Prenatal diagnosis of diastematomyelia.
BACKGROUND
Diastematomyelia, also termed split cord malformation, is a form of occult spinal dysraphism characterized by a cleft in the spinal cord. Prenatal diagnosis of this anomaly is possible by ultrasonography (US), and fetal MRI can be used to diagnose the type of diastematomyelia precisely. Diastematomyelia can be isolated or associated with other dysraphisms, segmental anomalies of the vertebral bodies, or visceral malformations (horseshoe or ectopic kidney, utero-ovarian malformation, and anorectal malformation). We present three cases of fetal diastematomyelia investigated using a multimodal prenatal work-up (US, MRI, 3D-CT).


METHODS
The first case, detected at 20 weeks' gestation, had a lumbar meningocele. At 30 weeks' gestation, direct US visualization revealed the division of the spinal cord into two hemicords. This patient illustrates an isolated type II diastematomyelia with a favorable prognosis. The second case, detected at 22 weeks' gestation, presented with disorganization of bony process of the vertebral column with a midline echogenic bony spur, asymmetrical hemicords, and a foot malposition. Fetal MRI at 26 weeks' gestation and CT/3D reconstructed at 32 weeks' gestation confirmed a type I diastematomyelia with orthopedic malposition. The third case, detected at 22 weeks' gestation, presented with widening of the lumbar canal and scoliosis. Prenatal work-up (US, MRI) disclosed other visceral malformations (pelvic kidney), which led to the assumption of a complex polymalformative syndrome. The pregnancy was terminated. Fetopathologic examination disclosed even more visceral malformations (anal atresia and unicorn uterus).
Sonigo-Cohen
Pascale
P
Pediatric Radiology Department, Hôpital Necker-Enfants Malades, 149 rue de Sevres, 75743 Paris Cedex 15, France. pascale.sonigo@nck.ap-hop-paris.fr
Schmit
Pierre
P
Zerah
Michel
M
Chat
Latifa
L
Simon
Isabelle
I
Aubry
Marie Cécile
MC
Gonzales
Marie
M
Pierre-Kahn
Alain
A
Brunelle
Francis
F
D000328:Adult; D005260:Female; D005315:Fetal Diseases / Q000000981:diagnostic imaging* / Q000196:embryology / Q000601:surgery; D005333:Fetus; D005865:Gestational Age; D006801:Humans; D021621:Imaging, Three-Dimensional / Q000295:instrumentation / Q000401:mortality; D007231:Infant, Newborn; D008161:Lumbosacral Region / Q000473:pathology; D008279:Magnetic Resonance Imaging / Q000295:instrumentation / Q000379:methods; D008588:Meningocele / Q000473:pathology; D009436:Neural Tube Defects / Q000000981:diagnostic imaging* / Q000196:embryology / Q000601:surgery; D011247:Pregnancy; D011296:Prenatal Diagnosis / Q000379:methods*; D016136:Spina Bifida Occulta / Q000473:pathology; D013116:Spinal Cord / Q000473:pathology; D016216:Ultrasonography, Prenatal / Q000295:instrumentation / Q000379:methods