2023 Volume 14 Issue 11 Pages 1408-1412
Introduction: Diastematomyelia is a rare disease that is often asymptomatic. Patients rarely display clinical symptoms, however, spinal cord tension increases with growth causing progressive neurological symptoms. Surgical treatments include septal resection, spinal cord detachment and duraplasty, nevertheless, if the condition coincides with congenital scoliosis, these operations are often later followed by corrective fusion surgery in a two-stage manner. In the present case, corrective fixation was performed in a one-stage surgery with a resection limited to bony septum only. The case indicates that under careful monitoring, there are cases that can be corrected with minimal invasion.
Case report: An 11-year-old girl was referred to our hospital with a spinal abnormality after scoliosis school screening. Somatic findings showed no abnormalities in muscle strength or sensory ability, however, her Cobb angles at T1-T4-L1 were 19° and, 44°, a Lenke Type 1 deformity, with abnormal vertebral arch morphology at T11 to L1 and fused vertebrae at T9 to T10. The skeletal maturity was classified as Risser Grade 4 and thumb ossification composite index (TOCI) stage 7 and she was considered to be still in the growth period. In addition, CT/MRI examinations revealed a spinal cord split with a bony septum at T12. The patient was diagnosed with a congenital scoliosis with diastematomyelia and a one-stage surgery was performed. While monitoring under free-running MEG and MEP, the surgery first removed the bony septum that split the cord, and then using O-arm navigation, fused posterior spines for scoliosis treatment. The surgery was completed in 6 hours and 39 minutes with bleeding of 50 ml. After the operation, the Cobb angles at T1-T5-L2 were corrected to 10° and 8° with no postoperative complications.
Conclusions: The present case reports congenital scoliosis with type 1 diastematomyelia. The case suggests that under a careful monitoring, such scoliosis that are characterized by modest correction angle without neurological symptoms may be corrected in a one-stage surgery with minimal invasion, when the resection is limited solely to the bony septum.
