Segmental Spinal Dysgenesis: Case report.

, Park, Song Rae; , Park, Jin Hong; , Sung, In Young

Case Report

Journal of the Korean Academy of Rehabilitation Medicine 2007;31(1):127-130.

Park, Song Rae , Park, Jin Hong , Sung, In Young

Department of Rehabilitation Medicine, Asan Medical Center, University of Ulsan College of Medicine, Korea. iysung@amc.seoul.kr

분절성 척추이형성증 －증례 보고－

박송래, 박진홍, 성인영

울산대학교 의과대학 서울아산병원 재활의학교실

Abstract

Segmental spinal dysgenesis (SSD) is a rare congenital abnormality in which a segment of the spine and spinal cord fails to develop properly. Segmental vertebral anomalies involve the thoracolumbar, lumbar, or lumbosacral spine. We report two cases of SSD associated with other spinal dysraphism. Radiologic investigations revealed segmental dysgenesis of thoracic spinal cord without an associated vertebral bony anomaly in one case and segmental dysgenesis of the cervico-thoracic cord with diastematomyelia in the other case. The neurologic picture depends on the severity of the malformation and on its segmental level along the longitudinal embryonic axis. The pathogenesis of SSD syndrome is not clear but its cause is probably related to a segmental maldevelopment of the neural tube. (J Korean Acad Rehab Med 2007; 31: 127-130)

Key Words: Segmental spinal dysgenesis, Congenital abnormality