Posterior Occipitocervical Fixation and Intrathecal Baclofen Therapy for the Treatment of Basilar Invagination with Klippel-Feil Syndrome: A Case Report.

Klippel-Feil syndrome (KFS) is characterized by the congenital fusion of the cervical vertebrae and is sometimes accompanied by anomalies in the craniocervical junction. In basilar invagination (BI), which is a dislocation of the dens in an upper direction, compression of the brainstem and cervical cord results in neurological defects and surgery is required. A 16-year-old boy diagnosed with KFS and severe BI presented with spastic tetraplegia, opisthotonus and dyspnea. CT scans showed basilar impression, occipitalization of C1 and fusion of C2/C3. MRI showed ventral compression of the medullocervical junction. Posterior occipitocervical reduction and fusion along with decompression were performed. Paralysis gradually improved postoperatively over 3 weeks. However, severe spasticity and opisthotonus persisted and intrathecal baclofen (ITB) therapy was initiated. Following this, opisthotonus disappeared and spasticity of the extremities improved. Rehabilitation therapy continued by controlling the dose of ITB. Five years after the surgery, self-propelled wheelchair driving was achieved and activities of daily life improved. The treatment strategy for patients with BI and congenital anomalies remains controversial. Posterior reduction and internal fixation using instrumentation were effective techniques in this case. Spasticity control achieved through a combination of surgery and ITB treatment enabled the amelioration of therapeutic efficacy of rehabilitation and the improvement of ADL.

Baclofen Stability up to One Year in In Vivo Intrathecal Infusion Pumps.

OBJECTIVES: Commercial intrathecal baclofen treatment (ITBT) infusion pumps are recommended to be refilled within a maximum of 180 days, thus necessitating at least twice-yearly outpatient visits and refill injections. In particular, pumps with 40-mL reservoir volumes would allow much longer refill intervals. We investigated baclofen stability in active implanted ITBT infusion pumps in vivo with refill intervals up to 367 days to study the feasibility of lengthening refill intervals beyond six months. MATERIALS AND METHODS: We obtained 25 baclofen samples from 19 patients receiving ITBT with varying pump refill intervals. All patients had a baclofen infusion system delivering undiluted 2 mg/mL baclofen at continuous rates of 96.1 to 673.7 µg/d with a concentration of 2.002 mg/mL. Baclofen concentrations of the infusate samples acquired during the refill procedures were analyzed using a validated high-performance liquid chromatography with diode-array detection (HPLC-DAD) assay, later complemented with repeat assay with pH and physical measurements. We also present the validation data of the HPLC-DAD assay. RESULTS: During the mean refill interval of 247 days (SD 90, range 54-367 days), the mean change in baclofen concentration was -0.0156 mg/mL (-0.8%, SD 0.14, range -0.30 to 0.32 mg/mL, paired t-test p = 0.57, t24 = 0.57). Only a low negative correlation was found between the baclofen concentration and the refill interval (Pearson's r = -0.32, p = 0.12). CONCLUSIONS: We could not show a significant change in baclofen concentration over the time studied; 2 mg/mL baclofen ITBT refill intervals could be lengthened to up to one year-the theoretical maximum refill interval in our cohort would have been 489 days. Further studies with larger sample sizes and other baclofen brands are warranted.

Neuromodulation and quality of life for patient with spasticity after spinal cord injury.

RATIONALE: Spasticity develops in 80% of spinal cord injury cases and negatively affects the patents' quality of life. The most common method of surgical treatment for severe spasticity is a long-term intrathecal baclofen therapy (ITB). Long-term spinal cord stimulation is another possible treatment technique. This paper aims to evaluate the changes in quality of life for patients with spasticity who have been treated with neuromodulation (SCS or ITB) in 12 months after the surgery, as well to compare the changes in quality of life for patients who have been treated with spinal cord stimulation and those who received long-term intrathecal baclofen therapy. MATERIALS AND METHODS: The influence of spasticity, experienced by the patients with a spinal cord injury, on their quality of life was analyzed before the surgery and 12 months after it. The severity of the spinal cord damage was determined with the scale of the American Spinal Injury Association (ASIA); spasticity was evaluated with the modified Ashworth scale, Penn Spasm Frequency Scale; pain levels were determined with visual analogue scale (VAS), anxiety and depression levels - with HADS. Functional activity of the patients was evaluated with the help of the Functional Independence Measure (FIM). RESULTS: The treatment results for 33 patients (25 men and 8 women), aged from 18 to 62, are presented. After the trial stimulation, the patients were randomly assigned to either SCS or ITB group (18 and 15 people respectively). The decrease of spasticity in both experimental groups caused lower levels of pain, less functional dependency on other people, lower stress and depression rates and, as a consequence, better quality of life and social adaptation. The obtained results for SCS and ITB groups are statistically similar.

Calcification of the pump pouch in patients receiving ITB therapy: A rare complication affecting refill procedure - Analysis of two cases.

BACKGROUND: Intrathecal baclofen therapy (ITB) is an effective treatment for reducing spasticity but can be associated with various complications, including infection and implant malfunction. METHODS: This retrospective cohort study analyzed refill reports, complications, and functional outcomes in 40 consecutive patients with intractable spasticity or dystonia undergoing ITB. RESULTS: Among the 40 patients, 8 experienced complications, including two cases of calcification of the baclofen pump pouch and surrounding tissue, a rare complication not extensively described in the literature. DISCUSSION: Calcification, in addition to port access difficulties, could lead to drug delivery failure. We hypothesize that calcification may result from microtrauma or needlestick injury to the subcutaneous tissue and muscle fascia. The length of time the pump stays in the pocket could also contribute to favoring this phenomenon. CONCLUSION: As the number of patients receiving ITB increases, physicians must be aware of potential life-threatening complications. The risk of pouch calcification should be further investigated and considered in managing patients undergoing ITB, as it could significantly impact patient care.

A multicenter initiative to reduce intrathecal baclofen pump surgical site infection: a Cerebral Palsy Research Network quality improvement project.

OBJECTIVE: Intrathecal baclofen (ITB) therapy is an effective treatment for spasticity and dystonia in children with cerebral palsy (CP). However, ITB pump surgery is associated with one of the highest rates of surgical site infection (SSI) in medicine, leading to significant morbidity and expense. Surgical protocols have reduced the rate of SSI in children with other CNS implants, and single-center protocols have been effective in ITB surgery in pediatrics. The authors describe the first multicenter quality improvement (QI)-driven standardized protocol for ITB pump surgery in children with CP across the Cerebral Palsy Research Network (CPRN), implemented with the aim of reducing ITB-associated SSI. METHODS: SSI was defined as a culture-positive infection, ITB pump system removal for suspected infection, or wound dehiscence with exposed hardware. Each center reported historical infection rates for at least 3 years before initiating the SSI protocol (preintervention phase). After initiation of a 13-step surgical protocol, a consecutive series of 130 patients undergoing 149 surgical procedures for ITB at four CPRN tertiary pediatric neurosurgery centers were prospectively enrolled at surgery during a 2-year study period (intervention phase). QI methodology was used, including development of a key driver diagram and tracking performance using run and control charts. The primary process measure goal was documented compliance with 80% of the protocol steps, and the primary outcome measure goal was a 20% reduction in 90-day infection rate. Patient characteristics were collected from the CPRN Research Electronic Data Capture registry, including age at surgery, BMI, Gross Motor Function Classification System level, and pattern of spasticity. RESULTS: The aggregated preintervention 90-day ITB SSI rate was 4.9% (223 procedures) between 2014 and 2017. During the intervention phase, 136 of 149 ITB surgeries performed met inclusion criteria for analysis. The mean documented compliance rate with protocol steps was 75%, and the 90-day infection rate was 4.4%, with an average of 42 days from index surgery to infection. CONCLUSIONS: This is the first multicenter QI initiative designed to reduce SSI in ITB surgery in children with CP. Ongoing enrollment and expansion of the protocol to other CPRN centers will facilitate identification of patient- and procedure-specific risk factors for SSI, and iterative plan-do-study-act cycles incorporating these data will further decrease the risk of SSI for ITB surgery in children.

Ultrasound-Guided Intrathecal Baclofen Pump Refilling Method for Management of Spasticity in a Complex Clinical Case.

Intrathecal baclofen (ITB) administration is a common method in managing spasticity. The location of the reservoir fill port (RFP) is identified manually in most cases. However, it can be difficult due to a variety of factors, such as the formation of excess subcutaneous cellular tissue and scar formation overlying the RFP and rotation or inversion of the pump. Consequently, multiple failed attempts accessing the reservoir increases pain and risk of fatal complications (e.g., infection and withdrawal syndrome from pocket filling). We describe a successful ultrasound-guided pump refilling case after multiple failed attempts by the conventional method. This groundbreaking instrument assists this minimally invasive procedure while limiting iatrogenic injury in the treatment of spasticity. The presentation of this case shows the utility of ultrasound as an important tool to guide the procedure and prevent adverse events in a spasticity management consult.

Neuromodulation as a basic platform for neuroprotection and repair after spinal cord injury.

Spinal cord injury (SCI) is one of the most challenging medical issues. Spasticity is a major complication of SCI. A combination of spinal cord stimulation, new methods of neuroprotection and biomedical cellular products provides fundamentally new options for SCI treatment and rehabilitation. The paper attempts to critically analyze the effectiveness of using these procedures for patients with SCI, suggesting a protocol for a step-by-step personalized treatment of SCI, based on continuity of modern conservative and surgical methods. The study argues the possibility of using neuromodulation as a basis for rehabilitating patients with SCI.

Streptococcus dysgalactiae-related intrathecal baclofen therapy infection: how to avoid withdrawal?

Intrathecal baclofen therapy is commonly used for neurologically spastic patients. One of the major complications is hardware infection, which generally requires urgent removal of the pump and the intrathecal catheter, with the risk of severe baclofen withdrawal. We have recently been facing this situation and propose another solution with adapted antibiotic therapy, removal with immediate replacement of the intrathecal catheter, initially connected to an implanted port to continue baclofen administration. A new pump was secondarily implanted, after successful treatment of acute bacterial meningitis due to Streptococcus dysgalactiae.

Unusual mechanical failures of intrathecal baclofen pump systems: symptoms, signs, and trouble shooting.

INTRODUCTION: Although intrathecal baclofen (ITB) therapy is an effective treatment for spasticity, it has several disadvantages and a risk of complications. METHODS: We present six pediatric patients who suffered from unusual mechanical failures of intrathecal baclofen pump systems. RESULTS: With these case-vignettes, we provide a systematic approach on how to interpret the symptoms of ITB complications and an advice which further diagnostic and therapeutic steps to follow. We underline the seriousness of baclofen overdose, underdosing or withdrawal.

Oculo-dento-digital Dysplasia Presenting as Spastic Paraparesis Which Was Successfully Treated by Intrathecal Baclofen Therapy.

A 42-year-old man with a history of migraine and bilateral syndactyly presented with numbness of the extremities and shaking legs, which thus prevented him from working as a carpenter. A neurological examination revealed spastic paraparesis with pathological reflexes on all four extremities. Oculo-dento-digital dysplasia (ODDD) was suspected based on his medical history and characteristic facial appearance including small eye slits, thin mouth, and pinched nose with anteverted nostrils. Genetic tests revealed a gap junction alpha 1 (GJA1) gene mutation and confirmed the diagnosis of ODDD. His spastic paraparesis was resistant to oral antispastic medication, however, his symptoms successfully improved after the initiation of intrathecal baclofen therapy, which thus allowed him to return to work.

Intrathecal baclofen therapy for Lesch-Nyhan disease: illustrative case.

BACKGROUND: Lesch-Nyhan disease (LND) is a very rare metabolic disorder involving the purine salvage pathway. LND manifests hyperuricemia, self-mutilation, cognitive impairment, and movement disorders such as spasticity and dystonia, whose control is difficult pharmaceutically. OBSERVATIONS: Intrathecal baclofen (ITB) therapy was received by a 22-year-old male for generalized dystonia. His paroxysmal abnormal dystonic posturing reduced after surgery, making the task of caregivers easier despite the unchanged assignment on the dystonia scale during a follow-up period of 4 years. LESSONS: ITB may be a safe and feasible option for dystonic symptoms and difficulty with nursing care in patients with LND.

Spinal cord stimulation and intrathecal baclofen therapy for patients with severe spasticity after spinal cord injury.

RATIONALE: Spasticity is one of the main complications after the spinal cord injury (SCI). Most commonly, severe cases of spasticity are treated surgically with intrathecal baclofen therapy (ITB). Spinal cord stimulation for chronic pains (SCS) serves as an alternative for ITB. Both methods have their benefits and limitations. This study is aimed at development of a personalized SCS and ITB treatment algorithm for patients with severe cases of spasticity after SCI. MATERIALS AND METHODS: The paper analyzes the treatment results of 66 patients with severe spasticity after SCI (50 men and 16 women, age ranging from 18 to 62), average age is 36.03±12.29 y.o. Patients who chose surgery as a spasticity treatment option, received experimental stimulation, and after muscle tone reduction to a comfort level they were surgically implanted with a SCS system for chronic pain management. Patients with negative response to experimental stimulation were tested for baclofen and, based on the results, had a baclofen pump implanted. The patients were examined after 1, 3, 6 and 12 months. RESULTS: Surgical implantation of a SCS system was performed for 18 patients, ITB was used for 15 patients. After first 3 months of observation both groups demonstrated a significant improvement of spasticity index, but the SCS patients had better results. However, 6 months later the MAS scores, frequency of spasms and reflexes in both groups were the same. After 12 months of observation the ITB group exhibited a significant improvement of the MAS scores, compared with the control group, and reached the results, similar to the SCS group. CONCLUSIONS: Surgical treatment of patients with severe spasticity after SCI should start with experimental spinal cord stimulation, and, in case of a positive response, be followed by SCS system implantation. Patients with positive response to the experimental stimulation exhibit a significantly prolonged response to treatment, without substantial differences from ITB patients.

[Intrathecal baclofen therapy in brain injury and recovery of persistent vegetative state. Apropos of a case]. / Baclofeno intratecal en daño cerebral y recuperación de estado vegetativo persistente. A propósito de un caso.

Patients with brain injury and spasticity are candidates for intrathecal baclofen therapy (ITB) when maximal doses of oral antispastic drugs fail. Some authors have described an improvement in the level of consciousness in patients with brain injury and disorder of consciousness treated with ITB for spasticity. We present the case of a 43-year-old patient with brain injury, spasticity, and permanent vegetative state (PVS) who showed an improvement in the level of consciousness after ITB for spasticity. We performed an ITB infusion test, assessing the spasticity with the Modified Ashworth Scale (MAS) and level of consciousness with the Coma Recovery Scale-Revised (CRS-R) and observed an improvement in the spasticity and the level of consciousness. Consequently, the ITB pump was implanted and the patient recovered from PVS to minimal conscious state (MCS). We conclude that ITB is indicated in patients with brain injury and spasticity. We suggest the improvement in the level of consciousness as a possible additional benefit. There is a lack of evidence to recommend ITB in patients with altered level of consciousness.

Intrathecal Baclofen Therapy in Russia: National Register, the Effect of One-Year Usage.

Patients with severe muscular spasticity still represent the most complex and resistant to therapy group of neuro-rehabilitation patients. In a few years, in Russia, intrathecal baclofen therapy has appeared to be the most effective method for such spasticity. For the first time the authors developed and implemented in clinical practice "Prospective register to treat spastic states using intrathecal baclofen therapy in Russian Federation" aimed at therapy classification of spastic patients: to reveal management characteristics, assess treatment outcomes and frequency of occurrence of adverse events that will finally help specify the need for the method employment in real clinical practice. The article presents the findings of a one-year usage of Register, which enabled to make a preliminary evaluation of intrathecal baclofen therapy in Russia.

Intrathecal baclofen therapy for treatment of spasticity in infants and small children under 6 years of age.

PURPOSE: The aim of this study is to prove the efficacy and safety of intrathecal baclofen therapy in infants and children below 6 years of age by retrospective analysis of our pediatric cohort of 135 primary pump implantations. METHODS: Between 2007 and 2018, 17 patients with pump implantations were below 6 years of age. Data were acquired retrospectively with a follow-up of 12 months to 11 years regarding complications. RESULTS: The youngest infant was 11 months at implantation with a bodyweight of 6, 4 kg, and 63 cm length. Surgical complications were comparable to published literature and mainly involved the catheter (2 catheter dislocations and 1 catheter transection) and one pump infection resulting in 4 revision surgeries in 3 patients. One baclofen-related apnea during titration and an overdose after refill were treated conservatively. Using a subfascial implantation technique, we observed neither skin ulceration nor pump infection since 2007. In a growing child, catheter slides are common and related to growth, scoliosis, spine surgery, and surgical failure. CONCLUSION: Intrathecal baclofen therapy in infants and small children is as safe and effective as published for older pediatric patients; therefore, intrathecal baclofen can be considered in all infants as long as an 8-cm incision fits into the triangle of the anterior superior iliac spine, costal margin of the 10th rib, and navel. We suggest the utilization of subfascial surgical technique for implantation pump and catheter. Titration of intrathecal baclofen should be performed slowly to avoid bradycardia in infants. This is a retrospective study (level of evidence 4).

The use ultrasound guided for refilling intrathecal baclofene pump in complicated clinical cases: A practical approach.

Muscular spasticity due to neurological disorders is a heavy cause of severe pain and disability for many patients, compromising the independence and quality life. Baclofene is a good tool to guarantee patients independence and pain control. Anyway in chronic therapy oral treatment become unsatisfactory. In all these cases, intrathecal baclofen therapy (ITB), after sub fascial implantation of intrathecal pumps is used as an important long term treatment to reduce spasticity. After pump implantation the drug reservoir must be refilled periodically in order to maintain the reduction of spasticity and avoid the symptoms and signs of withdrawal. ITB refilling, which involves the insertion of a needle through the skin until the access port of the pump, is often hard, mainly due to the layer of abdominal fat, spasticity, suboptimal pump positioning, pump rotation or inversion, and scar formation over the implantation site. To avoid the difficulties of ITB refilling radiography or other invasive supportive examinations are sometimes needed. We reported here our experience and we suggest a simple method to use the ultrasound in refilling with particular attention to some cases with complications after implantation with a difficult approach in refilling. We used the ultrasound examination to identify the access port of her pump so as to avoid multiple needle punctures and infections and radiation exposition. Ultrasound-guided technique may facilitate ITB refill in technically challenging cases. With ultrasound ITB was easily detectable and was quite simple to identify the exact point of needle injection. In the last years different new applications for ultrasounds are emerging. In our opinion the use of Doppler ultrasounds in the study of muscles and nerves represent an emerging tool for the physician's neurological rehabilitation.

Pregnancy and Breastfeeding during Intrathecal Baclofen Therapy - A Case Study and Review.

We report the case of a young woman who received intrathecal baclofen therapy (ITB) and subsequently became pregnant and had a normal delivery. A 28-year-old woman with flexion myelopathy had anterior decompression with fusion at C4/5 and C5/6 levels. Clinical symptoms improved after surgery. However, when she was 29 years old, her symptoms steadily advanced to Modified Ashworth Scale 3 spasticity level in the lower legs, with pain in both of them and urinary retention tendency. Since a 25 µg intrathecal baclofen injection improved her symptoms, an ITB pump system was implanted. After surgery, lower limb spasticity and urinary retention improved. Two years after ITB pump implantation, the patient married and became pregnant. The patient intended to have normal delivery but the induction of labor was ineffective and childbirth was completed by Cesarean section with lumbar anesthesia. The infant's Apgar score was 8 at 1 min and 9 at 5 min, and birth-weight was 2,704 g. We measured the baclofen concentration in the patient's breast milk using high-performance liquid chromatography/tandem mass spectrometry. The level of baclofen in the breast milk was very low (0.617 ng/ml) and the predicted pharmacological effect on the infant was judged to be negligible. No withdrawal symptoms or muscle tone abnormalities were found after birth. Our findings indicate that ITB therapy could be considered for young women with severe spasticity, even if they plan to have children.

Occlusion of the Ascenda Catheter in a Patient with Pump Twiddler's Sydrome: A Case Report.

INTRODUCTION: Intrathecal baclofen (ITB) therapy is an effective way to manage spasticity in numerous conditions, including multiple sclerosis, stroke, and cerebral palsy. While pump failure is a common complication of ITB, improvements in device design have led to reduction of complications. In particular, the Ascenda catheter from Medtronic, Inc. was designed to resist kinking and associated complications; indeed, no incidences of catheter twisting or occlusion have been reported in literature prior to this case. CASE REPORT: We report a case of a 32-year old gentleman who presented to the clinic with symptoms of baclofen withdrawal 19 months after he had a programmable pump implanted for spasticity. During the diagnostic evaluation it was discovered that the patients pump had flipped in his abdominal pocket. He was taken to surgery to reorient the pump, during which time it was noted the catheter was tightly coiled on itself occluding flow. The twisted catheter was excised and replaced with a new segment.His symptoms subsequently resolved. CONCLUSIONS: Although catheter occlusions have subsided since the approval of the Ascenda catheter, pump twiddler's syndrome remains a risk factor for this complication. This is the first report describing this syndrome in a patient with the Ascenda catheter.

Intrathecal Baclofen Pump Implantation for Type 2 Gaucher Disease.

Gaucher disease (GD) is the most common type of lysosomal storage disease, with type 2 being the most severe subtype. Type 2 GD patients suffer significant progressive neurological impairment, including spasticity, opisthotonus, seizure, and apnea. The recently developed enzyme replacement therapy (ERT) has shown therapeutic benefit for GD. However, as the enzymes do not cross the blood-brain barrier, ERT does not ameliorate neurological impairment in GD. Intrathecal baclofen therapy (IBT) is indicated for spastic neurological diseases, such as cerebral palsy, and studies have shown its therapeutic benefit in improving several manifestations of GD, such as scoliosis caused by muscle spasticity and respiratory function. To date, the potential benefits of IBT for treating lysosomal storage diseases such as GD have not been examined. Here we provide the first report of a patient with type 2 GD treated with IBT, and demonstrate its therapeutic benefit in ameliorating the neurological aspects of this disease.