We report first trimester cystic hygroma colli with subsequent resolution a
nd development of a fetal akinesia deformation sequence. Neuropathological
examination of the brain showed intra- and extracellular white matter edema
while spinal cord, peripheral nerves and muscles were normal. Hygroma coll
i as the first echographic sign of subsequent severe fetal akinesia sequenc
e without muscular dystrophy as seen in the Lethal Multiple Pterygium syndr
ome has not been previously reported.