Management of Type I Split Cord Malformation: Surgical Technique and Clinical Outcome

Background: Split cord malformations (SCM) are rare congenital anomalies in which the spinal cord is split into 2 hemicords with 2 dural tubes separated by a rigid septum (type I) or 2 hemicords separated by a fibrous septum in a single dural tube (type II). Objectives: Evaluation of surgical technique, timing, indications, clinical outcome and complications in treatment of SCM (type I). Material and Methods: Retrospective review of 9 patients with type I SCM (Diastematomyelia) operated upon in Cairo University Hospitals between January 2006 and December 2008. Patients were 6 females and 3 males, age ranged between 1.5 and 12 years (mean 5.5 years), skin manifestations were present with different types ranging from 11.1-55.5% each, orthopedic deformities ranged between 33.3% to 44.4%, neurological deficits were 11.1-66.6% and associated spina bifida aperta were present in 3 patients (33.3%). One of the 9 patients was asymptomatic and discovered accidentally. Preoperative MRI revealed thick filum terminale in 4 patients (44.4%) and tethered cord in all 9 patients (100%). The level of the diastematomyelia was lumbar in 66.6% of cases, dorsolumbar in 22.2% and dorsal in 11.1%. Results: Neurological deficits improved as follows; paraparesis (83.3%), sphincteric dysfunction (66.6%) while sensory deficits improved in only 50%. Radiologically, postoperative CT revealed proper excision of the bony septum, MRI showed proper position of the conus medullaris in 3 patients (33.3%) at 12 months interval. Patients were followed up clinically and radiologically for a mean period of 20 months. Postoperative complications were encountered in 3 patients (33.3%) and all were temporary and resolved conservatively within 3 weeks. Conclusion: Microsurgical intervention is the definitive treatment for cases of diastematomyelia being effective in preventing future neurological deficits, with good postoperative improvement and low incidence of complications. Surgery should be performed once the diagnosis is made. Prophylactic surgery is recommended in asymptomatic accidentally discovered lesions. Correspondence to: Dr. Hazem M. Kamal, E-mail: [email protected]