gms | German Medical Science

Objective: Split cord malformations (SCMI and II) in children and adults are rare findings and well-known cause for tethered cord syndrome. We expect a wide variety of different forms. When complaints and symptoms occur surgery with the goal of untethering the cord is usually indicated. Our last patient is a young girl with a very complex spinal deformity, a bony spur originating dorsally and progressive symptoms of cord tethering. We present the findings to underline the wide complexity of these malformations.

Methods: In preparing the treatment of the child we reviewed our patient group with special focus on the type and anatomical origin of the septum or spur dividing the spinal canal. Clinical, radiological and intraoperative findings of the girl were reviewed and compared to neuropathological and neuroradiological descriptions. Follow-up is complete. Data of 10 other patients treated were used for the purpose of comparing different forms of split cord malformation seen by us before.

Results: In a group of 10 patients (8 children and 2 adult patients) operated for diastematomyelia a girl 9 years of age was admitted last. She was treated abroad in 2010 for open spina bifida. The girl now suffered from symptoms of cord tethering including pain and progressive motor deficits. First MRI imaging was performed in 2017 but the child was not introduced to a neurosurgeon at that time. X-ray, MRI and CT scans in 2020 showed complex vertebral deformities and a bony spur was noted originating dorsally from the dysplastic lamina of L2 and extending almost to the backside of the dysplastic vertebral body. The spur was removed and untethering of the cord could be achieved. Postoperative course was uneventful and symptoms are gradually resolving. In a second combined operative procedure spinal deformity and kyphoskoliosis were treated few months later.

Conclusion: When clear dividing structures in the midline are detected a dorsal approach possibly including laminoplasty may be the primary option. In our last patient with a spur dorsally originating from the lamina only a wide dorsal opening was effective enough. Surgery for split cord malformations is clearly indicated in symptomatic patients but it is a challenging procedure due to the often widely distorted anatomical structures.