CASE 10456 Published on 21.01.2013

A case of diastematomyelia with minor dysplasias of the posterior elements of lumbar spine

Section

Neuroradiology

Case Type

Clinical Cases

Authors

Kouskouras K2, Giannakopoulou L1, Dimitriadou A2, Sarli A2, Antoniou N2, Drevelegas A2

1. Euromedica Pilis Axiou
2. AHEPA General University Hospital of Thessaloniki
Eratiras 39, 55133 Thessaloniki, Greece;
Email:linagiannakopoulou@yahoo.gr
Patient

31 years, female

Categories
Area of Interest Neuroradiology spine ; Imaging Technique MR, CT
Clinical History
A 31-year-old female patient presented with worsening low back pain and progressive neurologic deficit of her lower limbs, after a blunt traumatic injury of her spine a few months ago. Her medical history appeared otherwise unremarkable.
Imaging Findings
The patient underwent a lumbar spine MRI examination, which revealed diastematomyelia, with splitting of the distal spinal cord and conus medullaris into two hemicords within a single dural tube, with no osseous spur or septum between them. Multiple minor osseous dysplasias of the posterior elements of the lumbar spine were also identified during the following CT examination, including bilateral spondylolysis at the level of L5 vertebra without spondylolisthesis, spina bifida at the level of L1 and L2 vertebrae with accompanying synosteosis of their spinous processes - resulting in scoliosis - and a small osseous sinus track at the same level (L1-L2) with a possible lipoma of the posterior paraspinal soft tissues. No pathological findings were reported from the intervertebal disks.
Discussion
Diastematomyelia is a form of spinal dysraphism characterised by partial or complete sagittal clefting of the spinal cord into two symmetrical or asymmetrical hemicords, which usually reunite caudally [1, 4]. Each hemicord contains one central canal and gives rise to the ipsilateral segmental dorsal and ventral nerve roots.
Diastematomyelia occurs more frequently in the thoracolumbar region and the majority of the cases are recognised during childhood [1]. It is considered as a result of abnormal development of the notochord during embryogenesis [5]. Many other congenital malformations may be identified along with diastematomyelia, such as other dysraphisms (meningocele, myelomeningocele, spinal lipoma, dermal sinus, neurenteric cyst), anomalies of the vertebral bodies (kyphosis, scoliosis, spina bifida, hemivertebrae, butterfly vertebrae) or visceral malformations (kidney, rectum, uterus) [2, 5]. Diastematomyelia is frequently focal and variable in length (2-15 vertebral segments) [1]. Conus medullaris is typically low in position (below L2 level). The two hemicords are usually supplied by two seperate anterior spinal arteries [1].
There are two types of diastematomyelia: type 1, in which an osseous or osteocartilaginous spur-septum seperates the two hemicords (thus creating two dural sacs, each one containing one hemicord) and type 2, in which the two hemicords are contained in a single dural sac, with no osseous spur between them [3, 4]. Type 1 diastematomyelia is frequently symptomatic, whereas type 2 often progresses asymptomatically [5]. The signs and symptoms of diastematomyelia may appear at any time during life, including low back pain, sensory and motor problems, foot and spinal deformities and incontinence. Hairy patch, haemangioma, lipoma and teratoma often co-exist [2, 5].
Modern imaging techniques, such as MRI, CT and US are able to reveal the extent of the spinal cord splitting and the possible associated vertebral deformities [2].
Asymptomatic patients do not undergo surgery. Surgical decompression is ordered in symptomatic patients, with progressive neurologic deficits or a new onset of symptoms related to the situation [5].
Differential Diagnosis List
Diastematomyelia with no osseous spur.
Diastematomyelia with oseeous spur
Diastematomyelia with no osseous spur
Final Diagnosis
Diastematomyelia with no osseous spur.
Case information
URL: https://www.eurorad.org/case/10456
DOI: 10.1594/EURORAD/CASE.10456
ISSN: 1563-4086