Congenital generalized fibromatosis: a review of the literature and report of a case associated with porencephaly, hemiatrophy, and cutis marmorata telangiectatica congenita.
In congenital generalized fibromatosis (CGF), fibrous nodules involve the skin, bone, and viscera, and the mortality rate is as high as 80% because the visceral nodules can significantly obstruct and compress vital organs. If the fibromas involve only the skin and skeleton and not the viscera, the disease is known as congenital multiple fibromatosis, and the prognosis is excellent. In both conditions, which may be variants of the same disease process, the fibromas resolve completely and spontaneously. There are no previous reports in the literature of CGF associated with cutis marmorata telangiectatica congenita (CMTC), hemiatrophy, or porencephaly with hemiparesis, although CMTC has been seen in conjunction with hemiatrophy. These associations may be coincidental.
D001284:Atrophy / Q000150:complications; D001927:Brain Diseases / Q000150:complications; D005145:Face / Q000473:pathology; D005260:Female; D005350:Fibroma / Q000150:complications / Q000151:congenital*; D006801:Humans; D007223:Infant; D013684:Telangiectasis / Q000150:complications* / Q000151:congenital