Intrathecal baclofen therapy for Lesch-Nyhan disease: illustrative case

2021 ◽  
Vol 1 (1) ◽  
Author(s):  
Takeshi Satow ◽  
Masafumi Ogawa ◽  
Taro Komuro
Keyword(s):  
Intrathecal Baclofen ◽  
Illustrative Case ◽  
Purine Salvage ◽  
Intrathecal Baclofen Therapy ◽  
Self Mutilation ◽  
Dystonic Posturing ◽  
Feasible Option ◽  
Generalized Dystonia ◽  
Rare Metabolic Disorder

BACKGROUNDLesch-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.OBSERVATIONSIntrathecal 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.LESSONSITB may be a safe and feasible option for dystonic symptoms and difficulty with nursing care in patients with LND.

Subfascial Implantation of Intrathecal Baclofen Pumps in Children: Technical Note

Neurosurgery ◽  
2001 ◽  
Vol 49 (3) ◽  
pp. 753-757 ◽  
Author(s):  
Brian Harris Kopell ◽  
Debra Sala ◽  
Werner K. Doyle ◽  
David S. Feldman ◽  
Jeffrey H. Wisoff ◽  
...  
Keyword(s):  
Soft Tissue ◽  
Pediatric Population ◽  
Intrathecal Baclofen ◽  
Implantation Site ◽  
Implantation Technique ◽  
Soft Tissue Coverage ◽  
Tissue Mass ◽  
Intrathecal Baclofen Therapy ◽  
Skin Breakdown

Abstract OBJECTIVE Indwelling intrathecal drug delivery systems are becoming increasingly important as a method of neuromodulation within the nervous system. In particular, intrathecal baclofen therapy has shown efficacy and safety in the management of spasticity and dystonia in children. The most common complications leading to explantation of the pumps are skin breakdown and infection at the pump implantation site. The pediatric population poses particular challenges with regard to these complications because appropriate candidates for intrathecal baclofen therapy are often undernourished and thus have a dearth of soft tissue mass to cover a subcutaneously implanted baclofen pump. We report a technique of subfascial implantation that provides greater soft tissue coverage of the pump, thereby reducing the potential for skin breakdown and improving the cosmetic appearance of the implantation site. METHODS Eighteen consecutively treated children (average age, 8 yr, 7 mo) with spasticity and/or dystonia underwent subfascial implantation of a baclofen pump. These children's mean weight of 42.9 lb is less than the expected weight for a group of children in this age group, ranging from 4 years, 8 months, to 15 years, 7 months. In all patients, the pump was inserted into a pocket surgically constructed between the rectus abdominus and the external oblique muscles and the respective anterior fascial layers. RESULTS At an average follow-up of 13.7 months, no infection or skin breakdown had occurred at the pump surgical site in any of the 18 patients. CONCLUSION At this early follow-up, the subfascial implantation technique was associated with a reduced rate of local wound and pump infections and provided optimal cosmetic results as compared with that observed in retrospective cases.

393 LONG TERM FOLLOW-UP AFTER CONTINUOUS INTRATHECAL BACLOFEN THERAPY FOR SEVERE SPASTICITY

2010 ◽  
Vol 4 (S1) ◽  
pp. 112-112
Author(s):  
E. Anastasiou ◽  
A. Tzortzopoulou ◽  
A. Alexandropoulou ◽  
K. Rizos ◽  
A. Karakosta ◽  
...  
Keyword(s):  
Intrathecal Baclofen ◽  
Intrathecal Baclofen Therapy ◽  
Severe Spasticity ◽  
Long Term Follow Up

Long-term follow-up on continuous intrathecal Baclofen therapy in non-ambulant children with intractable spastic Cerebral Palsy

2013 ◽  
Vol 17 (6) ◽  
pp. 639-644 ◽  
Author(s):  
Georges F. Vles ◽  
Dan L. Soudant ◽  
Marjanke A. Hoving ◽  
R. Jeroen Vermeulen ◽  
Laura A. Bonouvrié ◽  
...  
Keyword(s):  
Cerebral Palsy ◽  
Intrathecal Baclofen ◽  
Spastic Cerebral Palsy ◽  
Intrathecal Baclofen Therapy ◽  
Long Term Follow Up

Sustained effect of high-dose intrathecal baclofen in primary generalized dystonia: A 2-year follow-up study

1997 ◽  
Vol 12 (6) ◽  
pp. 1100-1102 ◽  
Author(s):  
N. J. Diederich ◽  
C. L. Comella ◽  
G. Matge ◽  
G. Becker ◽  
F. Schiltz ◽  
...  
Keyword(s):  
Intrathecal Baclofen ◽  
High Dose ◽  
Follow Up Study ◽  
Sustained Effect ◽  
Generalized Dystonia

Analysis of complications in 430 consecutive pediatric patients treated with intrathecal baclofen therapy: 14-year experience

2014 ◽  
Vol 13 (3) ◽  
pp. 301-306 ◽  
Author(s):  
Francesco Motta ◽  
Clara E. Antonello
Keyword(s):  
Adverse Events ◽  
Temporal Distribution ◽  
Intrathecal Baclofen ◽  
Csf Leak ◽  
Children And Adolescent ◽  
Intrathecal Baclofen Therapy ◽  
Single Center Study ◽  
Different Origins ◽  
Pump Implantation

Object This single-center study investigated adverse events that occurred in children and adolescent patients treated with intrathecal baclofen (ITB) therapy for spasticity and/or dystonia. Methods In a 14-year period, 430 consecutive patients with a mean age of 13.3 ± 5.9 years received ITB over a mean follow-up period of 8.6 ± 3.8 years (range 12 months to 14 years). Eighty-nine percent of these patients had cerebral palsy. Major complications, defined as those that required a surgical intervention, were infections, CSF leaks, and device problems related to the catheter or pump. Assessing infections, the authors compared the 2 groups of patients implanted with an ITB system by either the subcutaneous or subfascial technique. The temporal distribution of events related to the catheter was also considered. Results At least 1 complication was present in 25% of the patients: 9.3% experienced an infection, 4.9% a CSF leak, 15.1% a problem with the catheter, and 1% a problem related to the pump. Five percent of the assessed patients suffered more than 1 complication. The rate of infections was significantly lower (p < 0.001) in patients with the pump placed subfascially compared with those with the pump placed subcutaneously. A higher rate of infection was found after pump replacement compared with the first pump implantation (10.6% vs 6%, respectively). Catheter problems were the most common complication and occurred more frequently during the 1st year after the implant. Conclusions While ITB is an effective treatment to manage spasticity of different origins, adverse events may occur and need to be managed. The surgical procedure should be meticulous and different techniques may have a diverse impact on the infection rate, which is the most critical complication. Despite the adverse events that occurred in this study, the majority of patients were satisfied with the treatment received.

Arthroscopic Biceps Tenodesis: Technique and Results in Six Dogs

2005 ◽  
Vol 41 (2) ◽  
pp. 121-127 ◽  
Author(s):  
James L. Cook ◽  
Keith Kenter ◽  
Derek B. Fox
Keyword(s):  
Surgical Management ◽  
Biceps Tendon ◽  
Interference Screw ◽  
Cannulated Screw ◽  
Biceps Tenodesis ◽  
Fixation Methods ◽  
Feasible Option ◽  
Successful Outcomes ◽  
Tendon Pathology

Biceps tenodesis was performed using an arthroscopic-assisted technique on six dogs diagnosed with chronic bicipital tendon pathology. The technique was performed using two different fixation methods (i.e., cannulated interference screw, cannulated screw and tissue washer). All six dogs had successful outcomes in terms of return to full function at a mean follow-up time of 11.7 months after surgery. Arthroscopic biceps tenodesis is a feasible option for surgical management of biceps tendon pathology, and it may have advantages over open tenodesis and open or arthroscopic tenotomy. Further study is needed before definitive recommendations regarding indications, complications, and prognosis associated with arthroscopic biceps tenodesis can be made.

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