Results after surgery for lumbosacral lipoma: the significance of early and late worsening.

We retrospectively reviewed 32 patients operated on for lipoma of the conus and lipomyeloschisis, the two main anatomical subtypes of congenital lumbosacral lipomas associated with tethered cord syndrome. Surgery was proposed to patients when they were symptomatic, and in most cases for progressive symptoms. The evolution of the different symptoms was studied separately. In most patients, symptoms improved or stabilized after surgery; in some, however, postoperative worsening, at least of some of the symptoms, was seen. This postoperative worsening became apparent either early or late after the operation, and was not associated with surgical trauma or postoperative complications. We suggest it was caused mainly by the natural course of the disease (especially in the case of the orthopedic deformities), and in some cases by retethering. Our series is not large enough to detect statistical significance for the different symptoms or for the anatomical subgroups. Importantly, according to our analysis by the different symptoms, the operation did not seem to protect the patients from later development of new deficits. This can be interpreted as an argument against prophylactic surgery in asymptomatic patients.

Intravesical application of a stable oxybutynin solution improves therapeutic compliance and acceptance in children with neurogenic bladder dysfunction.

PURPOSE: To improve patient compliance with and acceptance of intravesical oxybutynin therapy for neurogenic bladder dysfunction we developed a stable oxybutynin solution that eliminates the complicated crushing procedure. MATERIALS AND METHODS: From January 1995 to January 1997 we prospectively evaluated 15 children with a mean age of 6.1 years with persistent detrusor hyperactivity or significant side effects on oral oxybutynin therapy who received intravesically 0.2 mg./kg. (maximum 5 mg.) of a stable oxybutynin solution (5 mg./5 ml., pH 5.85) twice daily. RESULTS: The oxybutynin solution remained stable up to 24 months. In 13 of the 15 children therapeutic compliance was excellent. Detrusor hyperactivity decreased and systemic side effects were absent or minimal. After 4 and 24 months mean cystometric bladder capacity plus or minus standard error of mean increased from 114+/-15.2 to 161+/-26.6 and 214+/-21.7 ml. (p <0.01), mean ratio of cystometric-to-expected bladder capacity increased from 0.88+/-0.12 to 1.18+/-0.14 and 1.24+/-0.16 (p <0.01), and end filling bladder pressure decreased from 57.0+/-7.1 to 25.6+/-4.4 and 30.8+/-4.4 cm. water (p <0.01), respectively. CONCLUSIONS: Intravesical instillation of a specially prepared oxybutynin solution is safe and reliable in children with persistent detrusor hyperactivity or side effects on oral oxybutynin therapy. Eliminating the complex crushing preparation of the solution by the child or parent has made this therapy easy to use and acceptable in the long term.

Intravesical oxybutynin for neurogenic bladder dysfunction: less systemic side effects due to reduced first pass metabolism.

PURPOSE: To unravel why intravesical oxybutynin is more effective and causes significantly fewer systemic side effects than oral oxybutynin in the treatment of neurogenic bladder dysfunction, we tested the hypothesis that the absorption and metabolism of oxybutynin are changed after intravesical instillation. MATERIALS AND METHODS: A high-performance liquid chromatography assay was developed for both oxybutynin and its active metabolite, N-desethyl-oxybutynin. Plasma concentrations were quantified after intravesical (n = 11) and oral (n = 5) administration of oxybutynin in children under steady-state conditions. Pharmacokinetic parameters were calculated. RESULTS: Oral administration of oxybutynin (0.2 mg./kg./dose) resulted in peak plasma concentrations for N-desethyl-oxybutynin which were 7.4 +/- 1.3 times higher than corresponding values for oxybutynin (n = 5). Also the AUC (area under the plasma concentration time curve) values were higher for N-desethyl-oxybutynin compared with those of oxybutynin, the ratio being 10.8 +/- 1.0 (n = 5). Intravesical instillation (0.2 mg./kg./dose), on the other hand, resulted in reduced metabolite generation and peak plasma concentrations for N-desethyl-oxybutynin which were in the same range as those for oxybutynin, the ratio being 1.2 +/- 0.1 (n = 11). The ratio for the AUC values for N-desethyl-oxybutynin and oxybutynin was 2.1 +/- 0.2 (n = 11). CONCLUSIONS: The significantly lower AUC ratio of the N-desethyl metabolite over the mother compound, due to a reduced first pass metabolism, may explain the clinically relevant reduction of side effects that characterizes intravesical compared with oral oxybutynin therapy.

Alcoholism and emotional reactivity: more heterogeneous film-induced emotional response in newly detoxified alcoholics compared to controls--a preliminary study.

Alcoholics are thought to be characterized by irregular emotional responses, having trouble reaching an optimal level of emotional arousal. They therefore may use alcohol to restore emotional homeostasis. This study investigated whether recently detoxified alcoholics show different emotional responses as compared to controls. Film excerpts were used to induce emotions in 14 newly detoxified alcoholics (9 men, 5 women) and matched controls in a standardized laboratory setting. Subjective emotional (questionnaires) and physiological measures were employed. Depression and cognitive deterioration were controlled. Based on subjective ratings, alcoholics displayed greater variability of emotion; they displayed also fewer or no physiological arousal changes. Subjective emotional responses were exceedingly high or low. These differences were not accounted for by depression or cognitive deterioration. We hypothesize that alcohol could be used to restore an optimal level of emotional arousal. This homeostatic function of alcohol is yet to be clearly assessed.

Tethered cord syndrome in occult spinal dysraphism: timing and outcome of surgical release.

OBJECTIVE: To investigate the influence of neurosurgical intervention on the appearance of upper motor neuron (UMN) signs in newborns diagnosed with occult spinal dysraphism and tethered cord (TC) during the first month of life. METHODS: A prospective study (1990 to 1996) of 22 consecutive newborns with occult spinal dysraphism monitored for the appearance of UMN signs. Untethering was performed when neurologic or urodynamic investigation indicated the presence of UMN dysfunction. RESULTS: Of 22 patients, 10 remained free of UMN symptoms during follow-up (mean, 67+/-22 months). Untethering was performed in 12 of 22 patients because of the presence of UMN symptoms. In 7 of these 12 patients, there was a documented asymptomatic period of 13+/-11 months before the onset of UMN symptoms. Untethering at a mean age of 18+/-17 months restored normal neurologic and urinary function in all patients (mean postoperative follow-up, 25+/-16 months). Of the 12 children, 5 presented with UMN signs at birth. In these children, untethering was performed at a mean age of 9+/-5 months. In two of these five patients, UMN symptoms did not resolve after surgery, and ongoing conservative bladder treatment was required (mean follow-up, 37+/-14 months). In none of the 12 operated children did signs of retethering occur. CONCLUSIONS: A significant number (10/22) of children born with occult spinal dysraphism and TC did not develop UMN symptoms during follow-up; neurosurgical correction after the appearance of an UMN sign restored normal neurologic and urinary function in all children; and untethering in children presenting at birth with UMN symptoms resulted in poorer outcome.

Closed spinal dysraphism: a review on diagnosis and treatment in infancy.

This article reviews the clinical presentation, pathophysiology, diagnostic strategies, and therapeutic management of closed spinal dysraphism in infancy. Four groups of symptoms are distinguished: (1) cutaneous abnormalities, (2) lower motor neuron dysfunction due to congenital spinal and nerve root abnormalities, (3) upper motor neuron dysfunction due to tethering of the spinal cord, and (4) symptoms caused by associated malformations. Guidelines are proposed concerning timing and type of diagnostic investigations in infancy. This essentially encompasses a neurological assessment, including spinal ultrasonography and nuclear magnetic resonance imaging of the spine and the brain, and a urological assessment, including ultrasonography of kidneys and bladder, cystourethrography and a urodynamic study. As to the tethered cord syndrome it is concluded that first, already in infancy, a progressive neurological dysfunction can be detected; second, surgical untethering should be performed only upon appearance of upper motor neuron signs or upon progression of lower motor neuron signs. Despite its frequently asymptomatic course, the diagnosis of a congenital lumbosacral lipoma, and in the more general sense, of a closed spinal dysraphism, implies a periodic, multidisciplinary follow-up for life.

Determination of independent risk factors and comparative analysis of diagnostic methods for immediate type latex allergy in spina bifida patients.

BACKGROUND: Development of allergy to natural rubber latex in spina bifida patients is determined by several risk factors, such as age, number of interventions and atopic disease that are, however, interdependent. Furthermore, several diagnostic procedures have been analysed, but a comprehensive analysis of their diagnostic significance is lacking. OBJECTIVE: To determine the independent major risk factor(s) for development of natural rubber latex allergy and the most valuable diagnostic procedure. METHODS: In aselectively collected spina bifida patients, we correlated existing natural rubber latex allergy with age, sex, atopy and the number of hospitalizations and of surgical interventions in appropriately matched subgroups. Allergy to natural rubber latex was established by application of a latex glove fragment on the skin. Skin-prick tests with glove eluate, a natural latex extract and a commercial latex extract were carried out as were specific IgE measurements by radioimmuno assay (RAST-CAP). The results of the latex application test are compared with the other diagnostic methods. RESULTS: Out of 74 fully evaluated patients, 17 had a positive application test. The number of surgical interventions correlates strongly with the presence of natural rubber latex allergy (P<0.0002), independent of age, sex and presence of atopy. Skin-prick tests with unstandardized allergens made from known high allergenic latex gloves represent the most sensitive diagnostic method, with the highest negative predictive value and a specificity of 0.95. RAST-CAP was the next best method with a specificity of 0.93, a sensitivity of 0.89 and a negative predictive value of 0.97. CONCLUSION: The number of surgical interventions is the major independent determining factor for allergy to natural rubber latex in spina bifida patients. Unstandardized skin-prick tests are the most sensitive and specific diagnostic tool, but RAST-CAP is almost equally performant and therefore a valid alternative.

Treatment of neurogenic bladder dysfunction in infants and children with neurospinal dysraphism with clean intermittent (self)catheterisation and optimized intravesical oxybutynin hydrochloride therapy.

Clean intermittent (self)catheterisation (CIC) in combination with oral anticholinergic drugs (oxybutynin hydrochloride [OH] is the present standard therapy for neurogenic bladder dysfunction (NBD) with detrusor hyperactivity. However, complete suppression of detrusor contractions and complete urinary continence is not always obtained despite maximal dosage, and the high incidence of severe systemic anticholinergic side-effects often impairs therapeutic compliance, resulting in dose reduction or even discontinuation of therapy. The intravesical administration of OH has been shown recently to be an effective alternative for treating persistent detrusor hyperactivity, and occurrence and severity of systemic side-effects appeared to be significantly decreased. However, available data are limited from a paediatric view. Furthermore, it is our belief that the use of crushed OH tablets with consequent problems of impracticability accounts for the variable long-term patient compliance reported to be the only disadvantage to intravesical OH. Using an optimized drug preparation we demonstrate the superiority of intravesical OH for treatment of NBD in 15 children (range 0.6-13.75 years, mean 6.1) with incomplete detrusor activity suppression and/or intolerable systemic side-effects on oral OH therapy. Since the previous reported problems of impracticability and variable long-term patient compliance can be resolved by optimized drug preparation, we therefore conclude that the era of crushing OH tablets should be over in order to allow the intravesical OH therapy on a long-lasting and large-scale basis.

Conservative versus neurosurgical treatment of tethered cord patients.

We compared the outcome of neurosurgical release of a tethered spinal cord in 20 children with the neurological evolution of 21 other children known iwth a tethered spinal cord on nuclear magnetic resonance scan (NMR). Neurosurgery yielded stabilisation of the symptoms without any lasting improvement. Pre-operative conservative medical treatment had to be continued in all operated children. Three of the 20 operated children are showing signs of retethering despite appropriate neurosurgery, indicating that retethering should be considered as a major and frequent complication. In the majority of our children, symptomatic (re)tethering was recognised by the appearance of increased tendon reflexes and a progressive pes cavus, suggestive of an upper motor neuron disease involvement.

[Urodynamic, radiological and clinical studies in children with spina bifida]. / Etude urodynamique, radiologique et clinique chez des enfants avec spina bifida.

In 61 children with myelomeningocele urodynamic, radiological and clinical data are correlated. Bladder hyperactivity (low compliance and instability) was present in 77% of the cases. No correlation was observed with the clinical neurological level. However impairment of renal function by hydronephrosis or reflux occurred in 19 of the 33 cases presenting a bladder hyperpressure of more than 40 cm of water for bladder filling volumes lower than the bladder capacity accepted as normal for the children's age. Two thirds of urinary infections occurred in the children with hyperactive bladders. Detrusor-sphincter dyssynergia is observed in only 29% of cases.