Surgical correction of anterior urethral diverticula after hypospadias repair in children.

We present a new surgical technique to correct acquired anterior urethral diverticula after hypospadias repair. The correction involves a degloving circumferential skin incision, excision of the diverticulum, and closure of the urethra incorporating overlapping suture lines.

Use of distal Thiersch-Duplay urethroplasty for proximal hypospadias repairs in conjunction with short island pedicle flap.

Experience with 8 boys having proximal hypospadias with severe chordee and a foreshortened dorsal hooded foreskin is presented. Use of a distal Thiersch-Duplay tube was incorporated in addition to an island pedicle flap to achieve the correct meatal location on the glans. One boy with perineal hypospadias required both proximal and distal Thiersch tube with an island flap interposition. Follow-up of nine months to 3.5 years demonstrated excellent cosmetic and functional results with no recurrent chordee or urethral stenosis. The only fistula noted developed at the proximal Thiersch tube-island flap anastomosis in the boy with perineal hypospadias. Advantages of the aforementioned procedure include decreasing the risk of chordee on the basis of a foreshortened island pedicle flap, use of vascularized flaps, and completing the procedure in one stage with a satisfactory result.

Management considerations for treating vesicoureteral reflux in children with solitary kidneys.

OBJECTIVES: To evaluate the management approach for vesicoureteral reflux (reflux) into a solitary kidney. METHODS: Outcomes of all children with solitary kidneys and reflux managed between 1981 and 1996 were reviewed. Solitary kidneys were documented by nuclear renography and ultrasonography; reflux was graded after cystography. Management consisted of observation and antimicrobial prophylaxis or surgery by ureteroneocystostomy or subureteric injection of polytetrafluoroethylene (STING). Follow-up ranged from 3 months to 14 years and included serial cystography, sonography, and serum creatinine measurement. RESULTS: Twenty-one patients with a median follow-up of 26 months were identified. Etiologies included contralateral renal agenesis (14 children), multicystic dysplastic kidney (5 children), or nonfunctioning ureteropelvic junction obstruction (2 children). Low-grade (I to II) reflux was identified in 6 children, and high grade (III to V) was identified in 15. Reflux resolved in 20 patients. Five children with low-grade reflux were managed without surgery and demonstrated reflux resolution after a mean of 20.5 months. Renal function deteriorated in only 1 child. Ureteroneocystostomy was performed in 13 children with grades III to V reflux, and STING was performed in 1 child with grade II reflux. Every surgical patient maintained stable renal function and was infection-free during a mean follow-up of 56 months. Management by observation in 2 children with grades IV to V reflux resulted in spontaneous resolution in one and stable grade IV in the other. CONCLUSIONS: Reflux into the solitary functioning kidney may be managed by the same strategies used to manage unilateral reflux in children with two normally functioning kidneys: low-grade reflux by observation/ chemoprophylaxis until spontaneous resolution occurs, and higher grades by surgery to protect renal function; however, chemoprophylaxis and serial imaging may be used until well-defined indications for surgery are satisfied. Renal function should be monitored diligently.

Urine levels of transforming growth factor-beta 1 in children with ureteropelvic junction obstruction.

OBJECTIVES: To determine if there are measurable quantities of transforming growth factor-beta 1 (TGF-beta 1) in the urine of children with either normal or pathologic conditions of the urinary tract, specifically vesicoureteral reflux (VUR) and ureteropelvic junction obstruction (UPJO). We also sought to determine if the urine TGF-beta level could distinguish between renal obstruction and no obstruction. METHODS: Preoperative bladder urine from consecutive patients undergoing pyeloplasty (UPJO group; n = 13), ureteral reimplantation (VUR group; n = 11), or circumcision/orchiopexy (control group; n = 19) as well as urine from the renal pelvis of the UPJO group was collected. The urine level of TGF-beta 1 was measured using a quantitative sandwich enzyme immunoassay technique. RESULTS: Urine level of TGF-beta 1 was detected in each group: control (26.6 +/- 6.3 pg/mL), reflux (22.1 +/- 9.6), UPJO-pelvic urine (82.4 +/- 19.3), UPJO-bladder urine (31.2 +/- 8.2). The urine TGF-beta 1 concentration in pelvic urine in the UPJO group was significantly higher than that in bladder urine in children in the UPJO group (p = 0.03). TGF-beta 1 concentrations were similar from the bladder of children in all three study groups (p = NS). CONCLUSIONS: Urine TGF-beta 1 is detectable in children with normal and pathologic urinary tracts. The level of this urine marker is elevated in the renal pelvis of children with UPJO compared to the level in the bladder of either obstructed or nonobstructed upper urinary tracts.

Captopril (an inhibitor of angiotensin converting enzyme) inhibits obstructive changes in the neonatal rabbit bladder.

OBJECTIVES: To investigate whether angiotensin II has a role in the regulation of bladder smooth muscle growth and function, we developed a model of bladder neck obstruction (BNO) in the neonatal rabbit and investigated the effect of captopril (angiotensin converting enzyme inhibitor) on the obstructive changes in the developing bladder. METHODS: Partial BNO was induced in a group of 2-day-old rabbits (n = 8) by placing a loose 2-0 silk ligature around the vesicourethral junction. A second group of rabbits subjected to the identical partial BNO procedure (n = 8) was given captopril (1 mg/kg/day). Twelve days postobstruction, bladders from these animals, along with paired controls (n = 8), were harvested and assayed for total protein, DNA, and collagen content. RESULTS: Partial BNO resulted in a 170% increase in wet weight (P < 0.05), 132% increase in protein/deoxyribonucleic acid (DNA) ratio (P < 0.05), 75% increase in total DNA (P < 0.05), and 115% increase in total collagen (P < 0.05). When compared with obstructed animals, captopril administration significantly inhibited the increase in total DNA (P < 0.05) and reduced the amount of total collagen (P = 0.054). Examination of histology specimens demonstrated that captopril inhibited the serosal hyperplasia and collagen deposition associated with obstruction. CONCLUSIONS: These data demonstrate that captopril partially inhibits the changes in the neonatal rabbit bladder associated with obstruction, supporting the hypothesis that angiotensin II is involved in the regulation of bladder smooth muscle growth and collagen production.

Urinary undiversion in patients with myelodysplasia and neurogenic bladder dysfunction. Report of a workshop.

This workshop was conducted in an attempt to analyze critically the role of reconstruction of the myelodysplastic patient who had undergone urinary diversion and to develop guidelines for selecting those patients in whom urinary undiversion might be undertaken safely. The collective experience initially seems to be acceptable; however, the authors emphasize the gravity of the decision and the complexity of the evaluation which must be undertaken prior to embarking on such reconstructive surgery. Contrary to some reports, we believe that the defunctionalized bladder frequently can be evaluated. Further, many of the contraindications to urinary undiversion have been identified and several of the hazards involved therein can be avoided. We believe that the neurogenic bladder is no longer an absolute contraindication to undiversion. Our experience suggests that undiversion is a reasonable surgical treatment in select patient with neurogenic bladder dysfunction. But, the decision to remove a satisfactorily functioning conduit must not be undertaken lightly. Patients should be selected only after a thorough, detailed, and properly conducted evaluation. A protocol has been developed which will hopefully assist in this evaluation. Perhaps additional shared experience will further refine and delineate the circumstances appropriate for reconstruction of these patients.

Endoscopic injection of polytetrafluoroethylene for treatment of vesicoureteral reflux.

Subureteral injection of polytetrafluoroethylene (Teflon) for correction of vesicoureteral reflux was first utilized in 1981. Concerns about long-term effects remain, but the frequency of good immediate results and the ease of achieving a cure make this endoscopic approach a valuable alternative to open repair.

Management of myelomeningocele.

The care of the patient with myelomeningocele has improved over the last 15 years. Early diagnosis of urodynamic abnormalities and close monitoring of the urinary tract by ultrasonography have allowed the institution of prompt, effective therapy before upper-tract deterioration can occur. The wide acceptance of clean intermittent catheterization can in most circumstances eliminate the need for urinary diversion. Clean intermittent catheterization can alleviate reflux and in 85 per cent of children can promote continence. The adolescent and adult will continue to require observation and management to ensure their active place in society.

Endodermal sinus tumor: a clinical and pathological correlation.

Endodermal sinus tumors occur in the gonads and in a variety of extragonadal locations, particularly as the malignant component of sacral teratomas. Light and electron microscopic studies as well as the presence of alpha fetoprotein support the concept that these lesions arise from germ cells that migrate from the extraembryonic yolk sac. These tumors in all locations are highly malignant and metastasize to lymph nodes, lung, liver and bone. Peritoneal implants are seen from tumors in the ovary, testis, and following ventriculoperitoneal shunts for yolk sac tumors of the pineal gland. There is no satisfactory therapy for the endodermal sinus tumor, regardless of location, when there are distant metastases at the time of diagnosis; however, we have documented complete regression of metastases in one child following therapy with vinblastine, bleomycin, and cis-platinum. Nine of 13 boys with endodermal sinus tumors of the testis survived in this series of patients. All survivors were treated with orchiectomy and lymph node excision. Surgical excision combined with chemotherapy and radiation led to survivors in children with large extragonadal pelvic and retroperitoneal tumors. We recommend vigorous multimodal therapy with surgery and chemotherapy. Radiation is given to the primary lesion when it is too extensive for initial resection or to eradicate residual disease following chemotherapy. Alpha fetoprotein is a valuable tumor marker.

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.

Intravesical bladder stimulation in myelodysplasia.

Intravesical transurethral bladder stimulation has been used as a diagnostic and rehabilitative technique in children with a neurogenic bladder for 3 years. The program has expanded from 10 to 42 patients undergoing a significant number of therapy sessions. The technique presently is done on an outpatient basis. The child is catheterized for 90 minutes and undergoes electrotherapy for 60 minutes, ideally 3 to 5 days per week. An individual series consists of 15 to 30 daily sessions. Presently, 62 patients have been evaluated and 42 have had a least 1 complete series. This ongoing program has provided more data to classify better patients who can expect success with the program. Of the patients who presented initially with detrusor contractions and areflexia 80 and 33 per cent, respectively, can expect to void to completion or have full sensation so as to perform timely clean intermittent catheterization. A total of 21 patients underwent at least 3 series, and 38 per cent void with low pressure and total continence.

Intravesical transurethral bladder stimulation.

Intravesical transurethral bladder stimulation is a rehabilitative and diagnostic technique for the neurogenic bladder. The goal of therapy is threefold: to achieve the sensation of bladder filling or the urge to void, to initiate a detrusor contraction and to achieve conscious urinary control. The procedure combines the direct electrical stimulatory effect to bladder receptors, with the significant use of visual biofeedback by the patient's observation of a water manometric representation of their detrusor response. It appears that intravesical transurethral bladder stimulation may be helpful to determine diagnostically, the stimulus response of bladder function, and therapeutically, to rehabilitate the neurogenic bladder toward full control. It is not intended to replace clean intermittent catheterisation in all patients. However, it may allow selected children to void rather than be catheterised and may be used in conjunction with intermittent catheterisation to improve a child's level of continence.

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.

Treatment of testicular yolk sac carcinoma in the young child.

The majority of testicular tumors in children will be of germ cell origin and the predominant pathologic type is the yolk sac carcinoma. Initial treatment or radical orchiectomy is not in question. However, because of its presumed less virulent character, especially in children less than 2 years old, confusion remains as to adjunctive treatment for yolk sac carcinoma. We have studied retrospectively 21 children with testis tumors encountered within the last 10 years. Of these cases 12 were classified pathologically as yolk sac carcinoma. All children were less than 3 years old and 10 of 12 were less than 2 years old. Of the 12 cases 4 had positive lymph node dissections and 3 of these 4 children were less than 2 years old. Although chemotherapy is so effective its toxicity cannot be minimized. Therefore, adjunctive chemotherapy should be reserved for those patients who have lymphatic spread. Young children with yolk sac tumors are not immune to metastatic disease and should be treated with an extended unilateral lymphadenectomy for staging and appropriate treatment regimens.

The therapy of genital trauma by dog bite.

Genital trauma by dog bite rarely is reported. We present 2 cases of dog bite to the external genitalia involving male infants. Rabies precautions, tetanus prophylaxis and antimicrobial therapy are reviewed. Both children required surgical debridement and split thickness skin grafts for repair. Since pediatric genital trauma may be the result of negligence or even deliberate abuse, a thorough investigation of the child's environment is mandatory in such situations.

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.