Preputial plasty: a good alternative to circumcision.

Since 1991, boys needing surgery for tight nonretractile foreskin have been offered a choice of preputial plasty or circumcision, providing that there is no clinical evidence of preputial scarring. We compared two similar groups of 50 boys that underwent each procedure, through our routine audit and questionnaires sent to their parents. Of the boys with circumcisions, 20% required an overnight stay after the operation; 14% had anesthetic complications, and 6% required reoperation because of bleeding. Only 8% of patients with preputial plasty had an overnight stay, and no bleeding was observed. Parental assessment of both operations showed that morbidity was significantly less and of shorter duration for the preputial plasty group. Two patients in the preputial plasty group (4%) had recurrent narrowing of the foreskin caused by scarring and contraction of the incision. Parents were pleased with the long-term results of both procedures. This simple alternative to circumcision is easy to perform and allows full mobilization of the foreskin, preserving its function and providing an excellent cosmetic result.

Female hypospadias: a case report.

A case of proximal female hypospadias with urethral atresia is reported, and a nosological definition of this rare congenital anomaly is proposed. An attempt to clarify the difference between a urogenital sinus and a proximal or distal female hypospadias is made by examining the embryology of the urogenital tract.

Meconium ileus due to extensive intestinal aganglionosis.

Seven full-term infants with aganglionosis extending into the small bowel presented with clinical, radiological, and operative features of meconium ileus. Misdiagnosis resulted in inappropriate treatment. The correct diagnosis was eventually established by rectal suction biopsy, mostly after either recurrent intestinal obstruction or stomal dysfunction, and after cystic fibrosis had been excluded. For two patients, the results of rectal suction biopsies were initially misleading. Two infants died. Extensive intestinal aganglionosis should be considered a rare possibility in all infants with meconium ileus. In such cases, histological examination of the appendix may avoid this potential pitfall.

[Posterior urethral valve: determining factors of long-term results]. / Valves de l'urètre postérieur: facteurs déterminant les résultats à long terme.

Posterior urethral valves (PUV) is the most common congenital urine flow impairment in boys. Long-term prognosis involves: renal function impaired in 30 to 50% of PUV and leading to hyperdiuresis, low GFR and acidosis; bladder urodynamics impaired in 75% of PUV with abnormal urine storage, abnormal micturition and vesicoureteric reflux. Incontinence and recurrent urinary tract infections commonly reflect bladder and renal failures; abnormal bladder outlet leads to incontinence and abnormal ejaculation. The roles of antenatal treatments (vesico-amniotic shunts), neonatal treatments (resuscitation and endoscopic treatment of PUV) and long-term treatments (urinary diversions, bladder augmentation, alpha blockers, anticholinergic, dialysis and renal transplant) in the long-term outcomes of PUV are reviewed.

Management of urinary incontinence in neurogenic bladder.

The treatment of children with urinary incontinence caused by a neurogenic bladder presents a challenge to the paediatric urologist. Between 1965 and 1989, 527 patients with neurogenic bladder were treated and followed-up in the Department of Paediatric Urology of Debrousse Hospital. Of these, 46 were submitted to continent cystostomy, 39 to urinary artificial sphincter and 177 to clean intermittent catheterization (CIC). This last treatment is of paramount importance in girls and can be associated with complementary procedures if cervico-urethral resistance and/or bladder compliance are poor. Urethral lengthening (Kropp's procedure) in combination with CIC represents a major means of providing dryness in girls. Our technique of urethral lengthening is detailed and the place of CIC among other incontinence treatment is discussed.

The anatomical assessment of disorders of sex development (DSD).

The assessment of abnormal anatomy in cases of DSD is important to aid diagnosis, understand the aetiology and severity of the condition, guide management and assess the outcomes of treatment. In this paper we present a systematic approach to this assessment which will provide a means by which the multiple disciplines who manage patients with these rare and complex conditions can communicate and thus improve overall care.

A study of risk factors for hypospadias in the Rhône-Alpes region (France).

OBJECTIVE: Hypospadias is one of the most common congenital urogenital malformations in males with a significantly increasing incidence over the past 20 years. The causes of this insufficient virilization of the genital tubercle are essentially unknown. SUBJECTS AND METHODS: A hospital-based controlled study was realized with 225 hypospadias cases at Debrousse Hospital, Lyon, using a detailed questionnaire completed during a consultation with the patients' parents and those of controls of the same age. The chi(2), the P-value, the odds ratios and the 95% confidence interval were assessed. RESULTS: Hypospadias was found to be positively associated with genetic factors (as defined by the presence of other case(s) in the family in one case in four) and with neonatal low birth weight, fair-haired boys, maternal history such as viral infection during the first trimester, order of parity, toxaemia of pregnancy, delivery modality such as caesarean section, and environmental pollution. CONCLUSIONS: These results show that aetiological factors of hypospadias are likely to be related to three main fields which interact: genes, the placenta and environmental factors.

Salvage procedures for failed Benchekroun hydraulic valves: experience in four patients.

OBJECTIVE: To describe the complications encountered with the Benchekroun hydraulic valve and the salvage procedures used to convert it into a successful continent diversion. PATIENTS AND METHODS: Seven patients with spina-bifida (two males and five females) presenting with neurogenic incontinence were treated originally with a combined urethral lengthening (Pippi-Salle procedure), detubularized ileocystoplasty and Benchekroun hydraulic valve. Four of these patients presented with immediate (three) or subsequent (one) complications related to the devagination or the overdistension of the hydraulic valve, and stomal problems. Two techniques were used to create a catheterizable conduit with the intestinal material of the Benchekroun hydraulic valve; one used the inner tube of the valve and the other one used the appendix. These techniques are described and illustrated. RESULTS: All four patients made a satisfactory recovery and had no difficulty in catheterizing their bladder through the revised continent diversion. However, the follow-up of these patients was short (1-8 months) and a long-term assessment is required to evaluate these salvage procedures. CONCLUSIONS: The Mitrofanoff procedure remains our first choice to create a continent diversion in children with neuropathic bladders. However, when the appendix is not available or unusable, alternative procedures such as the Benchekroun hydraulic valve might be used to create a continent catheterizable conduit. Nevertheless, complications or failure of the Benchekroun valve and its variants (e.g. Guzman's technique) are common and salvage procedures are often necessary to recreate an efficient continent conduit.

[Bladder exstrophy]. / Extrofia vesical.

OBJECTIVE: To describe the procedures of bladder exstrophy closure, epispadias repair to achieve urinary control. The approaches to the failed exstrophy are also described. METHODS: Bladder closure soon after birth is performed with concomitant pelvic osteotomy. The different techniques are described. The Cantwell-Ransley technique is preferred for epispadias repair and the Young-Dees-Leadbetter procedure for bladder neck reconstruction. RESULTS: A success rate of 92% and a failure rate of 31% have been reported by different authors. CONCLUSION: To date there is no definitive solution in the treatment of bladder exstrophy. Continent urinary diversion may be warranted in some cases.

The use of a multipurpose stent in children.

OBJECTIVES: To assess the use of a multipurpose stent (the 'Blue stent', Angiomed Urosoft Pyeloplasty Stent, Bard, UK) in children undergoing pyeloplasty and ureteric reimplantation. PATIENTS AND METHODS: Between August 1994 and August 1996, the Blue stent was used in 50 renal units in 46 children aged 2 months to 12 years and 6 months. Twenty-five children underwent pyeloplasty, 11 had ureteric reimplantation for vesico-ureteric reflux (VUR), eight had ureteric reimplantation with remodelling for obstructed megaureters and in two patients it was used during the removal of stones. The mean follow-up was 18 months (range 6-30 months). RESULTS: After pyeloplasty, 22 patients (88%) had improved renal function and drainage with a decrease in hydronephrosis; two patients had a decrease in hydronephrosis only, one had an anastomotic leak and needed a repeat pyeloplasty and four developed a urinary tract infection (UTI). After ureteric reimplantation, VUR was not detected in any patient. Two patients had no change in drainage after remodelling and reimplantation of a megaureter, one was later diagnosed as having a neuropathic bladder and one child developed a UTI after ureteric reimplantation. The hospital stay was 3 days after pyeloplasty and 5 days after reimplantation. CONCLUSION: The design of the multipurpose Blue stent provides versatility; it can be used as a stent, and both an internal and external drain. Its use does not prolong the hospital stay. Insertion causes minimal trauma to the renal parenchyma, and removal is easy, pain-free and requires no anaesthesia. The complication rates in the present series compare favourably with other reported series.

Recurrent symptoms of urinary tract infection in eight patients with refluxing ureteric stumps.

OBJECTIVES: To evaluate and discuss the recurrent symptoms presented by eight patients with refluxing ureteric stumps. PATIENTS AND METHODS: Eight patients (three boys, five girls; mean age: 8 years, range 11 months-14 years) with refluxing ureteric stumps presented with recurrent urinary tract infections. Six developed symptoms mimicking pyelonephritis, even though the kidney had been partially [5] or totally [3] removed. The stump was removed in all patients. RESULTS: The stumps that were excised measured between 5 and 10 cm in length and were always dilated and inflamed. The results of histological examination and follow-up are reported. CONCLUSIONS: A dual approach is recommended when complete excision of the kidney and ureter is required. Symptoms of pyelonephritis are not pathognomonic of parenchymal infection but reflect infection above the vesico-ureteric junction. The distinction between supra-junctional and infra-junctional urinary tract infections seems more appropriate and is discussed.

Pathophysiology, diagnosis and management of prenatal upper tract dilatation.

Upper tract dilatation presenting as renal pelvis dilatation with or without ureteric dilatation is one of the more common abnormalities detected on prenatal ultrasound scanning. The majority of cases are due to pelvi-ureteric and vesico-ureteric junction anomalies, and vesico-ureteric reflux. Prenatal diagnosis with ultrasound may be aided by biochemical analysis of fetal blood and urine. The natural history of prenatally detected abnormalities remains under review. Precise diagnosis, and therefore prognosis, may not be made until after birth.

Antenatal and perinatal uro-nephrology: current questions and dilemmas.

The development of antenatal ultrasonography and the detection of fetal uropathies has created a new field for paediatric urologists. The embryology and physiology of the fetal renal tract is still poorly understood. It is, however, recognised that delays in the maturation of the renal system can lead to transient dilatation of the urinary tract. Structural and functional anomalies can also result in dilatation, but these are permanent. The ability to distinguish between the transient and permanent impairments to urine flow represents a diagnostic challenge. This review article discusses the pathophysiology of urine flow impairment, antenatal detection and the postnatal management of the common congenital uropathies.

Urinary continence after reconstruction of classical bladder exstrophy (73 cases)

OBJECTIVE: To evaluate urinary continence in children after reconstruction of classical bladder exstrophy. PATIENTS AND METHODS: Seventy-three children were treated between 1966 and 1990 at Debrousse Hospital in Lyon, France. Reconstruction involved a three-stage repair including bladder closure (with posterior iliac osteotomy), bladder neck reconstruction and penile reconstruction. RESULTS: Of 73 children with bladder exstrophy, seven underwent an initial urinary diversion and 66 a successful bladder closure at birth combined with posterior iliac osteotomy, 55 of whom underwent a bladder neck reconstruction and anti-reflux procedures. The Young-Dees procedure was performed in four cases, combined with the Jeff's technique in two cases and the Mollard technique in 49. Fifty-four of 55 (22 girls and 32 boys) were followed up for between 1 and 17 years. Results were classified as excellent (37 cases), good (11 cases) or failed (six cases), following assessment of continence, voiding capabilities, residual urine volume, urinary tract infection and urinary tract dilatation. Of these patients 69% had normal continence with transurethral voiding (girls, 77%; boys, 63%). Persistent incontinence after bladder neck reconstruction was related to insufficient outlet resistance, an abnormal bladder or a combination of the two. Repeated bladder neck reconstruction (seven patients), bladder augmentation (five patients), bladder neck suspension (one patient) and bladder neck reconstruction combined with augmentation (five patients) were performed. These operations were coupled with a Mitrofanoff procedure if further complications occurred. CONCLUSION: The outcome of bladder neck reconstruction was unpredictable. Achievement of a balance between intravesical pressure and outlet resistance was difficult and often required secondary endourethral procedures. Persistent incontinence after bladder neck reconstruction was related to insufficient outlet resistance, an abnormal bladder or a combination of the two. Repeated bladder neck reconstruction, bladder augmentation, bladder neck suspension and reconstruction combined with augmentation were performed. These operations were coupled with a Mitrofanoff procedure, which offered catheterization as a safe alternative if further complications occurred.

The left Monti-Malone procedure: Preliminary results in seven cases.

BACKGROUND/PURPOSE: The management of children who have congenital intestinal dysfunction and continuous feces soiling has been improved significantly by the Malone continent caecostomy. When the appendix is not available, the Monti tube represents a good alternative to create a catheterizable conduit. The authors report here 7 cases of left continent colonic access (left Monti-Malone). METHODS: From July 1999 to January 2001 7 patients have been operated on in our unit: 6 spina bifida, 1 cloacal exstrophy. Technically, a ring of descending colon of 1.5 to 2 cm width is isolated with its meso, the Monti's tube fashioned, and implanted into the left colon according to the Malone's technique. Antegrade bowel washouts start 3 weeks after surgery with standard saline (200 to 500 mL) +/- phosphate enemas. A period of 1 to 3 months is needed to adjust to the right enema regimen to get a satisfactory result. RESULTS: The follow-up range is 18 months to 1.5 months. One patient is excluded. The duration of the enemas varies between 10 and 30 minutes, and the number of enemas varies from 1 to every 2 or 3 days. Five children are clean and perform their antegrade enemas after dinner to avoid the embarrassment of post enema leakages. CONCLUSION: Although it is a short series with a short follow-up, the first results collected show a significant shortening of the duration of the enema with excellent outcomes in terms of continence.