Neuroradiology 16, 87-88 (1978)
Hearoradiologv
© by Springer-Verlag 1978
D i a s t e m a t o m y e l i a - Rare and U n u s u a l F e a t u r e s R. L. Gilmor 1,2 and S. Batnitzky 1 Departments of Radiology I and Neurosurgery 2, Indiana University Medical Center, Indianapolis, Indiana, USA
Summary. Diastematomyelia is an uncommon congenital anomaly of the spine. Four unusual variations of diastem a t o m y e l i a are presented. These include (1) double diastematomyelia, (2) adult presentation, (3) diastematomyelia w i t h o u t a s e p t u m and unusual t e r m i n a t i o n o f two halves o f the spinal cord, and (4) recurrence o f a fibrous band following removal o f an osseous septum.
Diastematomyelia is a rare congenital a n o m a l y o f the spine in which there is a longitudinal separation o f the spinal cord or conus medullaris by a fibrous or an osseous septum. Multiple vertebral anomalies including spina bifida, irregular laminar fusion, increased interpediculate distance, hemivertebrae, and varying degrees o f vertebral b o d y fusions are associated features.
Clinical Material In an 8-year period from 1970 to 1978 at Indiana University Medical Center, 11 patients were evaluated and treated for diastematomyelia. Four patients from this group had unusual variations of this malformation. Case 1. Female, 4 years of age, complained of left leg pain, had hypertrichosis, and a normal neurologic examination. Plain f'dms of the spine demonstrated osseous septa at the T 6 - 7 and T-12 levels, which were also confirmed by myelography (Fig. 1). Both lesions were treated by removal of the septa along with the associated dural sleeves.
Fig. 1. Myelography in a 4-year-old female demonstrating double diastematomyelia due to osseous septa at the T 6 - 7 and T-12 levels
Case 2. Female, 32 years old, presented with progressive paraparesis and a flaccid bladder. Myelography demonstrated a seprum of the canal at the T-7 level which was not apparent on plain films. At operation an osseous spicule was found at the same level.
out evidence of a septum. The fight half of the spinal cord terminated in the neural plate and the left segment of the cord entered the distal sacral canal.
Case 3. Newborn male with a lumbar myelomeningocele. There was good motor function of the left leg and good hip flexors on the fight but poor distal function. At operation, the spinal cord was noted to be divided proximal to the myelomeningoeele with-
Case 4. Newbom male with a midline lumbar sinus tract and a sacral dimple. There was a normal neurologie examination, myelography was done because of a widened interpediculate distance and revealed a defect in the canal at the L-4 level. At
0028-3940/78/0016/0087/$01.00
88 operation, a bony spicule was found at the L-4 level. The sinus tract was superficial and did not involve the dura or spinal canal. Ten years following his initial surgery he developed increasing lordosis and spastic gait. Repeat myelography demonstrated a defect at the L-4 level which at operation was found to be a fibrous band at the site of the original bony spicule.
Discussion The clinical features of this anomaly, including cutaneous abnormalities and varying involvement of the lower extremities, have been well documented [3, 5, 7]. Adults are rarely affected [1]. Double diastematomyelia is a very rare variation of an already rare congenital lesion. A total o f 8 reports in the literature document a double diastematomyelia [ 3 - 5 ] . Diastematomyelia without a septum was identified in only two cases in the literature [5]. The usual variation in Case 3 with one portion o f the cord terminating in the neural plate and the other continuing into the sacral canal could be explained by Gardner's hypothesis [2] of distention and varying degrees o f rupture o f the embryonal central canal. Recurrence of a diastematomyelia is not documented in the literature. In our Case 4, despite adequate surgical treatment, a fibrous septum developed. The recurrence can most likely be accounted for on tile basis of scar formation.
R. Gilmor and S. Batnitzky: Diastematomyelia
References 1. English, W. J., Maltby, G. L.: Diastematomyelia in adults. J. Neurosurg. 27, 260-264 (1967) 2. Gardner, W. J.: The dyastraphic states from syringomyelia to anencephaly. Amsterdam: Excerpta Medica 1973 3. Guthkelch, A. N.: Diastematomyelia with median septum. Brain 97, 729-742 (1974) 4. Hilal, S. K., Marton, D., Pollack, E.: Diastematomyelia in children, radiographic study of 34 cases. Radiology 112, 609621 (1974) 5. Keim, H. A., Greene, A. F.: Diastematomyelia and scoliosis. J. Bone Joint Surg. (Am.) 55A, 1425-1435 (1973) 6. Neuhauser, E. B. D., Wittenborg, M. H., Dehlinger, K.: Diastematomyelia, transfication of the cord or cauda equina with congenital anomalies of the spine. Radiology 54, 659-664 (1950) 7. Winter, R. B., Haven, J. J., Moe, J. H., Lagaard, S. M.: Diastematomyelia and congenital spine deformities. J. Bone Joint Surg. (Am.) 56, 27-39 (1974)
R. L. Gilmor, MD Department of Radiology Indiana University Medical Center Indianapolis, IN 46202, USA
Hearoradiologv
© by Springer-Verlag 1978
D i a s t e m a t o m y e l i a - Rare and U n u s u a l F e a t u r e s R. L. Gilmor 1,2 and S. Batnitzky 1 Departments of Radiology I and Neurosurgery 2, Indiana University Medical Center, Indianapolis, Indiana, USA
Summary. Diastematomyelia is an uncommon congenital anomaly of the spine. Four unusual variations of diastem a t o m y e l i a are presented. These include (1) double diastematomyelia, (2) adult presentation, (3) diastematomyelia w i t h o u t a s e p t u m and unusual t e r m i n a t i o n o f two halves o f the spinal cord, and (4) recurrence o f a fibrous band following removal o f an osseous septum.
Diastematomyelia is a rare congenital a n o m a l y o f the spine in which there is a longitudinal separation o f the spinal cord or conus medullaris by a fibrous or an osseous septum. Multiple vertebral anomalies including spina bifida, irregular laminar fusion, increased interpediculate distance, hemivertebrae, and varying degrees o f vertebral b o d y fusions are associated features.
Clinical Material In an 8-year period from 1970 to 1978 at Indiana University Medical Center, 11 patients were evaluated and treated for diastematomyelia. Four patients from this group had unusual variations of this malformation. Case 1. Female, 4 years of age, complained of left leg pain, had hypertrichosis, and a normal neurologic examination. Plain f'dms of the spine demonstrated osseous septa at the T 6 - 7 and T-12 levels, which were also confirmed by myelography (Fig. 1). Both lesions were treated by removal of the septa along with the associated dural sleeves.
Fig. 1. Myelography in a 4-year-old female demonstrating double diastematomyelia due to osseous septa at the T 6 - 7 and T-12 levels
Case 2. Female, 32 years old, presented with progressive paraparesis and a flaccid bladder. Myelography demonstrated a seprum of the canal at the T-7 level which was not apparent on plain films. At operation an osseous spicule was found at the same level.
out evidence of a septum. The fight half of the spinal cord terminated in the neural plate and the left segment of the cord entered the distal sacral canal.
Case 3. Newborn male with a lumbar myelomeningocele. There was good motor function of the left leg and good hip flexors on the fight but poor distal function. At operation, the spinal cord was noted to be divided proximal to the myelomeningoeele with-
Case 4. Newbom male with a midline lumbar sinus tract and a sacral dimple. There was a normal neurologie examination, myelography was done because of a widened interpediculate distance and revealed a defect in the canal at the L-4 level. At
0028-3940/78/0016/0087/$01.00
88 operation, a bony spicule was found at the L-4 level. The sinus tract was superficial and did not involve the dura or spinal canal. Ten years following his initial surgery he developed increasing lordosis and spastic gait. Repeat myelography demonstrated a defect at the L-4 level which at operation was found to be a fibrous band at the site of the original bony spicule.
Discussion The clinical features of this anomaly, including cutaneous abnormalities and varying involvement of the lower extremities, have been well documented [3, 5, 7]. Adults are rarely affected [1]. Double diastematomyelia is a very rare variation of an already rare congenital lesion. A total o f 8 reports in the literature document a double diastematomyelia [ 3 - 5 ] . Diastematomyelia without a septum was identified in only two cases in the literature [5]. The usual variation in Case 3 with one portion o f the cord terminating in the neural plate and the other continuing into the sacral canal could be explained by Gardner's hypothesis [2] of distention and varying degrees o f rupture o f the embryonal central canal. Recurrence of a diastematomyelia is not documented in the literature. In our Case 4, despite adequate surgical treatment, a fibrous septum developed. The recurrence can most likely be accounted for on tile basis of scar formation.
R. Gilmor and S. Batnitzky: Diastematomyelia
References 1. English, W. J., Maltby, G. L.: Diastematomyelia in adults. J. Neurosurg. 27, 260-264 (1967) 2. Gardner, W. J.: The dyastraphic states from syringomyelia to anencephaly. Amsterdam: Excerpta Medica 1973 3. Guthkelch, A. N.: Diastematomyelia with median septum. Brain 97, 729-742 (1974) 4. Hilal, S. K., Marton, D., Pollack, E.: Diastematomyelia in children, radiographic study of 34 cases. Radiology 112, 609621 (1974) 5. Keim, H. A., Greene, A. F.: Diastematomyelia and scoliosis. J. Bone Joint Surg. (Am.) 55A, 1425-1435 (1973) 6. Neuhauser, E. B. D., Wittenborg, M. H., Dehlinger, K.: Diastematomyelia, transfication of the cord or cauda equina with congenital anomalies of the spine. Radiology 54, 659-664 (1950) 7. Winter, R. B., Haven, J. J., Moe, J. H., Lagaard, S. M.: Diastematomyelia and congenital spine deformities. J. Bone Joint Surg. (Am.) 56, 27-39 (1974)
R. L. Gilmor, MD Department of Radiology Indiana University Medical Center Indianapolis, IN 46202, USA
