FIBROCHONDROGENESIS IN A 17-WEEK FETUS - A CASE EXPANDING THE PHENOTYPE

Fibrochondrogenesis is a very rare form of lethal short-limb dwarfism, with 8 cases described since it was first reported in 1978, It is bec oming clear that this condition has certain radiological and histologi cal characteristics that distinguish it from other skeletal dysplasias . We herein present a further case of fibrochondrogenesis diagnosed in a fetus of 17 weeks, which is the youngest patient reported so far, I n addition, the fetus showed severe micrognathia and a bifid tongue. T hese are not previously described manifestations, which extend the phe notype of this rare condition. (C) 1998 Wiley-Liss, Inc.