The hypospadias-specific health-related quality of life conceptual framework: a scoping review of the literature.

PURPOSE: Health-related quality of life (HRQoL) is an important yet understudied construct for individuals with hypospadias (HS). An important barrier towards understanding HRQoL and integrating it into research and clinical care for people with HS is the absence of an underlying conceptual framework to illustrate this construct. We propose a conceptual framework for HS-specific HRQoL based upon a scoping review of the HS literature. METHODS/MATERIALS: We conducted a literature review of articles published between 1989 and 2019. Our search in Embase and Pubmed used the keyword "hypospadias" in combination with "quality of life" and "patient-reported outcomes." We used thematic analysis of the resulting publications to identify core HRQOL domains. From these results and review of HRQoL literature in other pediatric populations, we developed a conceptual framework representing HS-specific HRQoL. RESULTS: We identified five domains of HRQOL previously studied in research with youth and adults with HS: penile appearance, voiding, social interaction, sexual health, and psychological or behavioral function. We propose a model of HS-specific HRQoL comprised of these domains and their areas of overlap, based upon the findings and conceptual mapping of our literature review. CONCLUSION: This novel conceptual framework provides a foundation for understanding disease-specific HRQoL in individuals with HS and may serve as a guide for the conduct of future qualitative studies of the HS population. The overlapping biopsychosocial domains illustrate the possible effects of HS on day-to-day life. This framework may guide future surgical, clinical, and behavioral interventions that aim to improve medical care and quality of life outcomes for HS patients.

Evaluating quality of patient-reported outcome measures in patients with hypospadias.

INTRODUCTION: There is growing recognition of the importance of patient-reported outcomes (PROs) in pediatric hypospadias. We have previously presented a conceptual framework for Hypospadias-Specific Health-Related Quality of Life (HRQoL), which posited 5 domains of HRQoL in this population. The framework components (domains) included penile appearance, voiding function, social function, psychological/behavioral function, and pubertal/sexual health. In this work, we investigated the established validity and relevance of PROs within each of these domains for patients with hypospadias. MATERIALS AND METHODS: We evaluated existing measures with published psychometric data, including validation data, in the hypospadias population. We also assessed the available data on each measure according to the guidelines of the Scientific Advisory Committee of the Medical Outcomes Trust (Table) in order to establish measure quality. We also examined the power of existing validation studies according to suggested guidelines for psychometric validation and factor analysis. DISCUSSION: Available validated measures in the hypospadias population have focused primarily on penile appearance and to a lesser degree on pubertal development/sexual health. There were no validated disease-specific measures with dedicated evaluations of other key HRQoL domains including voiding-related sequelae, social function, or psychological function. In addition, no single measure addressed all of the quality guidelines posed by the Scientific Advisory Committee. CONCLUSIONS: Current generalized measures for PROs lack relevance to the experience of hypospadias patients, and disease-specific assessments are often focused on penile appearance. Improving measure quality is necessary to optimize the value of our assessments and better help our patients with hypospadias.

Clinical outcomes after increasing bladder outlet resistance without augmentation cystoplasty in neurogenic bladder.

INTRODUCTION: Patients with neurogenic bladder (NGB) and urinary incontinence (UI) due to low bladder outlet resistance may require bladder neck procedures (BNPs) to achieve continence. These patients may also have reduced bladder capacity and or elevated detrusor storage pressures that require augmentation cystoplasty (AC). AC is not without complications that include risks for bladder rupture, urolithiasis, urinary tract infections and metabolic issues. Avoidance of AC would be helpful in patients with neurogenic urinary incontinence that have safe bladder parameters in the setting of low bladder outlet resistance. OBJECTIVE: To determine if pre-operative urodynamics could select children with NGBs and UI for isolated BNPs without AC. Additionally we sought to determine the safety of BNPs without AC and future need of AC with long-term follow-up. STUDY DESIGN: This is an IRB-approved retrospective analysis of all patients undergoing BNPs for management of neurogenic UI over a 17-year period. We separated these BNP patients into two groups: No AC + BNP (Group 1) vs. AC + BNP (Group 2). Our primary analyses focused on postoperative outcomes for patients in Group 1. Outcomes assessed included additional surgical procedures, urodynamic changes, development of CKD, new hydronephrosis (HDN) and vesicoureteral reflux (VUR). Secondary analysis included the timeline for the development of any bladder deterioration that necessitated AC in Group 1. RESULTS: 93 patients underwent BNP at a mean age of 10.8 years. Thirty did not have AC at the time of surgery (Group 1). These children had larger (p < 0.001) and more compliant (p < 0.001) bladders than Group 2 having simultaneous augmentation. At 6 years mean follow-up in Group 1 patients, three developed new reflux and three had new hydronephrosis. Nine (30%) had additional continence procedures. Twelve required (40%) AC at a mean of 23 months after the initial BNP. No patients had AC after 5 years. Detrusor end filling pressure increased 14.8 cm H2O (p = 0.028) and expected bladder capacity decreased 26.1% (p = 0.005) after isolated BNP. DISCUSSION: We found that from our cohort of patients who had normal bladder compliance and normal/near normal expected capacity preoperatively 40% required subsequent AC. We were unable to find pre-operative clinical parameters which predicted failure or conversion to AC. We found that 43.3% of our BNP without AC patients had no subsequent invasive procedures with mean 6-year follow-up. We found that none of our patients developed any degree of CKD. Finally, we found that the majority of patients that converted to AC after their BNP did so within the first 2 years after their initial BNP and no patients required augmentation 5 years post their initial BNP. This data validates that these patients require very strict follow up, particularly in the first 5 years after surgery. CONCLUSIONS: BNP without AC is safe in only a few selected patients with NGB. Despite preoperative selection, there are significant changes in bladder dynamics and 40% required subsequent augmentation. Bladder deterioration occurs early and generally in the first 2 years. Since there are no apparent reliable pre-operative variables predicting the need for subsequent AC, parents should be counseled regarding vigilant post-operative follow-up.

Do pediatricians routinely perform genitourinary examinations during well-child visits? A review from a large tertiary pediatric hospital.

BACKGROUND: The male genital examination is a common source of discomfort for the patient and medical provider. Performance of male genital examination is imperative; however, as many treatable diagnoses can be made. Undescended testicles (UDTs), hernias, testicular tumors, and urethral abnormalities are all potentially concerning findings which can be discovered on routine examination. OBJECTIVE: The objectives of this study are to determine the rate at which general pediatricians perform routine genitourinary (GU) examinations in the pediatric population and to determine the rate at which UDT are diagnosed or documented in the patient's history. The authors hypothesize the rate of pediatric GU examination during routine well-child visits to be in line with the previously reported rates in the adult literature. STUDY DESIGN: Nine hundred ninety-six consecutive male well-child visits conducted by general pediatricians at the study institution were reviewed. These visits were evaluated for documentation of a detailed GU examination as well as the presence of UDT from these examinations. In addition, past medical and surgical histories were reviewed to determine if a diagnosis of UDT was noted. RESULTS: Pediatricians at the study institution documented GU examinations 99.1% of the time during male well-child visits. Only 1.1% of the cohort had a documentation of UDT at any time point. Of the 11 patients with UDT, 6 boys (54.5%) had spontaneous descent with no referral to urology, whereas 5 (45.5%) required orchidopexy. DISCUSSION: Prior reports suggest 70-75% of routine office visits include a genital examination. None of these reports reviewed the pediatric population, thus making this review novel in this respect. In addition, the results are vastly different from these prior studies as the authors demonstrated over 99% of male well-child examinations included documentation of a thorough genital examination. A limitation of the study is its retrospective nature, which creates a lack of standardization across the data set. In addition, without being physically present in the examination room, one cannot discern whether an examination is simply being documented without actual performance because of the template format of the electronic medical record (EMR). Furthermore, the study was not designed to best evaluate the true rate of UDTs; therefore, the reported rate of 1.1% cannot be accurately associated with a particular age at diagnosis. CONCLUSIONS: Pediatricians do, in fact, document GU examinations on a routine basis. This finding cannot be taken with complete certainty as verification of actual examination performance is impractical. While the data demonstrated a lower than expected rate of UDT, depending upon age at diagnosis, this could indicate that although examinations are being documented, their accuracy may be diminished because of various factors at play in the healthcare system as a whole, including improper exam performance and EMR templates. Follow-up studies are required to verify these potentially changing rates of UDT and to determine if there is discordance between documentation and performance of GU examinations.

Comparing treatment modalities for transplant kidney vesicoureteral reflux in the pediatric population.

INTRODUCTION: Non-refluxing ureteral reimplantation is favored in pediatric renal transplantation to prevent complications, such as vesicoureteral reflux (VUR) in the transplant ureter. VUR resulting in febrile urinary tract infections remains a problem in this population, leading to repeated hospitalizations and increased morbidity. Revision of the vesicoureteral anastomosis can be a surgical challenge due to scar tissue and tenuous vascularity of the transplant ureter. Therefore, alternative options such as endoscopic injection of Deflux at the neo-orifice and surveillance with prophylactic antibiotics have emerged as potential treatment modalities for transplant ureter VUR. OBJECTIVE: The authors reviewed their experience of the management of VUR in the transplant ureter, comparing outcomes of various modalities. STUDY DESIGN: With Institutional Review Board approval, a retrospective chart review of all renal transplant patients from January 2002 to January 2017 was conducted. All patients with VUR on voiding cystourethrogram (VCUG) after surgery were identified. Indications for end-stage renal disease, urologic comorbidities, pretransplant VCUG, and operative details were recorded. After transplantation, febrile urinary tract infections, ultrasound findings, and any further interventions-surveillance, subureteral endoscopic injection of Deflux, or ureteral reimplantation-were documented along with their outcomes. RESULTS: Overall, VUR was identified in 35/285 (12.3%) transplant patients after a non-refluxing ureteroneocystostomy. VUR was managed with surveillance in 17/35 (49%), intravesical Deflux injection in 11/35 (31%), and immediate redo ureteral reimplantation in 7/35 (20%). Ten out of 11 patients undergoing Deflux injection had a postoperative VCUG. All patients developed VUR recurrence; the majority showed immediate failure and only 1/10 showed late recurrence. Of the immediate failures, 3/9 patients were maintained on prophylactic antibiotics, and 6/9 patients underwent ureteral reimplantation. In these six patients undergoing reimplantation after failed Deflux, 3/6 (50%) patients required additional surgeries: One patient developed recurrence of reflux and two patients developed ureterovesical junction obstruction. In contrast, no complications were seen in patients undergoing primary ureteral reimplantation. DISCUSSION: The study is limited by low numbers and a retrospective design. However, the results of this study differ significantly from the published Deflux series showing a success rate of more than 50% in the treatment of transplant kidney VUR. In fact, post-Deflux redo ureteral reimplantation was associated with an increased risk of postoperative complication. CONCLUSION: The use of Deflux in the post-transplant setting has poor results. In the study series, 11/11 patients demonstrated clinical and radiographic failure. Therefore, as an institution the authors do not recommend Deflux as first-line treatment of VUR in the transplant patient.

Pre-operative risk factors for stomal stenosis with Malone antegrade continence enema procedures.

INTRODUCTION: The Malone antegrade continence enema (MACE) procedure remains an effective tool in providing independence to patients with refractory constipation. Appendiceal, ileal and colonic segments are all used depending upon the patient's individual anatomy and surgeon's preference. Unfortunately, MACE stomal stenosis (SS) remains a frustrating complication. It was hypothesized that SS is associated with the type of efferent limb used for MACE construction. OBJECTIVE: To perform a retrospective risk analysis of the pre-operative factors that correlated with postoperative stomal stenosis. MATERIALS AND METHODS: The study reviewed 39 consecutive patients treated with MACE by a single surgeon at the present institution. Collected data included: the type of channel construction, stoma site, pre-operative body mass index (BMI) z-score, pre-operative diagnosis, and development of SS. Stomal stenosis was defined as a documented inability to catheterize, requirement of a Chait tube, or a subsequent stomal revision. An odds ratio (OR) analysis was performed to evaluate the association with the development of SS. RESULTS: Stomal stenosis developed in 19 patients (49%) with an average follow-up of 4.7 years. The majority of patients had a diagnosis of myelomeningocele (90%), and there was no significant difference in gender distribution (54% males, 46% females). The distribution types of MACE efferent limbs consisted of appendicocecostomy in 20 (51%), cecal-wall flap in 16 (41%) and ileocecostomy in three patients (8%). Neither type of efferent limb, nor stomal location were significant predictors of postoperative SS. Pre-operative obesity (BMI ≥95%), however, demonstrated a significant risk of SS compared to non-obese patients (OR 4.44, 95% CI 1.08-18.4, P = 0.04). DISCUSSION: This was a single-institution study of a relatively small number of patients. However, examining consecutive patients with a common surgeon can minimize technique variability. Also, the rates of stenosis in this cohort were higher than most. This may have been due to a more broad definition (unable to catheterize). These findings complement recent literature showing an increased complication rate for the obese pediatric patient. CONCLUSION: Stomal stenosis remains a frustrating complication following creation of the MACE stoma. In the present series, neither a cecal wall flap, nor the stomal site location contributed to SS. Obesity was the only identified statistically significant risk factor that led to SS.

Advantages of rectus fascial slings for urinary incontinence in children with neuropathic bladders.

PURPOSE: Many surgical procedures to improve outlet resistance in children with neuropathic bladders are obstructive and increase the detrusor leak point pressure. In contrast, fascial slings are designed to achieve continence by increasing the Valsalva or stress leak point pressure without altering the detrusor leak point pressure. We evaluate the effectiveness of fascial slings in achieving continence in pediatric patients with neuropathic bladder. MATERIALS AND METHODS: From October 1994 until February 1999, 10 females and 8 males with neuropathic bladder secondary to myelodysplasia or traumatic spinal cord injury underwent fascial sling procedures. Mean patient age was 14 years (range 8 to 18) and all were incontinent despite aggressive medical management. Urodynamic evaluation was performed preoperatively and postoperatively. Specific urodynamic measurements included detrusor leak point pressure, stress leak point pressure and detrusor compliance. Compliance was only compared in the 12 nonaugmented cases. RESULTS: With a mean followup of 21.2 months (range 6 to 57), preoperative and postoperative urodynamics revealed little change in mean detrusor leak point pressure (23.2 versus 23.22 cm. H2O) but a substantial increase in mean stress leak point pressure (41.6 versus 64.5 cm. H2O). Mean compliance was unchanged in the nonaugmented group (22.00 versus 26.78 ml/cm. H2O). Four patients (22.22%) remained wet after surgery, of whom 2 were successfully treated with a repeat sling procedure and 1 with collagen injection for an overall continence rate of 94.44%. CONCLUSIONS: Fascial slings can be effectively used in pediatric patients for neuropathic incontinence. Furthermore, stress urinary incontinence is corrected by increasing the Valsalva or stress leak point pressure with preservation of the detrusor leak point pressure. Preservation of detrusor leak point pressure is particularly advantageous because other forms of bladder outlet procedures achieve continence at the expense of increasing detrusor pressures, thus placing the upper tracts at risk for damage.

Multi-institutional experience with the gastrointestinal composite reservoir.

PURPOSE: We evaluated multi-institutional experience with the gastrointestinal composite reservoir in patients with metabolic acidosis, the short bowel syndrome, severe pelvic radiation and/or renal insufficiency. MATERIALS AND METHODS: At 4 institutions 33 patients underwent construction of a gastrointestinal composite reservoir, including 19 with the short bowel syndrome, 13 with metabolic acidosis and 7 who also had renal insufficiency. A total of 16 patients underwent conversion of a previous diversion and the remaining 17 received new urinary diversion. Charts were reviewed for the metabolic impact of the gastrointestinal reservoir as well as any long-term sequelae. RESULTS: At a mean followup of 54 months there was a significant (p < or =0.05) improvement in mean preoperative and postoperative serum chloride (106 versus 102 mEq./l.), serum bicarbonate (23.3 versus 25 mEq./l.) and serum pH (7.36 versus 7.4). Mean serum creatinine did not significantly differ during followup in patients with normal renal function or renal insufficiency. Complications were not different than those of standard intestinal or gastric reservoirs. CONCLUSIONS: The gastrointestinal reservoir has provided an excellent metabolic balance in a large series of compromised patients with few side effects. We believe that the gastrointestinal composite reservoir represents the urinary diversion of choice when standard intestinal urinary reservoirs cannot be created in the setting of metabolic acidosis, the short bowel syndrome and severe pelvic radiation. However, the value of the gastrointestinal composite in the setting of renal insufficiency remains undetermined.

Symptomatic ureteropelvic junction obstruction in children in the era of prenatal sonography-is there a higher incidence of crossing vessels?

OBJECTIVES: To determine whether prenatal sonography and early detection and correction of ureteropelvic junction obstruction (UPJO) has changed the incidence of crossing vessels as the etiology of obstruction in older children presenting with symptomatic UPJO. METHODS: We reviewed the medical records of all children and adolescents who underwent pyeloplasty for symptomatic UPJO between 1986 and 1999, during the era of widespread use of prenatal sonography. Operative notes were used to determine which patients had obstruction due to lower pole crossing vessels. RESULTS: Thirty-eight patients were identified who underwent pyeloplasty for symptomatic UPJO. Lower pole vessels were identified in 22 (58%) of 38 patients (P <0.0001 compared with historical controls). All patients underwent dismembered pyeloplasty and remained asymptomatic after surgery, with renal scans demonstrating excellent drainage and preservation of function. CONCLUSIONS: Prenatal ultrasonography has increased the incidence of crossing vessels as the etiology of UPJO in young children and adolescents presenting with symptomatic UPJO compared with the historical incidence of 11% to 15%. This finding may impact treatment recommendations with respect to endourologic management of the obstruction in this patient population. Currently, we recommend open dismembered pyeloplasty for young children and adolescents presenting with symptomatic UPJO.

Nephrostomy tube drainage with pyeloplasty: is it necessarily a bad choice?

PURPOSE: Despite continued controversy regarding the optimal method of urinary diversion after dismembered pyeloplasty in children, we have treated the majority of our patients with postoperative nephrostomy tubes and no stents. We report our experience. MATERIALS AND METHODS: The records of all patients who underwent surgery for ureteropelvic junction obstruction from August 1985 to October 1998 and were treated only with a nephrostomy tube after pyeloplasty were reviewed for hospital course, complications and postoperative followup. All patients had a perinephric Penrose drain as well as a Foley catheter placed for bladder drainage. RESULTS: A total of 137 pyeloplasties were performed in 132 patients, including 5 with bilateral ureteropelvic junction obstruction, using only nephrostomy tube drainage with an average followup of 2.1 years. Initial nephrostograms demonstrated good drainage across the repair with no extravasation in 91% of patients. Subsequent nephrostograms revealed a widely patent anastomosis in the remaining cases. No patient had postoperative obstruction, or required secondary pyeloplasty or nephrectomy. Urinary tract infection developed in 2 patients (1.5%). Mean hospitalization was 4.4 days. There was a significant difference in length of stay in the last 5 years compared to that in previous years (3.4 versus 5.8 days, p <0.05) and hospital stay continues to decrease. CONCLUSIONS: Use of only a nephrostomy tube after pyeloplasty resulted in few complications and an open anastomosis in 100% of cases. Nephrostomy drainage not only serves as a protective mechanism, but also allows easy access for radiographic studies before removal of the tube. In addition, nephrostomy tube drainage does not prolong hospitalization and the tube may be easily removed on an outpatient basis without further anesthesia.

alpha-Adrenergic blockade in children with neuropathic and nonneuropathic voiding dysfunction.

PURPOSE: Inadequate bladder emptying is a common urinary dysfunction in children. The role of alpha-blockers for managing bladder outlet obstruction remains relatively unexplored in children. Because of the well established impact of alpha-blocker therapy in men, we investigated its use for treating inadequate bladder emptying in the pediatric population. MATERIALS AND METHODS: We treated 17 children 3 to 15 years old with documented poor bladder emptying of various etiologies, including dysfunctional voiding, the Hinman syndrome, the lazy bladder syndrome, posterior urethral valves, myelomeningocele and the prune-belly syndrome, using the alpha-1 adrenergic receptor antagonist, doxazosin. The initial dose of 0.5 to 1.0 mg. nightly was increased according to patient response and as tolerated. Patients were followed weekly to monthly by symptomatic history, and urine flow and/or post-void residual urine volume measurement. Two patients with neurogenic bladder were also followed with cystometrography and leak point pressure determination. RESULTS: Bladder symptomatology and/or emptying improved in 14 patients (82%). Ten patients had decreased post-void residual urine during treatment and in 3 uroflowmetry showed increased maximum flow. Two patients with neuropathic bladder secondary to myelomeningocele had decreased leak point pressure on alpha-blocker therapy and in 2 with a history of posterior urethral valves new onset bilateral hydronephrosis completely resolved. Only 1 patient had mild postural hypotension, which resolved with dose reduction. CONCLUSIONS: Selective alpha-blocker therapy seems to be well tolerated in children and appears effective for improving bladder emptying in various pediatric voiding disorders at short-term followup. Long-term followup and further investigation are warranted to validate the potential role of alpha-blocker therapy in pediatric urinary dysfunction.

The gastrointestinal composite urinary reservoir in patients with myelomeningocele and exstrophy: long-term metabolic followup.

PURPOSE: We investigated the long-term metabolic effects of gastrointestinal composite urinary reservoirs in patients with myelomeningocele or exstrophy. MATERIALS AND METHODS: Seven patients with myelomeningocele or exstrophy who required complex urinary reconstruction in the setting of metabolic acidosis or the short bowel syndrome underwent construction of a gastrointestinal composite reservoir, including a staged and a single procedure in 3 and 4, respectively. Preoperatively and postoperatively serum electrolytes were measured, and urinalysis and urine cultures were performed in all patients. In 5 patients serum pH was compared preoperatively and postoperatively, and in all serum gastrin was measured postoperatively. RESULTS: At an average followup of 62 months (range 52 to 87) serum chloride and bicarbonate significantly normalized (p <0.05) in all 7 patients with bladder exstrophy or myelomeningocele. Serum pH also significantly normalized (p <0.05) in 5 patients at long-term followup. Serum gastrin and creatinine were normal and urinary pH fluctuated insignificantly throughout followup. None of the patients had urolithiasis or symptoms of the hematuria-dysuria syndrome. Periodic symptomatic urinary tract infections developed but none required chronic antibiotic therapy. CONCLUSIONS: Gastrointestinal composite urinary reservoirs appear to be beneficial for patients with myelomeningocele or exstrophy who have preexisting metabolic acidosis or the short bowel syndrome. Serum electrolyte neutrality is achieved during long-term followup. No patient had the hematuria-dysuria syndrome or urolithiasis.

Predictability of PSA failure in prostate cancer by computerized cytometric assessment of tumoral cell proliferation.

OBJECTIVES: To evaluate the relationship of DNA ploidy and cell proliferation (CP) with Gleason score (GS) and clinical outcome in prostate cancer. METHODS: Sixteen patients with benign prostatic hyperplasia (BPH) and 65 patients with prostate cancer classified by GS (four groups: 2 to 4, 5 to 6, 7, and 8 to 10) were studied. All patients with carcinoma underwent prostatectomy and were separated into prostate-specific antigen (PSA) failure and nonfailure groups (failure if PSA 0.1 ng/mL or more three times after surgery). Tumoral CP (Ki-67 inmunostaining and SG2M phase) and DNA ploidy were evaluated by computerized cytometry. RESULTS: BPH were diploid with low CP (8% SG2M cells or less). Carcinomas were either diploid with high CP (greater than 8% SG2M cells) or aneuploid. CP was significantly higher (P <0.001) in tumors with GS 7 or greater than in tumors with GS less than 7 (mean percent Ki-67 cells 18.3% versus 7.8%, respectively). PSA failure increased with GS (7.1% in GS 2 to 4, 21% in GS 5 to 6, 28.6% in GS 7, and 50% in GS 8 to 10), as well as with aneuploidy (18.5% in diploid tumors versus 72.7% in aneuploid tumors). Those experiencing PSA failure had significantly higher (P <0.001) CP than those not failing (mean percent Ki-67 cells 24% and mean percent SG2M 30.4% versus 8.7% and 13.5%, respectively). Cox regression analysis showed GS, DNA ploidy, Ki-67, and SG2M to each be univariately prognostic for time to PSA failure; however, Ki-67 and SG2M were more highly significant (P <0.0001 for both) than GS (P = 0.007) or DNA ploidy (P = 0.002). After adjusting for either SG2M or Ki-67 measures of CP, neither ploidy nor GS contained additional prognostic value. CONCLUSIONS: Tumor CP and DNA ploidy can be reliably determined in prostate cancer by computerized cytometry. On the basis of our preliminary results, CP correlates well with GS and predicts PSA failure better than DNA ploidy or GS.

Evaluation of flap valve as an alternative continence mechanism in the Florida pouch.

OBJECTIVES: To evaluate urodynamic findings in a successful flap valve (FV) continence mechanism in association with a continent colonic urinary reservoir (Florida pouch) and to compare the urodynamic findings of the FV mechanism with the doubly plicated (PI) standard anti-incontinence segment in the same reservoir. METHODS: Thirteen patients who successfully received the Florida pouch between 1988 and 1996 agreed to undergo urodynamic evaluation as part of a pilot study. Eight patients had a PI continence mechanism and a mean time from surgery of 51 months; 5 had a FV continence mechanism and a mean time from surgery of 14 months. Enterocystometry was performed with a trans-stomal Bard triple channel 7F catheter. Volume and pressure at first desire to empty (VFDE, PFDE), as well as maximal enterocystometric capacity and pressure (VMEC, PMEC), were recorded. Maximal outlet pressure (MOP) was recorded using the catheter withdrawal technique. RESULTS: PI and FV groups demonstrated the following mean values respectively: VFDE, 692.7 and 403 mL; PFDE, 19.5 and 19.2 cm H2O; VMEC, 876.5 and 515 mL; PMEC, 25.9 and 24.6 cm H2O; MOP, 57.5 and 51.2 cm H2O (reservoir empty) and 50.5 and 52.6 cm H2O (reservoir full); and functional length of outlet, 24.3 and 24.6 cm. MOP measurement demonstrated greater variability in the PI than in the FV group. CONCLUSIONS: Urodynamic comparison of these mechanisms reveals that MOP measurement was closer to the mean among FV than PI patients. In addition, the mean VFDE (692.7 mL for PI versus 403 mL for FV, P < 0.05) and the mean VMEC (876.5 mL for PI versus 515 mL for FV, P < 0.05) were significantly less in the FV group. Lower VMEC and less variability in MOP indicate that continence may be more dependent on MOP in the FV mechanism. A longer follow-up time and a larger number of patients will be of assistance in clarifying these findings.

Prenatal bladder outlet obstruction secondary to ureterocele.

We present 2 cases of prenatal hydroureteronephrosis and bladder outlet obstruction due to an obstructing ureterocele. Both neonates were stabilized and managed with early endoscopic decompression. Neither infant demonstrated significant function in the kidney ipsilateral to the ureterocele either before or after ureterocele puncture. There have been few cases reported of prenatal bladder outlet obstruction due to a ureterocele. Our limited experience has been poor with regard to salvaging the affected upper tract. Future definitive management will be tailored as more of these cases are documented.

The prenatal diagnosis of cloacal exstrophy.

PURPOSE: We assess and clarify diagnostic features for making the prenatal diagnosis of cloacal exstrophy. MATERIALS AND METHODS: We evaluated 9 patients born with cloacal exstrophy at our institutions (2 prospectively and 7 retrospectively) for diagnostic features on prenatal ultrasound studies. We also thoroughly reviewed the literature on 13 previous prenatally diagnosed cloacal exstrophy cases. Diagnostic criteria were assessed by combining the findings in our patients and those in previous reports. RESULTS: Of the 22 patients with prenatal ultrasound studies and cloacal exstrophy whom we analyzed 1 of our 9 and 2 in the literature had a cloacal membrane that persisted at 22 weeks of gestation. Major ultrasound criteria for diagnosing cloacal exstrophy prenatally are nonvisualization of the bladder, a large midline infraumbilical anterior wall defect or cystic anterior wall structure (persistent cloacal membrane), omphalocele and lumbosacral anomalies. Seven less frequent or minor criteria include lower extremity defects, renal anomalies, ascites, widened pubic arches, a narrow thorax, hydrocephalus and 1 umbilical artery. CONCLUSIONS: We propose major and minor criteria to assist in the prenatal diagnosis of cloacal exstrophy. Despite these major and minor criteria the certainty of establishing a prenatal diagnosis remains challenging. Persistence of the cloacal membrane beyond the first trimester in 1 patient was an exception to the classic concept of cloacal exstrophy embryogenesis. An accurate prenatal diagnosis requires validation of these criteria by further correlation of prenatal and postnatal observations.

Daytime functional bladder capacity as a predictor of response to desmopressin in monosymptomatic nocturnal enuresis.

OBJECTIVE: The objective of this study was to identify a cost-effective method of predicting a therapeutic response to desmopressin (DDAVP) by correlating daytime functional bladder capacity, age and urine osmolalities in patients with monosymptomatic nocturnal enuresis. MATERIALS AND METHODS: Thirty-five children out of 51 who initially presented to our institutions were included in the study. The remainder was excluded for lack of complete data. Constipation was actively addressed and was managed by administration of evening enemas for 3 consecutive days and dietary adjustment prior to initiating the study. Each micturition as measured throughout the day and the maximal daytime functional bladder capacity was determined as the largest void over a 2-day period. Urine samples were collected at home at 08:00, 16:00 and 22:00 (times that would best reflect the fluctuations in plasma vasopressin levels). Intranasal DDAVP was then administered, titrating the dose over a 2-week period. The initial dose was 10 micrograms and the dose was increased 10 micrograms every 3 days. RESULTS: The response to DDAVP was then evaluated and of the 35 children, 27 demonstrated a complete response to DDAVP (all at doses between 10 and 30 micrograms). These were then related to the possible predictive factors. There was a significant correlation between a high maximum daytime functional bladder capacity and response to DDAVP (p = 0.006). Similarly, age was also predictive of a good response to DDAVP treatment (p = 0.008). However, spot urine osmolalities were not predictive of a response to DDAVP (p > 0.1). CONCLUSIONS: Functional bladder capacity is a reliable predictor of response to desmopressin; children with larger capacities are more likely to exhibit a successful response. Older children have a better response rate than younger ones. Spot urine osmolality measured on specimens collected in the home setting is not predictive of response to desmopressin.