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Prenatal diagnosis of idiopathic infantile arterial calcification with hydrops fetalis
Author(s) -
Nasrallah F. K.,
Baho H.,
Sallout A.,
Qurashi M.
Publication year - 2009
Publication title -
ultrasound in obstetrics and gynecology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.202
H-Index - 141
eISSN - 1469-0705
pISSN - 0960-7692
DOI - 10.1002/uog.7438
Subject(s) - medicine , hydrops fetalis , polyhydramnios , intracardiac injection , gestation , prenatal diagnosis , calcification , fetal echocardiography , great arteries , obstetrics , gestational age , pregnancy , fetus , radiology , echogenicity , surgery , heart disease , cardiology , ultrasonography , genetics , biology
Abstract Idiopathic infantile arterial calcification (IIAC) is a rare and nearly always fatal disorder. To date, prenatal diagnosis has been reported in fewer than 10 cases. We describe a series of three cases in which the diagnosis of IIAC was made at 23, 25 and 29 weeks' gestation. All three cases presented with a normal anatomy scan at 20 weeks' gestation with an echogenic intracardiac focus. Follow‐up scans showed generalized hyperechogenicity and calcification of the walls of the large arteries, particularly the aorta and the iliac arteries. All cases developed hydrops fetalis with cardiomegaly and polyhydramnios later in gestation, resulting in intrauterine fetal death in two cases and neonatal death immediately following delivery in the third. This is the largest case series and the earliest gestational age of prenatal diagnosis of IIAC reported to date. When surveying for the disease, serial scans are important, perhaps from 20 weeks' gestation, with close examination of the iliac and aortic arteries. Detection of echogenic intracardiac focus could be an early marker in patients with a family history of the disease. Copyright © 2009 ISUOG. Published by John Wiley & Sons, Ltd.