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[人类尾芽畸形或:尾椎发育不全综合征。综述]

[Malformations of the human tail bud or: caudal agenesis syndrome. A review].

作者信息

Carstens C, Mai G, Greiner C

机构信息

Stiftung Orthopädische Universitätsklinik Heidelberg.

出版信息

Z Orthop Ihre Grenzgeb. 1994 Sep-Oct;132(5):345-56. doi: 10.1055/s-2008-1039836.

Abstract

This study presents radiological and clinical findings in 70 patients with aplasia of caudal spinal segments and associated anomalies of the lower extremities and the inner organs. From a functional point of view 5 different types can be classified--unilateral hemi-aplasia, bilateral aplasia with or without sacral plateau, complex malformations of the caudal spine and medial spinal aplasia. Foot deformities and anomalies of the inner organs are common findings regardless to the extent of spinal aplasia, whereas popliteal webbing is only seen in cases with involvement of the lumbar spine. The lack of osseous junction between the spine and the pelvis leeds to a lumbopelvic kyphosis. Additionally the iliac wings are rotated in the frontal plane with the result of a narrow pelvic outlet and a change in the geometry of the hip joints. Embryologically all types of caudal spinal aplasia can be referred to a damage of the human tail bud. However, the term "Syndrome of Caudal Regression" is misleading; with respect to the presented results it is proposed to call this type of spinal malformation "Syndrome of Caudal Aplasia".

摘要

本研究展示了70例尾椎节段发育不全及伴有下肢和内脏相关畸形患者的影像学和临床发现。从功能角度可分为5种不同类型——单侧半发育不全、伴有或不伴有骶骨平台的双侧发育不全、尾椎复杂畸形和脊柱内侧发育不全。无论脊柱发育不全的程度如何,足部畸形和内脏异常都是常见表现,而腘窝蹼仅见于腰椎受累的病例。脊柱与骨盆之间缺乏骨性连接会导致腰骶部后凸。此外,髂骨翼在额状面旋转,导致骨盆出口狭窄和髋关节几何形状改变。从胚胎学角度来看,所有类型的尾椎发育不全都可归因于人类尾芽的损伤。然而,“尾椎退化综合征”这一术语具有误导性;基于所呈现的结果,建议将这种脊柱畸形称为“尾椎发育不全综合征”。

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