尾侧退化综合征

F. Bouchenaki, K. Badache, N. Habchi, M. Benachour, S. Bakhti
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摘要

尾椎退行综合征(CRS)是一种罕见的畸形综合征,与不同程度的尾椎或腰骶椎发育不全有关。因此,这种椎体异常在临床上可被简化为单纯的尾骨发育不全,无任何缺陷,甚至腰骶发育不全,伴临床表现为括约肌疾病相关或不相关的运输障碍和涉及下肢的各种缺陷。该综合征还伴有其他矫形畸形,如下肢缩短和/或胃肠道异常,参见泌尿生殖系统和心血管。其发病率为每10万例分娩1至5例。其确切原因尚未确定,但它与产妇糖尿病的关系是很确定的。在我们的研究中,我们报告了来自不同诊所的5例CRS患者,他们的括约肌疾病在我们所有的患者中都被发现在前景中,其结果根据最初的临床图片而有所不同。MRI可以完善和确认先天性异常和相关病变的诊断。我们获得了75%的良好效果和25%的临床稳定;我们也不痛惜任何加重或死亡的案件。我们的兴趣是怀疑CRS的诊断;在产前开始时将其记录下来,并确定其严重程度和相关异常情况,以便为患者管理提供选择;因为一旦做出诊断,手术治疗就变得势在必行,因为可怕的神经系统后遗症会损害功能预后。
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Caudal Regression Syndrome
Caudal Regression Syndrome (CRS) is a rare malformation syndrome associating to varying degrees agenesis of the coccygeal or lumbosacral vertebrae.This vertebral anomaly can therefore be reduced clinically to a simple coccygeal agenesis without any deficit or even lumbosacral agenesis accompanying a clinical picture with sphincter disorders associated or not with transit disorders and various deficits involving the lower limbs.This syndrome is accompanied by other orthopedic malformations such as shortening of the lower limbs, and / or gastrointestinal abnormalities, see also genitourinary as well as cardiovascular.Its incidence is 1 to 5 cases per 100,000 births. Its precise cause has not yet been identified, but its relationship to maternal diabetes is well established. We report in our study 5 patients with CRS from different clinics whose sphincter disorders were found in the foreground in all our patients and whose results vary according to the inaugural clinical picture. MRI made it possible to refine and confirm the diagnosis highlighting the congenital anomaly and the associated lesions.We have obtained 75% good results and 25% clinical stabilization; nor do we deplore any case of aggravation or death. The interest is to suspect the diagnosis of CRS; document it at the start of the prenatal period and determine its severity and associated abnormalities in order to present options for patient management; because once the diagnosis is made, surgical treatment becomes imperative due to the formidable neurological sequelae compromising the functional prognosis.
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