gms | German Medical Science

Objective: Tethered Cord Syndrome (TCS) comprises a combination of neurological, musculoskeletal and urological disturbances caused by abnormal tension of the spinal cord. TCS is radiomorphologically characterized by a low lying conus, shortening of the filum and is associated with (open or occult) spinal dysraphisms, but can also occur in combination with other conditions such as Chiari malformation. Microsurgical detethering is the therapy of choice in symptomatic TCS, but ideal timing is still unclear. The aim of the current study was to analyze the outcome after surgical detethering with regard to age, preoperative symptomology, and neurological outcome.

Methods: Clinical records of all patients under the age of 20 years microsurgically treated at our department for tethered cord syndrome and presented to our outpatient clinic at least once for follow-up between 01/2003 and 10/2016 were retrospectively analyzed.

Results: 59 patients (27 male, 32 female) diagnosed with symptomatic tethered cord syndrome were included in the current study. Patients were postoperatively followed up for 2.6 years on average (0.2 – 9.7 years). Most common initial symptoms included bladder disturbances (89.8%), muscle weakness (83.1%), foot deformities (45.8%), and back pain (27.1%). Mean age at operation was 6.1 years (10 days – 20.0 years). 37 patients (62.7%) were diagnosed with myelomeningocele perinatally and had undergone repair within 4 months after birth; 7 of the remaining patients without open spinal dysraphism were diagnosed with occult spina bifida on MRI images performed for TCS related symptoms. All patients underwent microsurgical detethering under intraoperative electrophysiological monitoring. Intraoperatively, a spinal lipoma was resected in 10 cases, a dermal sinus excised in 3 cases. Perioperative morbidity was 11.8%: 3 cases of CSF leak, 4 cases of impaired wound healing requiring surgical revision. There was no postoperative worsening of symptoms. Bladder function improved in 67.3% of cases presenting with this symptom; recovery of urinary function tended to be more complete when patients were operated before the age of 4 years. Pain abated in 62.5% of patients. Motor function significantly improved in 67.3% of patients. Of note, while of the 17 patients that were too young to stand and walk at the time of operation (below the age of 12 months) 11 were ambulatory at the time of last check-up, only one of 9 patients with detethering after the age of 1 year regained the ability to ambulate.

Conclusion: Our results indicate that neurological outcome, especially recovery of urinary function, after microsurgical dethetering, is more favourable when surgery is performed early in life and when symptoms are mild. Further and prospective analyses are needed in order to validate these findings.