Intrathecal Access Through Suboccipital Port in Patients With Spinal Muscular Atrophy and Complex Spines: Case Series and Technical Note.

Introduction Many patients with the spinal muscular atrophy (SMA) have complex spinal anatomy, secondary to thoraco-lumbar spinal fusions. Their fragile musculoskeletal anatomy potentiates limb and joint injury if conventional spinal fluid access modalities are utilized. This creates a challenge when attempting to deliver intrathecal medications such as nusinersen (Spinraza®). Catheter placement in the cervical subarachnoid space with a caudally directed tip is potentially beneficial. This article describes our experience with Spinraza injections into the thecal space through a suboccipital port. This allowed for simple, chronic, and reliable cerebrospinal fluid (CSF) aspiration and intrathecal injections. Methods A total of 15 patients with SMA and complex spinal anatomy were implanted with a cervical subarachnoid catheter, connected to a suboccipital access port. We retrospectively reviewed the charts of these patients for clinical outcomes and complications. All patients then underwent serial port cannulation, aspiration of CSF, and injection of Spinraza following standard manufacturer dosage guidelines. Results The age range was 3 to 49. Two had type-1 SMA, 10 had type-2 SMA, and three had type-3 SMA. We were able to successfully cannulate the port, aspirate CSF, and inject Spinraza during all access attempts. Two incidents of subcutaneous CSF leaks were resolved through reoperation and one incident of transient CSF leak was resolved without surgical repair. Conclusion Patients with SMA requiring intrathecal injections of Spinraza can be treated safely and efficiently with this novel implantation technique. The complication rates are low and the injection time is dramatically lower than with conventional injection techniques.