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Fetal presentation of PHACES syndrome

PHACES syndrome, proposed by Frieden et al. [1996], consists of Posterior fossa CNS anomalies, Hemangioma, Arterial anomalies, Coarctation of the aorta and Cardiac anomalies, Eye anomalies, and Sternal defects. The condition has been reviewed extensively elsewhere [Gorlin et al., 2001]. Earlier, Leiber [1982] noted the syndrome in binary, tertiary, or quaternary combinations. Here, we present two cases of possible PHACES syndrome with fetal presentation. Case 1 involved the third pregnancy of a 29-year-old woman. The second pregnancy ended in the induced abortion of an anencephalic fetus. In the third pregnancy, an arachnoid cyst was suspected at 22 weeks gestation on ultrasound. A second ultrasound at 28 weeks gestation was consistent with Dandy– Walker malformation and abortion was induced at 30 weeks. Postmortem findings included Dandy–Walker malformation and aortic stenosis. Case 2 involved the third pregnancy of a 27-year-old woman. The second pregnancy ended in spontaneous abortion. A third trimester routine ultrasound suggested Dandy–Walker malformation and absent eyeballs. Abortion was induced at 36 weeks gestation. Postmortem examination showed macrosomia, prominent forehead, large anterior fontanelle, cryptophthalmia, excess cervical skin, absent optic nerves and optic chiasm, and Dandy–Walker malformation. To our knowledge, these are the first two cases described prenatally that suggest PHACES syndrome. Both of our cases had fetal presentation. Neither of our cases had hemangioma. However, almost all of them developed postnatally [Rossi et al., 2001]. Although various eye anomalies have been reported, particularly anterior segment abnormalities and microphthalmia, anophthalmia present in case 2, has not been previously described.