Sonigo-Cohen Pascale, Schmit Pierre, Zerah Michel, Chat Latifa, Simon Isabelle, Aubry Marie Cécile, Gonzales Marie, Pierre-Kahn Alain, Brunelle Francis
Pediatric Radiology Department, Hôpital Necker-Enfants Malades, 149 rue de Sevres, 75743 Paris Cedex 15, France.
Childs Nerv Syst. 2003 Aug;19(7-8):555-60. doi: 10.1007/s00381-003-0771-8. Epub 2003 Jul 4.
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).
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).
脊髓纵裂，也称为脊髓分裂畸形，是一种隐性脊柱裂，其特征为脊髓出现裂隙。通过超声检查（US）可在产前诊断这种异常情况，胎儿磁共振成像（MRI）可用于精确诊断脊髓纵裂的类型。脊髓纵裂可以是孤立性的，也可与其他脊柱裂、椎体节段性异常或内脏畸形（马蹄肾或异位肾、子宫卵巢畸形以及肛门直肠畸形）相关。我们呈现三例胎儿脊髓纵裂病例，这些病例采用了多模式产前检查方法（超声、MRI、三维CT）进行研究。
第一例在妊娠20周时被检测出，患有腰椎脊膜膨出。妊娠30周时，直接超声检查显示脊髓分裂为两条半脊髓。该病例说明了孤立性II型脊髓纵裂，预后良好。第二例在妊娠22周时被检测出，表现为脊柱骨突结构紊乱，伴有中线回声增强的骨棘、不对称半脊髓以及足部位置异常。妊娠26周时的胎儿MRI以及妊娠32周时的CT/三维重建证实为I型脊髓纵裂并伴有矫形位置异常。第三例在妊娠22周时被检测出，表现为腰椎管增宽和脊柱侧弯。产前检查（超声、MRI）发现了其他内脏畸形（盆腔肾），这提示可能存在复杂的多发畸形综合征。于是终止了妊娠。胎儿病理学检查发现了更多的内脏畸形（肛门闭锁和单角子宫）。
