Faculty Bibliography

OBJECTIVE:Tethered cord syndrome (TCS) is a significant cause of debilitation in patients with spinal dysraphisms or tumors. Management of TCS has historically centered on intradural detethering surgery, which is associated with a substantial risk of retethering, symptom recurrence, and repeat surgery. Vertebral column resection (VCR) has recently gained popularity as a treatment option via spinal shortening, but the literature on long-term outcomes is sparse. The objective of this study was to explore long-term clinical outcomes in a series of patients undergoing VCR for recurrent TCS. METHODS:A retrospective review was conducted of all consecutive patients undergoing VCR for recurrent TCS at a single center between 2014 and 2024. Demographic and radiological characteristics were recorded, including spinal column height reduction as well as symptom improvement rates regarding sensorimotor dysfunction, pain, and incontinence at the latest follow-up. Intra- and postoperative complications were noted, and pre- and postoperative symptomology was compared using McNemar's test at a significance level of p < 0.05 to identify improvements. RESULTS:Twenty surgeries in 19 patients (median age 19 years) involving 10 males (52.6%) were assessed. The most common etiology of TCS was lipomyelomeningocele (n = 7, 36.8%), followed by lipoma (n = 6, 31.6%). Patients underwent a mean of 3.4 previous detethering procedures. Osteotomy was most commonly performed at the L1 level (n = 11, 55.0%), and the mean spinal column height reduction was 16.5 mm. At a mean follow-up of 43.1 (range 12-101) months, resolution of symptoms was observed in 10 patients with back pain (71.4%; p = 0.043), 10 patients with leg pain (66.7%; p = 0.004), 9 patients with lower extremity weakness (75.0%; p = 0.008), 4 patients with urinary incontinence (44.4%; p = 0.371), and 1 patient with fecal incontinence (33.3%; p > 0.99). Complications included a CSF leak in 1 patient and repeat VCR in 1 patient. CONCLUSIONS:These results suggest that in select patients with recurrent TCS, VCR represents a treatment option with high rates of symptomatic improvement and a relatively low risk of retethering as observed over several years of follow-up. These results add to the body of literature supporting VCR and provide a compelling basis for future prospectively designed studies.

Crouzon's disease is a well-known disorder affecting multiple organ systems, specifically a craniofacial disorder with highly variable penetrance and severity of deformity. Crouzon's patients typically have anomalies of the skull base leading to gross distortion of the cranium and in some cases the cervicocranium. We present a 5-year-old girl with Crouzon's disease who suffered from an acquired Chiari I malformation after insertion of a ventriculoperitoneal shunt and a coexistent ventral odontoid panus. Both these lesions were causing cervicomedullary compression. The literature is controversial on the surgical management of anterior and posterior compression at the craniocervical junction. We review the literature on surgical options for decompression at the craniocervical junction and offer our surgical case as a treatment option for patients in this rare clinical situation