Department of Neurosurgery, Avicenne Military Hospital, Marrakech, Morocco and Mohammed V University, Rabat, Morocco.
World Neurosurg. 2020 Oct;142:301-302. doi: 10.1016/j.wneu.2020.07.055. Epub 2020 Jul 17.
This is a case report of a 2-year-old male who presented with walking disability and sphincter incontinence since birth. His mother has diabetes mellitus. The patient had a normal mental function, and his lower extremities were hypoplastic and akinetic. Spinal neuroimaging investigations revealed agenesis of the spinal column below T9 vertebral level with abrupt termination of the spinal cord at the T6 vertebral level. With this severe caudal regression syndrome (CauReS), cardiac, anorectal, urologic, and orthopedic consultations were made looking for other potential associated malformations. Unfortunately, no neurosurgical indication was suggested. CauReS is a rare congenital disorder in which there is abnormal fetal development of the lower spine and hypoplasia of the lower extremities. Neurosurgically, there are no clear guidelines to follow for patients with CauReS. Sometimes, surgical spinal cord untethering may be indicated in some selected patients to improve neurologic function.
这是一例 2 岁男童病例报告，他出生时即存在行走障碍和肛门失禁。其母亲患有糖尿病。患儿精神功能正常，下肢发育不良且活动受限。脊柱神经影像学检查显示 T9 椎体以下脊柱发育不全，脊髓在 T6 椎体水平突然终止。该患儿患有严重的尾部退化综合征（CauReS），心脏、肛肠、泌尿科和骨科会诊寻找其他潜在的相关畸形。不幸的是，没有建议进行神经外科手术。CauReS 是一种罕见的先天性疾病，表现为下脊柱异常发育和下肢发育不良。神经外科方面，对于 CauReS 患者尚无明确的治疗指南。有时，对一些特定患者进行脊髓松解手术可能有助于改善神经功能。
