Combined intrathecal baclofen pump revision to the cervical level and lumbosacral ventral-dorsal rhizotomy for severe medically refractory hypertonia: illustrative cases

BACKGROUND Medically refractory hypertonia (MRH) within the pediatric population causes severe disability and is difficult to treat. Neurosurgery for mixed MRH includes intrathecal baclofen (ITB) and lumbosacral ventral-dorsal rhizotomy (VDR). Surgical efficacy limitations can be mitigated by combining the two into a multimodal strategy. The authors examined outcomes following a multimodal neurosurgical strategy combining intraspinal ITB catheter revision to the cervical level and lumbosacral VDR. OBSERVATIONS Two patients with severe MRH resistant to ITB delivered through a thoracic catheter tip were identified: 1) a 16-year-old boy with quadriplegic mixed hypertonia and 2) a 17-year-old girl with secondary dystonia. The patient in case 1 experienced improvement in his Barry-Albright Dystonia Scale (BADS) score from 29 to 17 and lower-extremity modified Ashworth Scale score from 4 to 0 at 18 months postoperatively; the patient in case 2 experienced a decrease in her BADS score from 30 to 13 at 6 months postoperatively. Significant improvement in caregiving provisions, including patient positioning and transfers, was reported. LESSONS The authors highlight favorable outcomes using multimodal surgery in pediatric patients. Multimodal therapy is surgically feasible and better addresses MRH, particularly in patients in whom ITB monotherapy and polypharmacy have failed. Future studies with larger patient volumes are necessary to optimize indications and make more definitive outcome conclusions.