Mulliken John B, Marler Jennifer J, Burrows Patricia E, Kozakewich Harry P W
Department of Plastic Surgery, Children's Hospital and Harvard Medical School, Boston, Massachusetts 02115, USA.
Pediatr Dermatol. 2007 Jul-Aug;24(4):356-62. doi: 10.1111/j.1525-1470.2007.00496.x.
We describe six patients with an uncommon variant of infantile hemangioma that we have termed reticular, occurring in the extremity, which were associated with intractable ulceration, anogenito-urinary-sacral anomalies, and sometimes cardiac overload. The extreme end of the spectrum is exemplified by a male neonate who presented with a stained, enlarged, pulsatile lower extremity, and cardiac failure. He also had hepatic hemangiomas and ambiguous genitalia. Progressive soft tissue necrosis and bony destruction necessitated amputation. The histopathologic features differed from those of typical infantile hemangioma: infiltrative (not lobular) and involving fascia, muscle, and bone. The mid-spectrum is illustrated by five females with reticular infantile hemangioma of the lower limb, buttock, and perineum. Four of these infants had a ventral-caudal anomaly, including omphalocele, recto-vaginal fistula, solitary/duplex kidney, imperforate anus, and tethered cord; one infant also had hepatic hemangiomas. Deep ulcerations healed following corticosteroid therapy; one patient required skin graft for closure of a thigh wound. The minor end of the spectrum is exemplified a patchy lesion in the distal limb. The reticular variant of infantile hemangioma can be confused with other vascular anomalies in the limb, such as capillary malformation, cutis marmorata telangiectasia congenita, diffuse arteriovenous malformation (Parkes Weber syndrome) and capillary-lymphatico-venous malformation (Klippel-Trenaunay syndrome). The macular network-like appearance of the tumor and coexisting ventral-caudal structural anomalies is analogous to the association of posterior fossa brain malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, and eye abnormalities association in the craniofacial region.
我们描述了6例患有婴儿血管瘤罕见变异型的患者，我们将其称为网状婴儿血管瘤，发生于四肢，伴有难治性溃疡、肛门生殖泌尿骶骨异常，有时还伴有心脏负荷过重。该谱系的极端情况表现为一名男性新生儿，其下肢染色、肿大、搏动性，伴有心力衰竭。他还患有肝血管瘤和生殖器模糊。进行性软组织坏死和骨质破坏导致截肢。组织病理学特征与典型婴儿血管瘤不同：呈浸润性（而非小叶状），累及筋膜、肌肉和骨骼。谱系中间情况表现为5名患有下肢、臀部和会阴网状婴儿血管瘤的女性。其中4名婴儿有腹尾端异常，包括脐膨出、直肠阴道瘘、单/双肾、肛门闭锁和脊髓栓系；1名婴儿也有肝血管瘤。皮质类固醇治疗后深部溃疡愈合；1例患者大腿伤口闭合需要植皮。谱系较轻的一端表现为肢体远端的斑片状病变。婴儿血管瘤的网状变异型可能与肢体的其他血管异常相混淆，如毛细血管畸形、先天性大理石样皮肤毛细血管扩张、弥漫性动静脉畸形（帕克斯·韦伯综合征）和毛细血管淋巴管静脉畸形（克-特综合征）。肿瘤的黄斑状网络样外观以及并存的腹尾端结构异常类似于颅面区域后颅窝脑畸形、血管瘤、动脉异常、主动脉缩窄和心脏缺陷以及眼部异常的关联。
