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联体双下肢畸形。血管异常的血管造影显示。

Sirenomelia. Angiographic demonstration of vascular anomalies.

作者信息

Talamo T S, Macpherson T A, Dominquez R

出版信息

Arch Pathol Lab Med. 1982 Jul;106(7):347-8.

PMID:6896449
Abstract

A 1,730-g infant with severe caudal regression syndrome (sirenomelia) died shortly after delivery, after a 34-week gestation. Autopsy findings included multiple skeletal anomalies, renal agenesis, amnion nodosum, and a single lower extremity. Postmortem arteriography demonstrated a persistent vitelline artery and documented the vascular pattern within the lower extremity. To our knowledge, angiographic demonstration of the lower limb vascularity has not been described previously in sirenomelia. The angiographic findings support the pathogenetic concept of limb bud fusion and malrotation of the limb in sirenomelia after damage to the posterior axis mesoderm in early embryonic life. Postmortem arteriography is an inexpensive, retrievable, and easy means of documenting vascular anomalies in the fetus or infant with multiple congenital abnormalities.

摘要

一名妊娠34周后出生的1730克重婴儿,患有严重的尾椎退化综合征(美人鱼综合征),出生后不久死亡。尸检结果包括多处骨骼异常、肾缺如、羊膜结节和单一下肢。死后血管造影显示存在持续的卵黄动脉,并记录了下肢的血管模式。据我们所知,此前尚未有关于美人鱼综合征下肢血管造影表现的描述。血管造影结果支持了在胚胎早期后轴中胚层受损后,美人鱼综合征中肢体芽融合和肢体旋转不良的发病机制概念。死后血管造影是一种记录患有多种先天性异常的胎儿或婴儿血管异常的廉价、可获取且简便的方法。

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