Spared sacral function in patients with complete thoracolumbar myelodysplasia.
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Child↗
Biomedical subjects
F M Dyro
Publications and source records attributed to F M Dyro.
The neurosurgical implications of continuous neurourological surveillance of children with myelodysplasia.
Between 1979 and 1990, 148 newborns with myelodysplasia were followed with serial urodynamic studies and neurological assessment. Of the patients 59 (40%) exhibited changes in neurological status by age 5 years, of whom 28 (19%) showed signs of deterioration. Most changes occurred before age 2 years. A total of 22 children underwent repeat neurosurgical exploration because of a change in urethral sphincter innervation (17), deterioration of function of the lower extremities (3), or changes on computerized tomography or magnetic resonance imaging (2). Postoperative urodynamic evaluation demonstrated improvement in 11 children, stabilization in 9 and further deterioration in 2. The earlier that a change was detected and secondary surgery was performed, the better the outcome. These findings indicate that the neurological lesion in myelodysplasia is a dynamic disease process requiring continuous neurological, orthopedic and urodynamic surveillance. Early identification and prompt neurosurgical reexploration seem to arrest and even reverse the neurological deterioration that takes place in a substantial number of these children.
Child, Preschool↗
The prophylactic value of clean intermittent catheterization and anticholinergic medication in newborns and infants with myelodysplasia at risk of developing urinary tract deterioration.
OBJECTIVE: To determine if prophylactic use of clean intermittent catheterization and oxybutynin chloride is effective in preventing urinary tract deterioration in myelodysplastic children with high bladder pressure and detrusor-sphincter dyssynergia. DESIGN: Sequential, nonrandomized trial. SETTING: Referral-based urodynamics facility and myelodysplasia program at a major city pediatric hospital. PARTICIPANTS: Twenty-six of 71 consecutive newborns with myelodysplasia who exhibited these urodynamic findings were treated prophylactically over 5 years, whereas 56 of 105 consecutive newborns with the same findings treated during the previous 7 years were treated expectantly. INTERVENTION: Clean intermittent catheterization and oxybutynin therapy were begun when these specific urodynamic findings were detected. MEASUREMENTS AND RESULTS: Follow-up urodynamic studies and roentgenographic assessment of the urinary tract were performed periodically over 5 years. Oxybutynin eliminated uninhibited contractions in two of 14 newborns and lowered peak contractile pressure in the remaining 12. Oxybutynin also lowered bladder-filling pressure at capacity in all 12 additional neonates with only hypertonicity. Twenty-four (92%) of 26 children had normal kidney function and drainage during the observation period, two (8%) developed hydroureteronephrosis, and one of these two had vesicoureteral reflux. In a prior study of children similarly at risk who were treated expectantly, the roentgenographic appearance of the upper urinary tract had changed in 48%. Minimal side effects were noted with oxybutynin, and no adverse effects of clean intermittent catheterization were detected. CONCLUSIONS: Oxybutynin effectively reduces uninhibited contractions and lowers detrusor filling pressure, while clean intermittent catheterization allows bladder emptying at low pressures with no measurable side effects in these neonates. The overall effect maintains the integrity of the upper urinary tract in almost all myelodysplastic children at risk of urinary tract deterioration. Expectant therapy can no longer be advocated when these "at risk" children are identified because prophylactic treatment is so effective.
Boston↗
Urodynamic dysfunction in walking myelodysplastic children.
We evaluated urodynamically and radiologically 54 children with myelodysplasia and neurological deficits at or below the S1 level. Baseline urodynamic testing was normal in 13 patients (24%), while 12 (22%) had an upper motor neuron and 13 (24%) had a lower motor neuron type of dysfunction. A total of 7 patients (13%) had a mixed upper and lower motor neuron type, and 9 (17%) had only lower motor neuron dysfunction of the urethral sphincter with a normally contractile bladder. Followup studies varying in time from 1 month to 10 years showed a changing neurourological lesion in 29 patients (54%): 25 deteriorated while 4 improved. Of the 54 children 20 had hydronephrosis and/or vesicoureteral reflux. Incontinence was the major problem in 41 patients (75%), and was managed initially with pharmacological agents and/or clean intermittent catheterization, with 9 of the 41 eventually requiring surgery. Urodynamic assessment reveals a variable picture that does not correlate well with the apparent neurological examination. Despite the low level of the neurological deficit, many children may be at risk for urinary tract deterioration. These findings emphasize the importance of continuous surveillance and appropriate management in this group of myelodysplastic children who have the greatest potential for a normal life.
Child↗
Bladder functional changes resulting from lipomyelomeningocele repair.
From 1986 to 1991, 12 boys and 23 girls underwent surgery for lipomyelomeningocele removal. Of these patients 29 were 15 months old or younger (average age 3 months), while 6 were 4.5 to 19 years old (average age 10 years). Preoperative and postoperative urodynamic studies, including external urethral sphincter electromyography, were done on everyone. All 29 infants had a cutaneous lesion overlying the lower back and 14 had an abnormal neurological examination. Preoperative urodynamic studies were abnormal in 11 patients, consisting of an upper motor neuron lesion in 6, and a mixed upper and lower motor neuron lesion in 5. Postoperatively, 10 of 14 children with an abnormal neurological examination improved, while 9 of 11 with abnormal lower urinary tract function normalized. In 1 of 18 children (6%) with normal preoperative urodynamic studies detrusor-sphincter dyssynergia developed postoperatively. In all 6 older children urinary incontinence developed, and this led to the diagnosis. Everyone had an abnormal neurological examination and abnormal preoperative urodynamic studies. One child had a lower motor neuron lesion, and 5 had a mixed upper and lower motor neuron lesion. Postoperatively, the neurological examination improved in only 1 patient (16%), and the urological symptoms and urodynamic findings improved in another child. Lipomyelomeningocele has a progressive effect on lower spinal cord function because infants tend to present with fewer urinary manifestations and physical findings than older children. Individuals who escape early detection tend to have a more subtle cutaneous abnormality. As a result, older children are more likely to present with urological and neurological complaints. Surgical correction in infancy provides a degree of reversibility not seen in older children. It is imperative that early identification, evaluation and treatment be undertaken to prevent this progression and permanency of neurological changes and urinary dysfunction.
Adolescent↗
Modulation of the bulbocavernosus reflex during voiding: loss of inhibition in upper motor neuron lesions.
The bulbocavernosus reflex (BCR) is a sacral segmental reflex which is suppressed throughout most of micturition. The BCR was evaluated by recording the associated EMG activity using a concentric needle electrode placed in the external urethral sphincter. The influence of the central nervous system (CNS) on the BCR was studied in 29 neurologically normal individuals and in 33 patients with upper motor neuron (UMN) lesions and voiding dysfunction. Ninety percent (26/29) of the neurologically normal patients did not have a BCR during most of micturition. By comparison, all the patients with an UMN lesion had a positive BCR beginning early during voiding. The finding of suprasegmental modulation of the BCR during voiding demonstrated by external urethral sphincter EMG was a more sensitive although slightly less specific sign of UMN bladder dysfunction than detrusor sphincter dyssynergia; uninhibited bladder contractions were neither sensitive nor specific for UMN lesions.
Adolescent↗
The unrecognized neuropathic bladder of infancy.
Four infants presented with hydroureteronephrosis and incomplete emptying of the bladder secondary to detrusor-sphincter dyssynergia. None of the patients had other signs suggestive of neurological dysfunction. It is presumed but not proved that these infants manifested a urodynamic reaction in response to a perinatal insult involving the brainstem area. Long-term followup has demonstrated a persistent but nonprogressive neurourological disturbance.
Electromyography↗
Neurourological implications of the changing approach in management of occult spinal lesions.
Occult lesions of the spine in children are a rare but recognizable cause of neurogenic dysfunction involving the lower extremities, and lower urinary and gastrointestinal tracts. We report the preoperative and postoperative urodynamic findings in 40 children with varying spinal abnormalities. Of these patients 28 were neonates or infants (average age 8.7 months) and 12 were older children (average age 11.7 years). Preoperative urodynamic testing revealed normal function in 18 of 28 children (64 per cent) in the younger age group in contrast to 1 of 12 (8 per cent) in the older age group. Of the 10 infants with abnormal studies postoperative urodynamic findings returned to normal in 6, while 2 others remained abnormal but were improved. In contrast, of 11 older children with abnormal preoperative evaluations 3 (27 per cent) reverted to normal postoperatively. The neurourological changes seen in these occult lesions are variable, may occur at any age, are progressive and are potentially reversible by surgical correction but this reversibility diminishes with age.
Adolescent↗
Syringomyelia: electrophysiologic aspects.
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Electrophysiology↗
The changing neurourologic lesion in myelodysplasia.
Seventy-nine newborns with myelodysplasia were followed up for 1 1/2 to six years with serial roentgenographic, neurologic, and urodynamic evaluations. Twenty-nine infants (37%) demonstrated changes in external urethral sphincter innervation during the first three years of life. Of the group who changed, 896% (25/29 infants) did so in the first year, 10% (3/29 infants) in the second year, and 4% (1/29 infants) in the third. The risk factor for change was 32%, 6%, and 2%, respectively, during each of these three years. Fifteen infants (19% of the total) experienced neurourologic deterioration while 14 infants (18%) improved. Seven newborns underwent a second neurosurgical procedure resulting in improvement in four, stabilization in two, and further deterioration in one. These findings underline the importance of early and continued neurologic and urologic surveillance in children with myelodysplasia because it is a dynamic disease process.
Electromyography↗
Urodynamic assessment of children with cerebral palsy.
More than a third of the children with cerebral palsy present with dysfunctional voiding symptoms. Clinical evaluation and urodynamic study of cerebral palsy patients were performed to characterize the symptoms, to define the type of neurological deficit and to document its effect on voiding dynamics. We evaluated flow rate, and cystometrographic and external sphincter electromyographic findings in 57 children with cerebral palsy. Upper and lower motor neuron lesions were defined by standard criteria. Of the children 86 per cent had evidence of a pure upper motor neuron injury and 11 per cent manifested electromyographic findings suggestive of incomplete lower motor neuron sphincteric injury. The latter deficit could not be predicted on the basis of clinical neurological findings but it was suggested by a history of neonatal cyanosis. Treatment protocols achieved continence in more than 75 per cent of the children. The voiding dynamics in children with cerebral palsy have not been assessed previously. We have defined the lower urinary tract dysfunction in these patients, and provide a rational and effective plan of management.
Adolescent↗
Refractoriness of urethral striated sphincter during voiding: studies with afferent pudendal reflex arc stimulation in male subjects.
To assess the excitability of the striated sphincter under normal and abnormal conditions, electrostimulation of the periurethral striated sphincter via the dorsal nerve of the penis was done with the patient at rest and during voiding. Monitoring of simultaneous intravesical and intramembranous urethral pressures, and electromyographic responses of the striated sphincter was performed under fluoroscopic guidance in 14 male subjects. The urethral striated sphincter attained a state of relative refractoriness during detrusor contraction (voiding phase) and greater amounts of afferent stimulation were required to elicit sphincter contractile activity compared to the amounts required during resting states. Under conditions of a hyperactive detrusor with synergic voiding, the amounts of stimulation required to elicit striated sphincter responses were higher than those required in normal subjects. On the other hand, under conditions of striated sphincter dyssynergia, minute amounts of afferent stimulation were enough to produce sphincter contraction during voiding.
Electric Stimulation↗
Electrodiagnostic abnormalities in 15 patients with posttraumatic syringomyelia: pre- and postoperative studies.
A review of 15 patients with posttraumatic syringomyelia indicates that the most reliable electrodiagnostic criteria for the diagnosis of syrinx are the loss of motor unit numbers with increase in motor unit amplitude and duration nd synchronous firing. Prolongation of F wave latency in a previously stable patient is a useful observation. Return of function and improvement of F wave latencies can occur rapidly following decompression of the syrinx. Forty per cent of the patients studied had concomitant involvement of at least one peripheral nerve as one would expect invoking the double crush hypothesis.
Adult↗
Posttraumatic cervical syringomyelia. Incidence, clinical presentation, electrophysiological studies, syrinx protein and results of conservative and operative treatment.
In eleven years, 30 (3.2%) of 951 patients with spinal cord injury developed cervical syringomyelia. This condition was found in 22 (4.5%) of 488 posttraumatic tetraplegic and 8 (1.7%) of 463 posttraumatic paraplegic patients; the incidence was about 8 per cent in patients with complete tetraplegia. This study demonstrated the rarer clinical manifestations of syringomyelia, namely autonomic dysfunction, alterations in the sensory level with postural changes, the early occurrence of tendon areflexia and painless motor deterioration. Prolonged F wave latencies were present in all patients with a demonstrable syrinx and a higher protein content was found in the syrinx than in the cisternal fluid. Some of the symptoms and signs in a proportion of the patients treated conservatively remained stable without operative treatment over a number of years. Most of the patients in whom operation was performed for progressive motor weakness or severe pain had good postoperative results although a few developed late sensory or motor changes. There was no benefit in operating on a patient with a small syrinx.
Adult↗
Occult spinal dysraphism: a rare but detectable cause of voiding dysfunction.
Certain signs and symptoms should alert the clinician to a diagnosis of spinal dysraphism, one of the underlying causes of voiding dysfunction in children. The type of bladder and sphincter dysfunction found in eight children wit spinal dysraphism and the modes of therapy employed are discussed.
Adolescent↗
The unstable bladder in childhood.
A comprehensive study of children with voiding dysfunction, which included careful documentation of voiding habits, thorough radiologic investigation, and urodynamic evaluation was undertaken. Proposed etiologic factors, indications for therapy, and results of treatment are discussed.
Adolescent↗
Methyl ethyl ketone polyneuropathy in shoe factory workers.
Three cases of polyneuropathy in shoe factory workers are described. The etiologic agent is felt to be methyl ketone, a substance previously thought to be a safe solvent. The concentration of this substance in the ambient air of the patients was below the threshold limit value. This suggests that there may be a cumulative effect and that we should reexamine the threshold limit values set for all the organic solvents with respect to inhalation and absorption from skin surfaces.
Adult↗