A 5-year-old boy, born to a diabetic mother, presented with dribbling of urine since birth. Plain radiograph and MRI confirmed the diagnosis of caudal regression syndrome (figure, A through C).
(A) Lateral radiograph of lumbosacral spine reveals absence of distal sacrum (arrow). (B) Sagittal T1-weighted turbo spin echo MRI reveals abrupt termination of the conus medullaris with double bundle arrangement of nerve roots (arrow). (C) Sagittal T1-weighted turbo spin echo MRI reveals abrupt termination of the conus medullaris (arrow) and partial sacral agenesis.
Caudal regression syndrome results from abnormal canalization and retrogressive differentiation of the caudal cell mass in the early stages of gestation (33–40 days). In about 15%–25% cases, history of maternal diabetes mellitus is present. It encompasses a wide range of anomalies including variable degree of sacral dysgenesis, genitourinary anomalies, cardiac diseases, tethered cord, dermoid cyst, and diastematomyelia.1 Imaging reveals either high and abrupt termination of conus (type 1) or low-lying tethered cord (type 2).
AUTHOR CONTRIBUTIONS
Dr. Sharma: study concept and design, drafting and revision of manuscript. Dr. Jana: study concept and design, drafting and revision of manuscript, guarantor of study.
Footnotes
Disclosure: The authors report no disclosures.
- Copyright © 2011 by AAN Enterprises, Inc.
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