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胎儿脊髓纵裂的产前超声评估:两例I型脊髓分裂畸形

Prenatal ultrasound evaluation of fetal diastematomyelia: two cases of type I split cord malformation.

作者信息

Allen L M, Silverman R K

机构信息

SUNY Health Science Center at Syracuse, Department of Obstetrics and Gynecology 13202, USA.

出版信息

Ultrasound Obstet Gynecol. 2000 Jan;15(1):78-82. doi: 10.1046/j.1469-0705.2000.00019.x.

DOI:10.1046/j.1469-0705.2000.00019.x
PMID:10776019
Abstract

Isolated diastematomyelia is a rare form of spinal dysraphism characterized by a sagittal cleft in the spinal cord, conus medullaris and/or filum terminale with splaying of the posterior vertebral elements. This condition is the result of the presence of an osseous or fibrocartilaginous septum producing a complete or incomplete sagittal division of the spinal cord into two hemicords. It may be isolated or associated with other segmental anomalies of the vertebral bodies. Prenatal diagnosis of this anomaly is possible in the early midtrimester by sonography, thus allowing for early surgical intervention and a favorable prognosis. Two cases of fetal diastematomyelia diagnosed by prenatal sonography are presented, each demonstrating the typical sonographic features diagnostic of this condition. The first case, detected at 28 weeks' gestation, presented with disorganization of the bony processes of the vertebral column with a midline echogenic focus. The second fetus, diagnosed at 17 weeks' gestation, had a similar appearance with widening of the posterior elements and the presence of a midline echogenic bony spur. Postnatally, both infants underwent magnetic resonance imaging for a definitive diagnosis. Surgical repair of the defect was performed in the neonatal period in both cases.

摘要

孤立性脊髓纵裂是一种罕见的脊柱裂畸形,其特征为脊髓、脊髓圆锥和/或终丝出现矢状裂,同时伴有后方椎体结构分离。这种情况是由于存在骨或纤维软骨性中隔,导致脊髓完全或不完全地矢状分裂为两个半脊髓。它可能是孤立的,也可能与椎体的其他节段性异常相关。在孕中期早期通过超声检查可对这种异常进行产前诊断,从而实现早期手术干预并获得良好预后。本文介绍了两例经产前超声诊断的胎儿脊髓纵裂病例,每例均展示了诊断该病的典型超声特征。第一例在妊娠28周时被检测到,表现为脊柱骨结构紊乱,伴有中线强回声灶。第二例胎儿在妊娠17周时被诊断出,有类似表现,后方结构增宽且存在中线强回声骨嵴。出生后,两名婴儿均接受了磁共振成像以明确诊断。两例均在新生儿期进行了缺损的手术修复。

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