愛夫馬克(Ivemark)徵候群―九例先天性異常病例報告

Ivemark Syndrome-A Study of Congenital Anomalies in 9 Cases

陳建州(Ching-Chou Chen) ； 李瑤華(Yiu-Wah Li)

《中華放射線醫學雜誌》 11卷3期 (1986/09) Pp. 239-249

摘要

所謂愛夫馬克徵候群(Ivemark Syndrome)者，即指患有先天性無脾症的新生兒，常會同時合併先天發鉗性心臟病、內臟翻轉、或泌尿系統的異常，於1955年為愛夫馬克所發表。肇因於胎兒在母親懷孕的最初30至37天內，受到Warfarin等的傷害，但少有家族傾向，發生率約每2000屍體解剖中有一例。白人較黑人為多，男女比例為2:1或3:1。平均壽命約38天，很少超過兩歲，死因多為嚴重的心臟、血管、肺部的畸形所致。
自1981至1985的5年間，在台大醫院小兒心臟科證實為愛夫馬克徵候群者共9例，本文將其臨床徵候和放射線學所見，逐一詳細給予列表及說明。

並列摘要

From Dec. 1976 to Oct. 1985, within 9 years, we examined 9 patients with Ivemark syndrome. They were found to have congenital asplenia and complex cyanotic heart disease. Their average year was 1.6 years (range from 1 day to 6 years). Two patients expired, their age was 12 days and 9 months. One patient died during Blalock-Taussig operation that was performed on 4 of the 9 patients; 3 of 4 on whom Black-Taussig operation was performed has a follow-up of 7 months, 4 and 6 years, and they are still in a good health. But the prognosis of 3 of the 7 still alive patients cann't be performed, since they were discharged on earlier dates.
The radiology of Ivemark syndrome shows: (1) Anomalies of the chest, such as levocardia, dextrocardia, right aortic arch, left superior vena caca, pulmonary stenosis or atresia, bilateral minor fissure, bilateral epiarterial bronichi, etc. (2) Anomalies of abdomen, such as situs inversus, hiatal hernia, symmetrical (horizontal) liver, malrotation of intestine, etc.
The purpose of this paper is to prove the clinical and roentgenological findings of Ivemark syndrome, and compares the differences in the world literature.