gms | German Medical Science

Objective: Intrathecal baclofen (ITB) has been commonly used as treatment for dystonia. However, in rare cases, the use of ITB is not possible. For these patients, intraventricular baclofen (IVB) is a valid alternative. Up to now, most published case series made use of an endoscopic approach for implantation of the intraventricular catheter. Reducing the cortical manipulation to a minimum, implantation of IVB catheters with navigated frameless stereotaxy is the most minimal invasive approach feasible. For this, literature is scarce. Therefore, the aim of the study was to evaluate indications, surgical technique, complication rate and outcomes of IVB application with navigated frameless stereotaxy as an effective alternative for ITB in patients with severe generalized secondary and hereditary dystonia.

Methods: A retrospective analysis of all patients who were treated with IVB in our institution in the period between 2009 and 2019 was performed. All intraventricular catheters were implanted by navigated frameless stereotaxy. Intraoperatively, application ofcontrast agents was used to verify the correct placement of the catheter.

Results: Thirteen patients (six males, seven females) with severe generalized secondary and hereditary dystonia were treated with IVB. The mean age of the patients was 12.5 years (range 2-23 years). Indications for the use of IVB included preceding infection of the spinal catheter with intrathecal adhesions, insufficient ITB response, dystonic storms with a predisposition for spinal catheter dislocation, conservatively managed as well as surgically instrumented anatomical deformities of the spine and thereby maintaining the possibility to undergo a spinal surgery procedure in the future. Twelve catheters were located into the third ventricle and one catheter was positioned into the lateral ventricle. In our cohort, no perioperative or technical complication occurred. The mean follow up was 16 months. Dystonia improved in 12 of 13 patients. One child who did not respond to IVB had not responded to previous ITB as well. As side effects nausea and vomiting were observed after the implantation of IVB. One child developed a pump infection and required pump removal.

Conclusion: Our study indicates, thatIVB aided by navigated frameless stereotaxy is an effective, minimally invasive and safe therapeutic option with only few side effects in the management of rare cases with severe dystonia, when ITB drug delivery is not feasible.