gms | German Medical Science

Objective: Apart from the virtual spinal dysraphism, several malformations at the spinal level are well known in children with spina bifida. These include Arnold-Chiari malformation, syringomyelia, tethered cord and others. Almost no literature has yet been published concerning changes over time of these malformations. We analyzed serial spinal MRI scans of our patients with spina bifida with respect to operative consequences in order to obtain a better understanding of time course and clinical meaning of these malformations.

Methods: We currently take care of 53 children with spina bifida of which 41 could be analyzed. We reviewed 105 MRI scans (1.5 Tesla), T1 and T2 sagittal and axial weights, all without Gadolinium. Repetitive scans were performed depending on the specific clinical course or regularly every two to three years. Specifically we looked for occipito-cervical malformations (Arnold-Chiari), intramedullary malformations (syringomyelia, tumours), intraspinal lipomas and tethered cord.

Results: We analyzed 41 children (f=15, m=26). Thirty-three suffered from a lumbar or lumbo-sacral myelomeningocele. Seven patients showed a thoracic or thoraco-lumbar MMC and one patient had a cervical MMC. An Arnold-Chiari malformation was present in 27 children. None had to undergo an occipito-cervical decompression with the exception of one, who developed an arachnoid cyst at the level of foramen magnum and a paresis of the left arm. Twenty-one children developed a syringomyelia, one child required placement of a T-drain. In 7 patients MRI-scans showed decreasing syringomyelia, in 3 cases we noted an increasing finding without clinical deterioration. MRI-scans of 2 children displayed alternating proportions of the syringomyelia as well without a change in the clinical findings. Thirty-eight children had a tethered cord and in 4 children we performed detethering, three times because of worsening of gait function and one time for an operative correction of a scoliosis. We operated on four of 8 intradural lipomas and removed 4 dermoid cysts.

Conclusions: A decisions to operate or not was always an interdisciplinary approach with respect to clinical conditions of the specific patient; it was not only based on imaging findings. We generally advocate a more reluctant strategy especially concerning operations on Arnold-Chiari malformations or tethered cord. Regular clinical and imaging examinations are necessary for the management of these patients. We use electrophysiology to facilitate our decisions.