Baeza-Herrera C, Martínez-Aguilar G, Bravo-Becerra J M, Morales-Velazco F
Servicio de Cirugía Pediátrica, Hospital Pediátrico Moctezuma, D. F., México.
Bol Med Hosp Infant Mex. 1993 Nov;50(11):824-7.
Split notochord syndrome has been described in several previous case reports; however, we recently treated two patients with a previous undescribed variant of this syndrome. A 2,800 g male baby was admitted to the neonatal patients room with a non covered mass on the lumbo-sacral region, and a portion of intestine, with meconium being passed attached to it the legs were equine-varus and the anus was absent. Radiographs were consistent with a double spine defect (lumbar and sacral split notochord), and the patient was taken to the operating room for closure. A 2,600 g male baby was first seen in other hospital, and was admitted with a circular defect in the mid-line of the lumbosacral region. This defect was conformed by knee, leg, ankle and fingers. Radiographs showed bony structures consistent with incomplete lower extremity. During surgery, the skin overlying the mass was excised, showing a wide spine defect including stomach, duodenum, jejunum, ileum and colon, covered by a peritoneal membrane; there was not communication between this structure and the normal digestive tract.
以往已有多篇病例报告描述了脊索分裂综合征；然而，我们最近治疗了两名患有该综合征此前未描述变异型的患者。一名体重2800克的男婴被收治入新生儿病房，腰骶部有一个未覆盖的肿物，肿物上附着一部分肠道且有胎粪排出，双腿呈马蹄内翻足，肛门缺如。X线片显示符合双脊柱裂（腰骶部脊索分裂），该患者被送往手术室进行闭合手术。一名体重2600克的男婴最初在其他医院就诊，因腰骶部中线处有一个圆形缺损而入院。该缺损累及膝、腿、踝和手指。X线片显示骨质结构符合下肢发育不全。手术过程中，切除肿物上方的皮肤，可见一个广泛的脊柱缺损，包括胃、十二指肠、空肠、回肠和结肠，由一层腹膜覆盖；该结构与正常消化道之间无连通。
