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.

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.

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.

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.

Urinary continence in Müllerian duct anomalies.

Müllerian duct congenital anomalies such as Rokitansky-Mayer syndrome, Urogenital Sinus, Vaginal Atresia and Cloacal Malformation are relatively uncommon (1-5% of born female newborns). The complexity of these malformations has taken great interest regarding mainly the surgical procedure available for correction of genital abnormalities. However, the problem of urinary incontinence is still underestimated, and continence is often a goal difficult to achieve. Authors report 11 cases of congenital anomalies of Müllerian duct differentiation which have been observed in the last decade in pediatric age with special regard to preoperatory diagnostic procedures, urinary continence valuation after surgical correction, therapy chances for continence, considering the psychological implication of this unresolved abnormality in everyday-lifetime.

Pneumonia complicating abdominal sepsis: an experimental model of hematogenous contamination of the lung.

Pulmonary infection complicating intra-abdominal sepsis is a major clinical problem. An experimental model for intra-abdominal sepsis was created with implantation of gelatin capsules, containing 3 x 10(8) cfu E. coli strain no. 2554, in the peritoneal cavity of 20 rats (10 animals received and 10 did not receive antibiotic therapy with ceftriaxone) in order to verify the role of the primary site of infection in the pathogenesis of pneumonia. Ten rats were sacrificed to determine the relative pulmonary weight and 10 were submitted to simple laparotomy and insertion of a germ-free capsule (sham-operated group). In this group of animals there was only one death (10%). All the rats that received antibiotic therapy survived until sacrifice while all the rats that did not receive ceftriaxone died, 7 within the 2nd and 3 on the 6th postoperative day. Pneumonia and peritonitis developed only in the animals that did not receive ceftriaxone. Bacteriological findings of material obtained from peritoneal and pleural cavities revealed the same strain of E. coli used for the experiment, suggesting that bacteria involved in the pleuro-pulmonary infections may originate in the primary site of infection and that antibiotic therapy started at the moment of contamination, can prevent this major complication.

Continuous (6 hour) urodynamic monitoring in children with neuropathic bladder.

In adults the development of modern equipment for ambulatory monitoring permits long-term evaluation of the lower urinary tract which is more accurate than standard urodynamics (SUD). In children continuous urodynamic monitoring (CUM) has been used infrequently and therefore standardisation of the method has not been previously achieved, nor have the techniques and difficulties of performing 24-hour monitoring been solved. The aim of this study was to identify a technical method of CUM in children which was feasible and to verify its usefulness. For this purpose, we reviewed our preliminary experiences of CUM in children with neuropathic bladder. From March to November 1995 we made an outpatient study of the neuropathic bladders of 11 myelodysplastic children aged 1 to 18 years (average age 10.2 years). 7 of them were males and 4 females. All underwent SUD. With the children resting in bed, a 6-hour CUM (Lectromed MPR-2) was performed using a 4 Fr. microtip intravesical catheter (suprapubic in 3 infants and transurethral in 8 children) and an intrarectal catheter. The parents and/or a nurse monitored the fluid intake and micturition events and recorded the data in a diary. Based on the CUM experience in infants with non-neurogenic bladder dysfunction, the 3 suprapubic studies were done after 12 hours of urethral drainage in order to prevent leakage of urine. In all of the patients we were able to study 2 to 4 bladder fillings during a period of 6 to 8 hours (average 6.5 hours) observation. In 9 of the 11 children the CUM pattern was comparable to the SUD one but in the 2 remaining patients CUM showed uninhibited contractions. Higher uninhibited voiding contraction pressures were recorded in hyper-reflexic bladders. Our preliminary results show that it is feasible to perform CUM in children and that it has advantages over standard cystometry in the investigation of children with neuropathic bladder even if it is carried out for short term (6 hours).

Long-term urological follow-up of occult spinal dysraphism in children.

From 1980 to 1996 we followed up 65 children with occult spinal dysraphism (OSD) out of 85 observed in our urodynamic laboratory. The average age at diagnosis was 4.7 years (range: 1-21 years). In 19 cases (22%) the OSD was associated with anorectal anomalies (ARA). Urinary incontinence or voiding anomalies with urinary tract infection were the presenting symptom in 34 children (49%). At diagnosis all children underwent complete neuro-urological and urodynamic evaluation. Thirty-eight patients underwent neurosurgical correction. Vesicoureteral reflux (VUR) was present or developed in 17 patients: 15 underwent endoscopic procedures and 2 required surgery; 2 needed bladder augmentation because of upper tract and renal function deterioration. Urinary incontinence was treated mainly by intermittent catheterization and anticholinergic drugs, but endoscopic treatment was performed in 3 children. At long-term follow-up (2 to 14 years), socially acceptable continence was achieved in 78% of 57 children (8 could not be assessed because they were less than 4 years old). Upper urinary tract deterioration occurred in 15% and renal failure in 7.5%.

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.

Early detection of bladder dysfunction following posterior urethral valves ablation.

Urinary incontinence following Posterior Urethral Valves (PUV) ablation has been attributed in the past to sphincter injury, but it is nowadays accepted that bladder dysfunction (BD) plays a determinant role. In order to assess BD evolution, we have evaluated, from 1982 to 1994, 48 boys with PUV by urodynamics (UD) studies. Age of the patients ranged from 10 months to 15 years. A total of 65 examinations were performed. We considered four groups: 0-3 years; 4-7 years; 8-12 years and > 12 years. Uninhibited detrusor contractions (instability), end filling pressure (EFP), bladder capacity and voiding detrusor pressure were evaluated in order to assess the presence of BD distinguishing it in: hypercontractility, hypocontractility and low-compliant bladder. The results collected confirm a high percentage of BD in PUV boys (71%) and the evolution of hypercontractility versus hypocontractility in 60% of patients considered.

Neurovesical dysfunction due to spinal dysraphism in anorectal anomalies.

Spinal dysraphism (SD) has been found associated with functional abnormalities of anorectal anomalies (ARA). The incidence of SD in these children is probably underestimated and a complete neuroradiological investigation of the lower urinary tract function has not been carried out routinely. In a 2 years time frame we performed urodynamic (UDS) evaluations on 14 patients (8 males and 6 females) with ARA who showed SD at Magnetic Resonance Imaging (MRI) studies. We divided them into 2 groups by age: group A (5 to 18 months) and group B (3 to 12 years). The UDS findings were classified as upper (UMN) and lower motor neuron (LMN) lesion. Out of the 9 children included in group A, 5 showed normal urodynamic pattern, while 4 had pathological UDS findings suggesting UMN lesion. Among the older (group B) children only one had normal and 4 had pathological findings: 2 hyperreflexia suggesting UMN lesion and 2 external sphincter denervation suggesting LMN lesion. These data support the hypothesis that the neurovesical dysfunction found in children with anorectal anomalies results from a possible association with spinal dysraphism. An early morphological evaluation of the spinal cord is mandatory in all children with ARA prior to definitive surgical correction of the malformation. The low incidence of pathological UDS findings in small children if compared to older ones suggest that SD, although present, may be asymptomatic.

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.

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.

[pH-metric parameters potentially predictive of asthmatic symptomatology: clinical and statistical research]. / Parametri pH-metrici potenzialmente predittivi della sintomatologia asmatica: ricerca clinica e statistica.

The gastro-esophageal reflux (GER) usually causes digestive symptoms, failure to trive and/or respiratory symptoms. Furthemore the association between GER and asthma is well known. Nevertheless, the relationship between two pathologies and role of GER in aggravation of asthma are not well known. The aims of our study is to identify the peculiar pH-metric caracteristics of GER may be responsable of asthmatic symptoms in children. The study was conducted in 32 children. The patients were divided into two groups: Group A composed of 16 children suffering from non-allergic asthma characterized by prevalent nocturnal manifestation; Group B composed of 16 children suffering from GER, without respiratory symptoms. All patients underwent to 21 pH-monitoring. The pH-metric data collected in two groups are submitted to statistic analysis using the Student's "t" Test.

[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.

[Vertebral schisis in enuretic children: preliminary results of a statistical analysis]. / Schisi vertebrale nel bambino enuretico: risultati preliminari di un'analisi statistica.

It is well known that vertebral schisis is frequent in enuretic children but the true incidence in the normal population is not clear, because all series published are referred to children with associated urinary anomalies, who were submitted to voiding cystography and or intravenous pyelography. This determine a statistical bias. The aim of our study was to compare the prevalence of vertebral schisis in enuretic children and in the general pediatric population. Therefore, we chose 142 enuretic children without associated urological or neurological anomalies and a control group of 152 children, assumed as general population, who were submitted to spinal X-rays during screening for scoliosis or congenital dysplasia of the hip. Vertebral schisis was found in 65% (93/142) of enuretics and in 18% (28/152) of control group children. Maximum association between enuresis and vertebral schisis was found in primary monosymptomatic nocturnal enuresis (82%), while minimum association was found in children with secondary enuresis (57%). The difference in percentage of association enuresis-schisis was statistically significant between enuretics and control group and between primary monosymptomatic and secondary enuresis (p < 0.001). The results of this paper are simply add knowledges on the prevalence of the sacral schisis in enuretic children. But, to speculate the different prevalence in different types of enuresis, the results should confirm that the phenomenon of enuresis is multifactorial and the primary monosymptomatic and secondary enuresis have different etiological factor.

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.

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.

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.