[Caudal regression syndrome (report of 4 cases) (author's transl)]

Four cases of caudal regression syndrome with partial or complete sacrococcigeal agenesis are presented. One of them also had agenesis of the last lumbar vertebrae. Urinary, gluteae and inferior limb malformations were found in all four. Special emphasis is placed on the importance of the early knowledge of these malformations in order to avoid secondary effects to neurogenic bladder (urinary infections, vesico-ureteral reflux, hipofunctional kidney) caused by a delayed diagnosis. Maternal diabetes in one case and skeletal alterations (hexadactily, lack of one hand) in another were found among the family history. A very uncommon association with Goldenhar's syndrome and Klippel-Feil's syndrome in one case, and with "situs inversus totalis" in another are described. The possible ethiologies of the syndrome are being discussed.