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Year : 2000  |  Volume : 10  |  Issue : 4  |  Page : 255-257
Diastematomyelia


Dept of Radiodiagnosis, Command Hospital, Pune, India

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Keywords: Diastematomyelia, spinal dysraphism

How to cite this article:
Singh H, Maurya V, Saini M. Diastematomyelia. Indian J Radiol Imaging 2000;10:255-7

How to cite this URL:
Singh H, Maurya V, Saini M. Diastematomyelia. Indian J Radiol Imaging [serial online] 2000 [cited 2019 Jun 24];10:255-7. Available from: http://www.ijri.org/text.asp?2000/10/4/255/30575
Diastematomyelia is a rare form of spinal dysraphism characterized by a sagittal cleft of varying extent in the spinal cord, conus medullaris or filum terminale with splaying of the posterior vertebral elements. This condition is the result of the presence of an osseous, cartilaginous or fibrous septum producing a complete or incomplete sagittal division of the spinal cord into two hemicords. It may be isolated or associated with other segmental anomalies of the vertebral bodies [1].


   Case Report Top


A two-and-a-half-years-old child presented with abnormal gait and flat foot having mild foot drag on right side. This was noted by his parents from the time he started walking. There was no history of trauma, fever or seizures and no history suggestive of infantile hemiplegia.

On examination he had pes planus with the right foot slightly smaller than the left. He had a foot drop on the right side with mild wasting of the right calf muscles with decreased tone. Knee and ankle jerks were sluggish with plantar extensor on the right side. The right gluteal reflux was also sluggish. Bowel and bladder habits were normal. Routine blood and urine examinations were within normal limits.

A plain radiograph of the dorsolumbar spine in the frontal and lateral projections revealed widening of the interpedicular distance from D12 to sacrum retaining the medial convexity of the pedicles with varying degree of fusion defects involving the posterior elements and multiple bifid spinous processes [Figure - 1]. CT scan confirmed the plain film findings with a diagnosis of diastematomyelia. It revealed the spinal cord dividing into two hemicords within the same sac extending from D11 to L4-5, the right hemi-cord thinner than the left [Figure - 2],[Figure - 3]. The two hemi-cords were fused and tethered to the posterior elements of the L4 and L5 vertebrae. An ultrasound examination performed in the prone position due to examination performed in the prone position due to excessive bowel gas while in the supine position, through the interspinous lumbar spaces demonstrated the two hemicords lying within the echofree CSF, the right hemicord being thinner than the left [Figure - 4]. MRI confirmed the diagnosis of diastematomyelia. The cord was seen splitting into two hemicords superiorly at the level of D11 [Figure - 5] and extending upto the L4-5 vertebral where the two were fused just before the cord terminated. They were seen enclosed in the same dural covering [Figure - 6]. No osseous, cartilaginous or fibrous septum could be demonstrated in any of the imaging modalities.


   Discussion Top


Diastematomyelia is a dysraphic state of unknown embryogenesis, but is probably initiated by an accessory neurenteric canal. Narrowing of a disc space at the same level and interlaminar fusion are supportive evidence. Increased width of the spinal canal with retained medial convexity of the pedicles occurs with diastematomyelia and a bony spicule dividing the canal is confirmatory but in most cases the division is cartilaginous or fibrous [2]. No bony spicule, cartilaginous or fibrous division could be demonstrated in this case. Most cases are seen in association with other anomalies of the vertebral column such as spina bifida, kyphoscoliosis, butterfly vertebra and hemivertebra [3].

Diastematomyelia usually occurs between D9 and S1 levels [4],[5]. Cervical diastematomyelia is a very rare entity [6],[7]. In about 50% of patients, the hemi-cords are contained in a single dural sac as seen in this patient, while in the remaining they lie within separate dural sacs. In the latter, bony or fibrous spurs are usually found between the two sacs [8]. Associated intramedullary tumors with diastematomyelia have been rarely described [9] and associated conditions like tethered cord, inclusion dermoid, lipoma, syringohydromyelia and  Chiari malformation More Details have been described in literature [2],[10].

Prenatal ultrasound diagnosis of this anomaly is usually possible in the early mid third-trimester. An extra posterior echogenic focus between the fetal spinal laminae is seen with splaying of the posterior elements, thus allowing for early surgical intervention and a favorable prognosis [1],[11]. Depending on whether the diastematomyelia is isolated, with the skin intact or is in association with more serious neural tube defects, prenatal diagnosis of this condition is possible [1]. Apparently the fixation of the spinal cord by diastematomyelia seems not to be the primary cause of the "tethering cord syndrome". Other conditions, which are sometimes associated with diastematomyelia (myelodysplasia, dysraphia of the spinal cord etc), may be the primary cause of progressive neurological lesions [12].

Bony malformations and dysplasias are generally recognized on plain radiograph and if clinically indicated can be further elucidated by CT along with demonstration of spinal abnormalities. However MRI will generally allow adequate analysis of the spinal deformities and is the technique of choice for dysraphism requiring full elucidation [2].

 
   References Top

1.Allen LM, Silverman RK. Prenatal ultrasound evaluation of fetal diastematomyelia: two cases of Type I split cord malformation. Ultrasound Obstet Gynecol 2000; 15:78-82   Back to cited text no. 1  [PUBMED]  [FULLTEXT]
2.Brian EK. Neuroradiology of the spine. In: David S. Text book of radiology and imaging, 6th ed. New York: Churchill Livingstone, 1998: 1497-1499.   Back to cited text no. 2    
3.Sepulveda W, Kyle PM, Hassan J, Weiner E. Prenatal diagnosis of diastematomyelia: case reports and review of the literature. Prenat Diagn 1997; 17: 161-165   Back to cited text no. 3  [PUBMED]  [FULLTEXT]
4.Byrd SR, Darling CP, McLone DG. Developmental disorders of paediatric spine. RCNA 1991; 29: 4-5.   Back to cited text no. 4    
5.Bruberg JA, Latchaw RC, Emanuel K et al . Magnetic resonance imaging in occult spinal dysraphism. RSNA 1988; 26: 181-205   Back to cited text no. 5    
6.Anand AK, Kuchner E, James R. Cervical diastematomyelia: uncommon presentation of a rare congenital disorder. Comput Radiol 1985; 9: 45-49.   Back to cited text no. 6  [PUBMED]  
7.Herman TE, Siegel MJ. Cervical and basicranial diastematomyelia. AJR Am J Roentgenol 1990; 154: 806-808.   Back to cited text no. 7  [PUBMED]  
8.Castillo M, Mukherji SK. Imaging of paediatric head neck and spine. Philadelphia: Lippincott Raven, 1996: 630-635.   Back to cited text no. 8    
9.McMaster MJ. Occult intraspinal anomalies and congenital scoliosis. J Bone Joint Surgery (Am) 1984; 66: 588-601.   Back to cited text no. 9  [PUBMED]  
10.Han JS, Benson JE, Kaufman B, et al . Demonstration of diastematomyelia and associated abnormalities with MR imaging. AJNR Am J Neuroradiol 1985; 6: 215-219.   Back to cited text no. 10    
11.Anderson NG, Jordan S, MacFarlane MR, Lovell-Smith M. Diastematomyelia: diagnosis by prenatal sonography. AJR Am J Roentgenol 1994; 163: 911-914.   Back to cited text no. 11  [PUBMED]  
12.Banniza von Bazan U, Krastel A, Lohkamp FW. Diastematomyelia - a harmless finding or cause of late neurological disturbance? [Article in German] Z Orthop Ihre Grenzgeb 1978; 116: 72-80  Back to cited text no. 12  [PUBMED]  

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Correspondence Address:
Hariqbal Singh
Dept of Radiodiagnosis, Command Hospital, Pune 411 040
India
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Source of Support: None, Conflict of Interest: None


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    Figures

[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5], [Figure - 6]

This article has been cited by
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