156 Cervical Saccular Limited Dorsal Myeloschisis, So-Called “Cervical Myelomeningocele”: Long-Term Follow-Up of a Single-Center Series and Systematic Review

INTRODUCTION: Cervical saccular limited dorsal myeloschisis (LDM), the so-called “cervical myelomeningocele,” is a rare spinal dysraphism. LDM results from a delayed abnormality during the final stages of neurulation. METHODS: A retrospective study was conducted at our center. Information on patients who underwent surgery was extracted and combined with data from a previously published series from the same center. Also, pooled data from the literature on similar lesions were systematically reviewed. RESULTS: 38 patients aged 11.75 ± 28.64 months at surgery were included. The rates of neurological deficit, hydrocephalus, and CM-II were 26.32%, 39.47%, and 28.95%, respectively. The lesions were at the upper levels in 44.7% and lower-cervical levels in 55.3%, with 81.58% diagnosed with stalk-type and 18.42% with myelocystocele-type lesions. At the final follow-up, 81.57% achieved continence, and all 36 accessible patients were ambulated, consisting of 73.68% independent and 21.05% dependent. The rates of CM-II and hydrocephalus were insignificantly higher in patients with upper-level lesions, but those of neurological deficit, ambulation, and continence were not associated with level. The rates of hydrocephalus (p<0.01), CM-II (p<0.01), and neurological deficit (p=0.04) were significantly higher in the myelocystoceles. In the review, regression showed that older age at surgery (p=0.03) and associated spinal anomalies (p=0.04) were significant predictors of deficits. CM-II was significantly (p<0.001) more common in patients with myelocystocele-type lesions. CM-II incidence was higher in upper-level lesions (p=0.02). CONCLUSIONS: Patients with cervical saccular LDM had better outcomes compared with those patients with true myelomeningocele in more distal areas. According to the current series, most patients obtained ambulation and voiding continence, regardless of the level or type of lesion. Hydrocephalus, CM-II, and neurological deficits were more common in patients with myelocystocele-type lesions.