Anjankar Shailendra D, Subodh Raju
Department of Neurosurgery, Kamineni Hospitals Ltd., Hyderabad, Andhra Pradesh, India.
J Pediatr Neurosci. 2014 Sep-Dec;9(3):249-52. doi: 10.4103/1817-1745.147580.
Spondylocostal dysostosis (SCDO) is rare anomaly caused due to flawed embryological development of the axial skeleton during preliminary stages of gravidity, characterized by malformed vertebral column and ribs, abridged thorax and kyphoscoliosis. This entity was also reported as a "Jarcho-Levin syndrome" eponym by erstwhile authors, before the introduction of genetic based classification. A literature review showed only three cases of this clinical entity with lipomyelomeningocele. We report the fourth case report of an infant with SCDO with lipomyelomeningocele. His chest X-ray displayed absent left side 6(th)-8(th) ribs with peculiar fan like configuration, making the heart vulnerable to any direct injury. Special care has to be taken for such patients who need surgical procedure in the prone position.
脊椎肋骨发育不全(SCDO)是一种罕见的异常情况,由孕期早期轴向骨骼胚胎发育缺陷引起,其特征为脊柱和肋骨畸形、胸廓缩小以及脊柱侧凸。在基于遗传学的分类方法引入之前,该病症也曾被早期作者称为“贾科-莱文综合征”。文献综述显示,仅有3例该临床病症合并脂肪脊髓脊膜膨出的病例。我们报告了第4例脊椎肋骨发育不全合并脂肪脊髓脊膜膨出的婴儿病例。他的胸部X光片显示左侧第6至第8肋骨缺失,呈特殊的扇形结构,这使得心脏易受任何直接损伤。对于需要俯卧位进行外科手术的此类患者,必须给予特别护理。