Infectious complications of intrathecal baclofen pump devices in a pediatric population.

BACKGROUND: Intrathecal baclofen (ITB) is an effective therapy for spasticity and dystonia in pediatric populations; however, there are associated infectious complications. METHODS: Patients who had an initial ITB device implanted at our center were followed to determine the proportion of patients with infectious and noninfectious complications, identify risk factors for infection and describe the clinical presentations, treatment and outcomes of infectious complications. RESULTS: Over the 15-year study period, 139 patients had an initial ITB device placed. The mean age at placement was 13.6 years (range: 6 months to 41 years). In the first year of follow-up, 83% had no complications or secondary procedures, 17% had at least 1 secondary procedure and 5% had an infectious complication. The median time until infection was 14 days (mean 33 ± 42 days). Patients with secondary spasticity or dystonia were more likely to have infections than patients with cerebral palsy (86% versus 14%; P < 0.0001). In the 94 patients with a first secondary procedure, 29% had at least 1 other procedure and 8% had an infection in the 1 year follow-up. Overall, 24 patients had 27 infections; 22% superficial, 33% deep and 45% organ space. Staphylococcus aureus was isolated in 50% of those with cultures obtained. Explantation was required in 59% of patients with an infection and differed by infection type: superficial (17%), deep (44%) and organ space (92%) (P = 0.004). CONCLUSIONS: Infectious complications were relatively uncommon; however, when present, frequently led to the explantation of the ITB pump device.

Combined C-arm fluoroscopy and C-arm cone beam computed tomography for the evaluation of patients with possible intrathecal baclofen delivery system malfunctions.

BACKGROUND: Evaluating intrathecal baclofen (ITB) delivery systems for potential malfunction can be challenging. The catheter systems are prone to myriad complications that are frequently difficult to ascertain by conventional imaging techniques. Newer imaging technologies and their combinations can be used to identify such problems, define surgical indications, and focus operative planning. C-arm fluoroscopy and C-arm cone beam CT performed in one imaging session represents one such combination that has great utility. OBJECTIVE: We present a case series of ITB catheter evaluations using combined C-arm fluoroscopy (CF) and C-arm cone beam CT (CCBCT). METHODS: We retrospectively analyzed 7 pediatric patients who underwent ITB catheter systems evaluations by the use of combined CF and CCBCT. Study variables included indications for evaluation, imaging results, interventions, correlation of surgical findings with imaging, and clinical outcome. RESULTS: Three patients had intact and patent catheter systems. Four patients demonstrated various problems of the catheter systems, including disconnection, microfracture, fracture with segment migration, and subdural migration. Dosage adjustments improved all patients with normal studies. Surgery was guided by the imaging, and all operative patients improved after targeted interventions. Intraoperative findings correlated perfectly with imaging. CONCLUSION: Combined CF and CCBCT proved highly effective in the evaluation of our patients with potential ITB system malfunctions. This technique is advocated for such evaluations because it accurately defines problems with connectivity, integrity, and position of catheter systems. When surgical intervention is required, this information aids in operative planning.

EMG and MRI are independently related to shoulder external rotation function in neonatal brachial plexus palsy.

BACKGROUND: Few studies exist with regard to the ability of electromyography (EMG) and volumetric magnetic resonance imaging (MRI) of the infraspinatus muscle to complement the physical assessment of active global shoulder external rotation (GER) in the neonatal brachial plexus palsy (NBPP) population. Therefore, the purpose of this study was to evaluate the relationships of EMG and MRI with active GER based on analysis of the infraspinatus muscle. METHODS: Seventy-four NBPP patients (mean age, 5 y 1 m; range, 1 y 1 m to 13 y 3 m) who had undergone physical examination of the shoulder, EMG evaluation of the infraspinatus muscle, and shoulder MRI were included in this study. The outcome variable active GER was dichotomized into <0 degree active GER (poor) and ≥0 degree active GER (good). The interference pattern on EMG of the infraspinatus muscle was graded on a 6-point scale and dichotomized into ≤4 and ≥5. On shoulder MRI, infraspinatus muscle volume was measured. The infraspinatus muscle interference pattern and volume were compared with active GER. RESULTS: Interference pattern on EMG of the infraspinatus muscle was significantly related to the Mallet Score (P=0.0022), with a poor interference pattern associated with an approximately 7 times higher likelihood [odds ratio=7.391; 95% confidence interval (2.054, 26.588)] of poor active GER. Infraspinatus muscle volume decrease on MRI was also significantly related to active GER (P=0.0413), with each percent volume decrease corresponding to an increase of 0.094 in the odds of having a poor Mallet Score for active GER [odds ratio=1.094; 95% confidence interval (1.004, 1.193)]. CONCLUSIONS: The interference pattern of the infraspinatus muscle on EMG and the infraspinatus muscle volume on MRI are strongly related to active GER as assessed by the Mallet Score. Integrating clinical assessment with electrophysiological and imaging findings may improve the accuracy in evaluating shoulder dysfunction in NBPP and provide improved guidance in selecting interventions specific to the patient's pattern of deficits. LEVEL OF EVIDENCE: Diagnostic study, level II.

Acute progression of neuromuscular findings in infantile Pompe disease.

A 2-year-old girl with Pompe disease developed an acute worsening of muscle weakness during a hospitalization, and required intubation for an upper respiratory infection. Electromyography and nerve conduction studies produced results consistent with a severe chronic motor axonal peripheral polyneuropathy, with no evidence of reinnervation. Magnetic resonance imaging of the brain demonstrated generalized hypomyelination and parenchymal volume loss, whereas magnetic resonance spectroscopy suggested neuronal injury and hypomyelination. This case provides compelling evidence for a slowly progressive neurodegenerative process in patients with infantile Pompe disease, affecting the motor neurons. Routine electromyography, nerve conduction studies, and cranial magnetic resonance imaging should be considered to delineate the presence of a neurodegenerative process in infantile-onset Pompe disease.

Botulinum toxin A injections in children: technique and dosing issues.

Information is presented on the current state of administering botulinum toxin A injections in the pediatric population based on review of scientific publications cited in MEDLINE and PubMed that involved the use of botulinum toxin A in patients under the age of 19. Studies were selected if they contained nearly complete information on botulinum toxin dosing and injection technique within the article. In addition, consensus articles from pediatric task forces were selected for review. Information regarding botulinum toxin A dosing and injection technique was gathered from the material and method sections of the selected studies or from tables in consensus articles. Information was collected in regard to the total amount of botulinum toxin used, commercial brand of botulinum toxin used, dilution of the toxin, volume of toxin preparation, needle size, localization technique, adverse reactions, and whether sedation was used. There is currently a range of dosing and injections techniques in the literature when using botulinum toxin A in children. Body weight, muscle size, location of muscle, and degree of spasticity are all considered to determine total amount of botulinum toxin injected. Concentration and volume of the diluted toxin can affect the amount of spread of the toxin after injection. Injection techniques also vary and are not well reported. Use of electromyographic, electrical stimulation, or ultrasound guidance is employed in children for difficult to locate individual muscle groups. Use of sedation also varies between medical centers.

Hyperhidrosis in pediatric spinal cord injury: a case report and gabapentin therapy.

Hyperhidrosis is a relatively common condition with a multitude of causes. Spinal cord injury may be complicated by hyperhidrosis. Many forms of therapy have been reported for this phenomenon but few have been demonstrated to be efficacious. We describe a case of a child with hyperhidrosis from a spinal cord injury and report the first therapeutic use, to our knowledge, of gabapentin for hyperhidrosis.