[Diastematomelia (author's transl)].
21 cases have been observed and followed up for 10 years. All were children. Three types of anomaly were noted: Spinal curvature (mostly scoliosis), skin abnormalities in the mid line (hypertrichosis, angioma, skin dimples) and problems in the lower limbs (varus deformities of the feet, leg length discrepancy, muscular atrophy, neurological disorders). These signs, even if not all are present, must lead to a study of the vertebral bodies and to gas myelography whcih demonstrates a double spinal cord over a variable length from 2 to 14 segments. 15 children were treated surgically by mobilisation of the spinal cord and arthrodesis of the spine. 4 of them were made worse. In contrast, none of the 6 children treated conservately was made worse. The authors conclude that surgery should be reserved for recent spontaneous neurological deterioration and severe deformities of the spine.
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D007866:Leg / Q000002:abnormalities; D008297:Male; D009192:Myelography; D009422:Nervous System Diseases / Q000175:diagnosis; D009436:Neural Tube Defects / Q000175:diagnosis* / Q000601:surgery; D012600:Scoliosis / Q000175:diagnosis; D012868:Skin Abnormalities; D013116:Spinal Cord / Q000002:abnormalities
La diastématomyelie (ou syndrome de la double chorde): etude de 21 cas.