T. Michael

Efficacy of botulinum-a toxin in children with detrusor hyperreflexia due to myelomeningocele: preliminary results

OBJECTIVES The established treatment of children with neurogenic bladder consists of the use of anticholinergic drugs, such as oxybutynin and tolterodine, and clean intermittent catheterization four or five times a day. If anticholinergic drugs and clean intermittent catheterization fail, surgery is often necessary. With the intent of avoiding surgery, we investigated the effect of botulinum-A toxin for treating detrusor hyperreflexia in this group of patients. METHODS The subjects were 17 children (average age 10.8 years) who had detrusor hyperreflexia and were using clean intermittent catheterization four or five times a day. Urodynamic studies were followed by injection of 85 to 300 U of botulinum-A toxin into 30 to 40 sites in the detrusor muscle. Urodynamic follow-up was done 2 to 4 weeks after injection. RESULTS The mean reflex volume increased by 112.1%, from 95.00 plus minus 34.54 mL (range 47 to 147) to 201.45 plus minus 68.57 mL (range 77 to 310) (P <0.005). The maximal bladder capacity increased by 56.5%, from 137.53 plus minus 59.96 mL (range 59 to 242) to 215.25 plus minus 96.36 mL (range 60 to 380) (P <0.005). The maximal detrusor pressure decreased by 32.6%, from 58.94 plus minus 32.32 cm H(2)O (range 19 to 149) to 39.75 plus minus 26.12 cm H(2)O (range 7 to 100) (P <0.005). Detrusor compliance increased by 121.6%, from 20.39 plus minus 26.5 mL/cm H(2)O (range 4.5 to 40) to 45.18 plus minus 45.4 mL/cm H(2)O (range 5.3 to 100) (P <0.01). CONCLUSIONS Botulinum-A toxin injection into the hyperreflexive detrusor muscle seems to be very effective and might be a therapeutic alternative to anticholinergic drugs.

Alfuzosin in the treatment of high leak-point pressure in children with neurogenic bladder

Objective  To decrease the detrusor leak‐point pressure (LPP) of > 40 cmH2O in children with a neurogenic bladder, using the α1‐adrenergic blocking agent alfuzosin.

Urodynamic effects of propiverine hydrochloride in children with neurogenic detrusor overactivity: a prospective analysis

The management of stones in children is often controversial; extracorporeal shock wave lithotripsy appears to be very successful, with excellent clearance rates, and probably better than in adults. For this reason, many maintain that percutaneous nephrolithotripsy might not be required in children. Authors from Pakistan retrospectively assessed their use of percutaneous nephrolithotripsy, finding that it too yields satisfactory results.

Urodynamic effects of propiverine in children and adolescents with neurogenic bladder: results of a prospective long-term study.

OBJECTIVE To evaluate prospectively the efficacy and tolerability of propiverine for long-term treatment of neurogenic detrusor overactivity (NDO) in children. MATERIALS AND METHODS 17 children and adolescents with NDO (10 female, 7 male; average age at last consultation 13.0 years) were evaluated during long-term treatment with propiverine (0.8 mg/kg body weight/day). Outcome measurements included urodynamic parameters, continence, hydronephrosis and tolerability of propiverine. RESULTS Average follow-up was 3.6 years (range 2.0-5.9). The average maximum detrusor pressure was 33.2 ± 4.8 cmH(2)O and bladder compliance was 20.0 ± 5.4 ml/cmH(2)O at the last follow-up visit. Maximum cystometric bladder capacity (MCBC) within the normal range was attained in 11 patients; it was still reduced (average of 61% of expected MCBC) in the remaining 6. Incontinence occurred on average once per day. Hydronephrosis was classified for each renal unit separately: grade 0 was measured in 26 and 22 cases, grade 1 or 2 in 6 and 8 cases, grade 3 or 4 in 2 and 4 cases pre and post treatment, respectively. In 6/17 patients adjuvant intravesical oxybutynin was applied, in 4 out of these 6 patients more invasive procedures, such as untethering, augmentation cystoplasty or botulinum toxin injections, were necessitated. Propiverine monotherapy was well tolerated in 11/17 patients. No serious adverse events were encountered during the study period. CONCLUSION Long-term efficacy and tolerability of propiverine for NDO in children and adolescents is promising: clinically relevant improvements in key urodynamic outcomes were paralleled by improvements in incontinence score.

Klinische Variabilität bei 2 Patienten mit Aspartoacylase-Defekt (Leukodystrophie Typ Canavan)

Die Leukodystrophie Typ Canavan ist eine seltene autosomal rezessiv vererbte, neurodegenerative Erkrankung. Charakteristischerweise fallen die Kinder um den 3. Lebensmonat mit Megalenzephalie, statomotorischer Retardierung und verminderter Spontanmotorik auf. Der weitere Verlauf ist durch den Verlust der psychomotorischen und kommunikativen Fahigkeiten, progrediente Spastik, Blindheit, gelegentlich auch Taubheit und Krampfanfalle gekennzeichnet. Konnatale und Late-onset-Verlaufsvarianten sind ebenfalls beschrieben worden [3, 4]. Hagenfeld et al. [5] wiesen erstmals auf den Zusammenhang zwischen der N-acetyl-Aspartazidurie und einer Leukodystrophie hin. Matalon et al. [9] gelang durch den Nachweis einer spongiosen subkortikalen und kortikalen Degeneration in der Hirnbiopsie bei Patienten mit N-acetyl-Aspartatazidurie die Zuordnung zum Typ Canavan.

Dynamic Orthoses for Children with Meningomyelocele

For patients with meningomyelocele (MMC), the assessment of how the disability to walk will be influenced stands at the beginning of rehabilitation. From this assessment we can deduce a prognosis and the aims of rehabilitation, which should be defined within the first 6 months of a baby’s life (Fig. 1).

Therapie von BNS-Krämpfen mit Natriumvalproat: eine Alternative zur ACTH-Therapie?

Bei Bestimmungen der GABA-Konzentrationen im Liquor von Kindern mit verschiedenen Formen von Epilepsien war aufgefallen, das die Kinder mit BNS-Anfallen besonders niedrige GABA-Konzentrationen im Liquor hatten (Loscher u. Siemes 1985).