William E. Kaplan

Snodgrass hypospadias repair with vascularized dartos flap: the perfect repair for virgin cases of hypospadias?

PURPOSE Since its introduction, the Snodgrass hypospadias repair has been applied to virtually all forms of hypospadias repair. However, fistula rates have still been reported to be as high as 5% from large center, multiple surgeon studies and 16% from smaller center studies. We report on the use of the Snodgrass repair in conjunction with routine use of a vascularized dartos flap and 2-layer closure of the neourethra from multiple institutions. MATERIALS AND METHODS Records of patients who underwent a primary 1-stage hypospadias repair with the Snodgrass technique in conjunction with vascularized dartos flap coverage were reviewed. Nearly identical surgical technique was used by all 6 surgeons in each case, which included a 2-layer closure of the neourethra, preservation of the well vascularized periurethral tissue and routine use of vascularized dartos flap coverage. A total of 514 cases were identified, including 414 with distal and 100 with midshaft or proximal hypospadias. Stents were used in 292 of the 514 repairs. RESULTS Of the 414 distal cases there were no fistulas and 1 case of meatal stenosis. Of the 100 proximal cases there were 3 fistulas and 1 case of meatal stenosis. The overall complication rate was less than 1% for all cases combined. CONCLUSIONS This series represents the largest reported multi-institutional experience with the Snodgrass technique. When used in conjunction with vascularized dartos flap coverage, 2-layer closure of the neourethra and special attention to preservation of the periurethral vascular supply, this repair can be performed with a near 0 complication rate. We believe that this is the optimal repair for routine cases of hypospadias.

ERECTILE DYSFUNCTION IN PATIENTS WITH SPINA BIFIDA IS A TREATABLE CONDITION

PURPOSE Now that individuals with spina bifida live well into adulthood erectile dysfunction has become a recognized associated medical disorder. To our knowledge no study has dealt specifically with treatment of erectile dysfunction in men with spina bifida. Therefore, we conducted a prospective, blinded, randomized, placebo controlled, dose escalation, crossover study to determine the ability to treat erectile dysfunction in men with spina bifida with sildenafil citrate. MATERIALS AND METHODS Erectile dysfunction was diagnosed in 15 men 19 to 35 years old with spina bifida who were assigned to take 4 sets of tablets, 5 tablets per set, in a random order. All patients took 25 and 50 mg. sildenafil and 2 identical looking sets of corresponding placebos 1 hour before planned sexual activity. Efficacy was assessed by the effect of treatment compared to baseline, that is before treatment, on rating of erections (scored from 0 to 10), duration of erections, frequency of erections based on response to question 1 (scored from 0 to 5) of the International Index of Erectile Function and confidence to obtain an erection based on response to question 15 (scored from 1 to 5) of the International Index of Erectile Function. RESULTS Improved erectile function was reported while on sildenafil by 12 (80%) men compared to baseline and placebos. There was a significant dose dependent improvement of erectile function with both 25 and 50 mg. sildenafil compared to baseline (p <0.05), as mean erectile score increased by 50% and 88%, mean duration of erections increased by 192% and 266%, mean frequency of erections increased by 61% and 96%, and mean level of confidence increased by 33% and 63%, respectively. Furthermore, 50 mg. sildenafil provided greater improvement in all 4 parameters compared to 25 mg. The placebo results were not significantly different compared to baseline for any of the parameters. CONCLUSIONS Erectile dysfunction in patients with spina bifida is a medically treatable condition. Sildenafil is effective in this patient population and improves level of sexual confidence.

Does early pyeloplasty really avert loss of renal function? A retrospective review.

We reviewed 75 cases of isolated, unilateral pediatric ureteropelvic junction obstruction and 167 diuretic radionuclide renograms performed during the last 8 years. Differential function of the affected kidney was calculated using scintillation count data at 1 and 3 minutes, and the area under the renogram curve between 1 and 3 minutes. There was no significant difference among the 3 measures of per cent differential function for a given patient. Per cent differential function on the first renogram preoperatively neither declined as age at presentation advanced nor did it vary according to the clinical presentation. Similarly, the changes in postoperative per cent differential function were unaffected by the age at initial presentation, manner of presentation, occurrence of postoperative complications or the surgeon. These data do not support the concept that pyeloplasty for the isolated, unilateral ureteropelvic junction obstruction should be performed early to avert loss of renal function.

The Urological Manifestations of the Tethered Spinal Cord

The tethered spinal cord is an entity with potentially grave neurourological implications. Presenting urological symptoms include incontinence, symptomatic urinary tract infections and stool soiling. These symptoms may be the first to herald a tethered cord. The patients with previously repaired myelodysplastic defects are particularly prone to development of this lesion. We correlated urological history with preoperative and postoperative cystometry in 20 myelodysplastic children. In this study we noted that clinical symptomatology and urodynamic parameters improved in 60 per cent of those children who underwent cord untethering. A better result was evident in children whose condition was repaired promptly.

Complications of Intravesical Oxybutynin Chloride Therapy in the Pediatric Myelomeningocele Population

PURPOSE We report our experience with the intravesical administration of oxybutynin chloride with particular focus on the incidence and characterization of untoward effects and inconvenience of therapy. MATERIALS AND METHODS From 1990 to 1995, 23 children 5 to 11 years old with myelodysplasia were treated with intravesical oxybutynin chloride. Initial dose was 1.25 mg. in 5 cc sterile water instilled during routine catheterization 3 times daily, which was increased as tolerated and clinically indicated. Oral anticholinergic, antispasmodic and sympathomimetic medications were discontinued during therapy. We reviewed therapeutic indications, doses, frequency duration, reason for discontinuation and untoward effects. Patients/parents were surveyed for convenience of treatment as well as side effects and their timing with respect to drug administration and dose. RESULTS In 15 patients (65%) treatment was discontinued and oral formulations were resumed or other therapy was required due to side effects, ineffectiveness or inconvenience. Seven patients had untoward effects, ranging from facial flushing and dizziness to agoraphobia and hyperactivity. Six patients discontinued therapy due to side effects after 1 day to 2 years (mode 1 month) at doses of 1.25 to 5 mg., including 5 who previously had side effects from oral oxybutynin chloride. Inconvenience of therapy was noted irrespective of the degree of independence of the child for performing intravesical therapy. CONCLUSIONS Untoward effects and inconvenience are the most common reasons for discontinuing intravesical oxybutynin chloride therapy for neurogenic bladder dysfunction. Children who previously had side effects from oral oxybutynin chloride are more likely to have them during intravesical therapy.

The Female Urethral Syndrome: External Sphincter Spasm as Etiology

Many women suffer a constellation of urinary and pelvic symptoms commonly referred to as the urethral syndrome. Numerous medical, surgical and psychological treatment modalities have been used to alleviate the symptoms. Urodynamic techniques were used to study a group of women with the urethral syndrome. Based on the findings of external urethral sphincter spasm and/or pelvic floor hyperactivity the institution of diazepam therapy not only has provided clinical relief but also sphincter synergy as demonstrated by post-treaatment urodynamics.

The Endoscopic Correction of Reflux by Polytetrafluoroethylene Injection

Endoscopic subureteral injection of polytetrafluoroethylene (Teflon) was done in 38 patients (55 ureters) to correct vesicoureteral reflux. Followup excretory urograms and nuclear cystograms were available in 28 patients (40 ureters) 2 to 11 months after injection. Nearly 50 per cent of the patients had a neurogenic bladder. Injection was done for all grades of reflux, although the majority of cases had at least grade III reflux. After injection reflux either was eliminated or decreased in 76 per cent of the patients with neurogenic disease and in 87 per cent of those with a normally innervated bladder. This outpatient procedure is simple and brief to perform, and it is associated with minimal morbidity. As with any new antireflux technique longer followup is indicated.

Management of reflux in the myelodysplastic child

More than 75 per cent of the patients with spinal dysrhaphisms followed at our hospital are on intermittent catheterization. A previous report indicated that 62 per cent of 200 children with reflux and a neurogenic bladder either ceased to have reflux or reflux was downgraded while on intermittent catheterization and chronic antibiotics. We report on those children in whom either reflux did not stop while on the program, or who presented with high grades of reflux not amenable to cure by intermittent catheterization. During the last 4 years 25 children (40 ureters) required antireflux surgery. The criterion of repair was persistent reflux of at least grade IIB, associated with recurrent episodes of infection. A modified Leadbetter-Politano technique was used in 5 children (7 ureters) and the Cohen cross-trigonal technique was used in 20 children (33 ureters). A successful result, that is cessation of reflux and no obstruction, was achieved in 96 per cent of the patients. During the last 2 years the Cohen cross-trigonal technique has been used exclusively and there have been no failures. This successful result in 96 per cent of the children with neurogenic bladder indicates that while clean intermittent catheterization should be used primarily to relieve reflux, in a select group of children antireflux surgery should be done and clean intermittent catheterization should be continued.

Age Related Bladder Capacity and Bladder Capacity Growth in Children With Myelomeningocele

PURPOSE Currently the relationship between bladder capacity and age in children with myelomeningocele is inadequately understood, such that nomograms derived from neurologically normal children are inappropriately applied to the myelodysplastic population. The aim of the present study was to evaluate age related bladder capacity and bladder capacity growth in children with myelomeningocele, and compare them to those of age matched, neurologically intact children. MATERIALS AND METHODS Bladder capacity was determined by cystometrography in children with myelodysplasia. Regression analysis was used to derive a relationship between bladder capacity and patient age. Data were stratified by sphincter activity, bladder sensation and uninhibited contractions, and regression analysis was repeated. To evaluate serial growth change in bladder capacity divided by time yielded a yearly bladder capacity growth rate in patients who underwent 2 cystometrograms done at least 3 months apart before age 9 years. RESULTS A total of 506 children satisfied study inclusion criteria. Bladder capacity (BC) was related to age for the first 9 years by the linear equation, BC = 24.5 (age) + 62. This equation is approximately 25% less steep than published age related bladder capacity in neurologically intact children using the formula, 32 (age) + 73. After data stratification bladder capacity in children with sphincter activity, bladder sensation or no uninhibited contractions approached that of neurologically intact children. In contrast, children without sphincter activity or bladder sensation, or with uninhibited contractions had markedly smaller age related bladder capacity. Bladder capacity growth in the subgroup of 55 children in whom 2 cystometrograms were performed at least 3 months apart before age 9 years confirmed a mean gain in capacity of 24 cc per year. CONCLUSIONS Normal bladder capacity in children with myelodysplasia is approximately 25% less than in age matched, neurologically intact children. The bladder grows approximately 24 cc per year until age 9 years. When there is failure to store urine, bladder capacity is much smaller, while children with good storage features may attain the bladder capacity expected of age matched, neurologically intact children.

Intravesical Transurethral Electrotherapy for the Neurogenic Bladder

Intravesical transurethral bladder stimulation is a rehabilitative and diagnostic technique for the neurogenic bladder. The goal of therapy is 3-fold: 1) to achieve the sensation of bladder filling or the urge to void, 2) to initiate a detrusor contraction and 3) to achieve conscious urinary control. The procedure combines direct electrical stimulation of bladder receptors with visual biofeedback using patient observance of a water manometric representation of the detrusor response. Since November 1984 we have evaluated and/or treated 24 patients with a neurogenic bladder. An electrocatheter is passed transurethrally into the bladder and the bladder is filled to a third of its capacity with normal saline. We then deliver 2 to 6 mA. every 3 seconds for 90 minutes. Full treatment usually requires 30 to 90 sessions. Of the 24 patients 13 had only 1 evaluation and 10 had 3 to 39 sessions. Of those 10 patients 7 had significant detrusor activity that had not been present before treatment and 4 have a sensation to void. The remaining child underwent 110 sessions. He has controlled detrusor contractions resulting in a normal voiding pattern. It appears that intravesical transurethral bladder stimulation may be helpful diagnostically to determine the stimulus response of bladder function and therapeutically to rehabilitate the neurogenic bladder toward full control.