Caudal Regression Syndrome in a 12-Year-Old Boy Associated with Thecal Sac Ending at Fifth Lumbar Vertebrae Associated with Caudal Stenotic Dural Sac and Thickened Filum Terminale, Bifid Lumbar Vertebrae with Sacral Vertebral Agenesis: Pentads Defects

• Main Authors: Guru Dutta Satyarthee, Ashok Kumar Mahapatra
• Format: Article
• Language: English
• Published: Thieme Medical Publishers, Inc. 2017-02-01
• Series: Indian Journal of Neurosurgery
• Subjects: caudal agenesis; pentads
• Online Access: http://www.thieme-connect.de/DOI/DOI?10.1055/s-0036-1585459

Abstract Caudal regression syndrome is characterized by a spectrum of structural defects of the caudal vertebral region, varying from isolated agenesis or dysgenesis of coccyx to lumbosacral agenesis. It may be associated with congenital anomaly, spinal cord, distal genitourinary tract, and gastrointestinal tract. The authors report late presentation of caudal regression syndrome in a 12-year-old male student who had low backache and deformity of foot since childhood. He developed urinary incontinence by the age of 7 years. Magnetic resonance imaging revealed complete agenesis of lower three sacral and coccyx vertebral segments, with spina bifida of lower lumbar vertebra, stenosis of lumbar dural sac and thecal sac ending at L5, associated thickened filum terminale, and tethered cord. He underwent L2–L5 laminectomy with duraplasty and detethering of cord. Intraoperatively, markedly overcrowded lumbar and sacral nerve roots were observed. He noticed improvement of foot weakness and relief of backache following surgery.