Congenital Cystic Adenomatoid Malformation Type 4 and Aneurysm of the Vein of Galen: A Rare Coincidence or Possibly Related Association

Abstract

As far as the prognosis is concerned, it is important to diagnose the exact type of congenital cystic adenomatoid malformation (CCAM) in order to exclude associated anomalies, as well as the risk of development of malignancies in later life. The frequency of associated malformations of CCAM type 4 is unknown. We report a 4-month-old boy with CCAM type 4 and aneurysmal malformation of the vein of Galen (AVG). Although AVG is the most frequent arteriovenous malformation during childhood, this is the first case report, to our knowledge, of CCAM type 4 in association with an anomaly. In addition, we speculate that the relation between CCAM type 4 and AVG appears to be more likely a possibly related association rather than a rare coincidence, as both malformations develop at the same stages of embryonic life, and pathogenetically, apoptosis play a significant role in both entities. However, further studies are needed to validate this speculation placing emphasis on the association of the two anomalies, otherwise a rare coincidence cannot be excluded.