Gouedard C, Dupré-Goetghebeur D, Gagneur A, Sannier K, Misery L
Service de Dermatologie, CHU Brest.
Ann Dermatol Venereol. 2007 Nov;134(11):863-6. doi: 10.1016/s0151-9638(07)92833-1.
Benign natal haemangiomatosis is characterised by the presence of multiple congenital haemangiomas restricted to the skin. It is differentiated from diffuse neonatal haemangiomatosis in which there is both cutaneous and visceral involvement, with higher morbidity and mortality.
Two identical twins, I and II (monochorionic placenta, biamniotic), born prematurely at 30 weeks' amenorrhoea, presented twin-transfusion syndrome resulting in retarded intrauterine growth in twin I, the donor, and incipient anasarca in twin II, the recipient. Twin I weighed 960 g while twin II weighed 1 200 g. At birth, miliary haemangiomatosis was observed in both infants (16 haemangiomas in I, 19 in II). Abdominal ultrasound and whole-body MRI performed in the two children revealed multiple angiomatous hepatic nodular lesions in I. Subsequent routine clinical and ultrasound monitoring (hepatic and cardiac) showed increased size of the haemangiomatous lesions over the first 4 months followed by stabilisation and gradual regression. No systemic therapy was required. In twin I an episode of ulceration of a neck haemangioma occurred at 5 months and a favourable outcome was obtained on administration of topical hydrocolloid therapy.
Twin-transfusion syndrome affects 15 to 30% of monochorionic biamniotic pregnancies. It is a serious complication of twin pregnancies resulting from a dynamic process of interfoetal blood transfusion as a result of venous-venous or arteriovenous vascular anastomoses. In the present case, which appears to be the first reported case, it seems that these monochorionic twins, who shared the same placenta, presented haemangiomatosis simultaneously in utero, if we accept the hypothesis of grafting of emboli of placental microvessels in the formation of congenital haemangiomas.
良性先天性血管瘤病的特征是存在多个局限于皮肤的先天性血管瘤。它与弥漫性新生儿血管瘤病相鉴别,后者既有皮肤受累又有内脏受累,发病率和死亡率更高。
一对同卵双胞胎I和II(单绒毛膜胎盘,双羊膜囊),在停经30周时早产,出现双胎输血综合征,导致供体双胞胎I宫内生长迟缓,受体双胞胎II出现早期全身性水肿。双胞胎I体重960克,而双胞胎II体重1200克。出生时,两个婴儿均观察到粟粒状血管瘤病(I有16个血管瘤,II有19个)。对这两个孩子进行的腹部超声和全身MRI检查显示,I有多个血管瘤性肝结节性病变。随后的常规临床和超声监测(肝脏和心脏)显示,血管瘤性病变在最初4个月内增大,随后稳定并逐渐消退。无需进行全身治疗。在双胞胎I中,5个月时颈部血管瘤出现溃疡,局部应用水胶体疗法后取得了良好效果。
双胎输血综合征影响15%至30%的单绒毛膜双羊膜囊妊娠。它是双胎妊娠的一种严重并发症,是由于静脉 - 静脉或动静脉血管吻合导致的胎儿间输血动态过程引起的。在本病例中,这似乎是首例报道的病例,如果我们接受胎盘微血管栓子移植在先天性血管瘤形成中的假设,那么这对共享同一胎盘的单绒毛膜双胞胎似乎在子宫内同时出现了血管瘤病。