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Biomedical subjects

S B Bauer

Publications and source records attributed to S B Bauer.

At least 37 records · Page 2Linked to original sources

Normal urodynamics in patients with bladder exstrophy: are they achievable?

PURPOSE: Urodynamic study was performed in patients with exstrophy to determine the effect of bladder neck reconstruction and the ability to achieve normal urodynamics following surgery. MATERIALS AND METHODS: A total of 30 exstrophy cases at different stages of reconstruction were retrospectively reviewed in terms of bladder capacity, compliance, stability and presence of detrusor contractions following urodynamic study. RESULTS: Bladder capacity increased from a third predicted volume for age to half after reconstruction. Approximately 80% of patients had compliant and stable bladders before bladder neck reconstruction. Following bladder neck reconstruction approximately half of the patients maintained normal compliance with a smaller number maintaining normal stability. A quarter of patients maintained normal filling dynamics following bladder neck reconstruction, and 19% maintained normal filling and voiding dynamics after reconstruction. CONCLUSIONS: The majority of closed exstrophy bladders have normal filling dynamics before bladder neck reconstruction. Compliance and stability are impaired following bladder neck reconstruction. Approximately 25% of patients with exstrophy may maintain normal detrusor function following reconstruction. However, less invasive alternatives to the Young-Dees-Leadbetter bladder neck reconstruction should be sought.

Adolescent↗

Unilateral suprainguinal ectopic scrotum: the role of the gubernaculum in the formation of an ectopic scrotum.

A rare case of ectopic scrotum is described together with a review of the literature and a discussion of the embryological role of the gubernaculum in the formation and location of normal and ectopic scrota. We identified 16 reported cases of a suprainguinal ectopic scrotum, 4 cases of a femoral ectopic scrotum, 26 cases of penoscrotal transposition, and 19 cases of a perineal (accessory) scrotum. Although the gubernaculum is a prerequisite for the ultimate location of both the testis and scrotum, its role is complicated by the subsequent differential growth of the labioscrotal folds in which the gubernaculum is stabilized. If this interaction is disturbed, the result may be a suprainguinal ectopia, penoscrotal transposition or a perineal scrotum. A femoral ectopic scrotum, unlike the above, is the result of an aberrant gubernacular stabilization. While the etiology of these malformations is likely to be multifactorial, the existence of an inbred strain of rats characterized by a high incidence of an ectopic scrotum suggests a genetic component to this anomaly.

Animals↗

Urachal anomalies: defining the best diagnostic modality.

OBJECTIVES: Urachal abnormalities are uncommon and the literature is primarily comprised of case reports. Conclusions regarding the presentation and diagnosis of these abnormalities may be elucidated by reviewing a large experience. METHODS: The records of 45 patients with urachal abnormalities in the pediatric age group were reviewed from 1970 to 1997. This included 24 boys and 21 girls with an age range from 1 day to 20 years (average 4.0 years). The presenting complaint was periumbilical discharge in 19 patients (42%), umbilical cyst or mass in 15 (33%), abdominal or periumbilical pain in 10 (22%), and dysuria in 1 (2%). The diagnosis consisted of a urachal sinus in 22 children (49%), a urachal cyst in 16 (36%), and a patent urachus in 7 (15%). Various radiographic studies were used to establish the diagnosis. RESULTS: Patients with a urachal sinus had 16 voiding cystourethrograms performed (only 1 diagnostic), 9 sinograms (all diagnostic), 8 ultrasounds (4 diagnostic), and 1 excretory urogram (normal). Those with a urachal cyst had 8 voiding cystourethrograms (1 diagnostic), 5 excretory urograms (all normal), 4 ultrasounds (all diagnostic), and 1 computed tomography scan (diagnostic). Children with a patent urachus had 2 excretory urograms (both diagnostic), 1 voiding cystourethrogram (diagnostic), and 2 ultrasounds (normal). One baby with a patent urachus was diagnosed prenatally during ultrasound screening. The diagnosis was made by history and physical examination alone in 5 children and at the time of surgery in 1. Treatment consisted of surgical excision of the urachal abnormality with a cuff of bladder in 22 children, surgical excision without a bladder cuff in 22, incision and drainage of a urachal cyst (1%), and laparoscopic excision of a patent urachus with a bladder cuff in another (1%). There were three wound infections postoperatively. None developed any long-term sequelae. CONCLUSIONS: The diagnosis of urachal abnormalities can be made with certainty if a good physical examination and the appropriate radiographic test are performed. A patient who presents with periumbilical drainage should have a sinogram performed, which should be diagnostic for both a urachal sinus and a patent urachus. Any child who presents with a periumbilical mass should have an ultrasound performed, which should be diagnostic for a urachal cyst.

Adolescent↗

A single-system ectopic ureter draining an ectopic dysplastic kidney: delayed diagnosis in the young female with continuous urinary incontinence.

OBJECTIVE: To document the array of diagnostic modalities, the variety of errant diagnoses and treatments. and the time from initial presentation to ultimate diagnosis in girls with an ectopic single-system ureter draining an ectopic hypoplastic and/or dysplastic kidney. PATIENTS AND METHODS: Between 1990 and 1997, seven females were identified who had an ectopic hypoplastic and/or dysplastic kidney with an ectopically draining ureter, and who were treated at our institutions, either initially or upon referral. The nature and number of all diagnostic evaluations, previous diagnoses and treatments, and the time from presentation to definitive diagnosis were recorded. RESULTS: All seven females had a classical history of successful toilet training, a normal voiding pattern and continuous urinary incontinence. Typically, a solitary kidney was noted on the initial diagnostic evaluation by ultrasonography and/or intravenous urography. The mean (range) age at initial presentation was 3.2 (2-6) years. Additionally, voiding cysto-urethrography, urodynamics, radionuclide scintigraphy, computed tomography, magnetic resonance imaging and endoscopy were performed. The age at definitive diagnosis was 3-16.5 years and the mean (range) time from initial presentation to diagnosis was 5.7 (1-10) years. Nephroureterectomy was curative and all kidneys were dysplastic. CONCLUSION: Continuous urinary incontinence in females with a normal voiding pattern should prompt an evaluation for ureteric ectopia. When the initial evaluation yields the diagnosis of a solitary kidney, clinicians should be aware of the possibility of a hypoplastic and/or dysplastic, often ectopic, contralateral kidney with an ectopically draining ureter. Identification of this entity should allow curative surgical treatment.

Adolescent↗

Hypoplastic dysplastic kidney with a vaginal ectopic ureter identified by technetium-99m-DMSA scintigraphy.

Ectopic insertion of a ureter draining a hypoplastic dysplastic kidney is a significant cause of urinary incontinence in girls. In this case, such a kidney was detected with 99mTc-DMSA scintigraphy but not by intravenous pyelography. Scintigraphy facilitated further delineation of the anatomy with CT prior to nephrectomy. Based on this case and a literature review, we suggest that 99mTc-DMSA scintigraphy be performed early when evaluating girls with urinary incontinence.

Child↗

Current findings in diagnostic laparoscopic evaluation of the nonpalpable testis.

PURPOSE: We delineate the current findings and contribution of diagnostic laparoscopic evaluation in the management of nonpalpable testis. MATERIALS AND METHODS: We reviewed all cases in which laparoscopy was considered the management associated with a nonpalpable testis in a 4-year period. Since our previous series, we have performed a careful examination for the testis after induction of anesthesia but before committing to laparoscopy. We recorded testis position and quality, character of the vas deferens and spermatic vessels, type of management and contribution of laparoscopy. We also reviewed contemporary published series and collated the findings of studies performed elsewhere. RESULTS: We identified 263 nonpalpable testes in 225 patients between September 1992 and 1996. In 40 patients 46 testes (18%) were found during physical examination under anesthesia. Of the remaining cases considered appropriate for laparoscopy 215 with complete records were further analyzed. Only 12.6% could be considered missed on examination due to a viable testis distal to the inguinal ring. Of the testes 45.7% would have been found during inguinal exploration alone. In 9.8% of the patients there were intra-abdominal vanishing testes, while 4.2% had indeterminate cord structures on inguinal exploration that would have prompted abdominal extension without a laparoscopic demonstration that the vas and vessels entered the canal. A conventional inguinal incision would have provided optimal exposure for operative management in 34% of the testes. For testes distal to the internal ring when the vas and vessels were distinctly atretic we never identified a viable testis, while a normal appearing vas and vessel were associated with a 45% chance of a salvageable testis. Laparoscopy was informative regarding testis position in all cases in which it was performed. CONCLUSIONS: In 13.2% of the cases laparoscopic findings precluded unnecessary abdominal exploration. The typical surgical incision for inguinal exploration would have left the surgeon compromised in 66% of the cases compared to the approach optimized as a result of laparoscopic testicular localization. Of the patients 34% arguably did not benefit from laparoscopy versus inguinal exploration. A simple examination under anesthesia significantly decreases the number of uninformative laparoscopic evaluations, and it is well worth the cost of a few minutes of operative time.

Adolescent↗

Reservoir calculi: a comparison of reservoirs constructed from stomach and other enteric segments.

PURPOSE: The intestinal augmented bladder has an increased propensity to form urinary calculi. Predisposing risk factors include chronic bacteriuria, urinary stasis and mucus production. Gastric reservoirs have negligible mucus production and the ability to acidify the urine. We determined whether they also have a decreased incidence of stones. MATERIALS AND METHODS: We retrospectively reviewed the records of all patients undergoing augmentation cyptoplasty (215) or creation of a freestanding reservoir (44) between May 1976 and March 1996. Of these cases 83 were augmented with stomach and 179 were constructed from other intestinal segments, (that is ileal, ileocecal, sigmoid and/or a combination of these). Presenting diagnosis, patient age, gender, additional surgical procedures, interval to stone formation and calculous composition were recorded. Patients were excluded from study when there was less than 1 year of followup, as were those with renal or pre-augmentation bladder calculi. RESULTS: Stones formed in 32 of 207 patients (15%) after an average interval of 3.6 years (range 0.5 to 8.6). The majority of stones were composed of struvite (magnesium ammonium phosphate). Reservoirs containing gastric segments were significantly less likely to form calculi than those augmented with other intestinal segments (2 of 70 versus 30 of 137, p <0.0001 Fisher's exact test). Seven additional patients with pre-augmentation bladder stones who subsequently underwent gastrocystoplasty remain stone-free at an average followup of 3.0 years. CONCLUSIONS: Calculous formation is rare in reservoirs that incorporate a gastric segment. In patients with gastric augmentation stones appear only to develop when the patient is on histamine blockade or has a propensity to form stones that only favor an acidic environment, such as uric acid.

Adolescent↗

Correlation of cystographic bladder morphology and neuroanatomy in boys with posterior urethral valves.

PURPOSE: We have observed a difference in the radiographic appearance of the body of the bladder (trabeculated) and its base (smooth) in boys with severely obstructing posterior urethral valves. We wanted to determine if (1) this was a reproducible finding and (2) there was an anatomic and/or physiologic explanation for it. MATERIALS AND METHODS: We reviewed the initial voiding cystourethrogram in 47 boys with severe posterior urethral valves. The interureteric ridge was used as the division between the body and base of the bladder. The presence of trabeculation for each region was recorded. RESULTS: Ages ranged from 1 day to 6 years at the time of initial cystographic evaluation (median 14 days). The body of the bladder was trabeculated and the base smooth in 72 % (34 patients). In the remaining patients, both the body and base were smooth. In no patient was the base trabeculated. CONCLUSIONS: The cystographic morphology of the urinary bladder in boys with posterior urethral valves can be explained by its neuroanatomy. The body of the bladder, which contracts during voiding because of parasympathetic (cholinergic) stimulation, becomes trabeculated. The bladder base relaxes during voiding due to sympathetic (alpha adrenergic) stimulation and remains smooth. Thus, this difference in the cystographic appearance of the two parts of the urinary bladder reflects the normal innervation and the mechanics of micturition in boys with urethral obstruction.

Child↗

Vesicoureteral reflux in children: incidence and severity in siblings.

PURPOSE: We attempted to determine the incidence of vesicoureteral reflux in asymptomatic siblings of children with reflux at different ages and assess the incidence of renal damage in asymptomatic siblings with reflux. MATERIALS AND METHODS: We reviewed radionuclide cystograms of 482 consecutively referred siblings of children with vesicoureteral reflux, including 295 girls and 187 boys 2 weeks to 12.8 years old (mean age 2.8 years). Ultrasonograms and renal cortical scintigrams of children with reflux were evaluated. All siblings were considered asymptomatic by the referring physicians. RESULTS: The overall incidence of vesicoureteral reflux was 36.5%, and the incidence in girls and boys was 39.3 and 32.1%, respectively. Children 24 months old or younger had the highest incidence (45.7%) and the highest risk of bilateral reflux. From ages 25 to 72 months the incidence of reflux was 33.1% and in siblings older than 72 months it was 7%. Reflux of urine to the level of the renal pelvis was detected in 28.6% of all referred siblings. Renal damage was observed on sonography or scintigraphy in 4.7% of the siblings with reflux. CONCLUSIONS: The high incidence of vesicoureteral reflux through age 72 months indicates that it is important to screen siblings of children with reflux at an early age to prevent renal damage, which can occur in the absence of symptomatic urinary tract infection.

Age Distribution↗

Continent urinary diversion: the Children's Hospital experience.

PURPOSE: Continent urinary diversion has become increasingly important for treating childhood urinary tract pathology that cannot be managed by direct reconstructive techniques. We review our 9-year experience with continent diversion. MATERIALS AND METHODS: Since 1986 continent diversions were created in 74 patients 3 to 38 years old (mean age 13.7). The underlying pathological condition was the exstrophy/epispadias complex in 34 patients, neurological disorders in 23, malignancy in 13 and other congenital anomalies in 4. Followup averaged 5.2 years after the last procedure. Nonbladder reservoirs in 39 patients (53%) were fashioned from ileocolic (17), colic (7), gastrocolic (6), sigmoid (3), gastrosigmoid (2), ileosigmoid (2), ileal (1) and gastroileac (1) segments. When possible, the native bladder was incorporated into the reconstructive strategy. A total of 26 patients underwent bladder augmentation with intestine or stomach, including ileal (11), gastric (8), sigmoid (3), gastroileac (2) and ileocolic (2) segments. Nine other patients did not require bladder augmentation. Continence mechanisms were a flap valve (Mitrofanoff principle) in 50 patients, nipple valve in 15 and ileal plication (Indiana pouch) in 9. When the Mitrofanoff principle was used with a native bladder reservoir in 30 cases, outlet resistance was altered by bladder neck division (15), fascial sling placement (6) or Young-Dees-Leadbetter bladder neck reconstruction (2). In the remaining 7 patients the bladder neck remained intact. RESULTS: Excellent continence was obtained. The Mitrofanoff principle initially provided continence in 41 patients (82%). Six of the 9 incontinent patients were dry after a single revision. A total of 13 patients (87%) with nipple valves and 7 (78%) with Indiana pouches were dry, and the remaining 5 were cured after a single revision. Ultimately continence was achieved in 71 of the 74 patients (96%) after a maximum of 2 operations. Of the 48 complications in 29 patients the most common were difficulty in catheterizing (11), stones (11), infection (8) and upper tract deterioration (4). CONCLUSIONS: Many options exist for reconstructing complex anomalies. Choices must be individualized based on patient anatomy. The dry state may be achieved in most cases without resorting to a bag on the abdomen.

Adolescent↗

Estimating normal bladder capacity in children.

PURPOSE: An accurate estimation of normal bladder capacity can be helpful in evaluating the patient with genitourinary disease and in interpreting urodynamic data. Prior studies have provided initial estimates. We propose 2 new equations that are practical, easy to use and more accurate than those previously published. MATERIALS AND METHODS: We retrospectively reviewed the records of more than 5,000 children undergoing radionuclide cystography at our institution. Radionuclide cystography was conducted by instilling (99m)technetium pertechnetate via gravity drip in awake children. Bladder capacity was believed to be achieved when rate of inflow diminished to a minimal rate, initiation of voiding occurred or significant discomfort was indicated. Patients with vesicoureteral reflux, infravesical obstruction, urinary tract infection, dysfunctional voiding or other lower urinary tract pathology were excluded from the study. Linear and nonlinear regression modeling established the relationship between age and bladder capacity. RESULTS: A total of 2,066 children (598 boys and 1,468 girls) had normal radionuclide cystography and were included in the analysis. Analysis of variance demonstrated that increasing age was strongly predictive of bladder capacity (p <0.0001). Because a nonlinear model was the most accurate formula for all ages (4.5 x age(0.40) = capacity [ounces]), 2 practical linear equations were determined: 2 x age (years) + 2 = capacity (ounces) for children less than 2 years old, and age (years) divided by 2 + 6 = capacity (ounces) for those 2 years old or older. Although girls had larger capacities than boys, the rate of increase was not significantly different between them. CONCLUSIONS: The relationship between normal bladder capacity and age in children follows a nonlinear curve. This nonlinear relationship can be approximated by 2 practical linear formulas that are easy to remember and are derived from a larger population than any prior study. These formulas provided accurate estimations of bladder capacity when prospectively applied to normal patients.

Adolescent↗

Urodynamic and neurophysiologic evaluation of patients with diastematomyelia.

Diastematomyelia is a form of an occult dysraphism, which can present with lower urinary tract dysfunction. We present the findings in 21 patients with diastematomyelia evaluated at the Children's Hospital in Boston. Seventy-six percent of the patients were female. Eighty-six per cent had a cutaneous lesion present overlying the spine. Urodynamic and electrophysiologic studies showed abnormalities in 17 (81%) of 21 patients, of whom 13 (62%) had upper motor neuron dysfunction. Seventeen patients underwent corrective spinal surgery and none of the patients deteriorated on a follow-up urodynamic and electrophysiologic study. We recommend that patients with cutaneous, midline lower back lesions with or without lower urinary tract dysfunction be evaluated with a urodynamic and electrophysiologic study to characterize the extent of neurologic and urologic dysfunction.

Adolescent↗

Late effects of early surgery on lipoma and lipomeningocele in children less than 1 year old.

PURPOSE: We attempted to assess the usefulness of urodynamic testing for diagnosing lipoma and lipomeningocele, and the late effects of early neurosurgical intervention in children less than 1 year old. MATERIALS AND METHODS: A total of 28 patients in whom spinal surgery was performed before age 1 year for correcting lipoma and lipomeningocele underwent neurological and urodynamic evaluation preoperatively, and within 3 months and up to 14.5 years postoperatively. RESULTS: Preoperatively 15 patients (54%) had an abnormal neurological examination and 7 had an abnormal urodynamic study. Of the 13 patients with a normal neurological examination 3 had an abnormal urodynamic study. Overall 10 patients (37%) had an abnormal urodynamic examination preoperatively. Immediately after surgery 8 patients (29%) had improvement (2 of 15 with an abnormal neurological examination and 6 of 10 with an abnormal urodynamic study), whereas the condition of 9 (32%) worsened, including 4 of the 13 who were neurologically and 6 of the 18 who were urodynamically normal (1 in both categories). With time an additional 7 patients (25%) had progressive deterioration, including 6 urodynamically and 5 neurologically (4 in both categories), 2 (7%) had improvement (1 each urodynamically and neurologically) and the condition of 19 (68%) remained stable. Six patients (21%) underwent secondary spinal surgery for recurrent tethering. CONCLUSIONS: Early surgical repair seems to provide a degree of protection against later spinal cord tethering, and subsequent neurological and/or urodynamic deterioration (25% of our patients versus a reported 80% of those followed expectantly).

Female↗

Pressure at residual volume: a useful adjunct to standard fill cystometry.

PURPOSE: Determining the role of the bladder in incontinence and/or upper urinary tract deterioration involves the cystometrographic evaluation of detrusor compliance. Management decisions based on information provided by standard fill water cystometry assume that data reflect the physiological state of the patient but this is not always true. We report the use of pressure at residual volume as a physiological measurement to aid in the interpretation of data obtained during standard fill urodynamics. MATERIALS AND METHODS: All patients presenting to our urodynamic suite for standard cystometry between June 1994 and December 1995 underwent measurement of bladder pressure at insertion of the urodynamic catheter. Residual volume and pressure measured before draining this urine, that is pressure at residual volume, were recorded. The bladder was then filled at a rate equal to or less than 10% per minute of known or predicted capacity. When a volume equal to the residual volume had been instilled, filling pressure was recorded and the remainder of the standard urodynamic evaluation was completed. Uncooperative patients as well as those with an active infection or residual volume less than 10 ml. were excluded from further analysis. RESULTS: In 152 children pressure at residual volume was compared to filling pressure and the difference was analyzed. Filling pressure was higher than pressure at residual volume in the majority of cases. In 59 children there was a positive change (filling pressure greater than pressure at residual volume) of 5 cm. water or greater and in 13 there was a negative change (pressure at residual volume greater than filling pressure) of 5 cm. water or less. When only cases in which pressure at residual volume was greater than 5 cm. water were analyzed, 46 and 33% had a difference between filling pressure and pressure at residual volume of 5 cm. water or greater and 10 cm. water or greater, respectively. CONCLUSIONS: Pressure at residual volume is physiological, simple to determine and may provide additional information regarding storage characteristics of the bladder. In the context of the clinical history differences between pressure at residual volume and filling pressure may help to interpret standard fill cystometry data.

Adolescent↗

Urinary dysfunction in Duchenne muscular dystrophy.

In Duchenne muscular dystrophy (DMD), sphincter muscles tend to be clinically spared. However, urinary incontinence is occasionally reported, usually late in the course of the disease. We wished to determine the etiology of urinary dysfunction in patients with DMD. Seven boys with DMD and urinary dysfunction were examined by a neurologist and a urologist followed by urodynamic and electrophysiological assessment. Based on the results of these evaluations, patients were defined as having an upper motor neuron (UMN), lower motor neuron (LMN), or myopathic lesion. Five of the patients had UMN abnormalities consisting of either uninhibited contractions or bladder/sphincter dyssynergy. One patient had a LMN lesion with prolonged duration and high-amplitude motor units. No patient demonstrated myopathic motor units. Five boys had undergone spinal fusion for scoliosis. We conclude that urinary incontinence in DMD is most often due to UMN dysfunction and not due to a severe myopathy of the detrusor or external sphincter. The most likely causes of the UMN abnormalities are severe scoliosis or a complication of spinal fusion surgery.

Adolescent↗

Congenital posterior urethral perineal fistulae: a unique form of urethral duplication.

Three unique cases of urethral duplication, often referred to as congenital posterior urethral perineal fistulae, are presented. Presenting signs and symptoms, evaluation, and treatment are discussed, and the literature is reviewed. Although labeled as a fistula in the literature, this entity most likely represents a distinct type of urethral duplication. A functional dorsal urethra separates it from the other forms of urethral duplication.

Abnormalities, Multiple↗

Pediatric neurourology.

Children with voiding disturbances or neurologic dysfunction of the lower urinary tract present challenges to the practicing clinician. With careful clinical and radiologic evaluation, many of these disturbances can be managed. Complex situations, however, generally require expert urodynamic evaluation to delineate the cause of the bladder dysfunction and to guide management. These investigations combined with long-term follow up should provide new insights for further improvement in the management of neurogenic bladders in children. Urodynamic studies also have given us insights into the pathophysiology of abnormal voiding states secondary to dysfunctional bladders in children with posterior urethral valves, the prune belly syndrome, and the Hinman syndrome.

Child↗