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Biomedical subjects

S B Bauer

Publications and source records attributed to S B Bauer.

At least 19 recordsLinked to original sources
PubMed1992

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↗
PubMed1992

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↗
PubMed1992

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↗
PubMed1992

Perforation of the augmented bladder.

In 12 of 264 children treated with enterocystoplasty 15 spontaneous perforations occurred. Of the 12 children 9 had myelodysplasia. All segments of the gastrointestinal tract were used for the augmentation and most were detubularized. Surgery to increase bladder outlet resistance was done in 8 cases. At the time of each perforation 9 children had sterile cultures, however, 3 died of overwhelming sepsis. Presenting signs included abdominal pain in 8 cases, septic shock in 4 cases and shoulder pain in 4 older myelodysplastic children with diaphragmatic irritation from escaping urine. Cystography demonstrated a leak in 10 of 11 cases. Urodynamic studies revealed good compliance with low maximum filling pressure in 8 of 10 children. Hyperreflexia was noted in only 5 cases and outlet resistance greater than 85 cm. water was demonstrated in 5. Histological analysis showed changes in the bowel wall consistent with ischemia but suture granulomas were present in areas adjacent to the perforation site or thinned areas in biopsy or autopsy specimens. In addition to the theory that overdistention may cause enterocystoplasty perforation, current detubularization techniques may produce areas of relative ischemia, which become accentuated when the augmented bladder is distended beyond a reasonable volume.

Adolescent↗
PubMed1991

Vesical manifestations of chronic granulomatous disease in children. Its relation to eosinophilic cystitis.

Two children with chronic granulomatous disease involving the bladder are reported. Their clinical course was characterized by lower urinary tract symptoms, decreased vesical capacity, unilateral ureterovesical junction obstruction, and an intense eosinophilic infiltrate on biopsy specimens in each. Chronic granulomatous disease and eosinophilic cystitis appear to have remarkably similar clinical manifestations. A review of urinary tract involvement in chronic granulomatous disease and its comparison with eosinophilic cystitis is presented.

Child, Preschool↗
PubMed1991

The long-term results of artificial sphincters in children.

To determine the long-term efficacy of the artificial urinary sphincter, 36 consecutive children in whom a prosthesis was implanted between August 1978 and July 1985 were followed for a minimum of 5 years and re-evaluated within the last 3 months. Of the 36 sphincters 27 (75%) are currently in place; 2 of these 27 sphincters are dysfunctional due to fluid leakage. Of the 25 functioning sphincters 14 have required no further prosthetic operation, 10 needed 1 revision and 1 patient had 5 revisions. Fluid leakage, occurring in 12 patients, was the most common complication requiring reoperation. Twenty patients are completely continent (11 of whom without any medication) and 5 are occasionally damp. The over-all success rate at 2 and 5 years was 84 and 62%, respectively. The mean survival time of the prosthesis was 7.2 years and the mean standard interval was greater than 10.5 years. Patients with higher balloon pressures (81 to 100 cm. water) had more complications, and a decreased rate of success than those with lower pressure balloons (51 to 80 cm. water, p = 0.02). There was no difference in success rates between boys and girls (p = 0.98), although girls who had a prior bladder neck operation tended to have a higher rate of cuff erosion. Sphincters placed after 1980 (model AS800) had a lower reoperation rate than those placed earlier. In selected instances, the artificial urinary sphincter appears to be a viable long-term alternative for management of children with intractable incontinence who have either failed or are unsuitable for other methods of treatment.

Child↗
PubMed1990

The urodynamic consequences of posterior urethral valves.

We evaluated urodynamically 41 patients with posterior urethral valves because of signs or symptoms of incontinence (35), frequency (3), hydronephrosis (2) and infection (1). Findings included normal urodynamic evaluations in 3 patients, 2 had high voiding pressures secondary to outlet resistance and 1 had incontinence on the basis of external urethral sphincter damage. In the remainder 3 patterns of bladder dysfunction were identified. Myogenic failure with overflow incontinence occurred in 14 patients. In this group clean intermittent catheterization or Valsalva's voiding was used for emptying. Hyperreflexic bladders were seen in 10 patients. Pharmacological suppression of instability was effective in 5 of 7 patients treated; 1 required bladder augmentation. Eleven children had a small capacity bladder and poor compliance. Post-void residuals were low and these bladders were generally but not always stable. Pharmacological bladder relaxation was successful in 3 patients, 3 underwent augmentation, 1 did well with alpha-agonists and followup is unavailable on the other 4. These 3 patterns of bladder dysfunction represent an overlapping constellation of residual urodynamic abnormalities due to previous bladder outlet obstruction. Individual patients may show facets of several types of dysfunction associated with 1 predominant pattern.

Child↗
PubMed1990

Paratesticular rhabdomyosarcoma: results of therapy in 18 cases.

The management of paratesticular rhabdomyosarcoma has evolved more slowly than that for testicular germ cell tumors, mainly due to its lower incidence and the lack of standardized treatment protocols. With gradual refinements in staging and therapy the prognosis has improved. Between 1960 and 1988, 18 patients 2 to 18 years old were treated at the Children's Hospital. Management consisted of inguinal orchiectomy, staging evaluation and retroperitoneal lymph node dissection, followed by chemotherapy and radiotherapy according to the stage of the disease. In 11 patients (61%) the disease was confined to the scrotum, while 7 presented with spread to the retroperitoneal lymph nodes, including 3 children with more distant metastases. Histological study revealed predominantly embryonal characteristics except for 1 alveolar cell type. Chemotherapy consisted of actinomycin D in 2 patients, vincristine, actinomycin D and cyclophosphamide in 2, pulse vincristine, actinomycin D and cyclophosphamide in 10, and doxorubicin plus vincristine, actinomycin D and cyclophosphamide in 4. A total of 17 patients remain free of disease (median followup 4 years) with 1 death. The actuarial survival without relapse and over-all survival rate are 89 and 94%, respectively. We conclude that paratesticular rhabdomyosarcoma represents a favorable subgroup of rhabdomyosarcoma.

Actuarial Analysis↗
PubMed1990

Descent of the bladder neck: a urographic finding in denervation of the urethral sphincter in children with myelodysplasia.

The urographic position of the neck of the bladder in relation to the pubic symphysis was correlated with lower motor neuron (LMN) denervation of the urethral sphincter as detected with electromyography in 65 infants and children with myelodysplasia. Descent (prolapse) of the bladder (bladder neck below the upper margin of the pubic symphysis) was present in 30 (46.2%) patients. Prolapse was both more frequent and tended to be more severe in girls. Complete LMN denervation of the urethral sphincter was present in 25 (83.3%) of the 30 patients with prolapse and in only four (11.4%) of 35 with a normally positioned bladder. Conversely, 25 (86.2%) of the 29 patients with complete LMN denervation of the sphincter had prolapse as compared with only five (13.9%) of 36 patients with partial or no LMN denervation of the sphincter. Although not as precise as urodynamic testing, significant descent of the bladder neck is a reliable urographic finding of complete LMN denervation of the external urethral sphincter in infants and children with myelodysplasia.

Child↗
PubMed1990

Management of the obstructed urinary tract associated with neurogenic bladder dysfunction.

The child with neurogenic bladder dysfunction may suffer from lower urinary tract obstruction, which, if untreated, can result in upper urinary tract deterioration. It is prudent to identify those newborns and infants who are at risk for upper urinary tract deterioration and advise an appropriate treatment protocol. In order to identify those children at risk, a thorough examination is required, along with appropriate upper urinary tract imaging studies and urodynamic assessment of the bladder. Infants and children at risk for upper tract deterioration are those who have intermittent or continuous detrusor pressure elevation above 40 cm H2O. We have identified the safe period, which is the time during which the bladder remains at a pressure lower than 40 cm H2O. Our management protocol for each child is individualized and based on increasing the safe period. The safe period can be increased with a combination of intermittent catheterization, administration of medications, and, in some cases, surgical reconstruction. With compulsive evaluation and intensive management, many of the previous upper urinary tract changes associated with the obstructed neurogenic bladder can be averted. With the above factors in mind, we have defined the period of bladder filling and storage where the pressure is below 40 cm H2O as the safe period. The ideal bladder for maintenance of normal upper tracts would have a long safe period and low voiding pressure. Our treatment protocol for the neurogenic bladder is therefore dependent on identifying those children who have very short or nonexistent safe periods. These are the children who are at risk for upper tract deterioration.

Child↗
PubMed1990

Evaluation and management of urinary incontinence after surgery for posterior urethral valves.

Posterior urethral valves lead to an overlapping constellation of urodynamic abnormalities, often many years after the initial diagnosis and valve ablation. Voiding dysfunction and urinary incontinence in boys with a history of valves are seldom the result of sphincteric incompetence. Three major categories of bladder dysfunction may cause voiding symptoms: myogenic failure, detrusor hyperreflexia, and bladder hypertonia. The interaction of the severity and duration of valve obstruction may be important factors, but the determinants of ultimate bladder function in the setting of urethral valves remain unclear. A careful urodynamic assessment is needed to define the abnormal bladder and to select and evaluate therapy. Therapy should be designed to provide low-pressure urinary storage for a socially acceptable period of time as well as complete emptying of the bladder. Success may require several lines of treatment and demands patience, not only of the patient and his family, but of the physician.

Child↗
PubMed1989

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↗
PubMed1989

Congenital midureteral obstruction.

Congenital midureteral obstruction, caused either by a ureteral valve or stricture, is an exceedingly rare entity. When encountered, it is generally misdiagnosed as either primary megaureter or ureteropelvic junction obstruction, leading to a less than optimal surgical approach. Seven children with midureteral obstruction were seen over the past 17 years. Two of the patients presented with hydronephrosis on prenatal ultrasonography, and five with urinary tract infection. In only three was the diagnosis suspected on intravenous urography and voiding cystourethrography alone. Five of the seven cases had either antegrade or retrograde pyelography, prior to or at the time of their operative procedure, and in four of these a correct diagnosis was made. Notably, in five of the seven cases, recognition of the midureteral lesion prior to the surgical incision allowed the approach to be modified and a more appropriate technique (ureteroureterostomy) to be performed. The etiology of midureteral valve and stricture has been attributed to improper recanalization, insufficient vascular supply, or persistence of ureteral folds. Three of our patients had contralateral renal dysgenesis, suggesting an underlying ureteral bud abnormality.

Child↗
PubMed1989

The use of rectus fascia to manage urinary incontinence.

The surgical management of urinary incontinence in children is one of the most difficult challenges facing pediatric urologists today. We have critically looked at our experience using a rectus fascial sling to prevent incontinence. Eleven girls (6 to 22 years old) with urinary incontinence from neurogenic bladder dysfunction (9), surgical injury to the bladder (1) or urogenital sinus abnormality (1) comprise this study. All had failed regimens of pharmacological therapy and intermittent catheterization. Three patients had undergone prior bladder neck reconstruction and 2 an augmentation cystoplasty in an attempt to control the incontinence. Urodynamic studies revealed several reasons for the continued wetting in these individuals: a changing neurological lesion leading to a loss of innervation and concomitantly lowered urethral resistance in 2 patients, adequate urethral resistance at rest but decreasing resistance with bladder filling in 4, no increase in urethral resistance with increases in abdominal pressure in 4 and urethral instability (a decrease in resistance following a cough or Valsalva's maneuver) in 1 apparently neurologically normal girl. Eight patients are dry 3 to 24 months postoperatively on intermittent catheterization. All have demonstrated either an increase in urethral resistance at rest or an adequate level of resistance during filling of the bladder or a sudden increase in abdominal pressure. Of the remaining 3 patients 1 is dry for 2 to 3 hours but then leakage occurs, 1 is improved but damp and 1 patient is wet 3 months postoperatively. The use of rectus fascia to improve outlet resistance seems to be a viable alternative in the management of incontinence in selected female subjects.

Adolescent↗
PubMed1989

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↗
PubMed1989

Congenital obstructed megaureters in early infancy: diagnosis and treatment.

Fetal screening during maternal ultrasonography has changed the mode and age of presentation of congenital uropathies, particularly congenital obstructed megaureter. We studied 47 infants less than 8 months old with primary obstructed megaureter. Surgical correction in 42 patients with moderate to severe obstruction was performed at a mean age of 1.8 months in those detected prenatally and 3.8 months in those presenting after birth. All infants showed functional and structural urographic improvement with a mean followup of 2.3 years. Reflux was seen postoperatively in 8 patients, which subsided spontaneously in 3, continues to be followed in 2 and resulted in repeat reimplantation in 3. Five infants had mild obstruction, which was managed nonoperatively and 2 showed progressive obstruction, which required repair at ages 20 and 28 months. Repair of obstructed megaureters in early infancy improves renal drainage and offers the potential for preventing renal damage before the development of symptoms or infection. With proper attention to detail, excellent results may be achieved.

Dilatation, Pathologic↗
PubMed1989

Strictures in the ileal portion of ileocecal conduits in children and young adults.

Patients with ileocecal conduits are at risk for development of late complications. We present 3 patients who had strictures in the ileal portion of the ileocecal conduit, leading to deterioration of the upper urinary tract. The pathogenesis of these strictures appears to result from chronic inflammation. Long-term periodic upper urinary tract screening with ultrasonography is important for early detection of this complication. When new or increasing hydronephrosis is found a loopogram or antegrade pyelogram (if the ileocecal valve is competent) should be performed to evaluate the ileal portion of the ileocecal conduit.

Adolescent↗