History :
The 4+M/O girl was a case of diastematomyelia noted since birth without ans dysfunction noted at present. This time, she was admitted due to rapid enlargement of paraspinal mass in recent 5 days.

Image finding :
MRI of T-spine without & with contrast shows:

-Diastematomyelia with bony bridge and two hemicord are noted at T9-T10.
-presence of syrinx above the lesion
-No definite abscess is detected.

Diagnosis :
Spina Bifida with overlying hemangioma. Diastematomyelia.

DIASTEMATOMYELIA
=SPLIT CORD
=sagittal division of spinal cord into two hemicords, each of which contains a central canal, one dorsal horn + one ventral horn

Etiology:
congenital malformation as a result of split notochord; M:F = 1:3

Path:
--(a)2 hemicords each covered by layer of pia within single subarachnoid space + dural sac (60%); not accompanied by bony spur / fibrous band
--(b)2 hemicords each with its own pial, subarachnoidal + dural sheath (40%); accompanied by fibrous band (in 25%), cartilaginous / bony spurs (in

Associated with:myelomeningocele
-hypertrichosis, nevus, lipoma, dimple, hemangioma overlying the spine (26-81%)
-clubfoot (50%)
-muscle wasting, ankle weakness in one leg

Location: lower thoracic / upper lumbar > upper thoracic > cervical
-congenital scoliosis (50-75%) 5% of patients with congenital scoliosis have
-spina bifida over multiple
-anteroposterior narrowing of vertebral bodies widening of interpediculate distance
-narrowed disk space with hemivertebra, butterfly vertebra, block vertebra
-fusion + thickening of adjacent laminae (90%)
--(a)fusion to ipsilateral lamina at adjacent levels
--(b)diagonal fusion to contralateral adjacent lamina = intersegmental laminar fusion
-bony spur through center of spinal canal arising from posterior aspect of centra (<50%)
-thickened filum terminale >2 mm (>50%)
-tethered cord (>50%)
-low conus medullaris below L2 level (>75%)
-the 2 hemicords usually reunite caudal to cleft
-defect in thecal sac on myelogram

Cx :
progressive spinal cord dysfunction