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The role of bony pelvic anomalies in bladder exstrophy is long established and has generated many papers addressing walking problems. Biomechanical studies and kinematic gait analysis were performed on very young children.A direct kinetic gait evaluation has never been performed, nor has the effect of pelvis dimorphism on the upper body been studied.Controlled experimental study.Outpatients were studied at the time of periodic follow up.Nineteen patients with bladder exstrophy, age 14±8 years, and twenty-five healthy control participants, age 15±8 years, were enrolled in the present gait analysis study.Clinical evaluation and standard gait analysis were performed.Gait analysis deviations between exstrophy patients and controls and between patients that received pelvic osteotomy (OT--6 patients) and those that did not (no-OT--13 patients) were analyzed. Bladder exstrophy significantly affects kinematics and kinetics of trunk, spine, pelvis, knee and foot; in particular: in OT, trunk retroversion, pelvic retroversion and rotation, hip adduction angle and moment, knee flexion and its maximum power during loading response increased, whereas in no-OT, spine angle, pelvic posterior tilt, hip extension, and the external rotation of the foot progression angle increased. All the kinetics parameters analyzed in the study showed lower values in the patient group than in controls.. Walking in patients with bladder exstrophy is accomplished by retroversion of the pelvis and deviations mainly in the spine angle in no-OT and in knee flexion in OT.Gait analysis was shown to be an effective tool for the detection of walking deviations that should be identified early, prompting rehabilitative treatment in order to prevent spine and knee diseases.

– To make a new urinary reservoir which can be catheterised either through an external stoma or transurethrally, is the only way to achieve continence in quite a large number of irreversible structural and functional disorders of the bladder wall, also in pediatric age. Orthotopic are usually preferred to heterotopic pouches in pediatric patients, indications for the latter being few. Bladder exstrophy, neurogenic bladder from spina bifida, severe fibrosis of the bladder walls as a consequence of congenital outlet obstruction in prenatal age, such as posterior urethral valves and bilateral ectopic ureterocele, are the most frequent clinical indications, while bladder tumours are uncommon. Over the last 10 years we have perfomed 31 partial or total bladder substitutions in pediatric patients, 7 of which were continent urinary pouches with 2 in an ectopic position in the iliac fossa (Indiana pouch and Kock pouch). The 7 pouches were made in 4 bladder exstrophies (Indiana Pouch), 1 neurogenic bladder (gastric pouch with continent appendico-vesicostomy), 2 bladder tumours (Kock pouch). Results, in terms of continence and major or minor complications, are presented and discussed with particular reference to pediatric patients.

Objective Different etiopathological mechanisms of enuresis are today under study, and different therapies and drugs have been proposed. The Italian Multicentric Trial was undertaken in twelve pediatric and urological centers in order to assess the efficacy of two of the most popular drugs, desmopressin (DDAVP) and oxybutynin. Methods 114 enuretic patients were enrolled in the study. After a 2-week observation period, 66 patients with primary monosymptomatic enuresis were treated with DDAVP, 30 micrograms/day intranasally, for 6 weeks, 48 patients with enuresis and voiding dysfunction were randomly assigned to a protocol with oxybutynin alone or oxybutynin plus DDAVP. The efficacy of the two drugs was measured in terms of reduction of wet nights per week during the 6-week treatment period and a 2-week follow-up period. Children with 0-3 dry nights/week were considered as nonresponders. Results Patients with monosymptomatic enuresis treated with DDAVP reported a significantly lower number of wet night during treatment than during the baseline period, with 79% showing a 'good' (6-7 dry nights/week) or 'intermediate' response (4-5 dry nights/week). Of the patients with diurnal voiding disturbances and enuresis, those treated with oxybutynin alone had a 54% success rate. The patients treated with both oxybutynin and DDAVP showed a better response, with a 71% rate of success. Conclusions The efficacy of the two drugs is confirmed in patients carefully selected on the clinical basis of voiding disturbances. In patients with enuresis and voiding dysfunction, the reduced urinary output and the lower bladder filling rate due to DDAVP can reduce uninhibited bladder contractions, thus enhancing the oxybutynin action.

K. HJALMAS,*† SWEDEN; T. ARNOLD, NEW ZEALAND; W. BOWER, AUSTRALIA/HONG KONG; P. CAIONE, ITALY; L. M. CHIOZZA, ITALY; A. VON GONTARD, GERMANY; S. W. HAN, S. KOREA; D. A. HUSMAN, USA; A. KAWAUCHI, JAPAN; G. LACKGREN, SWEDEN; H. LOTTMANN, FRANCE; S. MARK, NEW ZEALAND; S. RITTIG, DENMARK; L. ROBSON, USA; J. VANDE WALLE, BELGIUM AND C. K. YEUNG, HONG KONG ON BEHALF OF THE INTERNATIONAL CHILDREN’S CONTINENCE SOCIETY (ICCS)

Vesico-ureteral reflux (VUR) is the most common urological malformation in pediatric age; nevertheless its optimal management remains controversial. Until early 80s, treatment guidelines for VUR recommended the use of antibiotic prophylaxis as initial therapy, with surgical repair for patients with persistent VUR. Endoscopic treatment of VUR has gained popularity and has proved successful in a high percentage of cases, but its role in the clinical practice remains to be established.In this paper, our series of 1029 patients and 1478 refluxing ureters, treated from January 1986 to June 2001, is presented. Reflux ranged from grade II to grade IV. In the first 14 cases Teflon was injected. After 1989 bovine collagen was used in 442 children and, since 1996, Deflux, a nonallergenic, biodegradable dextranomer, in 573 cases. All patients were clinically evaluated for possible voiding dysfunctions. All patients completed a 12 month follow-up period.After 1 or 2 injections, a voiding cystogram showed no VUR (or grade I) in 1123 ureters (76%). In grade II, III and IV success rates were, respectively, 87%, 73% and 48%. Complications were minimal (0.5%).These results confirm that endoscopic treatment of VUR is a valid alternative to "open surgery" and to antibiotic prophylaxis. Failure of treatment is usually due to dislocation of the implanted material, secondary to voiding dysfunction. In conclusion, we recommend the endoscopic treatment in the majority of VUR, for the short hospital stay, the absence of significant complications and the high success rate. In grade IV VUR, the use of endoscopic treatment is still questionable. Patients with voiding dysfunction should be identified and treated with appropriate therapy prior to attempt an endoscopic treatment of VUR.

Authors from Rome evaluated parental preference for treatment in children with grade III VUR. Parents were provided with detailed information about the three treatment options: antibiotic treatment, open surgery, endoscopic treatment. Most parents chose endoscopic management; with this in mind, the authors proposed a new treatment algorithm for VUR.To assess parental preference (acknowledged in treatment guidelines as important when choosing therapy) about treatments for vesico-ureteric reflux (VUR, commonly associated with urinary tract infection and which can cause long-term renal damage if left untreated), as at present there is no definitive treatment for VUR of moderate severity (grade III).The parents of 100 children with grade III reflux (38 boys and 62 girls, mean age 4 years, range 1-15) were provided with detailed information about the three treatment options available for treating VUR (antibiotic prophylaxis, open surgery and endoscopic treatment), including the mode of action, cure rate and possible complications, and the practical advantages and disadvantages. They were then presented with a questionnaire asking them to choose their preferred treatment.Most parents preferred endoscopic treatment (80%), rather than antibiotic prophylaxis (5%) or open surgery (2%); 13% could not decide among the three options and endoscopic treatment was recommended.Given the strong preference for endoscopic treatment we propose a new algorithm for treating VUR; endoscopic treatment would be considered as the first option for persistent VUR, except in severe cases where open surgery would still be recommended.

Fetal SBA (spina bifida aperta) has been operated upon in more than 213 cases in the USA in the last 8 years. Indications and results still remain controversial. Our group reproduced successfully Meuli's model in sheep and reviewed the literature on SBA and the Internet updated sites on the theme. Then we reviewed the follow-up of all cases operated upon postnatally in 20 years at a Neurosurgery Department in Rome. We published all the data regarding the three above-mentioned steps. This paper contains reflections on the three, and our opinion on the correct approach to prenatally diagnosed SBA, as far as fetal surgery is concerned.

To determine whether bladder dysfunction in boys with posterior urethral valves (PUV) changes from a uniform pattern of hypercontractility during infancy to the hypocontractility found in adolescence, by reviewing serial urodynamic studies.Thirty boys with PUV and no voiding symptoms underwent a total of 86 urodynamic tests (mean 2.8 each). The first urodynamic study was undertaken at 1-4 years of age in 15 boys and at 5-13 years in 15. They were re-evaluated at least 3 years later; 15 patients underwent the first and last urodynamic study, respectively, at a mean age of 2.8 and 7.7 years (group A), 10 boys at 6.2 and 8. 8 years (group B) and five at 9.4 and 15.2 years (group C). In 10 boys aged5 years the first and last pressure-flow studies (PFS) were analysed using an advanced analysis (PFA) to better identify hypocontractility.Bladder dysfunction was found in 21 of 30 (70%) boys at the first evaluation and in 18 (60%) at the last. In 25 boys the urodynamic pattern changed. Of the 15 boys in group A, 10 of 12 who had hypercontractility changed to normal (seven), low compliance (one) or hypocontractility (two), and two remained stable; two of the remaining three with normal urodynamic studies changed to hypocontractility, while one was unchanged. Among the 10 boys in group B, six with hypercontractility changed to normal (three) or hypocontractility (three); two with normal urodynamic findings and one with low compliance changed to hypocontractility. Of the five boys in group C who showed severe hypocontractility after puberty, three had a normal pattern, one low compliance and one hypercontractility before puberty. The PFA showed a 'weak' detrusor in four of the seven boys who were considered normal on standard PFS. At the urodynamic follow-up, the PFA pattern changed to a 'weak' detrusor in four boys who had a normal (two) or strong (two) detrusor at the first evaluation.Bladder dysfunction in boys with PUV changes during childhood and through adolescence. The urodynamic pattern of hypercontractility generally found soon after valve ablation gradually changes to hypocontractility in many boys and this pattern seems to be the rule after puberty. The evidence from this series supports the hypothesis that long-term detrusor hyperactivity in boys with PUV leads to detrusor failure, but a longitudinal 15-year follow-up from birth to puberty is needed to validate this concept.

To report our experience of the use of desmopressin to improve nocturnal dryness in patients who have undergone a staged reconstruction of the exstrophy-epispadias complex (EEC), who although continent by day, have nocturnal incontinence because their nocturnal urinary output exceeds their bladder capacity.Seven children (aged 8-12 years) who had undergone a staged reconstruction for EEC (six with classical bladder exstrophy, one with incontinent epispadias) were treated with intranasal desmopressin for persistent nocturnal incontinence despite daytime dryness. Previous additional procedures for continence had been self-augmentation in one and periurethral collagen injection in three others. The criteria for inclusion in the study were: normal renal function, no upper tract deterioration, no urinary tract infections, spontaneous voiding during the day with dry intervals between micturitions, a postvoid residual volume of10% of bladder capacity and night-time incontinence for 7 nights/week. Desmopressin was administered at bedtime at increasing dosages from 10 to 30 microg until effective. Body weight, arterial blood pressure, and serum electrolytes were measured, and all patients assessed using renal ultrasonography, a voiding diary and a nocturnal pad-test.Desmopressin at doses of 10-30 microg was successful in keeping all the patients dry. The nocturnal urinary output was decreased so that it did not exceed bladder capacity. There was only one minor side-effect (nose bleeding).In selected patients with EEC, desmopressin is effective in improving nocturnal dryness, with no significant side-effects.

A case of simultaneous heart and kidney transplantation is presented in a 12 years old girl. The patient suffered from dilated cardiomyopathy and end-stage kidney disease. Bilateral vesico-renal refluxes were corrected at the same time of the duplex transplantation. A multiorgan harvesting from a pediatric 8 years old donor was utilized. Follow-up is 40 months. Cytomegalovirus (CMV) infection and acute tubular necrosis from haemoglobinuria were transient complications. Hemolysis from bypass and extracorporeal circulation was involved as pathogenetic factor of the acute tubular necrosis. Cardiac and renal function remain excellent and the child is fully recovered and resumed a normal and active life. Combined heart and kidney transplantation may be of benefit in selected patients, also in pediatric age.

The gliding testis is a testicle located below the external ring, can be manipulated to the upper scrotum, but is prone to ascend to its original position. Histologic changes can be detected in these gonads by 7 years of age. We evaluated 427 consecutive prepubertal boys referred for cryptorchidism. One hundred and twenty-three had classical undescended, 71 ectopic, 55 retractile, and 178 (mean age 6 years 2 moths) gliding testes. The gliding testes were smaller than controlaterals in 24% of boys. All gliding testes were unilateral whereas bilaterality was 85% in the retractile group (p0.0001), 17.5% in the undescended (p0.001), and 10% in the ectopic group (p0.01). A history for one or more of the following: orchidopexy (3), hormonal treatment (5), late testicular descent (9), spermatic cord torsion (5), testicular pain (10), actual retractile testes (20) or actual gliding testis (58), was present in 93 (52.2%) of the fathers of the gliding group. Forty-seven (81%) paternal gliding testes were hypotrophic. Seventy-five boys with gliding testis underwent initial hormonal therapy with transient benefit, and 57 were operated on. Two anatomical findings are typical of the gliding testis: the absence of the gubernaculum and a processus vaginalis partially patent from the upper scrotum to the mid groin area. This feature explains the mobility of the gliding testis from the external ring to the upper scrotum. The absence of the gubernaculum may be responsible for a higher incidence of spermatic cord torsion in this population. The gliding testis is a distinct entity, representing the mildest degree of a true undescended testis. As hormonal treatment gives only transient results, orchidopexy should be considered before testicular damage occurs.

The 6-year follow-up of a patient affected by Wolfram's syndrome, a rare disease characterized by diabetes insipidus (DI), diabetes mellitus (DM), optic atrophy (OA), neurosensory deafness (D), atony of the urinary tract and other abnormalities (DIDMOAD or Wolfram's syndrome), is described. Our patient has diabetes insipidus, diabetes mellitus, abnormal audiograms, without subjective evidence of hearing loss, and dilatation of the urinary tract. Diagnosis was suspected at the age of 8 years. Diabetes mellitus was the first manifestation and treatment with insulin was necessary. Desmopressin therapy decreased dramatically the daily urinary output. In view of the significant morbidity and mortality from renal failure associated with recurrent urinary infections, we have drawn special attention to the urological manifestations of the syndrome. During the follow-up, the patients underwent some investigations, such as renal ultrasound and echotomography and cystourethroscopy. Outstanding results of these studies are severe bilateral hydronephrosis with dilatated ureters and loss of renal tissue. The particular finding is the presence of posterior urethral valves with obstructed bladder. The anatomical outlet obstruction are variable and may be disastrous. There may be failure to thrive, sepsis, anemia be disanal failure. In such instances corrective surgery could improve bladder and ureteral functions.

The clinico-pathologic association of nodular renal blastema, multicystic kidney and obstructive uropathy has been recently identified. We report on a female patient diagnosed as having unilater multicystic dysplasia by prenatal ultrasonography. The patient was nephrectomized at the age of 6 1/2 months. Examination of the resected kidney revealed multiple unilocular cysts in the cortex and hypoplasia of the homolateral ureter; histological study confirmed the presence of multiple cysts limited to the renal cortex, and revealed, among them, multiple cortical metanephric blastema cells islands. Our case supports a relationship between nodular renal blastema, cortical cysts and obstructive uropathy; ureter hypoplasia could cause intraluminal back pressure, with consequent abnormal development of the ampullae, normally endowed in nephronic anlagens induction, cystic tubular ectasia and persistence of nodular renal blastema. The peripheral location of renal nodular blastema and cysts supports a late error in nephrogenesis, at the time of formation of the last generation of nephrons.

Nowadays enuresis is a problem that pediatric urologists are often called to treat, since it affects 15 to 30% of school-age children. In 85% of affected children bedwetting is monosymptomatic, not accompanied by other voiding disorders or daytime incontinence. Treatment of choice is still highly controversial, as the physiopathology is not yet fully understood and the pathogenesis is multifactorial: genetic and psychological factors, sleep disorders, urinary reservoir abnormalities, urine production disorders can all play a part. Behavioural treatments (psychotherapy, bladder training and biofeedback, electric alarm) and pharmacological therapy (tricyclic antidepressants, anticholinergics, DDAVP) have been used with variable results. In our 1 year experience (54 enuretic children) DDAVP proved to be effective in reducing the number of wet nights per week in 79% of cases. Acupuncture, which we have been using for many years, also gave good results in 55% of treated patients. Long term success of DDAVP and acupuncture was respectively 50 and 40%. We discuss the probable pathophysiology and present our own results and those reported in the literature. It has to be stressed that an accurate diagnostic selection of patients and a better understanding of physiopathology are the basis of effective treatment of enuresis.

A rare case of asymptomatic pancreatic lipoma in an eleven-month-old child is presented. The abdominal mass was discovered occasionally during a grip episode. Preoperative tests proved the presence of a neoformation arising from the pancreas. It was multilobed, capsulated and located between colon transversus, spleen and stomach without infiltration of surrounding structures. The histological picture was that of a mature lipoma. The authors discuss the differential diagnosis versus other fat tumors.

During the period from March to September 1989, 40 children suffering from primary nocturnal enuresis, aged between 5 and 14 years, were included in a study to assess the comparative therapeutical efficacy of DDAVP and acupuncture. Children were divided into four groups of 10: group A was treated with DDAVP, group B was treated with acupuncture, group C was treated with DDAVP and acupuncture and group D was treated with placebo (control). The trial design included 3 periods: observation (2 weeks), treatment (8 weeks) and follow-up (4 weeks). Nineteen children completed the study. The efficacy of treatment, which was expressed as a percentage of dry nights, was high in both the DDAVP and acupuncture groups, when used separately. The combined treatment of DDAVP and acupuncture appeared to be the most efficacious both in terms of the percentage of dry nights at the end of treatment and in relation to the stability of results, even after the end of the study. The paper gives a detailed analysis of correlations between type of treatment and urinary osmolarity.

Post traumatic leptomeningeal cysts (P.T.L.C.) are capsulated liquor collections in the sub-arachnoidal space consequent to skull fractures. They occur typically in infancy and their deceitful onset needs an accurate X-ray follow-up in children with a history of cranial trauma. The authors present six cases of P.T.L.C. encountered in the last three years, recall the mechanism of formation, and stress the importance of standard radiological examination, and computed tomography for early diagnosis.

Urinary incontinence can only be considered a disease after a child has completely acquired spinal mechanisms of vesico-sphincteric nervous control. This normally happens for the majority of children between two and two-and-a-half years of age regarding voluntary daytime control, while complete night-time control is obtained in 90% of cases within the fourth year. By urinary incontinence we mean an involuntary leak of urine through the bladder neck. This can vary from an occasional leakage consisting of a few drops to a continuous and uncontrollable stream of urine. From 1981 to 1986 we evaluated, in the Pediatric Urological Service at Bambino Gesù Hospital, Rome, 426 children suffering from urinary incontinence. According to the etiology we divided the patients into two large groups: 1) patients with incontinence due to anatomic causes 56 (13%); 2) patients with incontinence due to dysfunctional causes 370 (87%).

The presentation of a rare case of leiomyoma of Meckel's diverticulum in a boy of 5 yr forms the starting point for a discussion of such cancers. In adult age, the complication is not so rare as the sporadic publications would suggest, incidence of tumoral forms being around 1 : 50 M.D. with a high malignancy frequency. In paediatric age, on the other hand, the tumoral degeneration of a M.D. is an exceptional occurrence: a review of more than 1,200 reported cases and personal experience (48 cases of M.D. in 15 yrs) confirm the rarity of the observation. The need to seek M.D. on each occasion and remove it to prevent the risk of tumoral degeneration in adult age is stressed. M.D. surgical removal tecniques are commented on and emphasis laid on the desirability of ample intestinal resection in cases of cancer of the diverticulum.