Frade F, Kadlub N, Soupre V, Cassier S, Audry G, Vazquez M-P, Picard A
Service de chirurgie maxillo-faciale et plastique, faculté de médecine Pierre-et-Marie-Curie Paris-6, hôpital d'enfant Armand-Trousseau, centre hospitalier STARTT, AP-HP, 26, rue du Docteur-Arnold-Netter, 75571 Paris cedex 12, France.
Arch Pediatr. 2012 Jan;19(1):55-8. doi: 10.1016/j.arcped.2011.10.004. Epub 2011 Nov 17.
The association of a pelvic hemangioma and malformations in the pelvic region are described as SACRAL or PELVIS syndrome. More recently, the acronym of LUMBAR syndrome has been used to describe the association of lower-body hemangioma and other cutaneous defects, urogenital anomalies, ulceration, myelopathy, bony deformities, anorectal malformations, arterial anomalies, and renal anomalies. We report herein 2 representative cases.
Two girls presented with infantile hemangioma associated with genital malformation. One case was associated with spinal anomalies and the other one with the anus in the genital position.
Segmented hemangiomas are commonly associated with extracutaneous abnormalities. By analogy with PHACE syndrome, PELVIS/SACRAL/LUMBAR syndromes describe the association of segmented hemangioma of the lower extremities associated with other trunk or lower-body malformations. The syndrome is often incomplete.
盆腔血管瘤与盆腔区域畸形的关联被描述为骶骨或骨盆综合征。最近,腰椎综合征这一缩写词被用于描述下半身血管瘤与其他皮肤缺陷、泌尿生殖系统异常、溃疡、脊髓病、骨骼畸形、肛门直肠畸形、动脉异常和肾脏异常的关联。我们在此报告2例典型病例。
两名女童表现为伴有生殖器畸形的婴儿血管瘤。1例伴有脊柱异常,另1例生殖器位置的肛门异常。
节段性血管瘤通常与皮肤外异常相关。类比PHACE综合征,骨盆/骶骨/腰椎综合征描述了下肢节段性血管瘤与其他躯干或下半身畸形的关联。该综合征往往并不完全。