*6-year-old girl with a hairy patch on her lower back from birth.
What is the most likely diagnosis?
Answer
Answer: Diastematomyelia
Case Discussion:
MRI reveals type I split cord malformation from T11 to L3, occult intrasacral meningocele (blue arrows), and tethered cord. There is bony spur (red arrow) with two hemicords in two dural sacs (green arrows).
Diastematomyelia (also known as a split cord malformation) is a type of spinal dysraphism when there is a longitudinal split in the spinal cord.
They are a congenital abnormality and account for ~5 % of all congenital spinal defects.
Classification
• type I: duplicated dural sac, with common osseous or fibrous midline spur and usually symptomatic
• type II: single dural sac containing both hemicords
References:
1. Davies SG. Aids to Radiological Differential Diagnosis. Saunders Ltd. (2009)
2. Özek MM, Cinalli G, Maixner WJ. The Spina Bifida, Management and Outcome. Springer Verlag. (2008)