Diagnosis and treatment of diastematomyelia in children: a perspective cohort study

Abstract

Study design Cohort study.Objectives The objectives of this study were examination, observation and surgical interventions of pediatric patients with diastematomyelia, a rare congenital deformity of the spinal cord. Setting Spine surgery and neurosurgery clinic under ministry of health, the Russian Federation. Methods Twenty children (1-18 years of age) with diastematomyelia participated in this study. Diagnosis was based on subjects' medical history, results of clinical examination, spine radiography in anteroposterior and lateral views, and neurophysiological examinations. Septum resection surgery was done in seventeen subjects. Results All the subjects had skin manifestations along the midline of the back. Neurological symptoms were noted in 17 subjects: paraparesis in 11, urinary incontinence in 2, monoparesis in 4, and spasticity in 2 subjects. Three subjects did not show any neurological deficit. Orthopedic examination revealed spinal deformities of various degrees of severity. In 4 subjects, partial restorations of neurological functions were observed; and in 1 subject, bladder incontinence developed after the surgery. Conclusions Patients who have limb length discrepancy, congenital scoliosis, skin manifestations should be examined for the presence of diastematomyelia. Patients having neither neurological deficit nor orthopedic deterioration should be monitored, and in case of deterioration, surgical treatment should be reserved. We advocate for performing resection of the septum in patients with diastematomyelia prior to surgical correction of scoliosis or orthopedic correction of lower limb deformities.

Contiguous diastematomyelia with tethered cord, intradural extramedullary dermoid tumor, and lipomyelomeningocele: A unique case of spinal dysraphism

“…The presence of bony spur indicates a necessity for surgery because of the associated progressive neurological deterioration in these patients. The bony spur should be removed prior to surgical correction of the spine 11 . There is some doubt as to whether untethering should be undertaken in such a patient or not.…”

Diastematomyelia differences in management of diastemetomyelia with associated abnormalities versus isolated diastemetomyelia: a case series

“…Thus, as soon as any neurological deterioration signs are detected, a prophylactic laminoplasty over laminectomy is preferred for better prognosis of the infant. 9 Cases which show no signs of neurological deterioration, should be meticulously observed and managed surgically if any signs of deficit occur.…”

A Case of Diastematomyelia Presenting With Minimal Neurologic Deficits in a Middle-Aged Patient

“…This disorder belongs to the group of spinal dysraphisms, along with other disorders such as meningocele, myelomeningocele, and spina bifida occulta. It also often coexists with these spinal dysraphisms in addition to others, or vertebral anomalies such as scoliosis, hemivertebrae, or butterfly vertebrae [1][2][3].…”

“…It is believed that diastematomyelia is caused by the presence of a bony, fibrous, or cartilaginous septum that moves dorsally towards the vertebral canal, arising from the posterior surface of the anterior wall during development. This leads to abnormal development of the notochord during the 15-18th days of pregnancy [1][2][3]. MRI is the diagnostic test of choice for this disorder [4].…”

“…The clinical presentation of diastematomyelia is similar to that of tethered cord syndrome, in that most patients present with neurologic disturbances such as back pain, asymmetric reflexes, progressive weakness, muscle atrophy, loss of sensation, paresthesias, bowel and bladder dysfunction, spasticity, or paresis. The reason for these deficits is likely due to damage and tension on the spinal cord due to limited space in the vertebral canal [ 1 , 2 ].…”