Long-term urological outcome of cloaca patients with multidisciplinary management.

PURPOSE: Urological management of Cloacal Malformation (CM) focuses on preserving renal function and continence. Study aim was to analyze urinary and intestinal outcomes in CM patients, considering the length of common channel (CC) and presence of occult spinal dysraphism (OSD). METHODS: Retrospective review of CM treated at our institution by a multidisciplinary team from 1999 to 2020. Patients with follow-up < 2.5 years were excluded. Length of CC, renal function, urinary and bowel outcomes, presence of associated anomalies (especially OSD) were evaluated. RESULTS: Twenty patients were included, median age at follow-up: 8 years (4-15). A long CC > 3 cm was described in 11 (55%). Chronic kidney disease was found in 3 patients. Urinary continence was achieved in 8/20 patients, dryness (with intermittent catheterization) in 9/20. Fecal continence was obtained in 3/20, cleanliness in 14 (under bowel regimen). OSD was present in 10 patients (higher prevalence in long-CC, 73%). Among OSD, 1 patient reached fecal continence, 7 were clean; 2 achieved urinary continence, while 6 were dry. CONCLUSIONS: Length of CC and OSD may affect urinary and fecal continence. An early counseling can improve outcome at long-term follow-up. Multidisciplinary management with patient centralization in high grade institutions is recommended to achieve better results.

Asynchronous Bilateral Ovarian Torsion: Three Cases, Three Lessons.

BACKGROUND: Ovarian torsion (OT) is a serious condition, and delay in surgical intervention may result in loss of the ovary. Children and adolescents who have suffered from ovarian torsion may be at risk for asynchronous torsion of the contralateral ovary. STUDY OBJECTIVE: Three cases of asynchronous bilateral ovarian torsion were reported to analyse clinical history of three patients, to review the current literature, and to draw a conclusion for future treatment. DESIGN: Case reports and review of the literature. RESULT: When a prepubertal girl presents with an ovarian torsion, several considerations have to be taken in account in order to preserve her future fertility; in particular, the pediatric surgeon/gynecologist has to preserve as much as possible the twisted ovary in addition to considering the fate of the contralateral ovary. SUMMARY AND CONCLUSIONS: Pelvic pain in a young girl has always raised the clinical suspect of an ovarian torsion; the possibility of asynchronous bilateral ovarian torsion is rare, but it is described in the literature and has catastrophic consequences; this condition has to be known and treated in the proper way by pediatric surgeons as well as by gynecologists in order to maximize the future fertility of the young patients.

Epidydimo-orchitis and anorectal malformations: when and in whom?

PURPOSE: Epididymo-orchitis (EO) is infrequently reported in anorectal malformation (ARM) cases. Therefore, it is difficult to assess its risk factors. METHODS: A total of 110 male patients who were operated on for ARM at the same Institution over a period of 13 years were contacted. Association was assessed between EO and the following: spinal dysraphism (SD), symptomatic VUR (VUR), and bowel management (BM) requiring enemas. The data were analyzed with the Chi-square test. RESULTS: A total of 89 patients were contacted. Ten cases of EO were found, and all occurred in patients with recto-urethral (RU) fistula after reconstruction. The patients' age at first episode ranged between 4 and 11 years. RU fistula patients experiencing EO (Group A, 10 patients) were compared with those without EO (Group B, 33 patients). VUR occurred in 9/10 cases in Group A and in 13/33 cases in group B (Chi-square 7.8658, p = 0.005038). SD was present in 4/10 cases in group A and in 13/33 cases in Group B (Chi-square 0.0434, p = 0.83491). A total of 8/10 cases in Group A and 12/33 cases in Group B were on BM (Chi-square 5.87, p = 0.0015). CONCLUSIONS: EO occurs in approximately in 20 % of male cases with ARM, and recto-urinary communication and should be considered the primary diagnosis in the presence of testicular pain. This could avoid unnecessary surgical exploration, and the family should be counseled about this subject.

Gait analysis in bladder exstrophy patients with and without pelvic osteotomy: a controlled experimental study.

BACKGROUND: 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. AIM: A direct kinetic gait evaluation has never been performed, nor has the effect of pelvis dimorphism on the upper body been studied. DESIGN: Controlled experimental study. SETTING: Outpatients were studied at the time of periodic follow up. POPULATION: 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. METHODS: Clinical evaluation and standard gait analysis were performed. RESULTS: 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. CONCLUSION: . 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. CLINICAL REHABILITATION IMPACT: 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.

A 20-year study of persistence of lower urinary tract symptoms and urinary incontinence in young women treated in childhood.

OBJECTIVE: To determine whether urinary incontinence (UI) and lower urinary tract symptoms (LUTS) persist over years, patients treated for UI and LUTS in childhood were re-evaluated in adulthood. MATERIALS AND METHODS: Forty-seven women (cases) treated in childhood for daytime UI/LUTS (group A) and nocturnal enuresis (group B) self-completed (average age: 24.89 ± 3.5 years) the International Consultation on Incontinence Questionnaire for Female with LUTS (ICIQ-FLUTS). ICIQ-FLUTS was self-administered to 111 healthy women (average age: 23 ± 5.1 years) from a nursing school as a control group. Data obtained from ICIQ-FLUTS and quality of life (QoL) score (0-10) were compared (Fisher's exact test) between patients and controls, and between group A (n = 28) and group B (n = 19). RESULTS: Prevalence of LUTS was higher in patients than in controls. The difference between patients and controls was statistically significant (p = 0.0001) for UI (34% vs. 7%) and feeling of incomplete bladder emptying (49% vs. 28%). QoL score was >5 in 59% of patients and 1% of controls (p = 0.0001). No significant differences were found between groups A and B. CONCLUSIONS: UI and LUTS are confirmed in young women who suffered for the same condition in childhood. Longitudinal studies are needed to assess if these symptoms persist or are newly onset.

Isolated fetal ascites secondary to persistent urogenital sinus.

Objective. To present a case of isolated ascites secondary due to urogenital abnormalities (urogenital sinus) without any other prenatal ultrasound marker. Method. A 36-year-old woman with prenatal isolated ascites delivered a female baby, weighing 2.285 g; ascites was drained at birth and the baby underwent several episodes of urinary retention prior to undergoing X-ray investigations. Results. A voiding cystourethrogram revealed a short urogenital sinus: a vesicostomy was performed. A vaginoscopy revealed double vagina with a large posterior vagina. A posterior sagittal anorectal pull-through with genitoplasty was performed at 2 years old with 1-year follow-up. Conclusions. Though rare, a urogenital abnormality is to be suspected in fetal ascites cases with negative viral tests and no cardiac anomalies. The most common ultrasound marker of such abnormalities (fluid filled cavity) may be missing because of complete drainage of urine through the tubes into peritoneum.

Acute oligohydramnios: antenatal expression of VURD syndrome?

OBJECTIVE: Oligohydramnios (OA) is nowadays regarded as one of the best markers of renal function (RF) impairment in bladder outlet obstruction (BOO) detected in utero. As such, its onset is usually early and progressive because of decline in fetal urine production. A series of acute OA complicating pregnancies with BOO has never been reported. METHODS: Over a 7-year period, 5 fetuses with in utero suspicion of BOO exhibited an abrupt decrease of amniotic fluid after the 30th week of gestation. RESULTS: All fetuses were delivered by cesarean section: diagnosis was posterior urethral valves in 3 cases, urethral atresia in 1, and prune-belly syndrome in 1. Urologic work-up demonstrated a unilateral vesicoureteral reflux dysplasia (VURD syndrome) in all 5 fetuses. RF at 1 year was normal in 4 fetuses and impaired in 1. CONCLUSIONS: Besides obstetrical reasons, OA may also have acute onset occurring in the presence of anomalies of the urinary tract; although diagnosis is almost always BOO, functional and anatomical characteristics of the urinary tract are those of VURD syndrome with a non-functioning, refluxing renal unit. The associated acute OA/VURD syndrome may represent a milder expression of a pop-off mechanism advocated in this syndrome with a more favorable prognosis than progressive OA detected early in pregnancy.

Pediatric spinal cord injury: approach for urological rehabilitation and treatment.

PURPOSE: As proposed in this report, early urological rehabilitative management of patients with spinal cord injuries (SCIs) is mandatory, in order to prevent a poorly compliant bladder with related upper urinary tract complications and secondary renal failure. Moreover, the approach to treating this traumatic condition in children must be as much rapid as appropriate. MATERIALS AND METHODS: We evaluated our experience of the last 5 years with 17 patients (12 males and five females), with a mean age of 9.6 years at injury (range 6 months-18 years), all affected by an SCI and with direct trauma involved in more than 50%. Mechanism of injury, lesional level, the mean interval between injury and bladder management onset, and the mean interval between bladder management onset and our last control were evaluated. A standardized diagnostic approach was instituted, and all patients received at least a video-urodynamic evaluation before and after the start of urological management. A continence score was established and evaluated before and at least 6 months after the application of rehabilitation treatments (catheterization, medication). Follow-up ranged from 12 to 60 months (average 29.6 months). RESULTS: Sixteen of the 17 patients showed, at first urodynamic evaluation, a neurogenic overactive bladder. Mean bladder maximum capacity was 287.7 ml+/-146.4 SD. Mean reflex volume and end filling pressure were 119.7 ml+/-76.4 SD and 44.6 cmH(2)O+/-25 SD, respectively. Detrusor sphincter dyssynergia was present in 16 out of 17 of the cases. All patients but one began self-catheterization and medication (anticholinergics). Urinary continence improved in all patients but one. An adjunctive endoscopic procedure for continence was carried out in five out of 17 cases. The upper urinary tract was involved in two out of 17 cases. CONCLUSIONS: A prompt and standardized urological approach to pediatric SCIs is mandatory. The aims of this initial management are the prevention of further secondary damage to the upper tract and the achievement of a socially acceptable degree of urinary continence as soon as possible after the traumatic event.

Percutaneous tibial nerve neuromodulation is well tolerated in children and effective for treating refractory vesical dysfunction.

PURPOSE: We evaluated pain tolerability and the preliminary results of percutaneous tibial nerve stimulation (PTNS) in children with unresponsive lower urinary tract symptoms (LUTS). MATERIALS AND METHODS: A total of 23 children 4 to 17 years old with LUTS refractory to conventional treatment underwent PTNS at 12, 30-minute weekly sessions. Ten patients had idiopathic overactive bladder, 7 were in nonneurogenic urinary retention and 6 had neuropathic bladder. Ten children were carefully evaluated for pain during needle insertion and electrical stimulation using certain scoring systems, namely the faces pain rating scale, Children's Hospital of Eastern Ontario pain scale, visual analogue scale and Questionario Italiano del Dolore. Evaluation was done at the first, sixth and last sessions. An anxiety-depression test was administered. All 23 children underwent clinical and urodynamic evaluation before and after treatment. RESULTS: All except 1 patient completed treatment. An anxious-depressive trait was found in 7 of 10 children/parents on anxiety-depression testing. Regarding pain, the faces pain rating scale never showed the severe pain face, the Children's Hospital of Eastern Ontario scale showed signs of pain at the beginning of each stimulation but not at the end, and the visual analog scale generally showed a low score with a further decrease during the first (p = 0.05), sixth (p = 0.03) and twelfth (p = 0.02) sessions. The Questionario Italiano del Dolore score was significantly related to the affective component of pain (p = 0.002) and it decreased between the first and last sessions. The 10 children with overactive bladder had symptom improvement in 80%, incontinence was cured in 5 of 9 and urodynamics showed normalization of cystometric bladder capacity in 62.5% with no more unstable contractions in those who became continent. Symptoms improved in 71% of the children in urinary retention. One of 3 and 4 of 7 patients had incontinence and post-void residual urine cured, respectively. Urodynamics showed an improved detrusor pressure at maximum flow (p = 0.009) and flow rate (p = 0.005). Symptoms and urodynamics did not significantly change in the neuropathic bladder group. CONCLUSIONS: PTNS is safe, minimally painful and feasible in children. It seems helpful for treating refractive nonneurogenic LUTS.

Impalpable testis and laparoscopy: when the gonad is not visualized.

The diagnostic accuracy of laparoscopy for impalpable testis is well recognized. However, in some cases, laparoscopic findings may be misleading, and a viable gonad may be missed with significant medico-legal implications. From January 1993 to December 2000, 202 patients with 219 impalpable testes were evaluated. In 95 cases, the gonad was immediately visualized, and in 5, the presence of a testis was documented by inserting the scope into the processus vaginalis. In the 119 remaining cases, no gonad was seen while entering the abdomen with the laparoscope. All patients with documented vas and vessels exiting the inguinal ring were surgically explored. Ten testes were found, 8 ectopic, with significant changes in shape and position, and 2 were canalicular. In the absence of hormone stimulation, no testes were found while exploring patients with cord structures coursing a closed inguinal ring and with contralateral hypertrophy. In 1 patient with absent vas and vessels, the testis was found at the lower renal pole while removing a dysplastic kidney. Despite technical refinements and an increase in clinical practice, a small percentage of viable testes may be missed with laparoscopic findings consistent with absent/vanished inguinal testis. Therefore, inguinal exploration is mandatory in all these cases.

Neurovesical dysfunction in children after treating pelvic neoplasms.

OBJECTIVE: To evaluate 10 years of experience, and thus define the occurrence and causes, of neurogenic lower urinary tract dysfunction in children with pelvic neoplasms treated by surgery. PATIENTS AND METHODS: From 1991 to 2000, 33 children were operated by the same surgeons for pelvic neoplasms; 11 were analysed, comprising four each with sacrococcygeal teratoma (ST) and ganglioneuroma, and one each with yolk sac tumour (YST), neuroblastoma and myofibroblastic bladder sarcoma (MBS). The other patients were not assessed because eight had died or were in severe progression, three were treated by bladder substitution and the others were lost to follow-up or refused a urological evaluation. All 11 children were evaluated at >/= 6 months after surgery with a questionnaire about bowel and voiding habits, a neurological and orthopaedic assessment, a noninvasive urodynamic study, renal ultrasonography and spinal and pelvic magnetic resonance imaging (MRI). All patients with signs of bladder dysfunction were evaluated by a pressure-flow study. The results were analysed for surgical approach and anatomical involvement, i.e. group A, extensive surgery for complete tumour excision in the sacral area (ST and YST); group B, surgery for tumour resection in the paraspinal ganglia area (neuroblastoma and ganglioneuroma); and group C, bladder tumour with partial bladder resection (MBS). RESULTS: Eight patients had signs or symptoms related to bladder sphincter dysfunction. One child refused the invasive urodynamic evaluation, leaving seven for analysis (two each ST and ganglioneuroma, one each YST, neuroblastoma and MBS). The urodynamic findings were normal in three children. On spinal and pelvic MRI a presacral lipoma with syringomyelia was discovered in one child with ST. Eight children had bladder dysfunction and two had no neurogenic damage (which was only in sacral tumours); in one child it was related to an upper motor neurone lesion from spinal dysraphism and in the other to a lower motor neurone lesion from surgical injury to the splanchnic nerves. Patients operated for paraspinal tumours had more bladder dysfunction but no signs of neurogenic damage, as did the patient with partial bladder resection. However in Group B, there may have been a transient or incomplete nerve injury in one patient. CONCLUSIONS: Deficits of parasympathetic, sympathetic and somatic innervation of the bladder and the urethra may occur in children after surgery for pelvic neoplasms, related to minor or major surgical trauma. In ST, a tethered cord may be associated with mixed neurogenic damage. Knowledge of bladder dysfunction in anorectal malformations, spinal dysraphism, etc. and the clinical protocol used in these patients also seemed to be useful for understanding the development of voiding dysfunction in patients with neoplasm.

Skin graft for 2-stage treatment of severe hypospadias: back to the future?

PURPOSE: Despite the introduction of more refined surgical techniques, the optimal treatment of the most severe forms of hypospadias remains to be determined. Single stage procedures, whether with the use of flaps or grafts, have long been regarded as the best approach, although the complication rate is nonnegligible with all procedures. MATERIALS AND METHODS: We report the use of a 2-stage repair with preputial graft interposition and subsequent tubularization of the urethral plate applied it to all severe cases of hypospadias with significant chordee or small glans. RESULTS: Both stages of the procedure were completed in 34 patients. Complications in 8 cases (23.5%) included 4 glans disruption in 4, coronal grove fistula in 2, urethral diverticulum in 1 and urethral stenosis due to balanitis xerotica obliterans in 1. Two pinhole fistulas also occurred which closed spontaneously. No complete disruptions or postoperative hematomas/bleeding was noted. Cosmetic and functional outcome after a minimum followup of 1 month was optimal in all cases with a normally located "slit" meatus and straight penile shaft. CONCLUSIONS: Although the controversy between use of grafts and flaps will probably continue forever, we believe that our 2-stage approach should be considered as a valid alternative for the most severe forms of hypospadias. Long-term results appear to outnumber the necessity of a learning curve for appropriate graft manipulation.

Adolescent varicocele: Tauber antegrade sclerotherapy versus Palomo repair.

PURPOSE: There is general agreement on treatment for varicocele in pediatric patients. Randomized prospective studies have shown that anatomical and functional lesions may be corrected. Due to the impossibility of seminal examination patients with moderate to large varicocele or ipsilateral testicular hypertrophy, characterized by a change in testicular consistency or symptoms, should undergo surgical correction. The best therapeutic approach is still under discussion. MATERIALS AND METHODS: At 2 centers 2 therapeutic approaches to varicocele treatment in pediatric patients were compared, namely the Palomo repair and antegrade sclerotherapy according to Tauber. The 89 patients from the same geographical area elected 1 procedure after an explanation. From the medical records we retrospectively evaluated operative time, postoperative analgesics, postoperative fever onset, complications, convalescence, recurrence and postoperative hydrocele. RESULTS: After Palomo repair in 45 patients there were 2 recurrences (4.4%) and 2 postoperative hydroceles (4.4%). Of 44 antegrade sclerotherapy cases 1 was converted to Palomo repair, there was no hydrocele formation and recurrence developed in 2 (4.5%). Testicular atrophy was not observed in any patient regardless of the method used. The cost of the procedure was lower in the sclerotherapy group. CONCLUSIONS: These data suggest that the failure rate was similar in both groups. The principal advantages of sclerotherapy are simplicity, decreased cost and lack of hydrocele formation.

Detrusor hypocontractility evolution in boys with posterior urethral valves detected by pressure flow analysis.

PURPOSE: We evaluated the natural evolution of detrusor voiding contractility in boys who underwent posterior urethral valve ablation using pressure flow analysis, which is a mathematical computerized analysis of pressure flow studies. MATERIALS AND METHODS: Among 30 boys with posterior urethral valves who were being prospectively followed, even if asymptomatic on serial pressure flow studies, 11 were included in our study. These 11 patients had had at least 2 evaluations performed between ages 5 and 15 years, a minimum interval of 4 years between the first and last examination, and all pressure flow studies records available for mathematical analysis of voiding contractility. The first examination had been done at ages 5 to 10 years (average 7 +/- 2.04) and the last one at ages 9 to 15 (12.5 +/- 2.5), including 6 evaluated after puberty. All but 1 patient underwent valve endoscopic resection as a newborn and none received urinary diversion. Voiding symptoms, post-void residual, cystometric bladder capacity and bladder instability were considered. Voiding phase maximal detrusor pressure and flow rate were evaluated and detrusor contractility was calculated by the pressure flow analysis parameters of contraction velocity, detrusor contractile power expressed as watt factor and Schafer's nomogram. Contraction velocity and contractile power factor were considered low if below 2 standard deviations of previously determined normal values. True hypocontractility was diagnosed when at least 2 pressure flow analysis parameters were low. RESULTS: True hypocontractility was detected in 3 of the 11 boys at the first examination and in 8 at the last pressure flow analysis. The remaining 8 and 3 cases of first and last examinations, respectively, were considered to have normal contractility even if 4 of the 8 and 1 of the 3 had 1 low pressure flow analysis parameter (covert hypocontractility). Detrusor contractility worsened in 6 patients, hypocontractility was detected at the first pressure flow analysis in 2, hypocontractility changed to normal in 1 and pressure flow analysis remained normal in 2. Of the 6 boys followed through puberty 5 had hypocontractility, including 3 with cystometric bladder capacity greater than 700 ml., high post-void residual and strained voiding. Of the 11 patients 8 had detrusor instability, including 7 with urge symptoms, at first evaluation which was not found at last examination. CONCLUSIONS: Pressure flow analysis extensively used in men has been confirmed as a useful tool to assess voiding contractility in children. The majority of boys with posterior urethral valves have progressive impairment of detrusor contractility at voiding many years after relief of obstruction. The pattern of hypocontractility, which is detected early on pressure flow analysis, follows a prolonged phase of instability in many cases and leads to an over distended bladder in most patients followed after puberty. Questions arise if this evolution may be prevented by early (pharmacological or rehabilitative) treatment and if it is partially determined by extensive use of drugs acting against unstable detrusor contractions.

Prevalence of hypercontractility in male and female infants with vesico-ureteral reflux.

PURPOSE: To establish whether infants with vesico-ureteral reflux (VUR) have bladder dysfunction, with difference in gender, age at presentation and severity. PATIENTS AND METHODS: 37 infants (24 male and 13 female) aged 2 to 24 months with II degrees to V degrees degree VUR underwent cystometry. Of those, 10 underwent natural filling cystometry. We considered: instability and maximal voiding detrusor pressure (VDP) to be "high" when it exceeded 90 cm H2O. We defined hypercontractility as high VDP and/or instability. RESULTS: The prevalence of hypercontractility was 75% (18/24) in male and 46% (6/13) in female infants (p<0.004). High VDP was found in 50% (12/24) of male and 7% (1/13) of female patients (p < 0.001); no significant difference was found between male (25%) and female ones (38%) with instability alone. The mean VDP was significantly higher in male than in female infants (p < 0.001), in patients < 1 year of age than in older ones (p<0.001) and in severe than in moderate reflux (p<0.006). The mean voiding detrusor pressure of male infants was higher in severe (108+/-46cm H2O) and bilateral (101.3+/-44cm H2O) than in moderate (76+/-24 cm H2O) and unilateral (73.7+/-24 cm H2O) and in infants < 1 year of age (101.7+/-42 cm H2O) than in older ones (70.2+/-21 cm H2O). Natural filling cystometry confirmed the results of standard urodynamic studies. CONCLUSIONS: Bladder dysfunction is confirmed also in infants with reflux, particularly in male younger patients, and it differs in gender. The pathogenesis of congenital reflux is not always a feature of malformation of the vesico-ureteral junction; therefore, patients with bladder dysfunction must be identified early.

The changing urodynamic pattern from infancy to adolescence in boys with posterior urethral valves.

OBJECTIVE: 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. PATIENTS AND METHODS: 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 aged > 5 years the first and last pressure-flow studies (PFS) were analysed using an advanced analysis (PFA) to better identify hypocontractility. RESULTS: 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. CONCLUSIONS: 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.

N-acetyl-glucosaminidase (NAG) excretion in partially obstructed weanling rats.

The understanding of pathophysiology of obstructed uropathy has been facilitated by animal models with partial ureteric obstruction. Some studies on partially obstructed adult rats have drawn attention to a biphasic pattern of obstructive uropathy: an initial 'destructive' phase and a 'steady' phase in which renal deterioration no longer occurs and in which relief of obstruction would be of no advantage. We aimed to verify if this pattern applies also to younger (weanling) rats with more immature kidneys, resembling those of the human fetus. We measured the NAG-values in the urine samples of partially obstructed animals at different intervals of obstruction and in those of controls. The biphasic pattern proved to be the same as in adult rats as was previously documented, but the turning point occurred earlier (between 10 and 15 days of obstruction). Furthermore, there is evidence of low level values of N-Acetyl-Glucosaminidase (NAG) in the early phase of obstruction (5 days), demonstrating that the increase of tubular enzyme is not due to the operation itself. There is evidence that, if the 'destructive' phase can be precisely identified by biochemical studies, this could help identifying those subjects who could benefit from relief of obstruction.

Endoscopic treatment for urinary incontinence in children with a congenital neuropathic bladder.

OBJECTIVE: To verify, in a retrospective analysis, the effectiveness of endoscopic treatment (collagen injection) in children with neurogenic bladder and neurogenic urinary incontinence. PATIENTS AND METHODS: From January 1992 to March 1997, 36 endoscopic collagen injections were performed to treat neurogenic urinary incontinence in 23 patients (mean age 10.9 years, range 6-17 at the time of the first procedure) selected on the basis of clinical status and the patient's motivation. Nineteen patients were affected by myelomeningocele and four had an occult spinal dysraphism. All patients underwent a preoperative cystometric urodynamic evaluation (without urethral pressure measurement) which showed detrusor areflexia in 12, normal reflexia in one and hyper-reflexia in 10 patients. Bladder compliance was considered good (> or =20 cmH2O) in 13 patients and low (10-20 cmH2O) in 10, four of whom had detrusor areflexia. Twenty-one children emptied their bladder by intermittent clean catheterization. The mean (range) follow-up was 19.2 (6-54) months. Twenty (group A) and 16 (group B) procedures, performed early and late in the series, were analysed separately to determine any increase in effectiveness with surgical experience. The evaluation criteria were; the increase in the 'dry' interval (between catheterizations) before and after treatment/s (delta dry); the patient's and parent's satisfaction; the number of endoscopic procedures and quantity of injected materials. RESULTS: There was an improvement (dryness of at least 2 h: delta dry of 1 h) in 13 of 23 patients after 1-3 (mean 1.5) procedures; 10 patients had a 0.2 h increase in delta dry after 1-3 injections and none was satisfied. There was no difference in the delta dry (0.9 vs. 0.7 h) between groups A and B, and/or the patient's/parent's satisfaction. Success rates differed with urodynamic patterns; seven of 10 patients showing no improvement had hyper-reflexia and three of the remaining 12 with areflexia had hypocompliant bladders, while nine of the 13 showing improvement had an areflexic detrusor and low-pressure bladders. CONCLUSION: The efficacy of the treatment depends largely on the urodynamic selection of patients (mainly those with detrusor areflexia and good compliance). The outcome may be improved further by increasing the quantity of injected material and with a longer follow-up, performing the procedure again if advisable. A close postoperative clinical (pad test) and personal observation is necessary to evaluate the outcome and thus optimize the strategy of treatment.

Detrusor hypocontractility in children with posterior urethral valves arises before puberty.

OBJECTIVES: To assess prepubertal boys with posterior urethral valves (PUV) using an analysis of pressure-flow studies to evaluate the voiding phase and thus determine if myogenic failure (hypocontractility) arises before puberty and if it can be detected early. PATIENTS AND METHODS: Eleven boys (8-13 years old) with PUV underwent urodynamics and the results were analysed using pressure-flow mathematical analysis (PFA) of the following variables of detrusor activity: contraction velocity (Vdet), detrusor contractile power expressed as power factor (WF) and Schafer's diagram, which differentiates a 'strong', 'normal' and 'weak' detrusor. Vdet and WF were compared with normal values previously determined in boys of similar age and considered 'low' if more than 2 SDs below the mean. The results of PFA were compared with standard pressure-flow studies and the three classical urodynamic patterns in boys with PUV, as determined by voiding symptoms. The subsequent PFA of seven of the 11 boys were also assessed as they had undergone previous urodynamics when < 8 years old. RESULTS: As assessed by the three patterns of dysfunction, two boys had bladder instability, two had low compliance and three had hypocontractility, with four boys being normal. From the PFA, the Vdet and WF were lower than normal, respectively, in seven and nine of the 11 boys; Schafer's nomogram showed a 'weak' detrusor in seven boys. The PFA suggested a pathology in four of five boys with symptoms and in three of six with no symptoms (two of the six showing a 'low' WF). Moreover, in older (11-13 years) boys, all five had a 'weak' detrusor, a 'low' WF and four a 'low' Vdet. Of the seven patients who underwent repeat PFA, three had a stable WF 3 years later, one (normal) worsened slightly and two were clearly worse, while one, who underwent late (at 3 years old) valve ablation, had an increased WF. CONCLUSIONS: The PFA showed hypocontractility in two-thirds of prepubertal boys with PUV, including asymptomatic patients. These findings confirm the hypothesis that bladder dysfunction in boys with PUV eventually causes detrusor myogenic failure and finally a postpubertal overdistended bladder.

[Total agenesis of the sacrum and neurogenic bladder dysfunction]. / Agenesia totale del sacro e disfunzione vescicale neurogena.

Total Sacral Agenesis (TSA) is a rare congenital anomaly of the lower vertebral column, frequently associated with bladder dysfunction. The diagnosis is often delayed until the evidence of neurological disorders addresses at neuro-radiological examination. In the last 9 years we observed 7 children, 3 males and 4 females, with TSA. The average age at diagnosis was 8 years (range: 1-15 years). Maternal diabetes was present only in one case. In 5 patients, urological ones have been the symptoms of presentation. In one case, TSA has been discovered because of the presence of a sacral mass (lipoma) and the subsequent development of club-foot. The neurological screening, performed in all patients with anorectal anomalies, led to the diagnosis of total sacral agenesis in a child with anteriorized anus. All patients have been evaluated by means of Magnetic Resonance Imaging, renal ultrasound, voiding cystography, renal nuclear scan and urodynamics. The functional evaluation of the lower urinary tract has shown a pattern of neurogenic bladder dysfunction in all children, while bilateral vesico-ureteral reflux has been detected in 2 cases. Intermittent clean catheterization (ICC) has been instituted at the diagnosis in all children. Surgery for continence and protection of upper urinary tract (bladder augmentation and bilateral ureteral reimplantation) has been performed in one patient. At a medium follow-up of 3 years (range: 1-5 years) 2 patients have been lost, normal renal function and urinary continence have been achieved in 4 and one child is continent but has renal failure. An early diagnosis and a correct neuro-urological evaluation and treatment are necessary to prevent urinary tract damage and achieve urinary continence in these patients. Symptoms, clinical findings and relationship with maternal diabetes are discussed. The diagnosis can be enough early if physicians pay attention to maternal diabetes, subtle neurological symptoms (voiding disturbance, constipation, club-foot) and careful physical examination of the back.