesias M E
Unidad de Dermatología, Hospital García Orcoyen, Estella.
An Sist Sanit Navar. 2004;27 Suppl 1:33-44.
Haemangiomas can be indicators or clue signs for serious syndromes. Although less well known than those related to vascular malformations, there are some syndromes of important diagnostic value that are associated with haemangomias. Early recognition of problematic haemangiomas, together with a prompt intervention, may help to minimise their future morbidity. Neonatal haemangiomas warrant a special follow-up, since their growth pattern might be unpredictable at such an early age. Several clinical presentations are relevant because of their risk of syndromic association: cervicofacial haemangiomas, especially the extensive ones, may be markers for severe dysmorphic conditions like the PHACE(S) syndrome. Those distributed in the beard area are occasionally associated with haemangiomas of the airway. Lumbosacral haemangiomas usually hide an underlying spinal dysraphism or anorectal and urogenital anomalies. Multiple cutaneous haemangiomas may be a sign of visceral haemangiomatosis, most often hepatic, which becomes complicated by cardiac insufficiency or thyroid disease. Finally, there are two vascular neoplasms of rapid and invasive growth - kaposiform haemangioendothelioma and angioblastoma or tufted angioma - which, unlike infantile haemangioma, are markers for the Kasabach-Merritt syndrome.
血管瘤可能是严重综合征的指标或线索性体征。尽管不如与血管畸形相关的综合征那么广为人知,但有一些具有重要诊断价值的综合征与血管瘤有关。早期识别有问题的血管瘤并及时进行干预,可能有助于将其未来的发病率降至最低。新生儿血管瘤需要特别随访,因为它们在如此早期的生长模式可能不可预测。几种临床表现因其与综合征相关的风险而具有相关性:头颈部血管瘤,尤其是广泛的血管瘤,可能是诸如PHACE(S)综合征等严重畸形状况的标志。分布在胡须区域的血管瘤偶尔与气道血管瘤有关。腰骶部血管瘤通常隐藏着潜在的脊柱裂或肛肠及泌尿生殖系统异常。多发性皮肤血管瘤可能是内脏血管瘤病的迹象,最常见的是肝脏血管瘤病,可并发心脏功能不全或甲状腺疾病。最后,有两种生长迅速且具有侵袭性的血管肿瘤——卡波西样血管内皮瘤和成血管细胞瘤或丛状血管瘤——与婴儿血管瘤不同,它们是卡萨巴赫-梅里特综合征的标志。