Clinical Sciences/Health Conditions
Ahmed Khawer, DO
Resident Physician
Johns Hopkins University
Baltimore, Maryland, United States
Ankur Jain, DO
Resident Physician
Johns Hopkins University
Baltimore, Maryland, United States
Amaan Sheikh, DO
Resident Physician
Johns Hopkins University
Baltimore, Maryland, United States
Daniel Khokhar, DO
Resident Physician
Johns Hopkins University
Baltimore, Maryland, United States
Dorianne Feldman, MD
Attending Physician
Johns Hopkins University
Baltimore, Maryland, United States
Daniel Krasna, MD
Attending Physician
Johns Hopkins Hospital
Baltimore, Maryland, United States
Autonomic Dysreflexia due to pain from non-standard intrathecal baclofen pump placement in a patient with Multiple sclerosis requiring explant.
Case Description: A 46 year old woman with multiple sclerosis, spastic quadriplegia, Ehlers–Danlos syndrome, and complex urologic and bowel surgeries presented with severe, refractory spasticity causing pain, sleep disruption, and impaired function. Her spasticity (MAS 4; Penn Spasm Frequency 4/4) was unresponsive or poorly tolerated with oral baclofen, tizanidine, dantrolene, botulinum toxin, and phenol neurolysis. An intrathecal baclofen (ITB) trial provided moderate benefit. A permanent ITB pump was later implanted in a nonstandard posterior-lateral flank position due to abdominal constraints. Her lower extremity tone remarkably improved, allowing oral baclofen discontinuation; however, persistent pump-site pain triggered recurrent autonomic dysreflexia (AD) refractory to conservative management. Multidisciplinary consensus attributed symptoms to pump positioning, and the device was explanted one month later. Following removal, autonomic dysreflexia resolved, but severe spasticity recurred despite maximal oral therapy.
Discussions: Intrathecal baclofen (ITB) therapy is effective for severe spasticity, but long-term benefit may be limited by hardware-related complications. While catheter or pump malfunction is well described, pain related to suboptimal or nonstandard pump placement remains under-recognized. Alternative pump locations have been reported when abdominal placement is not feasible, including anteromedial thigh implantation and relocation to thigh or flank sites, with variable success. Submuscular pump placement has been proposed for thin or cachectic patients to reduce erosion, extrusion, and pain. Although AD is classically associated with high-level spinal cord injury and is rare in multiple sclerosis, severe peripheral pain can trigger AD in non-SCI pathology.
Conclusions: This case demonstrates that intrathecal baclofen failure may result from pump-site pain rather than loss of efficacy. Nonstandard pump placement triggered recurrent autonomic dysreflexia, necessitating explantation despite spasticity improvement. Careful surgical planning and recognition of hardware-related pain are critical to prevent morbidity.