21-year-old female having history of swelling in lower back region since birth presented with lower back pain associated with radiculopathy since 3 months with no history of bowel/bladder incontinence. On clinical examination skin lined swelling was seen at lower back region. Detailed radiological examination was done.
Diastematomyelia is a spinal dysraphism in which there is partial/complete sagittal clefting of one or more segments of the spinal cord, conus medullaris, and/or filum terminale dividing the spinal cord into two hemicords which contains one central canal, one dorsal horn that gives origin to the ipsilateral segmental dorsal nerve root, and one ventral horn that gives origin to the ipsilateral segmental ventral nerve root. The involved segments may be contiguous or anatomically distant from each other [1, 2].
Depending upon the presence or absence of a fibrous partition or bony spur interposed between the hemicords there are two types of Diastematomyelia. In type 1, the two hemi cords are located within individual dural tubes separated by an osseous, fibrous or cartilaginous septum. In type 2 having a single dural tube contains two hemi cords, sometimes with an intervening ﬁbrous septum .
The severity of symptoms and signs varies greatly, ranging from cutaneous hairy patch and swelling at sacral region to gait disturbance with mild atrophy or weakness of one or both lower extremities, absent deep tendon reflexes, progressive paraparesis, and bladder or bowel dysfunction [3-5]. The diagnosis depends on presence or absence of bony spur and the associated features of spinal dysraphism such as spina bifida occulta, block vertebrae, widened interpediculate distance, hemivertebrae, and scoliosis which are commonly seen with type-I [6-8].
The open forms of spinal dysraphism, such as meningocele and myelomeningocele, can be identified on obstetric sonography. The occult forms, such as intracanalicular lipoma and a tethered cord can be identified through sonographic screening of neonates and infants . In late childhood and adults, plain radiographs of spine can give good details about abnormalities of spinal cord and vertebral bodies and bony spur. Computed tomography can demonstrate bony spur and its extent and relationship with vertebral bodies and vertebral anomalies more accurately. MRI with its better soft tissue contrast, allows visualization of extent of split in the cord and shows associated anomalies such as lipoma and syrinx. So accurate diagnosis can be made. T1WI is good for demonstrating lipomyelocele and filum terminale tethering. T2WI can show evidence of hydrosyringomyelia.
Treatment options vary. Tethering of cord by bony spur should be relieved immediately after diagnosis, especially before growth spurt as most of patients are symptomatic during growth spurt, possibly due to traction by spur on spinal cord. Some studies prefer removal of spur when patient becomes symptomatic.
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